UWorld Peds

Describe the 3 causes of neonatal conjunctivitis based on: - Age - findings - TMT

*Chemical* - Age >> (<24 hrs) - Findings >> Mild conjunctival irritation & tearing after silver nitrate ophthalmic prophylaxis -TMT >> Eye lubricant *Gonococcal* - Age >> 2-5 days - Findings >> ---Marked eyelid swelling ---profuse purulent discharge ---Corneal edema/ ulceration -TMT >> single IM dose of 3rd gen cephalosporin *Chlamydial* - Age >> 5-14 days - Findings ---Mild eye welling ---Watery, serosanguineous or mucopurulent discharge - TMT >> *PO macrolide* (AZ preferred)

Which Is the preferred tmt for Tourette's? (Anticholingergic OR Antidopaminergic OR SSRI)

Antidopaminergic - Most effective & include 1- DA depleting *Tetrabenazine* 2- Antipsychotic *Risperidone, haloperidol* 3- α2 agonists *guanfacine, clonidine* SSRI - Used to treat OCD which if often a co-morbid condition to Tourette's (but not a primary tmt) Anticholinergic > Not used in Tourette's

An 18-month-old presents for a check-up. The pt was born at full term w/out complications. Growth has been consistently along the 80th percentile for Ht & Wt. The mom reports, "He is healthy & seems happy just playing by himself, but I am concerned about him not talking." She says that he makes some sounds that are unintelligible, has a 1-word vocabulary of "no" & does not use "mama", "dada," or "bye-bye". The pt is able to walk & is starting to run. He can scribble & with help can use a spoon to feed himself. On exam, he does not make eye contact, respond to his name, or follow the doctor's gaze when toys are pointed out to him. Hearing testing is normal. Which of the following is the most likely explanation for this pt's behavior? (Autism OR Language disorder OR Speech sound disorder)

Autism - At 18-months most children should have --Vocab of 10-25 words -----(pt only uses 1 word) --Respond to their name -----(pt does not respond to name) --Make eye contact & engage in play w/ others -----(pt does not make eye contact & does not follow doctors gaze) -----(pt prefers to play alone) -Presentation- 1- Delayed language development 2- Unresponsiveness to his name 3- Impaired social communication & interaction 4- Absence of joint attention 5- Repetitive mvmts (rocking, hand-flapping) 6- Routines 7- Fixated interests Language disorder - AKA Stuttering → Characterized by persistent difficulties in comprehension & production of spoken & written language Speech sound disorder - Articulation disorder that would present similarly to language disorder (AKA stuttering)

An 8B, presents d/t abd pain & dark urine. His parents report that the symptoms began a week ago. He has no Hx of fever, nausea, or vomiting & is eating normally. He has not had any previous abd pain of trauma. Vitals are normal. Exam shows scleral icterus & mild abd tenderness. An abd mass is palpable in the RUQ. Labs show: - Bilirubin-------------6.5 - Direct bilirubin------3.4 - Amylase-------------138 - Lipase---------------152 US shows an extra hepatic cystic mass & a normal gallbladder. Which of the following is the most likely Dx? (Biliary Cyst OR Pancreatic pseudocyst)

Biliary Cyst - Presents at *age 10* as triad w/: 1- Palpable mass 2- Pain 3- Jaundice (obstructive cholestasis) & acholic stools ---(This present similarly to Acute pancreatitis However, pt has no vomiting or radiating epigastric pain -> which is the most common presenting symptom of acute pancreatitis) UltraSound findings - *Extra-hepatic, single cystic dilatation* of the bile duct Pancreatic pseudocyst - Complication of pancreatitis → characterized by ---1) palpable epigastric mass -------[pt has a RUQ mass] ---2) ↑ pancreatic enzymes --------(Pt presents w/ increased pancreatic lipase & amylase) ---3) Timeline - Typically develops several weeks after acute pancreatitis; pt has no signs of acute pancreatitis (vomiting, pain that radiates to back)

A 16B, presents to the office in December d/t knee swelling that began earlier that day following soccer practice, although he does not recall any specific injury. The pt has mild associated pain & stiffness. In addition, over the last several months he has experienced fatigue w/occasional flu-like symptoms & variable joint pains. The pt has no sig PMHx. He is the captain of his high school soccer team & spent the summer at a soccer camp in Maine. The pt is sexually active w/ his gf & uses condoms. Vitals are normal. Cardiopulmonary exam is normal. The pt is able to bear wt & has a grossly normal gait. Exam of the right knee shows palpable warmth & mild effusion but no tenderness. ROM testing shows a decrease in flexion in the right knee compared to the left. Other joints are normal. Plain radiographs of the knee joint reveal no bony deformity. Aspiration of the knee joint yields yellow-colored translucent fluid w/ a leukocyte count of 20K (50% neutro's) & no organisms on gram stain. Which of the following is the most likely cause of this pt's swelling? (Borrelia infection OR Staph joint infection OR Disseminated gonococcal infection)

Borrelia infection -Pt most likely presents w/ Late Lyme disease -A) RF ---1) Travel to endemic areas -------(Maine) ---------Can present Late i.e., month-to-years -B) Signs/symptoms ---1) Acute monoarticular arthritis following months of migratory arthralgias ------------Arthritis is a monoarticular or asymmetric Oligoarticular arthritis -> most commonly involving the knee ---2) Encephalitis ---3) peripheral neuropathy -C) Associated labs ---1) Synovial fluid avg of leuko's 25K ---2) Gm stain -> neg -------(Must Dx w/ ELISA or Western blot) Disseminated gonococcal infection - Also presents w/ chronic joint pain in a sexually active young Pateint - However, key differences are: ---1) Usually causes septic arthritis ---1) Joint fluid would be purulent w/ higher NEUTROPHIL cell count ---2) pt would have POLY-ARTICULAR arthralgias usually w/ tenosynovitis & pustular skin lesions ---3) Knee would feel warm, tender to the touch ---4) Gm stain would be positive Staph - Can also cause joint pain - However, key differences are: ---1) Joint fluid would be purulent w/ higher NEUTROPHIL cell count ---2) Gram stain would be positive ---3) Knee would feel warm, tender to the touch

An 8F, presents d/t nasal congestion for 2 wks. Her nasal discharge has become thick & green, & fever started today. On PE, the pt has mucopurulent nasal drip & tenderness over the maxillae. The remainder of the exam is normal. A Dx of acute sinusitis is made, & antibiotics are initiated. The pt has had 2 prior episodes of sinusitis this year. PMHx is sig for recurrent Giardia infection & a hospitalization last year for step pneumo bacteria; prior to this, the pt was in good health. She has had asthma since age 3 but takes no meds other than bronchodilators as needed. Ht & Wt are currently at the 5th percentile for age. All immunizations are up to date. Her developmental milestones were achieved on time. CBC shows: - Hb-------------12 - Plts-------------180K - Leuko's--------7,5K What is the most likely Dx? (Brutons OR CVID)

CVID - Pt is a girl ----Brutons is X-linked!!! - presents with: 1- recurrent respiratory (pneumonia, sinusitis, otitis media) 2- Recurrent GI (Giardia, salmonella, campy) May also present w/: 1- Autoimmune disease (RA, Thyroid) 2- Chronic lung disease (Bronchiectasis) 3- GI disorders (chronic diarrhea, IBD-like conditions) Brutons - It is X-linked, so it would not been seen in girls

A 9F, presents for eval of recurrent vomiting. The pt has no symptoms today but has had nausea, vomiting, & diffuse abd pain for several days each month over the last 8 months. When the vomiting occurs, it typically begins early in the morning, recurs 10-15 times/day, & lasts for 2 days. The vomiting is non-bloody & non-bilious. The mom states that the episodes were more frequent around the beginning of the school year & occurred 5 x's in a 2-month span. The last 2 times, the mom has taken off work b/c the daughter required hospitalization for IV hydration. The pt has no fever, headache, of diarrhea, during the episodes. Between episodes, she is active & eats well. Ht & Wt are at the 25th percentile for age, similar to her prior growth curves at her well visit 6 month ago. The pt is no distress & is well hydrated. Mucous membranes are moist, but the maxillary incisors have erosive dental caries. The abd is soft & nontender. Neuro exam is normal. Which of the following is the most likely cause of this pt's symptoms? (Bulimia nervosa OR Cyclic vomiting syndrome)

Cyclic vomiting disorder - Pt presents w/ *recurrent, predictable* pattern of acute & frequent vomiting that resolves spontaneously w/ no symptoms between episodes - Growth, exam, & eating patterns are all normal - Pts usually have a family Hx of *migranes* - Vomiting may be triggered by stress or infection & occur in intervals → pt would be asymptomatic between episodes Bulimia nervosa - Characterized by recurrent binge eating followed by inappropriate compensatory behavior to prevent wt gain - Pt also has no Hx of caloric restriction, binge eating, or preoccupation w/ body weight & shape

What is the diagnostic modality for meconium ileus & what is the finding on DRE?

Diagnostic modality -> Contrast enema DRE findings -> ABSENT Squirt sign

A 7B, presents d/t "yellow eyes" over the past few months. His parents state that he has been tired, pale, & frequently uninterested in playing. The boy's father has had similar symptoms throughout his life. The pt takes no meds. PE shows a tired-appearing boy w/ pale conjunctiva, sclera icterus, & generalized jaundice. The tip of the spleen is palpable. Labs show: - MCHC--------------38% - Total bilirubin--------3 - Direct----------------0.2 - AST------------------27 - ALT------------------32 PBS shows anisocytosis, spherocytes, & polychromatophilia. Coombs test is negative. Which of the following is the best next step in Eval? (Eosin-5-maleimide binding OR G6PD activity)

Eosin-5-maleimide binding > Pt has hereditary spherocytosis >> which typically presents as a *triad*: 1- Hemolytic anemia 2- Jaundice 3- Splenomegaly Relevant labs 1- *Increased MCHC* (N 36%) Key lab finding 2- *Increased bilirubin* 3- *Spherocytes* 4- Increased osmotic fragility test --- *Abnormal eosin-5-maleimide binding test* Flow cytometry ---Acidified glycerol lysis test G6PD > Can present w/ hemolytic anemia & jaundice following oxidative stress *MCHC would not be elevated*

What is the best test to Dx Duchenne muscular dystrophy? (Genetic testing OR Muscle biopsy)

Genetic testing - Gold Standard for Dx of Duchenne muscular dystrophy - Pathophysio ----Dystrophin gene on xp21 gene Muscle biopsy - Would show fibrosis & fatty infiltration that supports the Dx → but is not gold standard

A 4B, presents to the ER by his parents for eval of a fever & headache. Two days ago, the boy began to have a low-grade fever, cough, & congestion. Last night, his fever became higher, & he became fussy, more difficult to console, & less active. He slept poorly & woke up w/ a headache unrelieved w/ OTC pain meds. The pt vomited twice prior to arrival. He has sig PMHx but has not received regular health maintenance or vaccinations. Temp is 102.9F, pulse is 110/min, RR are 24/min. PE shows an irritable child. Pupils are equal & reactive; fundoscopic exam is limited by photophobia. The oropharynx is erythematous. Nuchal rigidity is present & bilateral lower extremities flex when the neck is flexed. The remainder of the exam is normal. Labs show: 1- Glucose--------60mg/dL 2- Protein---------80mg/dL 3- RBCs-----------2 WBC's - Neutros---------10% - Lymphos--------70% - Monocytes-------20% which of the following pathogens is the most likely responsible? (Group B Coxsackie Vs Strep pneumo VS Crypto

Group B Coxsackie > Must be viral B/c lymphocytes are elevated Also 1- Normal/ slightly elevated protein 2- *Normal glucose* Strep pneumo Labs would show: 1- Increased neutros 2- Increased protein 3- *Decreased* glucose Crypto 1- Lymphocyte elevated 2- Increased Protein 3- *Decreased* glucose

What is the most common complication for sickle cell trait? (hematuria OR Splenic infarction)

Hematuria Splenic infacrtion - most common complication in Sickle cell disease

While working in the newborn nursery, you have the chance to counsel a first time mom. She had a healthy pregnancy & good prenatal care. The infant was delivered via normal spontaneous vaginal delivery & is doing well. You are counseling the mom on the benefits of breastfeeding, & she asks you what the differences are between human milk & formula. Which of the following is the true stmt? (Human milk protein absorbs better & improves gastric emptying OR The main advantage of formula is it decreases colic)

Human milk protein absorbs better & improves gastric emptying > Human milk is 70% whey & 30% casein which makes it more easily digestible The main advantage of formula is it decreases colic > Human milk decreases colic

A 17F, presents for eval of heavy menstrual bleeding. She typically has 4 days of heavy bleeding, often requiring 5-6 pads/day. The pt awakens multiple times a night to change her pad & often soaks through her clothing & bed sheets. She has mild cramping on the first 2 days or her menses that is controlled by ibuprofen but has no intermenstrual bleeding or chronic pelvic pain. Menarche was at age 12; menses were irregular the first couple of years but are now regular & occur every 26 days. The pt has no chronic medical conditions & has had no surgery. She has a male partner & uses condoms. Vitals are normal. BMI is 23. On bimanual exam, the uteri is small & mobile; no adnexal masses or tenderness is present. Labs show: - Hb------------------9.6 - MCV----------------76 - Plts------------------300K - PT-------------------13sec -aPTT-----------------38sec Urine pregnancy test is neg. Which of the following is the most likely cause of this pt's heavy menstrual bleeding? (Immature HPO axis OR Impaired platelet adhesion)

Impaired Plts adhesion - Pt presents w/ Von Willebrand disease (vWD) → which causes impaired Plts adhesion - Most often presents as increased bleeding during menstruation in young females Immature HPO axis - Pt would have a PMHx significant for irregular menstrual periods & anovulatory bleeding -----(pt has had regular menstrual periods)

Which of the following are RF for development of respiratory distress syndrome? (IUGR OR Maternal DM)

Maternal DM -A) RFs for RDS include: ------1) Prematurity (MOST IMPORTANT) ------2) Male sex ------3) Perinatal asphyxia ------4) Maternal GDM -------------delays maturation of surfactant production d/t high levels of insulin ------5) CS w/out labor IUGR > Associated w/ DECREASED risk of RDS ---(intrauterine stress is thought to stimulate early fetal lung maturity)

What is the best medication for an 8 year old boy with ADHD? (Atomoxetine OR Methylphenidate)

Methylphenidate Atomoxetine > Can be considered if there is a family preference for *non-stimulant meds* or in pts w/ *substance abuse in Hx*

A 10B, presents w/ a headache, vomiting, & visual disturbances. The pt has had 2 wks of persistent headache for which he was given OTC analgesics w/out relief. He has also had daily episodes of non bloody, non-bilious emesis. Neuro exam shows bilateral upper eyelid retraction & limitation of upward gaze w/ a preference for downward gaze. Pupils react sluggishly to light but respond appropriately to accommodation. Strength, sensation, & deep tendon reflexes are normal. Which of the following lesions are most likely responsible for this pts symptoms? (Craniopharyngioma OR Pinealoma OR Neuroblastoma)

Pinealoma > Features of pinealomas include: *Parinaud's syndrome (first three)* 1- *Limited upward gaze* 2- *Upper eyelid retraction (Collier sign)* 3- *Pupils non-reactive to light but reactive to accommodation* 4- Obstructive hydrocephalus --*papilledema* --*Headache, vomiting* --Ataxia Craniopharyngioma > suprasellar masses that can compress optic chiasm but not Parinaud's syndrome Neuroblastoma > Usually located in the adrenal gland >> intracranial tumors are rare

A 3F, presents to the ED by her babysitter for eval of burns. The babysitter states that she was bathing the child when the child began crying & was quickly removed from the water. The pt lives w/ her biological parents, who are at work, & has been cared for by this babysitter for the last 6 months. Exam shows an alert child who is crying inconsolably & in severe pain. The back, buttocks, & legs have patches of blistered, waxy, red, skin. If found in this pt, which of the following burn characteristics would be the most concerning for child abuse? (Presence of splash marks OR Sparing of flexural crease)

Sparing of the flexural crease > Usually involve the buttocks, back, & legs > seen b/c ankle, knee, & hip flexion at the time of forced immersion > stocking or glove burn distribution w/a sharp line of demarcation & uniform burn depth may also be seen > Absence of splash marks also seen

What is the preferred tmt in otitis externa? (topical cipro OR Topical nystatin)

Topical Cipro - Preferred tmt ± topical glucocorticoid Topical nystatin - Used for fungal infections but not in ear

A 2-hr-old B, has developed worsening cyanosis over the past few mins. The infant was delivered vaginally at term after an uncomplicated pregnancy. Birth Wt was 9-lbs. Apgar scores were 7 & 8 at 1 & 5 mins. O2 was administered by hood but does not relieve the cyanosis. Further exam shows tachypnea, subcostal retractions, a normal S1, & a single loud S2, & no murmur. Which of the following is the most likely cause of the infants cyanosis? (Tetralogy of fallot OR Transposition)

Transposition - Pt most likely presents w/ transposition of great arteries -A) Etiology ---1) Most common congenital cyanotic heart disease in neonatal period -B) Signs/symptoms ---1) Cyanosis w/in 24 hrs of life -------a) Tachypnea -------b) Subcostal retractions -C) Murmur -----1) Single S2 ---------a) d/t absent pulm component of S2 ---------b) NO MURMUR -D) XR findings -----1) Narrow mediastinum "egg on string" -E) TMT ---1) PG's to keep the PDA open Tetralogy of fallot - Also presents w/ cyanosis at birth & a single S2 sound - However, key differences are: ---1) Presents w/ a systolic/holosystolic murmur --------- Pulmonary stenosis-like murmur (crescendo-decrescendo) murmur at left upper sternal border ----------------[pt has NO MURMUR] ---2) It is usually seen w/ associated congenital disorders including Di George -------(pt has no symptoms of Di George) ---3) Cardio exam would reveal ---------a) Early systolic click ---------c) MURMUR ---4) CXR findings ---------1) Boot shaped heart

Name the immuno-deficiency disease where this tmt would be used? 1- Cultured thymus tranplant 2- Immunomodulator therapy 3- IVIG infusion

1- Cultured thymus transplant →DiGeorge 2- Immunomodulator → Interferon-γ →CGD 3- IVIG → CVID or Brutons

A pt with amenorrhea has a pelvic US which reveals a prepubertal uterus w/ small ovaries. Labs show: - FSH----------------60 (N<40) - LH------------------30 Which of the following is the most Likely underlying cause of this pts condition? (Congenital absence of an X chromosome OR Abnormal development of the Mullerian duct)

Congenital absence of an X chromosome - Pt has *Turner Syndrome* which results in *Streak ovaries* (small ovaries w/ minimal or no follicles) - Pts have pubic hair b/c TS does not affect the adrenal glands Hormone levels - ↓ estrogen levels (inhibit neg feedback on HPO axis) → ↑FSH & ↑LH levels

A 2-month-old baby presents b/c of vomiting & diarrhea. She became ill three days ago when she developed a low-grade fever & diarrhea. She refuses to eat, but was drinking some until yesterday when she began vomiting. In the last 24 hrs she has had one wet diaper. Temp is 100.4F, Pulse is 110/min, RR is 25/min, BP is 90/50. On exam, she is lethargic w/ shrunken eyes. Her mucous membranes are dry & capillary refill is 3-4 seconds. Which of the following is the next best step in the MGMT? (20mL/kg of normal saline IV OR 20mL/kg of 1/2 normal saline IV)

20mL/kg of normal saline IV > *Isotonic* crystalloid is the only crystalloid solution recommended for IV in children

What are the most common causes of meningitis in children: - <1 month - ≥ 1 month

<1 month 1- GBS 2- E.coli (+ other Gm-) 3- Listeria 4- HSV ≥1 month 1- Strep pneumo 2- Neisseria

What describes a pts long-term outcome following an orchioplexy? (The pt has no sig long-term risks OR The risk if testicular cancer is increased OR The risk of testicular torsion remains increased)

The risk of testicular cancer is increased > Orchioplexy is performed at 1 yr to avoid complications >> w/out surgery, an undescended testicle has a higher rate of testicular torsion >> however, *testicular cancer risk remains elevated*

A 7B, presents d/t 2 days of sore throat, poor appetite, & malaise. Several classmates have missed school d/t similar symptoms. The pt has no cough, rhinorrhea, or nasal congestion. He takes no meds, & has NKA. Immunizations are up to date. Temp is 102F, BP is 110/70, Pulse is 130/min, RR is 16/min. On exam, the tonsils are swollen & covered w/ thin white exudates. Small, tender anterior cervical LN's are palpated. Rapid Strep antigen detection is negative. Which of the following is the most appropriate next step in mgmt? (Amox OR Throat culture OR Heterophile antibody testing)

Throat culture -Pt presents w/ *bacterial pharyngitis* -A) Signs/Symptoms -----1) ACUTE onset of symptoms -----2) Tonsillar erythema & (thin) exudates -----3) Tender ANTERIOR cervical LN -----4) Palatal petechiae -----5) Acute onset sore throat -----6) Headache -----7) Nausea, vomiting, malaise, -----8) Abdominal pain -----9) Odynophagia, ----------(may lead to poor appetite) -----10) Lack of a cough ------11) Scarlantiform rash ------12) Swollen uvula -B) MGMT -----a) Diagnostic tests --------1) RADT (rapid antigen detection test) ------------a) Postive RADT -> antibiotics ------------b) Negative RADT -> throat culture --------2) Throat culture -----b) TMT ---------1) Penicillin V -> Adults ---------2) Amoxicillin -> children ---------3) IM penicillin G -------------(children who cannot tolerate oral meds) Amoxicillin - First step is to get throat culture Heterophile antibody testing - Used to Dx infectious mononucleosis from EBV - Also presents w/ present with fever, exudative pharyngitis, and lymphadenopathy - However, key differences include: ----1) 1-4 week PRODROMAL period​ ----------[GAS has an ACUTE ONSET] ----2) Severe fatigue ----------[pt does not have] ----3) Hepatosplenomegaly ----------[pt does not have] ----4) POSTERIOR> anterior LNs affected in EBV ---------[pt has ANTERIOR > posterior, more specific to GAS]

What are the expected values for - Reticulocyte count (High/low) - Direct Coombs (Negative/positive) - MCHC (high/low) in hereditary spherocytosis?

- Reticulocyte count → High → In all types of hemolytic anemia, the BM produces increased reticulocytes - Direct Coombs → negative → no antibodies - MCHC → high → elevated d/t membrane loss & RBC dehydration

Med side effect -Pt presents w/ intracranial HTN → AE of isotretinoin → presents w/: --1) Throbbing headaches (initial symptom) → worse when lying flat & improves w/ standing --2) Headache may be accompanied w/ -----Pulsatile tinnitus (whooshing sound), -----Nausea, -----Vomiting, -----Blurry vision (papilledema) -----Cranial nerve palsies (CN VI) Cluster headaches -Pt would have excruciating, unilateral headaches that occur near the eye/temporal region -Most common in age 20-50 -Papilledema does not occur

A 16B, presents for eval of headaches. For the last month, he has had daily, throbbing pain in the frontal region that is worse in the morning & occasionally associated w/nausea & vomiting. Acetaminophen provides some relief. The pt also had several brief episodes of blurriness in his left eye during football practice last week. In addition to acetaminophen, e takes oral isotretinoin for severe acne. Temp 98.1F, BP 130/80, pulse 70/min, & RR are 15/min. The pt is sitting up & cooperative w/ the exam. The pupils are equal & reactive to light. Eye exam shows bilateral optic disc edema & decreased visual acuity. The visual fields are full. There is no neck stiffness. Motor exam reveals 5/5 strength, 2+ deep tendon reflexes, & a normal plantar response. Sensory exam is unremarkable. Which of the following is the most likely cause of this pts symptoms? (Cluster headaches OR Med side effect)

Keratosis pilaris -Characteristics include: --1) Presents as small, painless papules → a rough skin texture & mottled perifollicular erythema ---Can be pruritic & may get exacerbated by cold, dry weather --2) Can occur anywhere on the body but most common on the posterior surface of the upper arm Milaria -AKA heat rash -Most common in newborns & young children -Manifests as small, thin-walled vesicles to patches of erythematous papules/pustules

A 16F, presents d/t a skin condition. For the last 3 years, she has had small "pimples" on the posterior surface of her upper arms. The lesions are associated w/ mild pruritus during the winter months but are otherwise asymptomatic, & the pt is primarily concerned d/t their appearance when she wears short-sleeved tops. PMHx is unremarkable, & the pt takes no meds. Exam findings are shown. Which of the following is the most likely Dx? (Keratosis pilaris OR Miliaria)

Renal & bladder US -Pt most likely has posterior urethral valves -A) Signs/Symptoms ----1) Low Abd pain/Abd distention (distended bladder) ----2) Dysuria (Poor urine output) --------a) Can also present w/ Increased frequency & urgency ----3) Malodorous urine ----4) Respiratory distress → (hypoxia, tachypnea, decreased breath sounds) -----------a) oligohydraminos -----------b) lung hypoplasia ----5) Lethargy/failure to thrive ----6) Low-grade fever -B) MGMT ----a) Diagnostic -------1) Renal & bladder US -----------a) Dilated bladder -----------b) Bilateral hydoureters & hydronephrosis --------2) Voiding cysturethrogram→ (DIAGNOSTIC) -----------a) Only used if US is normal --------3) Cystoscopy → (can also be DIAGNOSTIC) ----B) TMT ---------1) Conservative ------------a) Bladder drainage & electrolyte correction ---------2) Low-dose TMP-SMX → PROPHYLAXIS ------------a) Until resolution of reflux Upper GI series -Used to evaluate for malrotation - Also presents w/ abd distention and/or diffuse abd pain -However, key differences include: ---1) Pt would have Sudden onset bilious vomting -------[This pt has REGURGITATION (spit up)] ---2) Pain would be out-of-proportion to the physical exam ---3) Malrotation DOES NOT commonly cause respiratory distress

A 2-day-old boy, is evaluated in the newborn nursery. The pt was born at an estimated 40-wks gestation to a 26-y/o primigravida who received no prenatal care. He has been taking 30 mL (1 oz) of a cow's milk-based formula every 3-hrs since birth. The mom says the boy seems uncomfortable. She is worried about dehydration b/c he has been spitting up formula after his last few feeds & has only had one damp diaper & a single stool since birth. Birth Wt was 7-lbs, 1-oz & Wt today is 7-lbs, 3-oz. Temp is 98.1F, pulse is 150/min, RR are 66/min. Pulse Ox is 88% on room air. Lung exam shows diminished breath sounds bilaterally. Cardiac exam is normal. The abd is distended. The urethral meatus appears intact. A small sacral dimple w/intact skin overlies the coccyx. His skin is warm & pink. CXR reveals diminished lung volumes bilaterally w/ normal heart size. The pt is placed on supplemental O2. Which of the following is the best next step in Evaluation of this pt's condition? (Renal & bladder US OR Upper GI series)

Obtain US of the RUQ -Pt presents w/biliary atresia → characteristics include: --1) Manifests in first 2-8 wks of life --2) Jaundice + Scleral icterus (w/ ↑ DIRECT hyperbilirubinemia) --3) Light-colored stools Important labs 1) ↑↑ direct hyperbilirubinemia Diagnostic 1) US of RUQ → findings include: ---a) Abnormal/absent gallbladder ---b) Absent CBD ---c) Triangular cord sign (fibrous remnants seen above the porta hepatis) 2) Liver biopsy → to help distinguish from other cholestatic conditions Recommend continued breastfeeding -Correct if pt had breast milk jaundice → pt would increased indirect bilirubin ----(Pt has increased DIRECT bilirubin) Send genetic testing for Crigler-Najjar -Pt would have increased indirect bilirubin ----(Pt has increased DIRECT bilirubin)

A 2-wk-old boy, presents d/t to persistent yellowing of the skin & eyes. In the first few days of life, his parents were told the pt had jaundice that would likely improve over time. However, they feel that his color has not changed significantly. The pt was born at term, & his parents declined the Hep B vaccine b/c the mom's serologic studies confirmed she is immune. The boy is exclusively breastfed & voids frequently. He has 3-4 BM's, & the stools are light yellow. The pts only med is Vit. D. Wt today is 7.7-lbs, increased from 7.5-lbs at birth. Exam reveals scleral icterus & jaundice of the face & chest. The abd is soft w/normal bowel sounds. The remainder of the exam is normal. Total bile is 10.0 & direct is 7.4. AST & ALT are normal. Maternal blood type is O, Rh(D)+. Which of the following is the best next step in MGMT? Obtain US of RUQ OR Recommend continued breastfeeding OR Send genetic testing for Crigler-Najjar)

AZ -Pt presents w/ whooping cough d/t pertussis -A) Signs,Symptoms --presentation includes 2 phases of progression: -----------Phase 1 -Catarrhal phase (Mild cough & rhinorrhea) ---Phase be be short/mild or absent in children Phase 2 -----------Phase 2 (Paroxysmal Phase) -----1) Severe cough w/ life-threatening apnea -----2) Posttussive emesis --------(Pt starts coughing harshly than stopped breathing) ---2) Gagging, gasping, cyanosis -B) Complications -----1) Apnea -----2) seizures -C) MGMT ---a) TMT ------1) Supportive TMT → (PRIMARY TMT) ------2) Macrolide antibiotics (prophylaxis & TMT) -----------(AZ, clarithromycin, erythromycin) Amoxicillin - Bordetella is a Gm(-) bacteria, amoxicillin would be ineffective against it

A 3-wks old, presents to the ED d/t apnea & cyanosis. Her mother reports multiple episodes over the last 48-hrs of gagging & gasping for air. During the last event, the pt started coughing harshly during a feed than stopped breathing. She became cyanotic for approximately 12 seconds, then quickly returned to normal. The pt has a 1-wks Hx of clear rhinorrhea & increasing cough w/ episodes of emesis. Temp 99F, BP is 85/52, pulse is 100/min, RR 45/min. She is well developed & active. There is nasal congestion & clear rhinorrhea. Lung sounds are clear except for referred upper airway congestion. Cardiac & abd exams are normal. Which of the following is the most appropriate empiric med for this pt? (Amoxicillin OR AZ)

Bilateral hip US -Pt presents w/ transient synovitis → presentation includes: --1) Typically presents in kids 3-8 --2) Typically after a viral illness --3) Afebrile/low-grade fever --4) Limited mobility of affected joint but able to bear weight --5) Inflammatory markers -/↑ (slightly) Diagnostic -Bilateral hip US CT scan of abdomen -Would be used when psoas abscess is suspected -Pt would present w/ pain radiating to the hip that worsens w/ extension & improves w/ flexion [however, pt "wails in pain" when his hips are flexed]

A 3B, presents to the ED d/t refusal to walk. The pts mother first noticed a mild limp 3 days ago after picking him up from day care. The boy tends to climb & jump on furniture, so she initially attributed the limp to rough play. The symptoms worsened today & the pt refuses to stand, falling when help up by his mother. He has no sig PMHx but has had a runny nose & fever intermittently for 2 months since starting day care. Temp 100.4F, pulse 90/min, RR 24/min. Pulse Ox is 100% on room air. The pt appears comfortable while lying supine on the exam table. He has clear rhinorrhea w/several soft & mobile anterior cervical LN's approximately 1-cm in diameter. The abd is nontender & the liver edge is palpable. The hips are flexed bilaterally. During mvmt of the left hip, the pt wails & resists. Sensation is normal, & reflexes are intact. Babinski sign is neg bilaterally. Labs show: -Hb------------------------11.5 -Plts-----------------------380K -Leukos-------------------15K --Neutros-----------------80% --Lymphos----------------20% -ESR-----------------------30 mm/hr Which of the following is the best next step in MGMT? (Bilateral hip US OR CT scan of abdomen)

Uveitis -Pt presents w/Juvenile idiopathic arthritis → characteristics include: --1) Oligoarticular (more common in age 2-4) or Polyarticular (more common in 2-10/14) --2) Limp that is worse in the AM --3) No systemic symptoms --4) Pain may be minimal, but can have joint swelling Complications 1) Uveitis IBD -Not associated w/ JIA -Associated w/ spondyloarthritis

A 3F, presents for eval of right knee swelling. Her mother first noticed the swelling & a limp 2 months ago but attributed them to roughhousing w/ her brother. Typically, the limping was present in the mornings & resolved by midday. However, while bathing the pt last night, her mother noticed that the right knee was more swollen that usual. The knee is not painful, & the pt has had no fever or rashes. Temp is 99.5F. She can bear wt on both legs but has a noticeable limp. The right knee is swollen & slightly warm. The overlying skin appears normal & intact. There is no tenderness to palpation. Peripheral pulses are normal; sensation is intact. This pt is at greatest risk for which of the following complications? (Uveitis OR IBD)

Decreased excretion -Pt most likely has Biliary atresia → characteristics include: 1) Presents at 2-8 wks of life 2) Jaundice w/ Increased DIRECT hyperbilirubinemia 3) Pale stools 4) Hepatomegaly 5) Splenomegaly (late finding, age > 3 months) Pathophysio -Newborns have decreased bilirubin excretion d/t bile duct obstruction (cholestasis) Labs 1) Elevated Direct bilirubin 2) Elevated LFT's TMT 1) Kasai procedure (hepatoportoenterostomy) → diverts Bile to SI → most successful if performed < 2 months 2) Liver transplant Decreased conjugation -Pt would have ↑↑ Indirect bilirubin

A 4-wks-old girl presents d/t yellowing of the skin. The pts first noticed the symptoms a week ago & the pts skin has become progressively more yellow. She was born at term to a primigravida mom via spontaneous vaginal delivery. The pregnancy & delivery were unremarkable, & the pt was discharged at 48 hrs of life. She is exclusively breastfed & is gaining wt appropriately. Bowel mvmts occur once/twice daily, but stool has become increasingly light in color & is now pale gray. Exam reveals scleral icterus & jaundice to the umbilicus. The liver is palpable 4-cm below the coastal margin & the spleen tip is palpable. Labs show: -Hb---------------------------11 -Total bili---------------------12.8 -Direct bili--------------------8.1 -SGOT (AST)------------------52 -SGPT (ALT)-------------------70 Which of the following is the most likely primary mechanism for this pts hyperbilirubinemia? (Decreased conjugation OR Decreased excretion)

Newborn inability to metabolize breast milk -Pt presents w/ Galactosemia → presentation includes: --1) Presents typically in first week of life --2) Vomiting → poor nutrition leads to ------Lethargy ------Hypotonia ------Dehydration (shrunken fontanelle) --3) Jaundice --4) Hepatomegaly --5) Cataracts Maternal non-adherence to vaccination schedule -If the pt presented w/ the symptoms above & it was d/t a congenital infection → we would suspect rubella -However, the pt is missing key symptoms of a rubella infection including microcephaly & a heart defect (PDA)

A 5-day-old girl presents to the ED d/t fussiness. The pt was born at home to a 36F, G5P5, via spontaneous vaginal delivery. The mother received no prenatal care. The newborn has been breastfeeding exclusively & has had non-bloody, non-bilious vomiting after feeds for the past day. Head circumference & length are at the 50th percentile. Wt is at 20th percentile. Temp is 99F & pulse is 180/min. The newborn poor tone & is jaundiced. The anterior fontanelle is sunken w/ over-riding sutures. Eye exam shows bilateral cataracts. Ears are in the normal position, & the palate is intact w/ a normal tongue. Cardiopulmonary exam is normal. The liver is palpable 4-cm below the costal margin. Which of the following is the most likely cause of this pts presentation? (Maternal non-adherence to vaccination schedule OR Newborn inability to metabolize breast milk)

Vitamin deficiency -Pt most likely has rickets → presentation includes: --1) RF's -----Exclusive diet of breast milk w/out Vit D supplementation --2) Symptoms -----A) Craniotabes (skull bones that depress w/ pressure) -----B) Widened wrists (swelling of wrists) -----C) LE bowing (genu varum) → only seen in older pts once the children have become wt bearing Non-Accident trauma -New/old fractures would be expected on XR

A 6-month-old Boy, present for a well-child visit. The pt sits w/ support, mouths toys, & responds to his name. He has not yet started babbling or using a pincer grip. The pt drinks 24-28 oz of donated, pasteurized breast milk obtained through a local milk bank. He was adopted from Uganda at age 2-mnths. The only available birth Hx is the the pt was born full-term & that labor & delivery were uncomplicated. FMHx is unknown. Immunizations are up to date. He has no sig PMHx & takes no meds. Ht & Wt are at the 20th & 40th percentiles, respectively. Head circumference is at the 30th percentile. The pt has no dysmorphic facial features. The anterior fontanel is open & flat, & the skull bones are soft & flexible to pressure. Bilateral swelling of wrist is present. There is no bowing of the LE's. Cardiopulmonary & abd exams are normal. A wrist XR is shown. Which of the following is the most likely cause of this pts XR abnormality? (Non-accidental trauma OR Vitamin deficiency)

Obstructive sleep apnea -Characteristics include: --1) Manifestations worsen at night → & better during the day --2) Gasping for air --3) Can eat & drink w/out gagging --4) Can keep up with peers --5) Daytime sleepiness -----Inattention -----Learning difficulties -----Behavioral problems --6) Enuresis --7) Parasomnias Choanal atresia -Also presents w/ noisy breathing -However, other symptoms include: ---1) Mainly occurs in neonates (congenital) → more common in girls > boys ------(pt is 6 years old) ---2) Unilateral ---3) Cyanosis (worsens when feeding, improves w/ Crying) ---4) Unilateral discharge (presents later in life) Laryngomalacia -Also presents w/ noisy breathing → However: --1) Mainly occurs in neonates (congenital) -----(pt is 6 years old) --2) Intermittent, wet, inspiratory stridor → loudest at 4-8 months (resolves by 12-18 months) ----(pt is 6 years old) --3) Stridor worse during resp infections & supine --4) Stridor loudest when feeding or sleeping (may improve w/ crying)

A 6B, presents d/t noisy breathing. Approximately 6-mnths ago, his older brother asked to be moved to a different room b/c the pt's noisy breathing was keeping him up at night. His mother reports that it sounds like he is gasping for air. During the day, the pt breathes thru his mouth & eats & drinks w/out gagging or choking. He is able to keep up w/ his peers during outdoor play. The pt takes no meds & is up to date w/ vaccinations. He lives w/his mother, father, older brother, & the family cat. No one in the household smokes. Ht is at 40th percentile & Wt is at 25th percentile. Which of the following is the most likely Dx? (Laryngomalacia OR Choanal atresia OR Obstructive sleep apnea)

No long term complications -Pt has a greenstick fracture (pt only has one side of the bone fractured) -Once prompt reduction & immobilization is done, & the fracture is fully healed, the pt should have no long term complications Limited range of supination & pronation -This is complication of malunion after a severely displaced radial fracture → pts fracture is only mildly angulated Limb length discrepancy -This would be a complication of a fracture involving the growth plate (physis) leading to growth arrest

A 6B, presents to the ED d/t an injury to his left arm. About an hour ago, he was jumping on the bed when he bounced off & landed on his outstretched hand. He cried & has not wanted to use the arm since then. The pt has no sig PMHx. Exam shows mild edema of the left distal forearm w/ intact sensation & pulses distally. Palpation of the forearm produces sharp pain. The pt refuses to move the wrist. XR is given. The forearm is immobilized. The pt is at greatest risk for developing which of the following long-term complications? (Limb length discrepancy OR Limited range of supination & pronation OR No long term complications)

Medulloblastoma -Characteristics include: --1) Second most common pediatric tumor --2) Cerebellar dysfunction -----Truncal/gait ataxia -----Dysmetria, intention tremor --3) Obstructive hydrocephalus -----Abducens palsy -----Headaceh, vomiting -----Papilledema Craniopharyngioma -Presents w/ Bi-temporal field vision loss (Tunnel vision) -----(Pt has abducens palsy)

A 6F, presents d/t multiple falls while running in the past few months. Over the past month, the pt has also had intermittent headaches, w/increased frequency in the past 2 weeks. The headaches are worse in the morning & have occasionally been accompanied by nausea & vomiting. Exam shows a tired-appearing young girl. The pt walks w/a wide-based gait & has difficulty w/ heel-to-toe walking. Eye exam reveals bilateral abducens nerve palsies & papilledema. The remainder of the cranial nerve exam is nominal. Strength is 5/5 in bilateral upper & lower extremities. Sensation is intact & reflexes are 2+ bilaterally. Babinski testing reveals plantar flexion of the toes. Which of the following is the most likely responsible for this pts symptoms? (Craniopharyngioma OR Medulloblastoma)

Supportive care only -Pt most likely has VIRAL rhinosinusitis → Viral illness is characterized by: -A) Signs/Symptoms -----1) No/Low fever or early resolution -----------(pt had a fever the first day but has since been afebrile) ------2) Mild symptoms -----------(generally well appearing, mild facial pain) -B) MGMT ----1) TMT -------a) Supportive ------------(Improvement & resolution by days 5-10) Oral antibiotics -TMT for BACTERIAL sinusitis -A) Signs/Symptoms -----1) High Fever & lasting ≥ 3 days -----2) New/recurrent fever after initial improvement -----3) Persistent symptoms ≥ 10 days ---------[pt has none of these symptoms so antibiotic TMT is not indicated]

A 7B, presents for eval of rhinorrhea. 3-days ago, the pt developed rhinorrhea & a cough that is worse at night. For the past day, he has had intermittent, mild pain around the upper cheeks, particularly when leaning forward. He had fever on the first day of illness but has been afebrile for 48-hrs. The pt appetite is decreased, but he is drinking fluids & voiding normally. He has no sig PMHx & takes no meds. Temp 99.7F, pulse is 100/min, RR are 20/min. The pt is well-appearing & comfortable on exam. The tympanic membranes are clear. Nasal discharge is profuse & yellow-green in color. The posterior oropharynx is erythematous. Palpation over the upper cheeks bilaterally causes mild discomfort. Cardiopulmonary exam is unremarkable. The abd is soft w/no organomegaly. Which of the following is the best next step in MGMT of this pt? (Oral antibiotics OR Supportive care)

Myotonic dystrophy -Presentation includes: --1) Myotonia (delayed muscle relaxation - Grip myotonia) --2) Progressive muscle weakness (Face, hands) --3) Childhood form → -----Cognitive & behavioral problems -----Often presents at age 10 -----Excessive daytime sleepiness --4) Infantile form → Hypotonia, arthrogyrposis Cerebral palsy -Motor dysfunction is characterized by hypertonicity & hyper-reflexia [not myotonia]

A 9B, presents d/t poor academic performance. The pt receives a special needs education, including speech therapy. His teachers have had difficulty keeping his attention in class, & his grades are declining. Over the last few weeks, the pt has been falling asleep at his desk. He watches TV in the evenings until bedtime at 9PM & wakes at 7AM every morning. He feels tired most days & is occasionally sad b/c his parents recently divorced. BP is 104/70, & RR 26/min. Wt & Ht are 50th & 20th percentile. Tonsils are 1+. Cardiopulmonary exam is normal. The pt has difficulty opening his hands after squeezing the examiners fingers. There is full ROM in the bilateral extremities. Which of the following is the most likely explanation of this pts clinical presentation? (Myotonic dystrophy OR Cerebral palsy)

CHARGE syndrome C → Colomba H → Heart defects (TOF, VSD) A → Atresia Choanal R → Retardation of growth/development G → Genitourinary abnormalities E → Ear abnormalities (hearing loss) -Also ---Anosmia ---Cleft palate ---Hypotonia VAaCTERL syndrome V → Vertebral Aa → Anal atresia C → Cardiac defects T & E → TracheaEsophageal fistula R → Renal L → Limb

A newborn boy is being evaluated in the nursery for respiratory distress. He was born an hour ago at 37 weeks via spontaneous vaginal delivery to a primigravida mother who had scant prenatal care. Delivery was uneventful. During skin-to-skin contact, the noticed his face turn blue. The pt was simulated & began crying, which improved his color. Wt, length, & head circumference are in the 25th-50th percentile. Temp 98.2F, pulse 105/min, RR 48/min. Pulse Ox is 97% on room air. As the crying stops, Pulse Ox increases to 80%. The ears are short & wide w/no earlobes. The right is shown. Oropharyngeal exam reveals a cleft palate. The pt has a loud, harsh, holosystolic murmur best heard at the left lower sternal border. The testes are not palpable. The skin & extremities appear normal. Which of the following is the most likely Dx? (CHARGE syndrome OR VAaCTERL syndrome)

Urology eval -Pt present w/ hypospadias (described as ventrally displaced ureteral opening) -A) Signs/Symptoms --1) Urethral meatus located at the coronal margin --2) Dorsal hooded foreskin --3) Penile curvature -B) MGMT ---1) Urological eval Coagulation Studies -Indicated for a pt that may have inherited a coagulopathy (pt's dad has hemophilia - However, he can't inherit this from his dad b/c its X-linked)

A term newborn is undergoing routine eval in the nursery. The boy was born 12-hrs ago via spontaneous vagal delivery to a 24F. His mom had appopriate prenatal care, & her pregnancy was uncomplicated. Delivery was unremarkable, & the boy has had a normal nursery course. He received the Hep B vaccine, erythromycin eye ointment, & intramuscular Vit. K after delivery. FMHx reveals factor VIII deficiency in the boy's paternal grandfather. Vitals are normal. PE shows a sleeping newborn w/ normal tone. A 1/6 systolic murmur is best heard at the left sternal border. Peripheral pulses are strong. Genitourinary exam shows a penis of normal length w/no curvature. The urethral meatus is located at the coronal margin, & there is a dorsal hooded foreskin. Testes are present bilaterally w/in the scrotum. The pt urinates through the urethral meatus during the exam. The father is present & requests that a circumcision be performed. Which of the following is the best next step in MGMT? (Coagulation studies OR Urology eval)

A 4-month-old B, presents for a routine eval. He spent 3 wks in the NICU for mgmt of hypoglycemia & poor feeding secondary to macroglossia. The pt has had no sig PMHx. He is currently at the 99th percentile for Wt, Lt, & head circumference. PE shows an enlarged tongue & a reducible umbilical hernia. His RUE & RLE are sig larger in circumference than the LUE & LLE. The remainder of the exam is normal. In addition to routine vaccinations, which of the following is the best next step in mgmt of this pt? (Abd US OR TSH)

Abd US - Pt presents w/ Beckwith-Widemann syndrome -A) Signs/Symptoms ------1) Fetal macrosomia ----------(rapid growth until late childhood) ------2) Omphalocele or umbilical hernia ------3) Macroglossia ------4) Hemi-hyperplasia ------5) Hypoglycemia -B) Associated complications -> ↑ Risk for ------1) Wilms tumor ------2) Hepato blastoma -C) MGMT ------a) Diagnostic -----------1) Abd US & AFP -> SCREENING ---------------every 3 months from ages 4-8 for Wilms tumor & development of blastoma TSH - Would check in cases of suspected hypothyroidisms - Hypothyroidism also presents w/ macroglossia & umbilical hernia - However, key differences are: ---1) hypothyroidism DOES NOT present w/ macrosomia, hypoglycemia, hemi-hyperplasia -------[pts RUE & RLE are larger than the LUE & LLE]

Compare/Contrast serous otitis media (+effusion) and acute otitis media? (Inflammatory signs, Otoscopic findings)

Acute otitis media -A) Signs/Symptoms ------1) Inflammatory signs -----------a) Otalgia/otorrhea -----------b) Systemic symptoms -------2) Bulging Tympanic membrane ---Hearing loss ) - Decreased tympanic mobility on pneumatic insufflation (indicates middle ear effusion) Serous otitis media -A) Signs/Symptoms -----1) Absence of inflammation, otalgia, otorrhea -----2) Retracted/neutral tympanic membrane

A 11F, perviously healthy, presents w/ a severe headache. The headache began gradually approximately 2 hrs earlier, is bifrontal, & is slightly worse on the right side. The girl feels as though "someone is pounding on my head." Approximately 30mins before her headache started she noted changes in her vision & saw "dark spots" while watching TV. Her mom says that the pt has no recent fevers of illnesses. She has no Hx of night-time awakening or preceding head trauma or injury & no numbness, tingling, or weakness. The girl had never had headaches until 2 wks prior to presentation, when she had a similar episode. On the way to today's appointment, she had a single episode of emesis & still complains of nausea. Temp is 99F, BP is 92/54, Pulse is 88/min, RR is 18/min. The pt's body mass index is at the 60th percentile for her age & gender. She is lying quietly in bed with her eyes closed. When asked to sit-up, she moves very slowly & carefully. Her pupils are equal, round, & briskly reactive to light, she is unable to tolerate a fundoscopic exam. CV, resp, Abd, & neurologic exams are normal. Which of the following is the most appropriate next step in MGMT? (Administer ibuprofen & counsel the family OR Perform Lumbar OR Perform EEG)

Administer ibuprofen & counsel the family - Pt most likely presents w/ *migraines* -A) Etiology -----1) Most common form of headaches in the Peds population -----2) Occur <20 in 50% of cases -B) Signs/Symptoms -----1) Usually Unilateral (can be bilateral), frontal & of shorter duration -----2) Photophobia & phonophobia -----3) Nausea & vomiting -----4) Flushing, tearing, rhinorrhea -----5) +/- Aura -----------(pt saw dark spots 30 mins before headache onset) -C) MGMT -----a) TMT ---------1) Supportive -> (FIRST LINE -> CHILDREN < 12yrs) -------------Lying in dark room w/ cool cloth on forehead ---------2) NSAIDs or acetaminophen ---------3) Triptans Perform lumbar - Appropriate if suspected meningitis - Meningitis can also cause a headache & photophobia - However, key differences include: ----1) NOT ASSOCIATED w/ photophobia ---2) Missing key characteristics of meningitis including -------a) Nuchal rigidity -------b) Meningeal signs/Altered mental status -------c) Fever Perform EEG - only it pt had seizures

Poor dental hygiene -Pt presents w/ a cervical lymphadenitis → presentation includes: 1) An enlarged, markedly tender, warm, erythematous lymph node 2) ± Fever, but usually appear non-toxic Etiology 1) Children <5 y/o → Staph/strep 2) Children >5 y/o → Anaerobic ---Associated w/ poor dental hygiene Recent cat scratches by a kitten -Describes Bartonella infection -Also presets w/ lymphadenitis - However, key differences include: ---1) pt would also have a PAPULAR LESION at the site of injury ---2) Most cases are caused by a cat scratch -------[pt has no Hx of cat scratch]

An 8B, presents to the ED d/t neck swelling & fever. He first developed left-sided neck swelling 2-days ago. Over the last 24-hrs, the area has become sig larger, & the pt cries if the area is touched. He developed fever this morning but has otherwise been well. Temp is 102F. PE shows a red, tender, & fluctuant anterior cervical mass approximately 4-cm in diameter in the left anterior LN chain. Incision & drainage are performed, & the pt is admitted to the hospital for IV antibiotic therapy. Anaerobic bacteria are cultured from the aspirated purulent fluid. Which of the following RF's most likely contributed to this pts condition? (Poor dental hygiene OR Recent cat scratches by a kitten)

A 7-week-old B, presents w/ persistent hypoxia. He has been in the NICU since birth. He was born at 29 wks d/t placental abruption & weighed 2.5-lbs. Six hrs after delivery, the pt developed tachypnea, grunting, & cyanosis; a CXR revealed ground-glass opacities. Surfactant was administered & mechanical ventilation was initiated. He remained on mechanical ventilation for the first 3 weeks of life. Ever since ventilation was discontinued, the pt has remained hypoxic & continues to require O2 administered by cannula. Temp is 98F, pulse is 124/min, RR is 50/min. He has awake & alert. No rhinorrhea or pharyngeal erythema is found. S1 & S2 are normal; a 1/6 systolic murmur is present at the left lower sternal border. Scattered rhonchi & rales are present in both lungs. The abd is soft w/out organomegaly. There is not extremity edema. CXR appears hazy bilaterally & demonstrates slightly decreased lung volumes. What is the most likely Dx? (Bronchiectasis OR Bronchopulmonary dysplasia)

Bronchopulmonary dysplasia - Pt most likely presents w/ Bronchopulmonary dysplasia -A) RFs 1- Prematurity 2- Low birth wt (<2.5lbs) 3- NRDS -----NRDS at birth which required mechanical ventilation) 4- Mechanical ventilation & prolonged O2 exposure Bronchiectasis - Generally associated w/ CF - NOT ASSOCIATED w/ prematurity of RDS

A 15B, presents d/t worsening articulation & gait instability. His parents say that his speech has been increasingly difficult to understand over the last 2 months, but he has had no difficulty over the last month. He is falling more frequently & had to quit his basketball team. He has no chronic conditions or allergies. The pt takes no meds & does not use alcohol or illicit drugs. BP is 120/70, & pulse is 80/min. MSK exam shows scoliosis. Neuro exam shows dysarthria & a wide-based, unstable gait. There are absent deep tendon reflexes of the bilateral extremities. MRI of the brain & spinal cord shows marked atrophy of the medulla & dorsal communes of the spinal cord. The pt is at greatest risk of mortality from which of the following conditions? (Cardiac dysfunction OR Resp failure)

Cardiac dysfunction - Pt presents w/ Friedrichs ataxia -A) Signs/Symptoms ----1) Progressive ataxia in adolescence ----2) Dysarthria ----3) Limb weakness ----4) Loss of deep tendon reflexes ----5) Progressive gait & limb ataxia ----6) Loss of vibration & position sense ---------(d/t deterioration of the dorsal spinal column) ----7) Skeletal abnormalities ---------(Kyphoscoliosis, Pes cavus, high arched feet) -B) Complications -----1) Hypertrophic cardiomyopathy ----------(w/ increase risk of arrhythmia & HF usually leading to death by age 40) -C) MGMT -----a) Diagnostic ----------1) XR/MRI ---------------a) Cervical cord atrophy Respiratory failure - Associated w/ Guillen-Barre → presents w/ ascending paralysis after exposure to animals -----(would not cause medulla or dorsal column findings)

A 6B, presents d/t bleeding gums for the past 3 months. The pt brushes his teeth regularly & visits the dentist twice per year; his most recent appointment 6 months ago was normal. The pt has become easily agitated over the last 3-4 wks & is not as active as he previously was. Ht is below the 5th percentile & Wt is at the 25th percentile for age. Several small patches of hypopigmentation are noted on the trunk. The thumbs appear hypo plastic & thenar eminences are flattened. Labs show: - Leuko's-------------3K - Hb------------------6.5 - MCV----------------112 - Plts------------------40K Which of the following is the most likely cause of this pt's condition? (DNA repair defect OR Inherited Autosomal dominant anemia)

DNA repair defect - Pt presents w/ Fanconi anemia -A) Deficiency -----1) DNA repair defect & BM failure -B) Signs/Symptoms -----1) Short stature -----2) Hypo/hyper pigmented macules -----3) Hypoplastic thumbs -------(flattened thenar eminences, polydactyly) -----4) Genitourinary malformations -C) Labs ------1) Pancytopenia ------2) Thrombocytopenia ----------(bleeding of gums) -----3) Macrocytic anemia Inherited Autosomal dominant anemia - Describes the inheritance pattern of diamond-blackfan - Also presents w/ macrocytic anemia, genitourinary malformations, & Thumb abnormalities - However, key differences include: ---1) Thumb abnormalities are typically TRI-PHALANGEAL --------[pt has HYPO-PLASTIC thumbs] ---2) Anemia presents as PURE RED cell aplasia (only MCV and Hb would be affected ---------[pt presents w/ PANCYTOPENIA] ---3) DOES NOT typically present w/ thrombocytopenia --------[pt has increased bleeding d/t thrombocytopenia]

An 18-hour-old B, is evaluated in the ED for low muscle tone since birth. He was delivered at home to a 19F, at approximately 40 wks gestation who received no prenatal care. Since birth, the infant has breastfed 4 times, has a weak suck each time, & falls asleep a few mins after he begins nursing. Wt is 5.1-lbs. The eye have up-slanting palpebral fissures, & the mouth is open w/ a protruding tongue. The palate it narrow but intact. Cardiopulmonary exam is normal. The abd is soft w/ no hepatosplenomegaly. When the infant is held under the arms, his legs are extended & he almost slips through the examiners' hands. Which of the following is the most likely underlying Dx for this pt? (Beckwith-Wiedemann syndrome OR Down syndrome)

Downs - Children of young women can be affected → often present w/: 1- Low birth Wt (<5.5 lbs) 2- Dysmorphic features (up-slanting palpebral fissures, epicanthal folds, single palmar crease) 3- Macroglossia 4- Hypotonia *weak suck*, *slip through examiners hands* ----(key symptom to differentiate between Down syndrome & Beckwith-Widemann) Beckwith-Wiedemann - Characterized by: 1- Macrosomia 2- Hemihyperplasia 3- Omphalocele 4- *Macroglossia* ---(both present w/ macroglossia) ---NO HYPOTONIA

A 14F, presents to the ED d/t progressive weakness in the LE for 3 wks. Her parents first noticed clumsiness, which has progressed to difficulty walking over the last week. The pt had a fever, cough, & congestion about 1-month ago & has otherwise been well. She has a Hx of high plantar arches requiring orthotics & mild scoliosis. The pt takes no meds & has no allergies. Her parents & 2 older siblings are healthy. Vitals are normal. On exam, she was a wide-based gait & has difficulty maintaining balance w/out assistance. Strength is 2/5 in the LE & 5/5 in the UE. There is decreased vibratory & position sense in the LE. MRI shows cervical spinal cord atrophy. When discussing the most likely Dx w/ the pt's parents, which of the following stmts is most accurate? (Genetic testing is recommended to confirm the Dx OR A predisposition to malignancy is typical of this progressive disease OR Anti-viral therapy will reduce symptoms OR This is a non-progressive condition that usually improves w/ supportive care)

Genetic testing is recommended to confirm the Dx - pt most likely presents w/ Friedrich's ataxia -A) Signs/Symptoms ----1) Progressive ataxia in adolescence ----2) Dysarthria ----3) Limb weakness ----4) Loss of deep tendon reflexes ----5) Progressive gait & limb ataxia ----6) Loss of vibration & position sense ---------(d/t deterioration of the dorsal spinal column) ----7) Skeletal abnormalities ---------(Kyphoscoliosis, Pes cavus, high arched feet) -B) Complications -----1) Hypertrophic cardiomyopathy ----------(w/ increase risk of arrhythmia & HF usually leading to death by age 40) -C) MGMT -----a) Diagnostic ----------1) XR ---------------a) Cervical cord atrophy A predisposition to malignancy is typical of this progressive Disease - Describes Ataxia telangiectasia - Also presents w/ ataxic gait - However, key differences include ---1) Ataxia usually presents at 18-24 months -------[pt is 14 years old] ---2) Telangiectasia presents a 3-6 years old -------[pt has not Hx of telangiectasia] Anti-virals will reduce the symptoms - Describes viral encephalitis - Which also presents w/ focal neuro deficits (such as motor or sensory deficits) - However, key differences are: ----1) Encephalitis presents w/ Altered mental status --------(Altered behavior and personality changes) ------------[pt does not have] ---2) Malaise, headache, vomiting, neck pain, fever ------------[pt has none of these symptoms] This is a non-progressive condition that usually improves w/ supportive care - Describes Guillain Barre

A 1-month-old B, presents for a routine visit. His prenatal & birth Hx are unremarkable. Vitals & pulse OX are normal. Exam shows a harsh, III/VI holosystolic murmur over the left lower sternal border. Palpation indicates no thrill over the precordial region. Brachial & femoral pulses are 2+ & equal. The skin is pink & well perfused. ECG is normal. Which of the following is the most appropriate course of action? (Echo OR Reassurance)

Echo - Pt presents w/ a *VSD* → *Echo should be performed to determine the location & size of the defect & to rule out other defects* Reassurance - Small VSD's spontaneously close in 75% of children but must first do Echo - No workup is indicated only if murmur sounds like an innocent "flow" murmur of childhood → typically *grade I/II midsystolic ejection murmur*

A 16B, presents for pain & limited ROM. The pt has had previous episodes of sudden swelling in his right knee & does not recall them being associated w/ injury. This current episode of knee pain began 6 months ago. Initially the pain was controlled w/ heating pads & acetaminophen, but it has gradually worsened. over the last 2-3 months. The pt now has difficulty walking. He is worried that he will be unable to go to his annual camping trip w/ his family. The pt had an episode of prolonged bleeding after a tooth extraction several years ago. He is sexually active, has 2 lifetime partners, & uses condoms intermittently. Temp is 98F. On exam, the pt is alert & in no distress. The right knee is swollen & boggy w/ a small effusion present on passive motion. Which of the following is the most likely cause of this pt's knee joint? (Autoimmune cartilage destruction OR Hemosiderin deposition & fibrosis)

Hemosiderin deposition & fibrosis - Pt has *hemophilia* → Recurrent hemarthroses can result in long-term complications such as *Hemophilic arthropathy* Autoimmune cartilage destruction - Would be seen in *RA*

A 6B, presents to the clinic d/t 3 wks of itchy, red eyes. The pt has intermittent, mild, clear eye drainage & rubs his eyes frequently. His parents have noticed some light crusting of both eyes when the boy wakes up in the morning. The pt has a Hx of mild intermittent asthma for which he uses an albuterol inhaler. He had a clavicle fracture last year but has had no other medical problems. He lives w/ his parents, little sis, 2 cats, & a hamster. His sis has had rhinorrhea, cough, pharyngitis, & subjective fever for 2 days. Vitals are normal. Exam shows diffuse conjunctival injection in both eyes & mild eyelid edema. The child has no photophobia. Visual acuity is 20/20 in both eyes. Which of the following is the etiology of this pt's condition? (Adenovirus infection OR IgE mediated hypersensitivity OR Obstruction of the nasolacrimal duct)

IgE mediated hypersensitivity - Pt presents w/ *allergic conjunctivitis* -A) Signs/Symptoms ---1) Always affects both eyes ---2) Duration --------(< 30mins) ---3) Clear Watery discharge ---4) Ocular pruritus ---5) Edema of the conjunctivae & eyelids ---6) Mild crusting Adenovirus - Also a common cause of conjunctivitis - However, key differences include: ---1) Pt would've had a viral prodromal followed by 1-2 wks of watery mucoid eye drainage & eye redness --------[pt has no recent URI Hx] ---2) DOES NOT PRESENT w/ ocular pruritus -------[pt has red ITCHY eyes] ---3) Rarely lasts >2 wks --------[pt has had symptoms for 3 wks] Obstruction of the nasolacrimal duct - (AKA dacryostenosis) → most common in infancy (pt is 6) - Also presents w/ crusting of the eyelashes & tearing - However, key differences include: ---1) DOES NOT present w/ ocular itching or eyelid edema --------[pt has both symptoms] --2) UNLIKELY to present in BOTH eyes

An 8B, presents for a non-itchy rash on his face for the past 3 days. He has no fever, chills, sore throat or other symptoms. The pt has a history of mild eczema on his arms to which his parents apply topical petroleum jelly. He takes no meds & his immunizations are up to date. Vitals are normal. Exam shows erythematous papules, pustules, & honey-colored crusts around his mouth, nose, & neck. A few pustules are also present on his right index finger. Which of the following is the most likely Dx? (Hand-foot-mouth syndrome OR Impetigo)

Impetigo - Presents w/: 1- non-itchy rash 2- Painful postules & honey crusted lesions 3- Pt's w/ Hx of underlying skin issues (eczema, abrasions, insect bites) have increased risk 4- *NO* systemic symptoms Hand-foot-mouth syndrome - Characterized by vesicles on the posterior oropharynx, hands, and/or feet - [no honey-colored crusting]

An 8-month-old B, presents for eval of pale mucous membranes, irritability, & listlessness. The stool exam is neg for blood, ova, & parasites. Serum labs show: - Hb-----------6 - MCHC------25% - MCHb------16.5 - MCV---------68 - RBC DW-----22% (N 11.5-14.5) - REticulo's----0.6% - Plts----------230K - Leuko's------5,5K - Iron----------40 - TIBC---------460 (N 300-350) - Transferrin sat--8.7% (N 15-50%) - Total bili------0.9 PBS shows marked anisocytosis, Microcytosis, & Hypochromia. Which of the following is the most likely cause of this pt's anemia? (Iron deficiency OR Sideroblastic anemia)

Iron deficiency - Both Iron deficiency anemia & sideroblastic anemia present w/ irritability & listlessness - We must look at labs to determine which this pt has: -1) Labs (iron deficiency) -----a) DECREASED serum iron -----b) DECREASED transferrin -----c) DECREASED ferritin -----d) INCREASED TIBC Sideroblastic anemia - 1) Labs (similar to iron overload) ---a) INCREASED serum iron (60-100) ---b) INCREASED Transferrin ---c) INCREASED ferritin ---d) DECREASED TIBC - 3) Etiology ----a) Genetic → X-linked defect in ALA synthase gene ----b) Acquired → Myelodysplastic syndrome ----c) Reversible → Alcohol, Vti. B12 deficiency, Copper deficiency, Isoniazid, lead/zinc toxicity

A 4B, presents d/t sore throat. He first became ill 2 das ago w/fever & rhinorrhea. This morning, he developed a severe sore throat & refused to eat his mid-morning snack. The pt has DM I & has been hospitalized twice in the last year for diabetic ketoacidosis. He has had no childhood vaccinations. His mother smokes & often smokes inside the house. The pt is at the 50th percentile for Ht & 99th percentile for Wt. Temp is 100F. While sitting on the exam table, the pt appears distressed & leans forward w/ his chin thrust forward. Tympanic membranes are clear. Oropharyngeal exam shows multiple dental caries. The tonsils are mildly erythematous & 1+ bilaterally. The submandibular space & neck are soft. Lung exam reveals no wheezes or crackles. Which of the following is the most likely RF for this pt's condition? (Lack of immunizations OR second hand smoke)

Lack of immunizations - Pt presents w/ *Epiglotitis* → cause by *Hib* -A) Signs/Symptoms 1- Rapid onset *high* fever 2- Sore throat 3- Dysphagia *hurt to swallow* 4- Dystonia *muffled voice* 4- Drooling 5- stridor 6- Distress *Tripod/sniffing position, stridor* -B) MGMT ----a) Diagnostic tests --------1) XR findings --------------Thumb print --------2) Laryngoscopy -> (Diagnostic) --------------Cherry-Red, edematous epiglottis ----b) TMT --------1) Secure airway --------1) Vanco + 3rd gen cephalosporin --------------(ceftriaxone/cefotaxime) --------2) Rifampin -> (Post-exposure chemoprophylaxis) --------------indicated in households with either: -------------------(1) a child <1 year who has not completed the primary Hib vaccine series; -------------------(2) a child <4 years who has received an insufficient number of Hib vaccinations; or --------------------(3) an immunocompromised child <18 years Second hand smoke -A) RF for 1- Middle ear disease 3- Dental caries 3- Lower RTI (pneumonia) ---------[NOT A RF for epiglottis]

A 12B, presents d/t dark urine for the last 2 days. The pt has had no abd pain, dysuria, frequency, or urgency. Three wks ago, he had a fever & sore throat, which resolved after a week. The pt has no chronic conditions & takes no daily meds. FHx is sig for a paternal uncle w/ sensorineural hearing loss & a cousin who recently underwent renal transplant. Temp is 99F, BP is 150/90, Pulse is 84/min. PE shows an alert child w/ periorbital edema. Cardiac exam reveals no murmur. The lungs are clear bilaterally. 1+ pitting edema is present on the LE. Urinalysis shows: - Bacteria-----------N/A - WBC's-------------1-2/hpf - RBC's-------------many/hpf Which of the following is the most likely pathological finding in this pt? (Abnormal type IV collagen OR Mesangial IC deposits)

Mesanfgial IC deposits - Pt presents w/ *PSGN* → characterized by 1- *Mesangial IC deposits* 2- ↓ C3 & C4 levels 3- Usually presents 3-4 weeks after URI 4- Present w/ BOTH nephritic & nephrotic symptoms Abnormal type IV collagen - This is *Alport's* → which presents w/: 1- Sensorineural hearing loss 2- Ocular defects -----(*Pt does not have either of these, only relatives) 3- In boys <10 years old ----(pt is 12) 4- Nephritis ----(pt has signs of BOTH nephritic & nephrotic syndrome)

What is the most common cause of acute otitis externa in Diabetic patients? (Pseudomonas OR Mucor)

Mucor - Cause of sinusitis in diabetic pts → would present w/ black eschar from necrotic mucosa & can often be seen on the nasal turbinates Pseudomonas - Most common cause in non-diabetic pts

A 1-day-old girl is in the newborn nursery undergoing routine eval. She was born at term to a 24F by uncomplicated vaginal delivery. The Apgar scores were 7 & 1 at 8 & 5. The prenatal course was complicated w/ asymptomatic bacteriuria that was treated w/ an antibiotic. The mother took multivitamins daily throughout the pregnancy. PE shows bilaterally enlarged mammary glands. The labia are slightly swollen & blood-tinged vaginal discharge is seen. What is the best next step in MGMT? (Imaging of the ovaries OR Observation & routine care)

Observation & routine care - Baby is experiencing a physiological response to transplacental maternal estrogen exposure → benign transient findings that including: 1- Mammary gland enlargement 2- Non-purulent vaginal discharge 3- Mild uterine withdrawal bleeding Imagining of the ovaries - would be useful in premature thelarche presenting after infancy

A 9F, presents for assessment of "bad stomach aches." The pt has missed 5 days of school in the past few wks d/t intermittent abd pain that is "achy all over" & worse in the morning. She has not had associated nausea, vomiting, diarrhea, of constipation. Her appetite is poor. The mom reports that her daughter used to be talkative & outgoing, but now she declines invitations to play at friends' houses & instead takes naps after school. The pt has been distracted in class as of late & less engaged in group activities. The pt's father lost his job 3 months ago & has been home more often since then. The mom says, "Things have been difficult financially. It doesn't help that her father has started drinking again." On interview, the pt is withdrawn & makes poor eye contact. She avoids answering most questions about school or family & says, "Everything will be better when no one has to take care of me anymore." The pt has no sig PMHx. Vitals & PE are normal. Which of the following is the most appropriate next step in MGMT? (arrange for an in-home social work assessment OR Obtain an emergency psychiatric assessment)

Obtain an emergency psychiatric assessment - Pt presents w/ signs of *depression* → often seen with somatic symptoms, such as: 1- Stomachaches 2- Headaches 3- Acute psychosocial stressors (dad lost job) - Must determine if pt needs hospitalization → which would be required if she presented w/: 1- suicidal ideation 2- Poor social support 3- Lack of out-pt followup Arrange for an in-home social work assessment - can be helpful → but must determine risk of suicide in pt

An 11-month-old F, presents d/t 3 days of fever. The pt has also had rhinorrhea & nasal congestion for the past week. She recently completed a course of oral antibiotics for an ear infection 3 wks ago. The pt has no chronic conditions & takes no meds. She attends day care & her diet consists of formula & finger foods. Both parents smoke & both older siblings required tympanostomy tubes as infants. Temp is 102.9F. The pt is irritable but easily consoled by her mom. Otoscopy shows a bilateral bulging & pink tympanic membranes w/ poor mobility on insufflation. Crusted rhinorrhea is present at the nares. The oropharynx appears normal w/out lesions or sores, & lungs are clear to auscultation. The remainder of the exam is unremarkable. Which of the following is the most appropriate next step in MGMT? (Oral antibiotics OR Tympanostomy tube placement)

Oral antibiotics - Pt presents w/ AOM -A) MGMT -----a) TMT for AOM is: ---------1) Antibiotics -----------a) Amox -> First line -----------b) Amox-clavulanic acid (second line) ---------2) Tympanostomy tubes ------------a) Should only be considered in pts w/ Multiple episodes of AOM despite antibiotic tmt, criteria include: ------------1) ≥3 episodes in 6 mnths ------------2)≥4 episodes in 12 months ----------------------[pt has had 2 episodes ]

A 13F, presents for a routine health maintenance exam. She recently immigrated from SE Asia & has NK health concerns. Medical Hx includes a febrile illness a year ago that was followed by development of pain & swelling in the knees & wrists. At that time, the pt was treated w/ NSAID's & her symptoms resolved. Her last PE, 2 years ago, was prior to her immigration & was normal. She plays soccer & swims every weekend. BP is 110/70, pulses is 70/min & regular. Cardiac exam reveals a diminished S1 & a grade 2/6, high pitched, holosystolic murmur that is loudest at the apex & radiates to the left axilla. The lungs are clear to auscultation. The abd is soft, non-tender, & has no hepatosplenomegaly. Echo demonstrates mild mitral regurg. Which of the following is the most appropriate tmt for this pt? (penicillin OR No therapy)

Penicillin - Pt has Hx of rheumatic fever - She is at risk for developing rheumatic heart disease → so she should receive prophylaxis against rheumatic heart disease -A) MGMT -----1) IM benzathine penicillin G every 4 wks for 10 years/until pt turns 21 (whichever is longer) to prevent rheumatic heart disease

A 2B, presents to the ED after swallowing magnets 45 mins ago. The pt was playing w/ his older bro's toys when he ingested a few high-powered rare-earth magnets. He has no Chest or abd pain, nausea, or difficulty breathing. When the pt drank water, he had no pain w/ swallowing. He has no med conditions & has had no surgeries. Temp is 98.4F, Pulse is 88/min, & RR is 15/min. On exam, the pt is playful & not in distress. Cardiopulmonary exam is normal. The abd is soft & non-tender. The remainder of the PE is unremarkable. CXR reveals 2 small radio opaque objects in the distal esophagus; there is no mediastinal widening of sub diaphragmatic air. Which of the following is the best next step in MGMT? (Observe & repeat XR in 24 hours OR Perform endoscopic foreign body removal)

Perform endoscopic foreign body removal - ≥2 Magnets carry increase risk of necrosis, perforation, & fistulas → *require immediate endoscopic removal* Observe & repeat XR in 24 hrs - An asymptomatic pt w/ ingestion of a small, blunt object (coin) that is *not a magnet or battery* may be observed w/ repeat XR after 24 hrs to confirm progression of object - Observation CI in pt with ≥ 2 esophageal magnets d/t risk of necrosis & perforation

Differentiate in which conditions you would see - Persistence of RBC precursor nuclei - Precipitation of ribosomal ribonucleic acid On PBS?

Persistence of RBC precursor nuclei - Conditions where pt has no spleen of dysfunctional spleen (i.e, splenectomy) Precipitation of ribosomal ribonucleic acid - Lead poisoning (also basophilic stippling)

A 4B, presents by his mom d/t bruising. Over the last month, he has developed multiple bruises along his chest, back, & legs. The pt normally is very active & enjoys playing outside w/ his older siblings. However, he has been more tired than usual & takes longer naps. He has also had a poor appetite. This past week, his gums have been oozing blood during tooth brushing. The pt takes no daily meds. Last year, he had a tonsillectomy w/no complications. His paternal uncle has hemophilia. Vitals are normal. The pt appears pale & tired. Exam shows a few hematomas on the buccal mucosa. Scattered bruises are present on the shins, anterior chest, & back. Non-tender anterior cervical lymphadenopathy is palpated bilaterally. Abd exam shows hepatosplenomegaly. No joint swelling is present. Further eval of this pt would most likely reveal a deficiency in which of the following? (Factor VIII production OR Plts production)

Plts production - Pt presents w/ *ALL* - Most common childhood cancer → peaks at age *2-5 yrs* → presents w/: 1- Non-specific symptoms 2- Brusing 3- Petechiae 4- Mucosal bleeding 5- Bone pain 6- Lymphadenopathy & Hepatosplenomegaly 7- Pallor Factor VIII production - Typically presents w/ hemarthrosis (prolonged bleeding after minor trauma) - X-linked recessive and inherited from mom [does not matter that Uncle has] - pt did not have bleeding complications after tonsillectomy

You are called to the nursery to eval a newborn infant. The mom is a 24F, primigravida. Her pregnancy was complicated by preeclampsia. The infant was delivered at 39-wks via emergent CS d/t maternal HTN & non-reassuring fetal heart tones. On exam, the infant's Wt is 5-lbs 11-oz; placing him in the 5th percentile, Ht is 18inches, placing him in 5th percentile, & head circumference is 13 inches, placing him in the 10th percentile. The infant's head seems large for his body. There is paucity of sub-q fat. The remainder of the exam is normal. (Paucity is presence in small or insufficient quantities) This infant is at risk for having (or developing) which of the following? (Polycythemia OR Hyperthermia OR Hyperglycemia)

Polycythemia - Infants is small for gestational age -A) Criteria for "Small for gestational age" include: -----1) Birth Wt is <10th percentile ---------(pt is at 5th percentile) -B) Associated complications (small for gestational age) -----1) Hypoxia -----2) perinatal asphyxia -----3) Meconium aspiration -----4) Hypothermia -----5) Hypoglycemia -----6) Hypocalcemia -----7) Polycythemia -----------(in response to hypoxia) Hyperthermia - Infant has paucity of Sub-Q fat - This would lead to HYPO-thermia -----(Sub-Q fat helps keeps the baby warm) Hyperglycemia - HYPO-glycemia is associated w/ macrosomia - hyperglycemia is unlikely in a newborn infant

A 6-hour-old F, is evaluated in the newborn nursery d/t respiratory distress. She was born at 37 wks via spontaneous vaginal delivery after induction of labor for preeclampsia w/ severe features. Since birth, the baby has not been able to feed d/t tachypnea. Birth Wt is <3rd percentile for gestational age. Temp is 99F, BP is normal. Pulse OX is 95% on room air. Exam reveals a plethoric infant. The head is normocephalic w/ an opened & flat anterior fontanelle. Cardiac exam reveals sinus tachy & no murmurs. Pulm exam reveals tachypnea; breath sounds are clear throughout, & there are no retractions. The infant has normal female infantile genitalia. CXR shows clear lung fields & a normal cardiac silhouette. Labs show: - Hct---------------71% (N 55%- 65% - Plts---------------200K - Leuko's-----------20,5K - Glucose------------35 Which of the following is the most likely cause of this pt's symptoms? (Transient tachypnea of the newborn OR Polycythemia OR Dehydration)

Polycythemia - Pt has neonatal polycythemia - Defined as Hct level >65% -A) Etiology ------1) Excessive transfusion ------------Delayed umbilical cord clamping, ------------twin-to-twin transfusion syndrome ------2) Intrauterine hypoxia -------------poor placental perfusion d/t ------------------a) GDM, ------------------b) Smoking, ------------------c) HTN ------------------------(Mom has preeclampsia) --------3) Genetic --------------Trisomy 21 Transient tachypnea of the newborn - Also presents w/ difficulty breathing in premature children - However, key differences include: ---1) CXR findings --------a) Not consistent w/ this TTON -------------1) Increased pulm markings or *fluid in fissures* ---------------------[pt has normal CXR] Dehydration - can cause increased Hct levels → however, in first two days of life, dehydration is rare b/c neonates are born w/ excess extracellular water

A newborn boy is evaluated shortly after vaginal delivery for respiratory distress. He was born at 37 wks to a primigravid mother who had no medical problems. Second-trimester prenatal US showed bilateral hydronephrosis & oligohydraminos. Temp is 98F, Pulse is 144/min, RR is 50/min. Pulse OX is 84% on room air. Exam shows decreased aeration of both lungs & intercostal retractions. The child has flattened facies, abd distention, & clubfeet. What is the most likely underlying Dx? (Wilms tumor OR Posterior urethral valves)

Posterior urethral valves - Pt presents w/ posterior urethral valves -> leading to oligohydraminos -> leading to Potter sequence -A) Pathophysio of Potters sequence is: -----1) Bilateral renal agenesis (Posterior urethral valves) -----2) Failure of fetal renal excretion -----3) Oligohydraminos -----4) Multiple anomalies (Potter phenotype) and early death ----------a) Abnormal facies & limb deformities ----------b) Pulmonary hypoplasia ---------------(decreased aeration of lungs & intercostal retractions) -B) Etiology -----1) Most common cause of urinary tract obstruction in newborn boys -C) Pathophysio -----1) Dilation of bladder, ureters, & kidneys -D) Prenatal US findings -----1) Bladder distention -----2) Bilateral hydroureters -----3) Bilateral hydronephrosis Wilm's tumor - Can present w/ urinary symptoms & is Also associated w/ other congenital conditions that can lead to multiple abnormalities - However, key differences are: ---1) Wilms tumor presents w/: --------a) Abdominal mass or swelling (most common) --------b) Abdominal pain --------c) Hypertension --------d) Hematuria --------e) Fever --------------[pt has none of these] ---2) None of the conditions associated w/ Wilm's tumor presents w/ Abnormal facies & club feet -------a) WAGR -> (Wilm's tumor, Aniridia, Genitourinary symptoms, Retardation (mental-Motor)) -------b) Beckwith-Widemann -> (Macroglossia, Macrosomia, Omphalocele, Hemihyperplasia (formerly called hemihypertrophy)) -------c) Denys Drash -> (Gonadal dysgenesis (male pseudohermaphroditism), Early-onset nephrotic syndrome leading to renal failure) ---------------[pt has none of these]

A 4B, presents to the ED w/ fever & neck pain. He has had rhinorrhea, nasal congestion, & a sore throat for the past week. Today, the pt has refused to eat or drink. He has no chronic conditions & immunizations are up to date. Temp is 103.5F, pulse is 120/min, RR is 24/min. On exam, the pt appears tired & uncomfortable. Palpation of the neck reveals bilateral anterior cervical lymphadenopathy. The pt refuses to move his neck; passive extension is painful. His voice is muffled. Mouth opening is limited by pain, & the tonsils appear erythematous & symmetrically enlarged w/ gray exudates. The uvula is midline. Chest auscultation reveals good bilateral air entry w/out stridor or rhonchi. Lateral neck radiograph shows widened prevertebral space but no other abnormalities. Which of the following is the most likely Dx? (Peritonsillar abscess OR Retropharyngeal abscess)

Retropharyngeal abscess -A) Etiology ------1) Most often preceded by URI but can also result from direct spread of local bacteria (pharyngitis or tonsillitis) ------2) Most common in ages 6-mnths to 6-yrs -B) Signs/Symptoms ------1) Fever ------2) Muffled voice ------3) erythematous tonsils w/ gray exudate ------4) Painful passive neck extension ------5) Bilateral, anterior cervical lymphadenopathy -B) MGMT ------a) Diagnostic tests ----------1) Lateral XR findings -----------------Widening of pre-vertebral space Peritonsillar abscess - Also causes fever, muffled voice, & throat pain - However, key differences include: ---1) Typically presents w/ a Tonsillar mass -> that would cause UVULA DEVIATION ----------------[pt uvula is midline] ---2) Lateral XR findings -------NORMAL vertebral findings ----------------[pt has WIDENING OF PRE-VERTEBRAL SPACE]

A previously healthy 13B, presents for eval of delayed puberty. He is worried b/c he is the shortest male in his class. He feels well otherwise. On exam, he is 55in (144cm), & weighs 40kg (88-lbs). His mother is 65in (5ft 4in) tall & his father is 72in (6ft) tall. His sexual maturity score is a 2. A radiograph of his left hand is consistent w/ a bone age of 10yrs. Which of the following is the best next step in MGMT? (Begin growth hormone therapy OR Schedule a follow-up visit in 6 months)

Schedule a follow-up visit - Pt presents w/ *constitutional growth delay* -A) The most common cause of short stature & prepubertal delay in adolescents -B) Pathophysio -----1) Affected individuals have a normal birth Wt & Ht -> but between ages 6-month to 3-years the Ht growth velocity slows and drops percentiles in the growth curve -----2) Around 3 years of age the child regains the normal growth velocity and follows the growth curve at the 5th & 10th percentile -----2) Puberty & the adolescent growth spurt are delayed, but eventually occur -C) Bone age implications -----1) Hand XR shows a bone age VERY CLOSE to the pts age (usually 2years difference but can be up to 3 years difference) Begin growth hormone therapy - Growth delay is NOT TREATED w/ growth hormone → GH is used to treat GH deficiency (brain tumor) -A) TMT for delayed growth: -------Monthly administration of testosterone, when testosterone levels are <50ng/dL

A 9F, presents to the ED d/t sudden onset of uncontrollable, continuous writhing of the arms & hands & grimacing of the face. A month ago, she had a sore throat & fever that resolved after a few days w/out tmt. The girl & her family recently returned to the U.S. after living in Honduras for a year. The pt takes no daily meds & her vaccinations are current. Vitals are normal. On exam, the pt has multiple dental caries, & the oropharynx is non-erythematous & w/out exudate or tonsillar enlargement. Cardiac exam reveals a pericardial friction rub. Labs show an ESR of 65mm/h. ECG shows a prolonged PR interval & diffuse ST elevations. Infection w/which of the following organisms is the most likely cause of this pt's symptoms? (T.cruzi OR Strep. pyogenes OR Coxsackie)

Strep pyogenes - Pt presents w/ acute RF -A) Signs/symptoms ---1) Usually presents after a URI ---2) Characterized by JONES criteria --J → Joint pain (migratory arthritis) --O → carditis ----------a) friction rub, ----------b) prolonged PR interval, ----------c) diffuse ST elevations --N → Sub-Q nodules --E → Erythema marginatum --S → Sydenham chorea -B) Labs ---1) ↑ESR T.cruzi - Also can one a cause of heart abnormalities - However, key differences are: ---1) Heart abnormality most often associated w/ is cardiomyopathy (myocarditis) ---2) DOES NOT present w/ choreiform MVMTs Coxsackie - Also can one a cause of heart abnormalities (pericarditis) - However, key differences are: ---1) NOT ASSOCIATED w/ choreiform MVMTS ---2) Pt would have a Hx of slapped-cheek rash -------[pt has no Hx of rash]

A 1B, presents to the clinic to establish care after his family's recent move to the area. The pt was born at full term & has been growing & developing normally. Each day, he eats meat & pasta & drinks 300mL of whole milk; however, he does not eat fruit & veg. The pt takes no daily meds or vitamins. Wt & Ht are at the 50th percentile. Conjunctivae & mucous membranes are pale. Cardiac exam reveals no murmurs. Pulm exam is normal. The abd is soft & no hepatosplenomegaly is noted. CBC shows: - Hb-------------10 - RBC's-----------6.1mil - MCV------------60 - RBC DW---------13% (N 11.5%-14.5%) - Reticulocytes-----4% (N0.5%-1.5%) Which of the following is the most likely cause of this pt's anemia? (Fe+ deficiency anemia OR Thalassemia minor)

Thalassemia minor *Labs* - Microcytic anemia - *Normal RBC DW* - Mentzer index <13 - ↑/- reticulocyte count (d/t compensation of BM) Fe+ iron deficiency anemia - Pt eats a variety of foods - Usually caused by excessive cows milk intake (>700mL(24oz)/day) *Labs* - RDW would be increased ↓ Reticulocyte count - Mentzer index >13

A 5-month-old B, presents for fever. He was doing well until this morning, when he felt warm to his mother. He has had a "runny nose" & intermittent coughing but otherwise been breastfeeding well. His 3-year-old bro had an upper RTI last week. The infant takes Vit. D, & his vaccinations are up to date. Temp is 102F, BP is 90/60, pulse is 120/min, RR is 32/min. Pulse OX is 96%. Physical Exam shows crusted rhinorrhea at the nares. The pt's CXR shows an enlarged structure that looks like a sail in the anterior mediastinum - behind the sternum & in front of the heart, aortic arch, & trachea. What is this structure? (Right upper lobe infiltrate OR Thymus)

Thymus - Shaped like a *sail* - Normally visible on CXR in children <3 Right upper lobe infiltrate - typical locations for aspiration pneumonia → pts w/ aspiration pneumonia are usually *supine* & have swallowing dysfunction typically during choking or gagging that precedes resp distress

A 2-day-old B, is evaluated in the newborn nursery for jaundice. He was born to a 24F, primigravid who is in good health. The mother consumed a well-balanced diet & took prenatal vitamins throughout the pregnancy. The infant is breastfeeding poorly, w/a weak latch & suck. On exam, the pt has jaundice, scleral icterus, a large tongue, & a hoarse cry. His tone is normal but his activity is decreased. Labs show: - Total bili-----------15.3 - Direct bili----------0.7 - TSH----------------110 (N 0.5-5.0) - T4------------------0.6 (N 5-12) Which of the following is the most likely cause of this infant's condition? (Defective synthesis of thyroxine OR Thyroid dysgenesis)

Thyroid dysgenesis - Newborn presents w/ congenital hypothyroidism -A) Etiology -------1) Thyroid dysgenesis -------------a) The most common cause of hypothyroid in a newborn is -------------b) Can present as thyroid Aplasia, hypoplasia, ectopic gland -B) Associated Labs -------1) ↑ TSH -------2) ↓ T4 Defective synthesis of thyroxine - This can cause hypothyroidism - However, the KEY ETIOLOGY remains thyroid dysgenesis

A 3-month-old F, presents by her dad d/t fever & fussiness. A fever developed in the patient 2 days ago, & she has been receiving scheduled acetaminophen. The fever initially improved after meds but returned several hrs later. Now, the pt does not want to feed & is not finishing her bottles but has had no nausea or vomiting. She has no rhinorrhea, cough, or congestion. The pt was born at term & had no complications. She has intermittent constipation for which she takes prune juice. Immunizations are up to date. Temp is 102.9F, & pulse is 160/min. Exam shows a tired-appearing girl. The fontanel is open & flat. Tympanic membranes are clear w/ normal bony landmarks. The lungs are clear to auscultation bilaterally. The abd is soft & bowel sounds are present, but the girl cries during palpation of the lower abd. Which of the following is the best next step in mgmt of this pt? (abd US OR Urinalysis & urine culture)

Urinalysis & urine culture - Pt presents w/ a UTI -A) Signs/Symptoms ------1) Fever ------2) Fussiness ------3) Decreased urine output ------4) Poor feeding ------5) Abd discomfort ----------[pt cries upon palpation of the lower abd] -B) Associated RFs ------1) Constipation -C) MGMT -----1) Diagnostic --------a) Urinalysis & culture Abd US - Can be used to suspected urinary stasis or posterior urethral valves - However, would only be used in cases of RECURRENT UTI's -----[pt has not history of previous UTI]

A 5F, presents w/ fever, nasal congestion, & rhinorrhea that began 10 days ago. She has also had a dry cough during the day that worsens at night. Her symptoms do not seem to be improving. On exam, the child has erythema & swelling of the nasal turbinates w/ purulent nasal drainage. Dripping is seen in the posterior pharynx. The left cheek has mild swelling. The remainder of the exam shows no abnormalities. Which of the following is the most common predisposing factor for this child's condition? (Adenoidal hypertrophy OR Viral URI)

Viral URI - The most common predisposing factor for acute bacterial sinusitis is a viral URI → Contaminating bacteria cannot be cleared by mucocilliary clearance d/t mucosal inflammation from viral infection Adenoidal hypertrophy - Less common predisposing factor for acute bacterial sinusitis - Pt would have a Hx of noisy breathing/snoring, especially at night

A 9-month-old infant presents to the ED w/ lethargy & tachypnea. He was healthy before developing fever & diarrhea four days ago. He has been taking some formula, but has had two to three episodes of diarrhea w/ each bottle. He has lost 3 lbs since his routine check-up two wks ago. He has had one wet diaper in the past 24 hrs. On exam, temp is 102.5F, Pulse is 200/min, RR is 42/min, BP is 70/45. He is lethargic w/ decreased tone & decreased deep tendon reflexes. His mucous membranes are dry. Cardiopulmonary exam reveals tachycardia & tachypnea. Abd exam is unremarkable. Cap refill is 4 seconds. Labs show: - Na+-----------165 - K+-------------4.5 - Cl-------------108 - Bicarb--------14 - BUN----------20 - Calcium -----10 - Glucose-----98 Which of the following fluids should be used as a bolus in the resuscitation of this infant? (0.9% saline OR 0.45% saline)

0.9% saline > Pt presents w/ hypernatremia >> which presents w/ mainly neurological symptoms including: 1- *Lethargy* 2- *Altered mental status* 3- Irritability 4- Seizures 5- *Muscle weakness* 6- *Decreased deep tendon reflexes* TMT > When treating a pt w/ hypernatremia, the sodium most be *slowly returned to normal* >> goal is to stabilize him using a *bolus* >> when giving IV fluid boluses *only isotonic solutions should be used* 0.45% > not used as bolus b/c would lower the sodium too rapidly >> which may cause *cerebral edema*

Naproxen -Pt has Juvenile Idiopathic Arthritis → which has two types: -1) Polyarticular ---Second most common type ---≥5 joints affected w/in 6 months of disease ---Bimodal distribution → adolescent females and toddlers -2) Oligoarticular ---Most common type ---Affects <5 joins ---Uveitis ---Ages 2-4, F>M -3) Systemic ---Arthritis in ≥1 joint for ≥6 weeks ---Quotidian fever ≥2 weeks ---Evanescent rash ---Hepatosplenomegaly ---Lymphadenopathy TMT -1) Naproxen -2) Methotrexate Ceftriaxone - would treat lyme disease or gonococcal arthritis

A 14F, presents for leg pain. She first noticed ashiness in the ankles 3-months ago after starting daily walking routine on a local trail to improve her health. The pain spread to her knees, elbows, & wrists over the past month. Prior to this, the pt had been sedentary & spent most of her time watching TV. In addition to the walking routine, she has transitioned to a plant-based diet. The pt feels tired most days despite sleeping 8-9 hrs/night. Her Wt has improved from the 95th percentile to the 90th percentile over the past 3-months & her ht is stable at the 75th percentile. Temp is 99.1F, pulse is 80/min, & RR are 16/min. Cardiopulmonary exam is normal> Bilateral ankles, knees, elbows, & wrists have mild swelling & tenderness to palpation. Which of the following meds is most appropriate for this pts condition? (Naproxen OR Ceftriaxone)

Chronic rhino sinusitis > Pt has *meconium ileus* >> *associated w/ cystic fibrosis* (all newborns w/ meconium ileus have CF)>> which presents w/ 1- Obstruction at the *ileum* (dilated loops of small bowel >> free air above liver means perforation) 2- *Inspissated meconium* w/ a narrow undeveloped colon Early onset AZ disease - This describes duodenal atresia -Associated w/ Downs & Hypothyroidism ---(pt has none of these signs) -typically presents w/ 1- Squirt sign (forceful expulsion of stool after rectal exam 2- Obstruction at the rectosigmoid junction w/ a transition zone btwn the aganglionic rectum & proximal dilated colon

A 52 HOURS-old-female, in the newborn nursery develops vomiting & abd distention. She was delivered at 40 wks to a 30F by CS for failure to progress. Apgar scores were 8 & 9 at 1 & 5 mins. The pt has been voiding appropriately but has not yet passed meconium. Today, she has spit up all feeds & now has billious emesis. Her abd is tense & distended. Rectal exam shows no stool in the rectal vault & normal tone. Feeds are held & a nastogastric tube is placed for decompression. Upright abd radiograph shows multiple dilated loops of small bowel w/ paucity of air in the large intestine & rectum. Left lateral decubitus view shows free air above the liver, & an emergency laparotomy is performed. Viscous meconium is irrigated & evacuated primarily from the ileum w/ some from the colon. The colon is found to be diffusively narrow. Which co-morbidity of her underlying condition will this pt most likely develop? (Chronic rhino sinusitis OR Early-onset AZ disease OR Hypothyroidism)

Karyotype -Pt presents w/ hypospadias -A) Description -----(urethral meatus is ventral surface of the base) -B) Signs/Symptoms ----1) Underdeveloped penis ----2) Small glans and/or severe penile curvature (Chordee) -C) Complications -----1) Can lead to disorders of sex development (Androgen receptor mutation) -D) MGMT -----a) Diagnostic --------1) Karyotype analysis --------2) Pelvic US - PT also presents w/ signs/symptoms that are NORMAL IN NEWBORNS, including: ---1) Cyanosis in fingers & toes ---2) Acrocyanosis --------(cyanosis around mouth) ---3) I/VI flow murmur (benign pulmonary) → may radiate to axilla ---4) Liver size of 2-cm is normal in neonates --------(liver edge >3.5-cm is indication for liver US) Echo -Would be used in cases of ABNORMAL MURMUR - However, Pt has a faint I/VI murmur → However, it is characteristic of a BENIGN pulmonary flow murmur of the newborn

A term newborn is evaluated in the nursery 1-hr after a spontaneous vaginal delivery to a 28F, primigravida, whose pregnancy was uneventful. The pts Apgar scores were 9 & 9 at 1 & 5 mins. Vitals are normal. PE shows a newborn sleeping comfortably w/ overlapping coronal sutures. There is a I/VI systolic murmur at the left upper sternal border that radiates to the axilla. The lungs are clear bilaterally. The abd is soft, & the liver is palpable 2-cm below the costal margin. Genitourinary exam shows curvature of an undeveloped penis. The urethral meatus is at the ventral surface of the base & flanked by the surrounding scrotal sac. Gonads are not palpable. Exam of the back shows a sacral dimple w/ intact skin. The pt has normal fingers & toes. There is cyanosis in the digits. There is symmetric mvmt of all 4 extremities, & Moro & suck reflexes are normal. Which of the following is the best next step in eval of this pt? (Echo OR Karyotype)

A 5F, presents w/ joint pain & rash. Last week, the pt developed pain in her knees. The pain resolved after a few days, but now her ankles & wrists are tender. She has also developed a non-pruritic, pink rash, on her back. The pt had previously been healthy except for a sore throat a few wks ago that resolved on its own. Temp is 101F, Pulse is 85/min, RR is 20/min. Cardiac exam is normal. The wrists & ankles are stiff & tender to manipulation. Multiple large, well-demarcated, erythematous, non-pruritic patches w/ slightly raised outline are present on the trunk & proximal limbs. Labs show: - Leuko's--------6,5K - Neutro's-------56% - Lympho's------33% - Hb-------------12.5 - Plts------------380K - CRP-----------32 (N<3) - ESR-----------62mm/h Which of the following is the most likely Dx? (Acute rheumatic fever OR Henoch-Schonlein purpura OR Lyme disease

Acute Rheumatic Fever -A) Etiology ---1) Peak incidence at ages 5-15 ---2) > 2x's F>M -B) Signs/symptoms (Presents w/ MAJOR & MINOR signs --MAJOR signs-- ----1) typically occurs 2-4 wks after an episode of GAS pharyngitis* ----2) Jones criteria (major) --------J* → joints (migratory arthritis) --------O* → Carditis --------N* → Nodules (sub-q) -------E* → Erythema marginatum --------S* → Sydenham chorea --Minor signs-- 1- Fever 2- ↑ESR & ↑CRP 3- ↑PR interval Henoch-Schonlein purpura - Also presents w/ arthritic symptoms & a rash - However, key differences are: ---1) Presents w/ Palpable purpura -------[Pts rash not described as palpable/purpuric] ---2) Thrombocytopenia ------[pt has not thrombocytopenia] ---3) Arthritis -> that typically involves the LE joints knee/ankle -------[pt has arthritis that migrated from knee-to-ankle-to wrist] Lyme disease - Also presents w/ arthritic symptoms & a rash - However, key differences are: ---1) Pt would have a history of tick bite then symptoms 14 days later ---2) Rash would consist of single or multiple bullseye lesions (erythema migrans) that slowly expand ---3) pt would have MONO-ARTICULAR arthritis of the knee --------[pt has MIGRATORY arthritis knee-to-ankle-to wrist] ---4) NOT associated w/ a preceding URI illness --------[pt has Hx of pharyngitis]

An 11-month-old F, presents to the ER d/t a rash. Yesterday, the pt developed a rash on her face that has now spread over her entire body. The parents say that she has been ill for the past 3 days w/ fever, cough, rhinorrhea, & "pink eye." The pt has no other med conditions & has received all age-appropriate vaccinations as of age 6 months. She takes no meds & has no allergies. Approximately 3 wks ago, the family returned from a trip to the Philippines for her grandfather's funeral. The pt lives w/ her parents & several pets, including a dog, turtle, & a new kitten. Temp is 103.3F, BP is 80/50, Pulse 120/min, RR are 30/min. The pt appears tired & irritable w/ dry mucous membranes. The conjunctivae are injected w/out discharge. The lungs are clear to auscultation. Exam shows a blanching, erythematous maculopapular rash covering the face & entire body except for the palms and soles. Which of the following infection-control measures is most effective in preventing the spread of this pt's infection to others in the ED? (Airborne precautions OR Antibiotic prophylaxis OR Antiviral prophylaxis)

Airborne precautions > Pt presents w/ *measles* >> which presents w/ Maculopapular rash w/ cephalocaudal spread after a prodrome of cough, coryza, & conjunctivitis *Spares the palms & soles* >> spreads via *respiratory droplets* Antibiotics > Would be appropriate to prevent nesseria or bordatella pertussis Antivirals > Post-exposure prophylaxis only in VZV, Influenza, HIV

A 9B, presents to the clinic for eval of dark brown urine fora day. He has had no fever, dysuria, hesitancy, or urinary frequency. For the past 3 days, the pt has had rhinorrhea, nasal congestion, & a sore throat, but these symptoms are improving. A maternal uncle died from complications of a real transplant 5 years ago. Temp is 98.9F, BP is 132/94, Pulse is 90/min, RR is 14/min. The abdomen is soft, non-tender, & has no palpable masses. There is no suprapubic or CVA tenderness. Urinalysis shows hematuria. Serum creatinine is 1.4, & serum complement levels are normal. Renal biopsy reveals foam cells & longitudinal splitting of the GBM on EM. Which of the following is the most likely Dx? (Alport syndrome OR IgA nephropathy)

Alports syndrome - Typically presentation includes: ---X-linked -> *boy <10*, w/ *Gross hematuria* → after a recent URI ---Gross hematuria recurs periodically & may be accompanied by *HTN*, *Proteinuria*, & renal failure* ---Hearing Sensorineural hearing loss ---Eye Anterior lenticonus (conical lens protrusion) ---FHx of chronic kidney disease Findings - EM (Dx confirmation) → lamellated GBM *longitudinal splitting of the lamina densa* → *Basket weave* IgA nephropathy - can present w/ concomitant gross hematuria & URI/GI symptoms - Elevated creatinine & HTN may also be seen - Biopsy → *mesangial IgA deposits*

A 10F, presents w/ pain in multiple joints. Her pain began 3 months ago in her hands & has since progressed to involve her wrists, knees, & ankles. The pt previously danced competitively for the ballet team at school but has been unable to participate for the past month d/t pain. Her parents have been giving her acetaminophen w/out improvement. She has had no fever, rashes, Abd pain, or wt loss. The pt had a febrile seizure at age 4 but otherwise is healthy. Her maternal aunt has RA, & her grandmother had DM I. PE shows a comfortable young girl who grimaces d/t pain when asked to walk or use her hands. The hands, wrists, ankles, & knees are symmetrically swollen w/ mild erythema & warmth. Which of the following lab findings would most likely be seen in this pt? (Anemia OR Antibodies to DS DNA OR Antibodies to streptolysin O OR Hypogammaglobulinemia)

Anemia > Pt presents w/ *juvenile idiopathic arthritis* >> Which presents w/ 1- *Symmetric arthritis for at least 6 wks* 2- *Symmetric arthritis of >/=1 joint* (pt has 5) *In absence of other symptoms (fever, rash) Labs > reflect systemic inflammation >> co could show *Anemia* >> commonly seen d/t to chronic inflammation & iron deficiency 1- ESR 2- CRP 3- Hyperferritinemia 4- Hypergammaglobulinemia ---(other answer choice was HYPO-gammaglobulinmemia) 5- Thrombocytosis Antibodies to DS DNA > Specific to SLE - Pt has no other symptoms of SLE (butterfly/malar rash, pancytopenia) Antibodies to antistreptolysin O > Pt would have had Rheumatic fever >> but pt would've had pharyngitis & other criteria (Carditis, chorea, sub-q nodules, erythema marginatum)

A 6F, w/ Down's presents d/t behavioral changes. Over the past month she has stopped running, tripped frequently while walking, & accidentally knocked toys over more often than normal. The pt was toilet-trained at age 4, but recently began having frequent urinary accidents while playing & has started wearing a diaper again. PE shows an ataxic gait. Muscle tone is decreased throughout. Bilateral ankle deep tendon reflexes are hyperreflexic. When the soles of the feet are firmly stroked, the big toes move upward & the other toes fan out. Urinalysis is normal. Which of the following is the most likely cause of this pt's presentation? (Atlantoaxial instability OR Hypothyroidism OR tethered cord syndrome)

Atlantoaxial instability - Seen in >10% of pts w/ Down's → presents w/: 1- Weakness 2- Gait changes 3- Urinary/fecal incontinence 4- Vertebrobaisilar symptoms (dizziness, vertigo, imbalance, diplopia) 5- UMN findings *spasticity, hyperreflexia, Babinski* 6- Hypotonic Hypothyroidism > Associated w/ Down's → but presents w/: 1- Decreased growth velocity 2- Wt gain 3- Cold intolerance 4- Fatigue 5- Constipation 6- Declining school performance 7- Delayed reflexes *UMN do not occur* Tethered cord syndrome > Commonly associated w/ Spina bifida (not Down's) can cause: - Weakness - Decreased sensation - Urinary incontinence - hyporeflexia *UMN signs not seen*

A 19-day-old girl is brought to the ER d/t difficulty breathing. The infant was born to a term 24F, G2P2 following an uncomplicated pregnancy & had been breastfeeding well & voiding & stooling normally until 2 days ago. Since then, she has not been waking for feeds & has seemed much sleepier than usual. Today, she is fussy & difficult to console. The pt lives at home w/ her parents, 2-yr-old sis, & 3 cats. Temp is 95.2F, BP is 78/52, Pulse is 150, RR is 62. The infant has a full fontanelle, jaundice of the chest, mid-scleral icterus, & dry mucous membranes. Auscultation of the chest reveals mild tachypnea but clear lung fields. No murmur is present. Abd exam is normal. Neurologic exam shows a lethargic infant w/ decreased tone. Total direct bilirubin levels & a CBC w/ differential are pending. Which of the following is the next best step in MGMT? (Bacterial culture & antibiotics OR Temporary cessation of the breastfeeding OR CT scan of the head)

Bacterial cultures & antibiotics > Pt presents w/ neonatal sepsis (which can present w/ subtle & nonspecific findings) >> should be high on differential in infants: 1-*age <28 days* 2- *Decreased activity* (hypotonic) 3- *Poor feedings* May also present w/ 1- Fever or *hypothermia* 2- *Mild transient jaundice* 3- *Irritable & lethargic* Temporary cessation of breastfeeding > If pt had galactosemia would present w/: 1- Lethargy 2- Jaundice 3- Vomiting 4- Hepatomegaly *infant has MILD jaundice, no vomiting, no hepatomegaly* CT scan of head > Neonates do not experience herniation after lumbar puncture b/c their open fontanelles relieve intracranial pressure >> CT is not required before lumbar puncture in young infants

A 5B, present to the ER d/t right-sided hemiplegia. His parents say that he has been having increasing difficulty moving his right arm & leg over the past 3 hours. This morning, approximately 10 hrs prior, the child tripped on a toy while brushing his teeth & fell forward w/ the toothbrush in his mouth. He cried immediately, but his parents did not note any oral bleeding. The boy did say, "My throat hurts" throughout the day but was able to eat lunch. The child has a Hx of ADHD for which he takes methylphenidate. The boy's mom has a Hx of migraines, & his grandfather had a stroke at age 62. Neuro exam shows right hemiplegia, right hemianesthesia, & mild motor aphasia. Which of the following is the most likely cause of his condition? (Carotid artery dissection OR Congenital heart disease OR Homocystinuria)

Carotid artery dissection > Pt's symptoms of *hemiparesis & Aphasia* after an injury to the posterior pharynx are suspicious for an injury to the cervical internal carotid artery (located directly lateral & posterior to the tonsillar pillars >> pts may present with: 1- Neck pain 2- Thunderclap headache 3- Symptoms of ischemic stroke *Hemiparesis, facial droop, aphasia* Congenital heart disease > Certain cardiacs defects (ASD) can cause paradoxical emboli >> but Hx of trauma in an otherwise healthy child makes this Dx unlikely > Usually presents as sudden death in older adults Homocystinuria > Presents w/: 1- Lens dislocation 2- ID 3- Marfinoid features 4- Increased risk of arterial & venous thrombi *Dx is unlikely this child b/c symptoms started shortly after trauma to the posterior oropharynx

A 9 month old F, presents d/t to fever & poor feeding over the past day. During the last few days, the pt has become more fussy than usual & developed a fever yesterday. Today, she has not finished her formula bottles, & her stools have been slightly loose but non-bloody. Temp is 102.2F, Pulse is 142/min, RR 36/min. The fontanelle is open, soft, & flat. Tympanic membranes are clear bilaterally. Cardiac exam shows normal heart sounds; there are no murmurs, rubs, or gallops. The lungs are clear to auscultation bilaterally. The abd is soft & bowel sounds are present. The skin has no rashes or erythema. Urinalysis reveals positive nitrites & leukocyte esterase. Which of the following is the most appropriate med for this pt? (Cefixime OR Ciprofloxacin)

Cefixime > Pt presents w/ UTI >> Children are typically treated w/ *3rd gen cephalosporin* Cipro > Use in kids is limited d/t potential cartilage damage

An 18-month-old B, presents to the ED one hour after drinking liquid oven cleaner from an unlocked kitchen cabinet. His parents tried to give him water & milk, but he has difficulty swallowing. The boy has blood-tinged oral secretions. Vitals are stable. Exam shows an anxious child who is crying & drooling. His lips & chin are swollen & erythematous. He has no stridor & his breathing pattern appears normal. Lungs are clear to auscultation. His short is covered in oven cleaner. Which of the following is the best next step in mgmt? (Upper GI endoscopy OR Clothing removal)

Clothing removal > Steps in chemical burn of liquefaction necrosis are: 1- Secure airway, breathing, circulation 2- *Decontamination* (remove contaminated clothing & visible chemicals; irrigate exposed skin 3- CXR if respiratory symptoms 4- Endoscopy w/in 24 hrs Upper GI > follow steps outlined above

A 3B, is presents to ER by his stepdad d/t right knee swelling. The knee swelled immediately after the pt fell while playing outside. He is generally healthy & very active. His step dad reports that last week while climbing on the living room couch, he fell onto the floor & developed a large bruise to his left hip. Temp is 98.1F. Exam shows an uncomfortable-appearing boy w/ significant swelling of the right knee. The pt has pain w/ knee mvmt & limited ROM. An area of ecchymosis is seen on the left hip. There is also scattered ecchymosis to the anterior shins. The remainder of the PE is normal. CBC shows: - Hb------------13 - Plts-----------187K - Leukocytes---8.5K Radiograph of the right knee shows a large effusion but no fracture. Which of the following is the best next step in MGMT? (Coagulation studies OR Reassurance & observation)

Coagulation studies > Child has a Hx of *easy bruising* & acute onset of joint effusion following minor trauma (last week for bruise when climbing on couch) >> Raises concern for *hemophilia* >> may present w/: 1- *Hemarthrosis* (joint hemorrhage) Most common & typically presents following mild injury w/ pain & swelling in wt bearing joints (knees ankles) Reassurance & observation > Appropriate for minor contusions and/or sprains *hemarthrosis requires further eval & TMT to prevent recurrent bleeds*

What is Werdnig-Hoffman disease?

Congenital degeneration of anterior horns of spinal cord → LMN lesion >> causes floppy baby syndrome *other cause is botulism*

An 18-month-old B, presents to the ED d/t blood in his stool, which his parents noticed when changing his diaper. The infant has had previous bleeding & has been eating & drinking normally. He has a Hx of recurrent otitis media, frequent herpes labialis, & 2 episodes of pneumonia. Vitals are normal. On exam, The pt is well developed, well nourished, & has a fair complexion. He has eczema on his cheeks, trunk, & extremities. The remainder of his PE is unremarkable. Labs show a plts count of 24K, and leukocyte count of 9K. Peripheral smear confirms the low plts and the plts are small. Genetic testing confirms the Dx. Which of the following processes is most likely affected by this pt's mutation? (Cytoskeleton regulation OR DNA repair OR Maturation of T-cells)

Cytoskeleton regulation > Pt presents w/ Wiskott-Aldrich -A) Key deficiency ------1) Defective WAS protein -> leads to Impaired cytoskeleton regulation in leukocytes & plts -B) Signs/symptoms ------1) Eczema ------2) Microthrombocytopenia -----------(small plts, low plts count) ------3) Recurrent infections ------4) Fair complexion -C) Complications ------1) B-cell lymphoma (EBV+) ------2) Leukemia -D) MGMT -----a) Diagnostic --------1) Flow cytometry -> (Screening) --------2) Gene sequencing -> (DIAGNOSTIC) -----b) TMT --------1) IVIG ------------(hypogammaglobulinemia) --------2) Prophylactic antibiotics ------------a) TMP-SMX -> P.jirovecii ------------b) Acyclovir -> recurrent HSV infections --------3) Elective splenectomy -> thrombocytopenia & reduce risk of bleeding --------4) Rituximab -> autoimmune cytopenias DNA repair - Describes Ataxia telangiectasia - Also presents w/ Ataxia & immunodeficiency - However, key differences include: ---1) Presentation of symptoms -------a) Ataxia -> ~ 18 months -----------(Similar to pt) -------b) Telangiectasia -> (ages 3-6) -------c) Recurrent pulmonary infections (ages 6-9) ------------[pt is 18 months & HAS RECURRENT SINOPULMONARY INFECTIONS] ***SIDE NOTE -> TMT for Ataxia ------a) IVIG ------2) Prophylactic antibiotics Maturation of T-cells - Describes SCIDs

A 4-month-old boy presents to the ICU for respiratory distress d/t pneumonia. The infant developed cough & tachypnea 2 days ago w/ progressive worsening of hypoxia & work of breathing. He has had 2 months of poor wt gain & diarrhea, which have not improved despite changing to a hydrolyzed formula. The infant lives w/ his foster parents, who have 2 other children & a dog. The biological mom did not receive prenatal care after the first trimester. The child's birth wt was at the 60th percentile & current wt is at the 3rd percentile. Temp is103.1F, pulse Ox is 74% on room air. PE shows extensive oral thrush & generalized lymphadenopathy. Auscultation of the hear & lungs reveals a soft systolic ejection murmur & faint bilateral crackles. The remainder of the exam is normal. Labs show: - Hct-------------38% - Plts-------------260K - Leuko's---------18K - Neutro's---------50% - Lympho's--------45% Endotracheal tube aspirate shows P.jirovecii. Which of the following abnormalities is most likely present in this pt? (Decreased CD4+ lymphocytes count OR Deficiency of adenosine deaminase)

Decreased CD4+ lymphocyte count - Infection w/ P.jirovecii suggest CD4+ lymphocyte deficiency - Most likely d/t underlying HIV infection Deficiency of adenosine deaminase - Deficiency in SCIDs - SCIDs also presents w/ multiple infections - However, pt with SCIDS are NOT AT INCREASED RISK for P.jiroveci infections

A 2-day-old Asian F, in the newborn nursery has jaundice. She was born at 40 wks to a 30F, by uncomplicated vaginal delivery. Membranes ruptured spontaneously 1 hr prior to delivery. Prenatal lab testing was normal. BGS screen was negative. Maternal blood type is A+. The neonate has been exclusively breastfeeding w/ good latch, voiding appropriately, & passing meconium. Wt & Length are at the 50th percentile. Vitals are normal. On exam, jaundice of the face & neck is present. The remainder of the exam is unremarkable. Labs results at 24 hrs show: - total bilirubin-------7mg/dL - Conjugated--------0.4mg/dL Which of the following is the most likely cause of this newborn's condition? (Decreased hepatic uridine diphosphoglucoronate glucoronosyltransferase activity OR Deficiency of galactose-1-uridyl transferase)

Decreased hepatic uridine diphosphogluconurate glucuronosyltransferase *UGT* activity > 3 major causes of physiological Jaundice -> unconjugated hyperbilirubinemia are 1- ↑ Production 2- ↓ Excretion 3- ↑ enterohepatic circulation/ recycling *Asians have ↓ UGT activity* Deficiency of Galactose-1-uridyl transferase (GALT) - This enzyme deficiency describe galactosemia -Pt would have other symptoms besides jaundice (cataracts); including cataracts & hepatomegaly

Which of the following lab abnormalities is most likely associated w/ HUS? (Decreased platelet count OR Increased PT OR Postive direct Coombs test)

Decreased platelet count -HUS lab findings include: 1- Microangiopathic hemolytic anemia w/ *schistocytes on PBS, ↑Bilirubin* 2- *Thrombocytopenia* 3- Acute kidney injury (↑BUN, ↑Creatinine) Positive direct Coombs test - Negative in HUS b/c RBC's are mechanically destroyed *no Antibodies* Increased PT - PT is *normal* in HUS

A 1-hr-old F, is evaluated in the newborn nursery for respiratory distress. At 36 wks, the mother had PPROM w/ clear fluid. During induction of labor, non-reassuring fetal heart abnormalities were noted and an emergency CS was performed. Temp is 99F, Pulse Ox is 95% on 1L O2 via nasal cannula. Pulm exam reveals tachypnea, nasal flaring, intercostal retractions, and clear breath sounds. Cardiac exam shows tachycardia w/ normal heart sounds; femoral pulses are 2+ bilaterally. Abd is soft w/ no hepatosplenomegaly. CBC is normal. CXR reveals increased lung volumes & fluid in the interlobular fissures. Which of the following is the most likely etiology of this pt's symptoms? (Delayed absorption & clearance of alveolar fluid OR Alveolar collapse & diffuse atelectasis)

Delayed absorption & clearance of alveolar fluid - Describes the pathophysiology seen in transient tachypnea of the newborn -A) Signs/Symptoms ------1) Time Frame -----------Delivery-to-2 hours post-delivery (resolves w/in 2 days of life) -----2) Seen in TERM deliveries -B) RF's -----1) CS -----2) GDM -----3) Maternal asthma -C) MGMT -----a) Diagnostic ----------1) CXR findings ----------------Increased lung volumes & fluid in the interlobular fissures ----------------Bilateral perihilar linear streaking Alveolar collapse & diffuse atelectasis > Describes Neonatal RDS → d/t surfactant deficiency - Also presents w/ respiratory distress at birth - However key differences include: -A) RF ------1) PRETERM DELIVERY ----------(Presents w/ cyanosis after a premature birth) -B) MGMT -----a) Diagnostic tests ---------1) CXR findings ----------------Bilateral ground glass, reticular opacities opacities; ----------------Diffuse granularity ----------------Perihilar linear opacities

A 4-month-old B, presents d/t progressive lethargy, poor feeding, fatigue, & increasing pallor for the past 4 wks. Antenatal & birth Hx are unremarkable. The pt's diet consists mainly of breast milk. Immunizations are current. PE shows a webbed neck, cleft palate, Mild hypertelorism, flat nasal bridge, & pale mucous membranes. The thumbs appear triphalangeal. Cardiac exam reveals tachy w/ a systolic ejection flow murmur. Labs show: - Hb------------------4 - MCV----------------104 - Reticulocytes---------0.4% - Plts-----------------300K - Leukocytes-----------7,5K - Blood type------------A- Maternal blood type is O+ Which of the following is the most likely Dx? (Diamond-blackfan anemia OR Rh incompatibility)

Diamond-Blackfan Anemia -A) Signs/Symptoms -----1) Craniofacial abnormalities -----------(mild hypertelorism, flat nasal bridge, cleft palate) ----2) Neck abnormalities ----------(webbed neck) -----2) Triphalangeal thumbs -----3) Cardiac abnormalities ---------Faint systolic murmur -----4) Ophthalmological abnormalities -----5) Genitourinary malformations -----6) Growth failure ----------(both in utero and postnatal) -B) Complications -----1) Increased risk of malignancy -C) Associated Labs -----1) Profound Macrocytic anemia AKA (Macrocytic pure red cell aplasia) ------------(pt has a Hb of 4 & MCV of 104) -----2) Reticulocytopenia -----3) Normal plts & WBC's Rh incompatibility - Pt would hemolytic disease of newborn - This can also present w/ lethargy, poor-feeding, & fatigue & pallor - However, key differences include: ----1) Pt would have had signs IN UTERO -> (hepatosplenomegaly, ascites, polyhydraminos) --------[pt Antenatal Hx is said to be UNREMARKABLE] ---2) Jaundice would be present AT BIRTH ---3) Hemolytic disease of the newborn DOES NOT present w/ craniofacial/neck/hand abnormalities

A 3B, presents d/t 3 days of fever & painful swelling in his right groin. PMHx includes 4 previous episodes of cutaneous abscess & a lung abscess d/t staph, all of which required drainage & prolonged antimicrobial therapy. The boy's maternal uncle died in childhood from recurrent infections. The pt's temp is 101.3F. PE shows an enlarged, tender, fluctuant LN in the right inguinal area. There are several areas of scarring over previous drainage sites. The remainder of the PE is normal. Labs/CBC shows: - Hct------------40% - Plts------------320K - Neutros-------50% - Bands---------10% - Lymphos------35% Gram stain of fluid aspirate from affected LN reveals numerous organism-filled, segmented neutrophils. Cultures grow aspergillus. Which of the following tests would most likely confirm this pts Dx? (B-cells concentrations OR Dihydrorhodamine test)

Dihydrorhodamine test - Pt presents CGD -A) Deficiency ------Mutation prevents phagocytic oxidative burst (H2O2) & impairs intracellular killing -B) Signs/Symptoms ------1) Recurrent cutaneous & pulmonary abscesses w/ organism-filled neutrophils ------------(catalase positive organisms -> Aspergillus, Staph) ------2) Lungs ------------(pneumonis, empyema) ------3) Lymphadenitis) -C) MGMT ------a) Diagnostic ----------1) Dihydrorhodamine test/Nitroblue tetrazolium test B-cell concentrations - Describes the deficiency in x-linked (Bruton) Agammaglobulinemia - Also presents w/ immunodeficiencies & would affect males in the same family -However, key differences include: ---1)Infection type --------Brutons pts present w/ Recurrent Sinopulmonary & GI infections (pneumonia, otitis media, sinusitis) -----------[pt has recurrent infections w/ABSCESSES] ---2) Common organisms include step & H.flu -------[pt has recurrent infections w/ CATALSE POSTIVE ORGANISMS (STPAH/ASPERGILLUS]

A 4-month B, presents for "noisy breathing". His parents first noticed a harsh sound w/ inspiration at age 2 wks but report that is has gotten louder in the past 4 wks, especially when he is lying on his back. The noise seems to improve when the pt is held upright or during "tummy time." The infant has occasional, small spit-ups after feeds but is growing well along the 60th percentile for wt. He was born full term w/out complications during pregnancy on L&D. On exam, the infant is noted to have inspiratory stridor while supine that improves when he is prone. The remainder of the exam is normal. Which of the following is diagnostic test can confirm the most likely Dx? (Direct laryngoscopy OR French catheter in nose)

Direct laryngoscopy > Pt presents w/ *Laryngomalacia* (collapse of supraglottic tissues in inspiration) -A) Signs/Symptoms -----1) Inspiratory stridor that worsens when supine; prone position improves symptoms* -----2) peak at 4-8 months of age; M > F -B) MGMT -----a) Dx test ---------1) Laryngoscopy* -----b) TMT --------1) Most result in spontaneous resolution French catheter in nose - Test used in Choanal atresia - Also presents w/ noisy breathing in infancy - However, key differences include: ---1) Presents w/ CYANOSIS -> that -------Worsens w/ feeding -------Improves w/ crying -------[pt has STRIDOR that improves when prone & worsens when supine] ---2) More common in Females > males ---3) MGMT -------a) Dx test ------------1) French catheter -> (INITIAL TEST) ------------2) CT w/ intranasal contrast -> (DIAGNOSTIC) -------b) TMT ------------1) Placement of oral airway & lavage feedings ------------2) Surgical -> Definitive

A 14F, present d/t 8 days of fever, sore throat, & malaise. Her appetite has decreased but she is able to tolerate fluids. The pt has no cough, abd pain, nausea, or vomiting. Her mother gave her leftover amox for her symptoms, & the girl later developed a rash over her entire body. The pt has no sig PMHx & has taken Amox before w/ no rxn. Temp is 100.9F, BP is 110/70, Pulse is 84/min, RR is 12/min. Exam shows enlarged, erythematous tonsils bilaterally w/ white exudates & diffuse bilateral cervical lymphadenopathy. Heart sounds reveal a 1/6 systolic murmur along the left sternal border. The lungs are clear to auscultation bilaterally. The abd is soft & non-tender w/ mild hepatosplenomegaly. An erythematous, polymorphous, maculopapular rash is present over her entire body. Which of the following is the most likely explanation for the rash? (Acute RF OR EBV OR Strep scarlet fever)

EBV - Presents w/: 1- Fever 2- Tonsillar/pharyngitis w/ white exudates 3- Cervical lymphadenopathy 4- Hepatosplenomegaly 5- Development of a polymorphous/ maculopapular rash after taking Amox* even tho no previous Hx of previous rxn w/ Amox 6- Sig fatigue Strep scarlet fever - presents w/: 1- Fever 2- Pharyngitis 3- Maculopapular rash [[Rash occurs early in course of infection]] [[Hepatomegaly uncommon]] [[w/out tmt symptoms would've worsened by 8 days]] Acute RF - Pt would have to have a Hx of strep infection - Would be characterized by: 1- Arthritis 2- Carditis 3- Sub-q nodules 4- Erythema migrans 5- Sydenham chorea [[No maculopapular rash]] [[Carditis would not produce murmur]] [[No hepatosplenomegaly]]

An 8B, presents to the ED w/ 2 days of rhinorrhea, congestion, cough & wheezing that worsened acutely prior to arrival at the hospital. In the ED, he receives nebulized albuterol & ipratropium, IV corticosteroids, & IV mag sulfate. D/t lack of sig improvement in symptoms, the pt is admitted to the ICU & started on continuous nebulized albuterol & IV terbutaline. D/t persistent respiratory distress & poor oxygenation, bilevel positive airway pressure is started on hospital day 2. After 2 hrs of therapy, pulse OX shows 89% on 60% fraction of inspired O2, & the pt becomes agitated. There is no diminished air entry on chest auscultation & no wheezing is heard. ABG shows: - pH-----------------7.22 - PaO2---------------50 - PaCO2-------------62 What is the next step in MGMT? (Endotracheal intubation OR Nebulized racemic epinephrine)

Endotracheal intubation - Pt presents w/ signs of respiratory failure (Minimal wheezing, Difficulty speaking in full sentences) → despite escalation of medical TMT (BiPA) --This pt requires endotracheal intubation & mechanical ventilation Signs of worsening clinical status & impending respiratory failure are 1- Altered mental status 2- Persistent hypoxemia (despite high fractions of inspired O2) 3- Worsening hypercarbia 4- Resp acidosis Nebulized racemic epinephrine - typically used in severe croup

A 10B, presents d/t left knee pain & difficulty walking. His parents say that he developed knee pain several months ago, but it was mild & did not affect his mobility. Over the past week, the pt has had increasing pain that prevents him from playing soccer & basketball. He describes the pain as continuous, nonradiating, & sometimes worse at night. The pt has taken ibuprofen but it has not relieved the pain. He does not recall any preceding trauma. He has had occasional fever over the last month. Temp today is 99F. The pt has an antalgic gait & favors the right side. The left knee is swollen & erythematous. A tender, immobile mass is palpated above the left knee. Labs show: - Leuko's------------14,6K - ESR----------------40mm/hr X-ray of knee shows a central lytic lesion in the distal femur w/ cortical layering, a "mouth-eaten" appearance, & extension into the soft tissue. Which of the following is the most likely Dx? (Ewing sarcoma OR Fibrosarcoma OR Osteoid Osteoma)

Ewing sarcoma > 2nd most malignant in boys (after osteosarcoma) > Usually occurs in the *pelvis* or *diaphysis* of long bones > presents w/: 1- Subacute/ chronic *localized pain & swelling* 2- *Pain is often worse at night & w/ activity* & progresses rapidly 3- *Local erythema & warmth are common* 4- Systemic findings --*Fever* --*Leukocytosis* --*↑ ESR* (or other inflammatory markers) Imaging (X-ray) > Lamellated periosteal Rxn → central, poorly defined lytic lesion surrounded by concentric layers of new bone *onion skinning* > *Moth-eaten* appearance w/ *extension into soft tissue* > Periostal elevation Fibrosarcoma > Presents in males >30 as painful mass > X-ray → osteolytic lesions w/ well-defined or ragged, "mouth-eaten" margins Osteoid osteoma > Typically found in adolescent boys > Most commonly located in the proximal femur & causes pain at night that is responsive to NSAID's > X-ray → small round lucency

A 6B, presents to the ER w/ fever, purulent nasal discharge, & epistaxis. He has had daily fever & persistent nasal discharge for the past 10 days w/out improvement. The parents have had difficulty controlling intermittent epistaxis, which began 2 days ago. The pt has also had easy bruising for the past two months. His PMHx is sig for several recurrent pulm infections requiring hospitalization for parenteral antibiotic therapy, & previous sweat cl- testing was positive. The boy's father recently lost his job, & his parents report financial difficulties obtaining the pt's prescribed meds. Coag studies show: - PT---------25 sec (N 10-15) - aPTT-------35 sec (25-35) What is the pt's Dx and which of the following is most likely to be abnormal in this pt? (Factor VII or Fibrinogen)

Factor VII - Pt presents w/ acute sinusitis, recurrent resp infections, & a positive sweat test >> so pt has *CF* - Bleeding, easy bruising, epistaxis in CF is typically from *Vit. K deficiency* - Vit K deficiency will affect the Extrinsic path of coagulation (factors 3, 5, 7, 9, Protein C & S) -> which affect PT time Fibrinogen > not dependent on vit. K >> diseases include *severe liver disease & DIC*

A 1-month old B, presents for eval of blood-streaked stools. He was born at full term & has been breastfed exclusively. The infant usually has 3-5 yellow, seedy stools daily that have been bloody & loose over the past 3 days. He nurses every 2-3 hrs but spits up a small amount of breast milk w/ every feed. His mom recently completed a course on antibiotics for mastitis but otherwise has a regular diet & takes no meds. The pt's wt, length, and head circumference have been tracking along the 40th percentile. Vitals are normal. The infant appears well. PE shows diffuse eczema. A fecal occult blood test is positive. Which of the following is the most likely cause of this pt's condition? (Food protein induced allergic proctocolitis OR Meckel diverticulum OR Malrotation w/ midgut volvulus)

Food protein induced allergic proctocolitis > Pt likely has allergic rxn to milk or soy >> *non-IgE mediated immunologic response cause rectal & colonic inflammation >> typical presentation includes: -A) Signs/Symptoms -----1) *Well-appearing Child* -----2) *Painless, bloody stools* -----------(which may be loose during the first few months of life) -B) RF -> FH would include -----1) Allergies -----2) *Eczema* -----3) Asthma Meckel diverticulum > Also presents w/ painless hematochezia - However, key differences include ----1) Age of presentation -> Meckles typically present ~ 2 years of age ---2) DOES NOT typically present w/ loose stools Malrotation w/ midgut volvulus > Can cause bloody stools d/t intestinal ischemia - However, key differences include: ---1) Pt would appear ill d/t recurrent abd pain & bilious emesis ---2) Pt would have abd distention -----[pt has none of these symptoms]

A 12-month-old boy presents for a well-child visit. He was born at 39 weeks. Birth wt was 7 lbs 8 oz & length was 20 in. He was primarily breast-fed until last week, when he was transitioned to cow's milk. The pt can feed himself small pieces of table food w/ his thumb & first finger & drinks from a sippy cup. His only words are "mama", "dada", & "doggy". His parents are concerned about his growth b/c some children at his day care center seem taller. The pt weighs 26.5 lbs and is 30 in tall. On exam, he can pull himself up to stand & stand unassisted but requires holding onto objects for support while walking. He comes to his parents when called by name but does not speak during the exam. Which of the following is the most appropriate assessment in the pts development? [Growth, Motor, Language (Normal/Delayed)]

Growth (N) Motor (N) language (N) - saying one word other than "mama" & "dada" is typical language development at this age *Pt says doggy*

A 4-week-old F, presents for post-hospitalization follow-up. Her mother moved from Greece to the US for a diplomatic position a few months prior too giving birth. The pt was born at term, & the mother's prenatal course & delivery were uncomplicated. D/t hyperbilirubinemia, the pt was in the nursery for 3 wks of phototherapy. Bilirubin was normal at discharge. Since discharge, the girl's eyes & skin have remained yellow. Maternal blood type is AB+. Infant blood type is A+. The pt has been breastfeeding exclusively. Wt & Length were at the 50th percentile at 2 weeks of age & remain at the 50th percentile today. Labs show: - Hb------------9 - MCV----------78 - Reticulo's-----15% - Plts------------225K - Leuko's--------9K - total bilirubin--6.5 - Direct----------0.4 - MCHC---------40% (N 31-36) Which of the following is the most likely cause of this pt's condition? (Beta thalassemia OR G6PD deficiency OR Hereditary Spherocytosis)

Hereditary spherocytosis - Characteristic triad 1- Jaundice 2- Hemolytic anemia 3- Splenomegaly Labs 1- ↑ MCHC conc 2- Negative Coombs 3- Spherocytes on PBS 4- ↑ osmotic fragility (Acidified glycerol or eosin-5-maleimide) Beta thalassemia - Does not typically present until age *>6* (when fetal Hb decreases) - MCHC is usually decreased (↑ in pt) G6PD - X-linked → *Commonly seen in males* - MCHC would be normal (↑ in pt)

An 8B, presents for eval of bilateral leg & back pain. His pain started yesterday & has gradually worsened. He has had no relief w/ ibuprofen, acetaminophen, & warm packs. PMHx is sig for sickle cell, & he has had 5 prior hospitalizations for similar pain episodes in his back & legs. Hydroxyurea has been prescribed, but the Rx hasn't been filled d/t lack of insurance. Temp is 99F, BP is 110/70, Pulse is 132/min, RR are 20 min. PE shows an uncomfortable-appearing boy w/ conjunctival pallor. Palpation of the bilateral legs & back shows tenderness, although no erythema or swelling is noted. Labs show: - Hb---------------8.2 - Hct--------------26% - MCV-------------84 - Reticulo's--------8% - Plts--------------380K - Leuko's----------17K What finding is most likely to be present on this pt's PBS? (Bite cells OR Heinz bodies OR Helmet cells OR Howell-jolly bodies)

Howell-Jolly bodies > Sickle cell leads to *functional asplenia by age 5* (One dot in RBC's on PBS) Bite cells & Heinz bodies > Seen in G6PD *Heinz bodies* > (aggregates of denatured Hb; multiple dots in RBC's on PBS) *Bite cells* > Remnants of Heinz bodies when phagocytes extract the aggregates of denatured Hb Helmet cells (Schistocytes) > Fragments RBC's > *represent trauma to cells* >> seen in HUS, DIC, TTP

A 22-month-old G, presents d/t abd pain. Yesterday, she developed severe, intermittent abd pain & has been drawing up her legs when it occurs. The episodes last 15-20 mins & are occurring more frequently today. The pt cries & screams inconsolably during episodes but does not vomit or have any other symptoms. Her appetite is decreased & her last BM was 2 days ago. The pt recently began toilet training her & normally passes soft, formed stools daily in the toilet. Her last meal was oatmeal & apples at breakfast this morning. She attends daycare & has had 3 URI this winter as well as gastroenteritis 2 wks ago. The pt takes no meds, & her immunizations are up to date. Temp is 99F, BP is 100/60, Pulse is 116/min. Wt is at 35th percentile for age. Exam shows a tired-appearing girl w/ abd tenderness & a palpable cylindrical mass on the right side of the abd. Fecal occult blood test is negative. Which of the following is the most likely trigger for the pt's condition? (Hypertrophied Peter patches OR Inspissated stool OR Small-bowel diverticulum)

Hypertrophied Peyer's patches - pt presents w/ *intussusception* -A) Etiology -----1) the antecedent illness gastroenteritis is thought to cause hypertrophy of Peyer's patches -B) Signs symptoms -----1) INTERMITTENT pain Inspissated stool - Describes meconium ileus - Pt would have Hx of CF - Key differences ---1) DOES NOT present w/ intermittent pain Small-bowel diverticulum > Most common congenital anomaly that can serve as lead point - Common cause of meckles diverticulum which can lead to intussusception - However key differences include: ---1) Meckles presents in children ~ 2 yrs ---2) Meckles typically Presents w/ PAINLESS bleeding --------[pt has intermittent episodes of SEVERE PAIN]

A 3B, presents d/t fatigue & pallor. The pt was seen in another clinic a week ago for abd pain & diarrhea. A med was prescribed to help slow the diarrhea, which resolved, but the pt has been much less playful over the last day. Temp is 98F, BP is 110/70, Pulse is 120/min, RR 20/min. PE shows an uncomfortable-appearing child w/ scleral icterus. Cardiac exam shows mild tachy w/ no murmurs. A few ecchymoses are noted along the upper & lower extremities. There is pitting edema along bilateral LE to the knees. The reminder of exam is normal. Labs shows - Hb------------7.8 - MCV-----------84 - Plts-----------40K - Leukocytes-----13K Coag studies - PT------------12 sec - PTT-----------35 sec PBS shows schistocytes. What is this pts Dx and what is the most likely mechanism of this pt's condition? (Systemic activation of coag cascade OR Intravascular shearing of RBC's)

Intravascular shearing of RBC's - Pt most likely presents w/ HUS -> presentation includes: 1- Normocytic anemia 2- Thrombocytopenia 3- Edema *following a diarrheal illness* >>most commonly occurs 5-10 days after an acute, bloody diarrheal illness (Shiga) >> will present w/ microangiopathic hemolytic anemia Systemic activation of coag cascade > Would be seen in DIC >> which would have 1- Thrombocytopenia 2- Anemia 3- *Prolonged PT* 4- *Prolonged aPTT* (pt's is normal)

A 5B, presents to the ED for difficulty walking. The pt has had pain in his legs since the morning & "wouldn't even get out of bed." The father says that his son played 2 soccer games yesterday w/no pain or trauma. One wk ago, the pt has a low-grade fever & cough that have since resolved. ROS is positive for a non-pruritic rash on his back & legs for the past 2 days that his parents attribute to a new laundry detergent. He has had no nausea, emesis, wt loss, or night sweats. The pt has no sig PMHx & takes no meds. Temp is 98F, BP is 90/40, Pulse is 138/min, RR is 20/min. PE shows an uncomfortable appearing boy lying in bed who cries w/ any attempt to move his legs. A purplish, non-blanching rash is noted over his buttocks & posterior thighs. Abd exam is soft w/ active bowel sounds & mild diffuse tenderness to palpation w/out rebound or guarding. LE joints demonstrate no deformity, erythema, swelling, or warmth. The pt refuses to walk d/t pain in his legs. Labs show: - ph-------------7.0 - Protein--------trace - Blood---------trace - Glucose-------neg - Ketones-------neg - L.esterase-----neg - Nitrites--------neg The pt is admitted for further mgmt. Which of the following complications of the underlying disease is he most likely to develop? (Hemolytic anemia OR Intussusception OR Uveitis)

Intussusception - Pt presents w/ Henoch-Shonlein purpura (IgA mediated leukocytoclastic vasculitis) -A) Etiology -----1) symptoms often preceded by mild URTI -----2) Most common age 2-11 -B) Signs/Symptoms ---1) Palpable purpura -------a) purplish, non-blanching rash over extensor surfaces oh his arms, buttocks & posterior thighs ---2) ACUTE Polyarticular Arthralgia/Arthritis -------a) Can be severe & debilitating ---3) Abd pain --------a) Colicky, pain frequenlty w/ vomiting --------b) Pts w/ abd pain as a symptom have INCREASED RISK OF INTUSSUSCEPTION) ---4) Renal Failure --------a) similar to IgA nephropathy -C) Labs ------1) Urinalysis ----------a) RBC's/RBC casts ----------b) Protein -D) MGMT ------a) Diagnostic ---------1) Clinical -> (DIAGNOSTIC test) ---------2) Biopsy ---------------of Skin, kidney can support Dx ---------------Both will show IgA deposits ------b) TMT -----------1) Supportive ----------------(hydration & NSAID's) -----------2) Prednisone (systemic glucocorticoids) ----------------(for severe abd symptoms) -----------3) ACEIs -> HTN -----------4) Diuretics -> sig edema (nephritic syndrome) Uveitis - Presents as Complication of *Juvenile idiopathic arthritis - Also presents w/ Arthralgia/arthritis that is worse in the morning & rash that can present in proximal extremities - However, Key differences include: ---1) Usually preceded by HIGH FEVER SPIKES for 1-2x's/day ------[pt has no Hx of fevers] ---2) Arthralgia/arthritis in JIA presents GRADUALLY & is chronic (pt would have symptoms for >6 months ------[pt has ACUTE arthritis] --3) Rash in JIA presents as SALMON-COLORED MACULAR LESIONS -----[pt has PURPLISH NON-BLANCHING RASH which describes palpable purpura] Hemolytic anemia - May occur in pts w/ SLE - SLE can present w/ arthritis/arthralgias & a rash - However, key differences include: ---1) SLE presents mainly in females ---2) Rash in SLE is described a MALAR DISCOID rash & appears on face

A 10-month-old B, presents for a check-up. She is concerned that for the past several months he has cried & had screaming fits whenever another adult attempts to hold him. She plans to return to work next month & is worried about leaving him w/ a babysitter. The family recently moved to a new home so that she will be closer to her office. The pt crawls, pulls to a stand, and says "Ma." The mom tries to feed him range of food but he often refused to eat certain veg's. During the interview, the infant sits quietly on his mom's lap but cries when being weighed by the nurse. During the exam, the pt turns his head away from the physician, cries, & does not respond to his name. PE is normal, & the infant is at the 55th percentile for growth. Which of the following is the most appropriate response to the mother's concern? (It is likely that your son has stranger anxiety, which is normal at his age OR It is possible that your son is developing separation anxiety disorder)

It is likely that your son has stranger anxiety, which is normal at his age - Stranger Anxiety Typically starts at *6 months* - peaks at *8-9* months & *resolves by 2 years* - Children Typically cry when an unfamiliar person approaches even in presence of mom *Behavior is normal at this age* Separation anxiety - Separation anxiety → Part of normal development & generally resolves when the child develops object permanence *age 18-24 months* - Pt would have extreme anxiety when parents leave Separation anxiety disorder - Dx in older children who exhibit extreme & persistent anxiety w. separation & excessive worrying about losing major attachment figures

A 12-hour-old girl in the neonatal ICU has bilious emesis. She was born at 35 wks by vaginal delivery to a 22F who did not receive prenatal care & used cocaine during her pregnancy. The infant has urinated but has not had a BM. She was able to take 2 bottle feeds by mouth but has been vomiting green fluid since the third feed. Birth wt was 4 lbs 6 oz, which is small for gestational age. Temp is 98.4F, pulse is 160/min, RR is 40/min. The abdomen is distended. The rest of the exam is unremarkable. Abd XR shows a triple bubble sign & gassless colon. Which of the following is the most likely Dx? (Duodenal atresia OR Jejunal atresia OR Pyloric stenosis)

Jejunal atresia -A) Signs/Symptoms ---1) Bilious emesis ---2) Abd distention -B) Diagnostic -----a) XR --------1) Triple bubble sign -C) RF -----1) Use of vasoconstrictive meds -----2) *Cocaine* -----3) Tobacco → Duodenal atresia -A) Signs/Symptoms -----1) Abd distention -----2) Bilious emesis -B) Diagnostic -----1) XR -------a) Double bubble sign Pyloric stenosis -A) Etiology -----1) Most commonly presents at 3-6 wks of age* ---------(pt is 12 HOURS old) -B) Signs/Symptoms -----1) Non-bilious, PROJECTILE vomiting -C) PE findings ------1) Olive shaped mass palpable in the epigastric area

An 18-month-old boy presents to the ED for eval of a productive cough & fever. He has had 8 office & ED visits over the past year, all of which required antibiotics for Dx of sinusitis & pneumonia. He was delivered vaginally w/out complications. His older brother has had similar recurrent infections throughout his life. Temp is 102F, BP is 90/60, Pulse is 120/min, RR is 30/min. The pt's pulse Ox is 99% on room air. Exam of the oropharynx reveals mildly enlarged tonsils w/out erythema or exudate. Auscultation reveals rales & rhonchi in both lungs, & point of maximal impulse is displaced to the right side of the chest. What is the most likely Dx? (CF OR Kartagener syndrome)

Kartagener Syndorme > *Max impulse is displaced to right side of chest* describes *situs inversus* seen in Kartagener's >> presents w/ [[triad]]: 1- Recurrent sinopulmonary infections 2- Bronchiectasis 3- *+/- sinus inversus* CF > Distinguish from KS by the presence of pancreatic insufficiency & absence of dextrocardia

A 2B, presents to the ER by his parents for persistent, high fever over the last 6 days. The pt has been receiving ibuprofen & acetaminophen daily but has had minimal improvement. Two days ago, a rash developed along his trunk that has now spread to his arms and legs. Today, the pt has become increasingly irritable. He has no chronic medical conditions & has not received any vaccinations. Temp is 103F, BP is 110/60, Pulse is 120, RR are 24. Exam shows injected conjunctiva bilaterally w/ no discharge. The pt has fissured lips & an erythematous oropharynx. An erythematous, blanching rash covers the abdomen, arms, & legs. The hands are edematous, & the skin adjacent to the fingernails is peeling. There are 3 tender 2-cm, left-sided cervical LN. Labs show: - WBC-----------7/hpf - RBC------------0/hpf - Casts----------none - ESR------------58mm/h Which of the following is the most likely Dx? (Kawasaki OR Measles OR Rubella OR Scarlet fever)

Kawasaki > Presents in children <5 & characterized by: 1- Fever >/= 5 days *Plus >/= 4 of the following* 1- Conjunctivitis 2- Mucositis (erythematous, fissured lips; *strawberry tongue* 3- Rash (polymorphous) 4- Extremity changes (erythema, edema,) *desquamation of hands & feet* 5- *Cervical lymphadenopathy* > 1.5 cm node Labs 1- Evidence on inflammation *Elevated ESR* (leukocytosis, thrombocytosis, sterile pyuria) Measles > presents w/: 1- Fever 2- Cough 3- Coryza 4- Conjunctivitis 5- Kopek spots *rash spreads cephalocaudally* Rubella > *Mild disease* Presents w/ 1- Low grade fever 2- Lymphadenopathy 3- Fine, pink, maculopapular rash that rapidly spreads cephalocaudally *6 days if high fever would not be expected* Scarlet fever > Characterized by: 1- Pharyngitis 2- Fever 3- Sandpaper-like rash (that desquamates) 4- Strawberry tongue 5- Cervical lymphadenopathy > Rash in Scarlet fever is *most prominent over skin folds (groin, axillae)* > *No conjunctival injection or extremity swelling*

A 6B, presents for a follow-up for a limp. His symptoms began 2 months ago as a dull ache in the left knee w/ an intermittent limp. The parent brought him to an urgent care center at that time & were reassured that the lab eval & XR's of the knee & hips were normal. The pain has not worsened, but the pt now has a persistent limp w/ no other joint or muscle pain. He has been well except for a brief URTI w/ low-grade fever about 2 wks ago. He has a Hx of recurrent ear infections & had tympanostomy tubes placed 2 years ago. The pt takes no daily meds, & immunizations are up to date. Temp is 98.8F, BP is 95/65, Pulse is 80/min, RR is 16/min. BMI is at 60th percentile. Exam shows a well-appearing & alert child. ROM, particularly internal rotation & abduction, of the left hip is markedly limited. The remainder of the exam is normal. Which of the following is the most likely Dx? (Hematogenous osteomyelitis OR Legg Calve Perthes Disease)

Legg Calves Perthes - Pt most likely has Legg-Calves-Perthes disease - A complication of Legg-Calves-Perthes disease is idiopathic avascular necrosis of the femur -A) Etiology ---1) Presents in boys 4-8 w/ peak at age 6 -B) Signs/symptoms ----1) Chronic LE pain ---------(Insidious hip pain, limp) ---2) Positive trendelenburg sign --------(Restricted hip abduction, internal rotation) --------(Gait disturbance, i.e., Antalgic gait) ---3) Proximal thigh atrophy -C) Diagnostic -------1) XR ------------In Later stages → Sclerosis of the femoral head w/ flattening & fragmentation -------2) MRI -------------Avascular/necrotic femoral head Hematogenous osteomyelitis - Also presents w/ bone pain - However, key differences are: ---1) Osteomyelitis typically effects the metaphases of long bones (humerus, femur) ----------[pt has pain in hip] ---2) Presents ACUTELY ---------[Pt has CHRONIC pain] ---3) Fever ---4) Point tenderness ---5) Localized edema ------[pt is afebrile, & has no point tenderness or localized edema]

An 8 month B, presents to the ER w/ fever & vomiting. He has had a fever for the past 3 days, despite receiving acetaminophen & ibuprofen. Today, the pt has been refusing solid foods but is still breastfeeding. He is up to date on immunizations. The infant attends day care twice a week but has stayed home w/ his mom since becoming sick. Temp is 104F, BP is 92/48, Pulse is 120/min, RR are 30/min. On exam, the pt appears uncomfortable. He cries when the neck is flexed. The anterior fontanelle is bulging. The pupils are equal, round, & reactive to light. Extraocular mvmts are normal. There are no focal neuro deficits. CBC & blood culture are pending. Which of the following is the most appropriate next step in MGMT? (IV antibiotics OR Lumbar puncture)

Lumbar puncture - The pt has meningitis -A) MGMT includes (diagnostic test FIRST [usually], then antibiotics) -----a) Diagnostic test ----------1) lumbar puncture -------------(pts that have no neuro deficits) -------------(Pt has stable vitals & no papilledema) -------------(pt normal extraocular mvmts) ------b) CT -----------a) pts w/ neuro deficits (i.e., decreased mental status, papilledema) -----b) TMT -----------1) Vanco + 3rd gen cephalosporin ---------------(cefotaxime/ceftriaxone) ----------------(pts 1-to-50 years old) -----------2) Dexamethasone -> + TMT 1 ---------------- (if unknown bacteria/S.pneumo) -----------------(improves mortality & reduces neuro complications (hearing loss)) ------------3) Ampicillin -> + TMT 1 -----------------(Pts > 50 to cover listeria) ------------4) TMP-SMX -> + TMT 1 ------------------(pts allergic to penicillin) ------------5) Ampicillin + cefotaxime ---------------Ampicillin + aminoglycoside ------------------(Neonates < 1) -------------6) Vanco + Ceftazidime/Cefepime/meropenem -------------------(pts w/ neurosurgery or penetrating head trauma) IV antibiotics - Indicates use of Ceftazidime/Cefepime/meropenem (all are IV only) - Would only be used before Lumbar puncture or CT if pts was critically ill (Hypotension, Status epilepticus)

A 6F, presents for eval of pubic hair development. The girl's mom reports first noticing axillary hair a few wks ago while dressing her for school. The pt has also had breast & pubic hair development but no vaginal bleeding, headaches, emesis, or visual disturbances. She has no medical conditions & takes no meds daily. The pt had 2 long bone fractures at ages 4 & 5. FHx is unremarkable. PE reveals 2 large hyper pigmented macules w/ irregular contours on the left side of there back & chest. Axillae have secondary hair; there is no freckling. Bilateral breasts have budding & enlargement of the areola. There is coarse, dark pubic hair along the labia & the pubic junction. Which of the following is the most likely Dx? (McCune-Albright OR Peutz-Jeghers OR Sturge-Weber)

McCune-Albright > Pt presents w/: 1- Precocious puberty 2- Irregular cafe-au-lait macules *confined to one side of body* 3- *Recurrent fractures* d/t polyostotic fibrous dysplasia Physio >> Defect in *GNAS gene* >> leads to constant activation of G-protein activation >> leading to overproduction of pituitary hormones >> can lead to: 1- Thyrotoxicosis 2- Acromegaly 3- Cushings Peutz-Jeghers > Characterized by GI tract hamartomastous polyposis >> skin lesions are small (1-5m), perioral, mucocutaneous pigmented macules *Pts do not undergo precocious puberty* Sturge-Weber > Capillary venous malformations >> Characterized by: 1- ID 2- Seizures 3- Visual impairment 4- Skin lesion *Port-wine stain over the territory of the bigeminal nerve* *NO precocious puberty*

What is the major AE of hydroxyurea?

Myelosuppression

A 15F, presents b/c her menses have not started. She feels embarrassed b/c her friends have been "developing" & she looks younger than her peers. The pt has no headaches, visual changes, abd pain, or behavior changes. Her mom & maternal grandma began menstruation at age 13. The pt is not sexually active & is not on contraception. Ht is <3rd percentile & Wt is at the 20th percentile. Vitals are normal. PE shows no breast development, normal external female genitalia, & Tanner stage 1 pubic hair development. Urine B-hCG is neg. Which of the following is the best next step in mgmt of this pt? (Pelvic US OR Serum TSH level)

Pelvic US - Amenorrhea can result from either functional or anatomical problems of the hypothalamus, pituitary gland, ovaries, uterus, or vagina → so first step is *evaluate the female reproductive tract w/ pelvic US* to exclude anatomic abnormalities Serum TSH - Would be correct it pt had signs of hypothyroidism [[She does not have signs]]

A 7-day-old G, w/ truncus arteriosus in the neonatal ICU has poor feeding & abd distention. The pt has been hospitalized since her birth at 38 wks via normal spontaneous delivery, awaiting adequate wt gain prior to repair of her congenital heart condition. She has been taking oral formula & has regained her birth wt. Temp is 95F, BP is 60/30, pulse is 180/min, RR are 65/min. Pulse ox is 82% on room air. PE shows a lethargic, cyanotic neonate w/ moderate abd distention & absent bowel sounds. Bright red blood is visible in her stool. A nasogastric tube is placed for decompression & bilious fluid is recovered. Abd X-ray shows pneumatosis intestinalis (which represents extravasation of bowel gas into he damaged bowel wall & air in the portal venous system. What is the most likely Dx? (Intussusception OR Malrotation w/ midgut volvulus OR Necrotizing enterocolitis)

Necrotizing enterocolitis - Pt presents w/ pneumatosis intestinalis - This is a complication of necrotising enterocolitis -A) Signs/Symptoms (of NEC) ------1) Vital signs (Temp) instability ------2) Abd distention ------3) *Bloody stools* ------4) Lethargy ------5) Bilious emesis -B) RF ------1) Prematurity ------2) Very low birth wt (<3.3lbs) ------3) Enteral feeding ------------(formula > breast) ------4) Reduced mesenteric perfusion from either: ------------a) Congenital heart disease ------------------(pt has truncus arteriosis) ------------b) Hypotension* -C) MGMT ------a) Diagnostic ----------1) XR Intussusception > Classically presents at *age 6-36 months* ------(pt is 7 days old) > XR findings ----Target sign ---(pt has pneumatosis intestinalis) --Presents w/ relapsing/remitting pain as main symptom -----(pt does not have this symptom) Malrotation w/ midgut volvulus > typically presents w bilious vomiting & abd pain in <1 yrs - However, key differences include: ----1) XR findings ---------a) GAS-LESS ABDOMEN ------------[pts XR shows pneumatosis intestinalis]

An 18F, presents for a routine well-child visit. She reports skin lesions on her trunk that are not painful or pruritic but have increased in size & number over the past few years. She has no sig PMHx & takes no meds. Vitals are normal. PE reveals several large, uniformly pigmented, macular skin lesions. Eye exam reveals tan-colored lesions of the iris. The pt is at greatest risk of developing what? (Nerve sheath tumors OR Renal angiomyolipoma OR Renal Hemangioblastoma)

Nerve sheath tumors > Pt presents w/ NF 1 which presents w/: 1- Cafe-au-lait spots 2- Clustered freckles (axilla, inguinal) 3- Lisch nodules (raised, tan-colored hamartomas of the eyes) 4- Neurofibromas 5- Optic gliomas Renal angiomyolipoma > Associated w/ *Tuberous sclerosis* → which has multiple benign hamartomas of several organ systems > Skin findings include: 1- Facial angiofibromas 2- Hypopigmented macules (Ash leaf spots) 3- Shagreen patches Renal Hemangioblastoma > Associated w/ VHL disease → Causes: 1- Hemangioblastomas 2- Malignant tumors (RCC)

A 7B, presents for severe facial acne that has developed over the past few months. His mom tried treating it w/ topical benzoyl peroxide & a salicylic acid face wash w/ no improvement. She also noted a sig increase in her son's growth, & he is now much taller than his fraternal twin. The pt is otherwise healthy w/ no sig chronic med conditions & takes no meds. Ht is at 98th percentile, & Wt is at 85th percentile for age & sex. Neurologic exam is normal. Skin exam shows severe acne involving the face & shoulders. Exam reveals coarse pubic hair & axillary hair. Testicular volume is 2mL. Bone age is 2 std deviations higher than chronological age. Baseline LH levels are low & do not increase after stimulation w/ a GnRH agonist. Which of the following is the most likely Dx? (McCune Albright Syndrome OR Non-classic congenital adrenal hyperplasia)

Non-Classical CAH - Presents w/ all the same symptoms of CAH (precocious puberty): 1- secondary sexual characteristics 2- Advanced bone age 3- Low LH *Except* 1- Normal mineralocorticoid levels are maintained *no salt wasting* McCune Albright syndrome - Pt would have -1) Irregular cafe-au-lait macules -> that do not cross mid-line & look like "coast of Maine" -2) Fibrous dysplasia of bone ---Leading to weak bones, uneven growth, & deformity -3) Hyper-functioning endocrine abnormalities including: -----Precocious puberty -----Testicular abnormalities -----Hyperthyroidism -----Cushings -4) Mosaic disease d/t dysfunctional Gs G-protein

A 5B, presents d/t a 1-week Hx of generalized edema, fatigue, & abd pain. Otherwise, he has been well; his PMHx is unremarkable. The pt takes a daily multivitamin and no other meds. BP is 92/55, pulse is 90/min. Periorbital edema & 1+ pretibial edema are found on exam. The scrotum is mildly swollen but nontender. Abd exam Is unremarkable. Urinalysis shows: - pH--------------5 - Protein---------4+ - Blood----------neg - Casts-----------N/A - Crystals--------N/A Which of the following light microscopy findings would be expected is a kidney biopsy were performed? (Diffuse thickening of the basement membrane OR Normal findings)

Normal findings - Pt has *minimal change disease* → account for 80% of nephrotic syndrome in kids (ages ~2-3)→ which presents w/: 1- Edema 2- Fatigue 3- Abd pain 4- Proteinuria Pathophysio - T-cell mediated injury to podocytes Findings 1- *Biopsy* → Normal architecture* 2- *electron microscopy* → Diffuse effacement of foot process Diffuse thickening of the basement membrane - Thickened BM & subepithelial spikes are *pathognomonic for membranous glomerulonephritis* → one of the most common nephrotic syndromes in *adults* (rare in kids)

A 6B, presents d/t eyelid swelling. His mom noted a mosquito bite below his left lower eyelid 2 days ago. He developed mild swelling & redness of the eye lid yesterday & awoke today w/ his left eye swollen and shut. He had a fever this morning that was relieved w/ acetaminophen. The pt has also had decreased energy & took naps after breakfast & lunch yesterday, which was unusual. Temp is 101F. The left lower eyelid is erythematous, edematous, & tender to palpation. Visual acuity is 20/20 in both eyes. The pupils are equal, round, & reactive. There is limited left eye adduction; the right eye has a normal ROM. Labs show: - Hb-----------12.8 - Plts----------200K - Leukocytes--18K Which of the following is the most concerning finding in this pt? (Leukocytosis OR Ophthalmoplegia)

Ophthalmoplegia > Pt presents w/ orbital cellulitis -A) Pathophysio ------1) Caused by a contiguous extension of another infection ---------(i.e., sinusitis, dental abscess) -B) Signs/Symptoms -----1) Painful, tender eyelid -----2) Ophthalmoplegia ----------(Eye muscle weakness) -----3) Decreased vision -----4) Proptosis ----------(abnormal protrusion of the eye) -C) Complications ------1) Permanent visual impairment (optic nn) ------2) Intracranial ext (infection, trombosis) Leukocytosis - Is a common finding, & seen in ANY infection - Also part of presentation of pre-septal cellulitis - Which also present w/ eyelid erythema & swelling - However, key differences include ---1) NOT ASSOCAITED w/ ophthalmoplegia

A 13-day-old B, presents for a well-newborn visit. The infant was born at 38 wks via normal spontaneous vaginal delivery to a 27F, G1P1. He had an uncomplicated newborn nursery course w/ routine newborn care & was discharged w/ hs mother at age 2 days. The pt nurses for 15-20 mins on each breast every 2 hrs. He has approximately 7 wet diapers & 3 stool per day. Wt, Length, & head circumference are at the 75th percentile. Vitals are normal. On Exam, the anterior fontanelle is open, soft, and flat. Pupils are equal & reactive to light. Both eyes have mild eyelid swelling, conjunctival injection, & a scant amount of watery, slightly mucopurulent discharge. Parents report that this discharge began 2 days ago. The rest of the exam is normal. A sample of the eye discharge is sent for analysis. Which of the following is the most appropriate TMT? (Oral AZ OR Topical erythromycin OR Topical silver nitrate)

Oral AZ > pt presents w/ *neonatal conjunctivitis* >> secondary to Chlamydia >> which presents w/: 1- *Mild eyelid swelling* AND 2- *Watery or mucopurulent* Bilateral eye drainage 3- Chemosis (Conjunctival injection) >> Both in 2nd week of life *5-14 days* TMT > AZ >> *preferential TMT in infants < 1 month* Topical erythromycin > Routinely administered to all neonates as prophylaxis against gonococcal conjunctivitis but not for Chlamydial disease Topical silver nitrate > can be used to routine ophthalmic prophylaxis against gonococcal >> *banned in US* cause can cause chemical conjunctivitis in first 24 hrs of life

A 12F, presents d/t a worsening rash on her legs for 4 days. The initial rash was the size of a dime but has since grown w/ a raised edge & is increasingly itchy. She has applied moisturizing creams w/out improvement. The pt returned yesterday from a weekend gymnastics meet in Connecticut. She recalls that another girl on her team had a similar rash last week, but it looked more like a "bull's eye". The pt takes no meds & has no allergies. PE shows a rash on her left lower extremity that looks a scaly, erythematous, pruritic patch. Which of the following is the most appropriate therapy? (Oral doxy OR Topical Clotrimazole)

Oral Clotrimazole > Pt presents w/ *Ringworm* >> RF include: 1- Athletes who have skin-to-skin contact ---(pt is in gymnastics; so increase Likelihood to have person-to-person contact) ---(Team member had a similar rash) 2- Humid environment ---(gym locker rooms are a humid environment & breeding grounds for fungus) 3- Contact w/ infected animals (rodents) Rash presentation 1- Scaly, erythematous, pruritic patch w/ centrifugal spread 2- Subsequent central clearing w/ raised annular border >> may look like *Bull's eye* rash TMT > Topical anti fungal *First line* (Terbinafine, Clotrimazole) > Second line >> Griseofulvin Oral Doxy > Would be correct if pt has Lyme disease >> also described as bull's eye rash >> but 1- Transmission of Lyme disease requires > 48 hrs of tick attachment w/ rash appearing > 7 days later ---(pt has no Hx of camping or tick bite) ---(Pts Hx of gymnastics meet means a fungal infection is more likely) ---(Pts teammate had a similar rash; Lyme disease is not passed by person-to-person contact)

A 17B, presents d/t fever & facial pain. Two days ago, fever, myalgias, & fatigue began. This morning, pain & fullness developed in the right cheek. The pt has no sig PMHx & takes no meds. Immunizations are not up to date, but the family cannot remember which ones were missed. The pt is in high school & is on the school track team. He has a gf but is not sexually active. He does not drink, smoke, or use drugs. Temp is 100.9F. Exam reveals an alert, uncomfortable-appearing adolescent. Tenderness & fullness are present in the right cheek, which obscures the angle of the mandible. Bilateral tympanic membranes are clear. The oropharynx has no erythema, exudate, or tonsillar enlargement. No cervical LN are palpable. The pt is at greatest risk for developing which of the following complications? (Orchitis OR Myositis)

Orchitis > Pt presents w/ parotitis → and most likely has *mumps* → common complications include: 1-*Orchitis* 2- Pancreatitis 3- Aseptic meningitis (benign) 4- Sensorineural hearing loss Myositis > Particularly of the calves or thighs is associated w/ Influenza

A 16B, presents to the ED after falling off a bike & hitting his head on the ground. The fall was witnessed by friends who say that he lost consciousness for approximately 1 min but had no seizures. The boy vomited twice in the ambulance & has a mild headache. He has no other sig PMHx & takes no meds. BP is 121/67, & pulse is 78/min. Exam shows a small bruise & an abrasion on his forehead. GCS is 15. The rest of the exam is normal. Which of the following is the most appropriate next step in MGMT? Order a head CT w/ contrast OR Order a head CT w/out contrast)

Order a head CT w/out contrast - Contrast can confound the appearance of blood

A 1B, presents d/t poor Wt gain. Mom is concerned that the pt is smaller than children of the same age at his day care. Since birth, the mom has trialed various infant formulas d/t recurrent "greasy" & loose stools. The pt recently started drinking cow's milk; he drinks about 20 oz of whole milk & eats table food such as meats, fruits, & grains 3-4 times/day. He has also had recurrent nosebleeds over the past month. The pt's PMHx is sig for 3 prior hospitalizations for bronchiolitis, & his mom states that his cough persisted for a month after his last discharge. He & his mom live her BF, who smokes. PE shows a thin child w/ dried blood on his nasal turbinates. Bilateral wheezes are heard on chest auscultation. Scattered ecchymoses are noted on his extremities. What is the most likely cause of his failure to thrive? (Lactose intolerance OR Pancreatic insufficiency)

Pancreatic insufficiency -A) Etiology -----1) Associated w/ CF -> one of the most common causes of failure to thrive -B) Signs/Symptoms -----1) Recurrent sinopulmonary infections -----2) Pancreatic insufficiency -> leading to ----------a) Loose, greasy stools ----------b) Vit deficiency (Vit. K & Vit. D) ----------------(Vit. K leads to ecchymoses & increased bleeding epistaxis ---------------------(pt has both ecchymosis & epistaxis) Lactose intolerance - Can also present w/ Loose stools - However, key differences are: ---1) Loose stool in lactose intolerances are NOT TYPICALLY "greasy" ---2) Typically presents w/ abd cramping & bloating -------[pt does not have these symptoms] ---3) NOT ASSOCIATED w/ recurrent sinopulmonary infections and/or CF

A 12B, presents to the ED b/c "he cannot walk anymore." The pt has had a pins-and-needles sensation & weakness in both feet for the past few days. This morning, he could not stand or move his legs & has bilateral thigh pain. Two wks ago, the pt had a febrile diarrheal illness that resolved on its own. He has had no headache, nausea, vomiting, or recurrent diarrhea. He & his family are vegetarians, & they recently visited a petting zoo. The pt has no Sig PMHx & vaccines are up to date. On arrival, temp is 98.1F, BP is 150/90, Pulse is 92/min, RR is 16/min. Pulse OX is 99% on room air. PE shows a symmetric flaccid paralysis of the LE & absent ankle & patellar deep tendon reflexes. Sensation is intact to light touch on both legs. The UE are weak & hyporeflexic. There is no photophobia or nuchal rigidity. Which of the following structures is primarily impaired in this pts' condition? (Dorsal & lateral spinal columns OR Peripheral nerve fibers)

Peripheral nerve fibers - Pt presets with *GBS* → presents w/: 1- Direct contact w/ animals 2- Ascending weakness → often accompanied by paresthesias & neuropathic pain (begins in LE & ascends to involve the respiratory & bulbar muscles *respiratory failure* 3- Absent deep tendon reflexes 4- Autonomic symptoms (BP/HR instability, ileus) Dorsal & lateral spinal columns - Describes Vit. B12 deficiency

A 16F, presents d/t a rash. The pt developed a pruritic red lesion on her back 5 days ago that has slowly grown. She has since developed multiple new lessons on the chest & back. The pt has tried OTC topical antibiotics w/ no improvement. She also had a fever, headache, & malaise about a week ago, which resolved after 2 days. She has sig PMHx, takes no meds, & has no allergies. The pt is a wrestler for her high school team. She is sexually active & uses combo OCP. Temp is 99F. Cardiopulmonary exam is normal. Skin exam shows multiple 0.5-1.0 cm erythematous macules w/ minimal scaling on the chest & back oriented along lines of tension. There is a larger 3-cm erythematous patch on the upper back w/ scaling. The rash spares the palms & soles. Which of the following is the most likely Dx? (Nummular eczema OR Pityriasis rosea)

Pityriasis rosea > Common skin condition that typically presents from early adolescence to young adulthood >> Usually preceded by a *viral prodrome* >> Classically presents w/ a *herald patch* (erythematous, annular lesion on the trunk >> which may increase in size & develops scaling around edge) >> W/in on week clusters of small, erythematous, oval lesions appear on the trunk >> These scaly macules & papules are *distributed obliquely along lines of tension* (in Christmas tree pattern) most noticeable on the back MGMT > Reassurance or relief of pruritus Nummular Eczema > Chronic rash characterized by *dry erythematous & intensely pruritic patches on the extremities - pt has an *acute, mildly pruritic* rash on the *trunk* ---No Herald patch & back is NOT COMMON presentation

A 2 month old G, comes in for a well-child visit. Her parents are concerned about the back of her head being flat on the right. The infant usually sleeps on her back in a crib but she sometimes naps in her car seat or stroller. She is breastfeeding well & receives Vit. D supplementation. Birth history is sig for a full-term CS d/t to breech. PE shows flattening of the right occiput w/ anterior displacement of the right ear & forehead. The pt demonstrates a preference to tilt her head to the left whole rotating the chin to the right. When the head is turned to the right, there is a firm mass in the inferior portion of the left sternocleidomastoid. The mass does not transilluminate. The hips are stable w/ no clicks or clunks. Which of the following is the most likely underlying cause of this pt's presentation? (Congenital lymphatic malformation OR Postural deformity of the neck OR Premature closure of a cranial suture)

Postural deformity of the neck > Pt presents w/ *congenital torticolis* >> which is a *postural deformity/* >> presents w/: 1- *Neck mass* (or SCM thickening) 2- *Contralateral head tilt* 3- *Contralateral chin deviation* >> typically comes to attention at age *1-8 mnths w/ a head preference to one side which entails *flattening of the head on that side* as well as *anterior displacement of the ear & forehead* ipsilateral to the flattening Congenital lymphatic malformation > This is a cystic hygroma (seen in turners) - Located in the *posterior triangle of the neck* - would show *Fluctuant mass that transilluminates* Premature closure of a cranial suture > *AKA Craniosynostosis* >> results in skull deformity but *not anterior displacement of the ear & forehead* > Mass would not be seen

A 5F, presents for eval of pubic hair. Her mom noticed dark hair under the girl's arms & in her pubic area several days ago when helping her bathe. The pt has no headaches, visual changes, abd pain, or behavioral changes. She is obese & currently on a diet & exercise program. Several maternal relatives have DM II. The pt's ht has been tracking along the 50th percentile & wt at the 97th percentile. PE shows a cooperative girl w/ mild facial acne. No breast buds are palpable. She has dark, coarse hair across the pubis & under both axillae. Which of the following is the most likely Dx? (Idiopathic precocious puberty OR Premature adrenarche)

Premature adrenarche - caused by early activation of adrenal androgens → manifests w/: 1- oily skin 2- body odor 3- *Acne* 4- *Pubic & axillary hair* 5- *normal bone age* (bone age was not mentioned in the stem so we can assume it is normal) - Estrogen & testosterone levels remain normal Idiopathic precocious puberty -Precocious puberty would be breast bud development before age 8 in girls -A subset if precocious puberty is central precocious puberty → characterized by: 1- Advanced bone age 2- Elevated FSH & LH 3- True precocious development (Breast development)

A 2 day old G, develops seizures & a bulging anterior fontanelle. Temp is 97.6F, pulse is 180/min, Capillary refill time is >4 sec. Cardiac exam is sig for tachy. Neurologic exam shows rhythmic contractions of the upper extremities, tongue thrusting, & lip smacking. Labs shows: - glucose---------72 - Hb--------------9 A packed RBC transfusion is ordered. Phenobarbital is given IV, & the seizure acitivity resolves. Cranial US is performed & reveals bilateral hemorrhage involving the germinal matrix, lateral ventricles, & surrounding brain parenchyma. Which of the following conditions most likely led to the pts' presentation? (AV malformation OR Prenatal infection OR Prematurity)

Prematurity > Pt presents w/ intraventricular hemorrhage >> whose pathophysio includes: 1- *Ruptured germinal matrix vesels* 2- *Prematurity* 3- Very low birth wt AV malformation > Can cause Lt-to-Rt shunting of the brain >> but hemorrhage is not usually seen w/ brain AV malformations Prenatal infection > No prenatal infections would cause intraventricular hemorrhage

Which of the following features would be most consistent w/ a Dx of absence seizures? (Cessation by calling her name OR Presence of eye fluttering) Compare w/ inattentive staring spells

Presence of eye fluttering - Absence seizures are characterized by: 1- Occurrence during all activities 2- Length <20sec 3- Lack of response to vocal or tactile stimulation ----(CESSATION BY CALLING HER NAME -> The seizure should not cease when the pts name is called) 4- Presence of automatisms (eye fluttering, lip smacking) Inattentive staring spells 1- Occurrence primarily during "boring" activities 2- Variable length, often >1 min 3- Response to vocal or tactile stimulation 4- Lack of automatisms

A 1-wk old F, presents to the ED by ambulance d/t fever & irritability. She was born at term via precipitous vaginal delivery & had an uncomplicated nursery course. Prenatal labs included a positive GBS screen, for which the mom received intrapartum antibiotics. The infants mother & father are both age 16, & the father is not involved in the child's care. The infant lives w/ her mother and maternal grandparents in their home. The mom is present on the ED along with both of her parents. Temp is 101F, & pulse ox is 87% on room air. The infant is intermittent apneic & appears cyanotic. Anterior & posterior fontanelles are bulging. PE reveals tachypnea, intercostal retractions, & clear breathing sounds bilaterally. The infant's exam is concerning for meningitis & the child requires intubation & mechanical ventilation; however, the mom refuses the procedure. Which of the following is the best next step in MGMT? (Attempt to contact infant's dad for consent OR Proceed w/ mechanical ventilation w/out consent)

Proceed w/ mechanical ventilation w/out consent > Exception to informed consent is *emergency, life-threatening conditions* in which parent or legal guardian cannot or *does not* provide consent & delaying tmt would result in harm to the pt > Parents have the responsibility to make decisions in the *best interest of their child* therefore *cannot refuse life-saving tmt* > If not time sensitive >> then this committee and board would be called >> however *pt has unstable airway* so can proceed w/ tmt Attempt to contact infant's dad for consent > Consent not required in emergency situation

A 30-min-old boy is in the NICU w/ central cyanosis. He was born at 28 wks by vaginal delivery to a 16F. The pregnancy was complicated by premature labor, & the mother received glucocorticoids & magnesium sulfate shortly after the onset of labor. Membranes ruptured spontaneously 6 hrs prior to delivery; amniotic fluid was clear. The boy initially emerged vigorous w/ spontaneous respirations but rapidly developed resp distress. Brith wt was 2 lbs 4 oz. Temp was 96.8F, under radiant heat, BP is 65/35, Pulse is 140/min, RR is 70/min. Pulse Ox showed 75% on room air & improves to 90% w/ 35% FiO2 by continuous positive air pressure ventilation. PE shows grunting, intercostal & subcostal retractions, nasal flaring, & shallow respirations. Bilateral breath sounds are diminished. A CXR shows diffuse, fine, reticular granularity & perihilar linear opacities bilaterally. Which is the most likely Dx? (Respiratory distress syndrome OR Transient tachypnea of the newborn)

RDS -A) RF ------1) Prematurity (usually <30-32 weeks) -B) Signs/Symptoms ------1) Severe resp distress with cyanosis -C) MGMT ------a) Diagnostic tests ----------1) CXR findings ------------Diffuse, reticulogranular (ground-glass) appearance ------------diffuse granularity* , ------------low lung volumes ------------Perihilar linear opacities* -D) Pathophysio -------1) Diffuse alveolar collapse -> shown by: ------------Tachypnea ------------Grunting* ------------Nasal flaring* -------------Retractions* -------------Hypoxia & cyanosis Transient tachypnea of the newborn - Also Presents w/ Tachypnea & respiratory distress shortly after birth - However, key differences include -A) Signs/Symptoms -------1) Presents in TERM infant ----------------------[pt is PREMATURE] -------2) CXR findings ------------Prominent vascular markings ----------------(Bilateral perihilar linear streaking) ---------------------[pt has perihilar linear opacities] ------------Increased lung volumes ---------------------[pt has LOW lung volumes] ------------Flat diaphragm ------------Fluid in the inter-lobar fissures Prominent vascular markings

A mom brings in her 4yr-old daughter d/t concerns about her adjustment to preschool. The pt was taken away from her birth mom, who was abusing cocaine & alcohol, at the age of 2 & moved to an orphanage. The girl was adopted by her current family 6 months ago. Prenatal Hx is unknown. The adoptive mom reports that the pt doesn't interact w/ classmates & prefers to play by herself. At home, the girl hardly speaks / the family & stays in her room, where the mom has seen her hoarding toys & food under the bed. The pt is able to dress herself, copy a triangle, write letters of her name, & walk Dows the stairs w/out aid. Her mom says, "Sometimes she doesn't pay attention or listen to me. At other times she will start to cry for no reason. She doesn't like being picked up; nothing I do seems to comfort her." On exam, the pt is sitting quietly, playing w/ various toys in the doctor's office. She makes some eye contact. Speech is normal, but answers are brief w/ little facial expression. Vitals & PE are normal. She is in the 50th & 60th percentile for wt & ht. Which of the following is the most likely Dx? (Adjustment disorder OR Reactive attachment disorder)

Reactive attachement disorder > Pt has Hx of 1- Neglect 2- Social withdrawal 3- Lack of positive response to comfort 4- Decreased emotional responsiveness >> usually develops in young children in a setting of abuse, neglect, prolonged institutionalization, or inconsistent care Adjustment disorder > Dx of exclusion > this child's presentation is better explained w/ reactive attachment disorder

A 4-month-old B, presents for a routine exam. This is the pt's first visit as the mother recently moved to the area. Review of medical record shows that the only parental concerns at age 2 months were excessive gassiness & spitting up, both of which have resolved. The pt was born full term & has no chronic conditions. Vaccinations are up to date. His parents recently divorced, & now he lives w/ his mom & 9-year-old sis. The sis has ADHD & anxiety. The pt's Ht & Wt are at the 50th & 75th percentiles, respectively. On PE, he is smiling & reaching for the examiner's stethoscope. He holds his bottle for a few seconds & then drops it on the ground. The pt can sit w/ support & roll from front to back. Flat, non-blanching, non-tender patches over his scrotum & buttocks are seen on PE. Which of the following is the most appropriate next step in MGMT? (CBC & Coag studies OR Reassurance)

Reassurance - Pt presents w/ *congenital dermal melanocytosis* (Mongolia spots) → which present as *flat, blue-gray patches* that are usually found in infants over the *lower back & buttocks* - No tmt is necessary; fades spontaneously CBC & coag studies - Unexplained bruising or bleeding warrants a CBC & coag study → However, bruises are usually *tender, varied in color, & fade quickly*

A 1-day-old B, is undergoing eval in the nursery. He was born via vaginal delivery to a primigravid at 38 wks. The mom took a daily prenatal vit. & had routine prenatal care. Pregnancy was complicated by GDM, which was controlled by diet. L & D were unremarkable. Birth Wt was 8 lbs 2 0z. The boy Is awake & alert w/ normal tone & a strong sucking reflex. The lungs are clear to auscultation, & the heart has a normal rate & rhythm w/out murmurs. The abd is soft, nontender, & non distended. The penis is uncircumcised, & the urethral meatus is visualized at the tip of the glans. The scrotum is sig swollen w/ no discoloration. Both testis are palpated w/in the scrotal sac. A light shone behind the scrotum transilluminates the swelling. Which of the following is the most appropriate next step in MGMT? (Aspiration of fluid OR Reassurance & observation OR Scrotal US)

Reassurance and observation > Pt presents w/ a hydrocele - They are common in neonates & present w/ painless unilateral or bilateral scrotal swelling -A) MGMT ------1) TMT ---------a) Observation --------------(should spontaneously resolve by age 1) Aspiration of fluid > Not recommended d/t possible infection Scrotal US > Would be appropriate if pt presented w/ a scrotal mass that does not transilluminate

A 6-month F, presents for a well visit. The pt can sit w/out support & recently started babbling. Her diet consists of breast milk & pureed fruits & veggies. She was born at 35 wks d/t preterm labor, & her neonatal course was notable for a 1-wk hospital stay d/t difficulty feeding. She was hospitalized for bronchiolitis at age 3 months for 4 days. The pt takes no meds & has NKA. Immunizations are up to date. Ht & Wt track along the 55th & 40th percentiles. She is alert & interactive, smiling at the examiner & her mom. Ophthalmoscope exam reveals a white reflex in the left eye & a red reflex in the right eye. Extraocular mvmts are intact, & pupils are equal & reactive to light. Corneal light reflex is symmetric. The rest of the exam is normal. Which of the following is the most likely cause of the pt's exam findings? (Retinoblastoma OR Retinopathy of prematurity)

Retinoblastoma -A) Ophthalmoscope exam findings ------1) white reflex ----------Indicates retinoblastoma -B) Etiology ------1) Most commonly Dx'd at age <2 -C) Signs/Symptoms ------1) Strabismus ------2) Nystagmus ------3) Vision impairment ------4) Ocular inflammation -D) MGMT -------a) Diagnostic test ------------MRI of the brain & orbits Retinopathy of prematurity - Usually presents in pts < 30 weeks & <1.5 pounds ------[pt was born at 35 weeks & was not underweight] - NOT Associated w/ WHITE reflex

An 18-month-old F, presents the ED after a seizure in day care. PMHx is sig for 3 ear infections in the past 10 months & one previous seizure thought to have been febrile in origin. FMHx is sig for Alzheimers disease in the pt's paternal grandfather & retinoblastoma in a paternal cousin. The pt's mom says, "My daughter used to say "mama & papa" & babble away but has begun to talk less in the past few months." The pt has also become "needy," wanting to be fed by others instead of using a spoon herself. The pt is afebrile & Vitals are normal. On PE, the pt makes eye contact w/ the physician but speaks no words. She repeatedly rubs her hands together & has a clumsy, broad-based gait. Which of the following is the most likely Dx? (Landau-Kleffner Syndrome OR Rett syndrome OR Tuberous sclerosis

Rett syndrome -A) Etiology -----1) More commonly presents In girls -B) Mutations -----1) Mutations in the *X-linked MECP2 genre -C) Signs/Symptoms -----1) Regression of speech -----2) Loss of purposeful hand mvmts ----------(Inability to feed herself) -----3) Gait abnormalities -----4) Development of stereotypical hand mvmts -----------(at age 6-18 months) ------5) Deceleration of head growth (early sign) ------6) Other presentations : ------------a) Autistic features ------------b) Sleep disturbances ------------c) Periodic breathing abnormalities, ------------d) Seizures Tuberous sclerosis - Can also present in childhood w/Seizures, Cognitive deficits & Autistic features - However, key Differences are ---1) Key characteristics of TS are missing, including: --------a) Skin involvement --------------(Hypopigmented macules, angiofibromas, shagreen patches, forehead plaques) --2) DOES NOT typically present w/ ataxia, & regression Landau-Kleffner - Also presents w/ regression of language skills & severe epileptic attacks - However, key differences include: ---1) Symptoms manifest at 3-6 years -------[pt is 18 months]

A 5B, w/ fever & joint pain presents by his mom. The child was Dx'd w/ Strep pharyngitis 2 wks ago & treated w/ a 10 day course of amoxicillin. He completed the full course of antibiotics & did not miss any doses. Two days ago, he developed fever 102F, & seemed fatigued w. decreased oral intake. Today he complains of right knee pain & asked to be picked up rather than walk on his own. His mom says that he has had no trauma or injury to the joint. On exam, temp is 103F, BP is 94/52, pulse is 106/min, RR 16/min. The pt is holding his right knee in a flexed position. His knee is swollen & tender to palpation. He has significant pain w/ active ROM & w/ weight bearing. CBC shows: - Leukos--------16K - Hb------------12 - Plts------------260K Immune studies - CRP----------62 (N</=8) - ERS----------44 (N 0-15) Blood cultures are pending. XR of the right knee shows effusion & soft-tissue swelling. Which of the following is the most appropriate next step in MGMT? (Empiric IV antibiotics OR Right knee arthrocetnesis)

Right knee arthrocentesis > Pt presents w/ septic arthritis >> *Often preceded by URI* >> presents w/: 1- *Acute onset fever & joint pain* 2- *Fatigue or malaise* 3- *Refusal to bear wt d/t pain* 4- *Pain w/ active & passive ROM* Labs 1- *Elevated ESR & CRP* 2- Elevated WBC's 3- Synovial fluid WBC > 50K *Arthrocentesis is both diagnostic & therapeutic >> should be performed ASAP d/t risk of permanent joint destruction* TMT Birth to 3 months > Most likely staph, GBS, Gm- bacilli >> - Naficillin/ vanco PLUS Genta/ cefotaxime Older than 3 months > Staph, Group A strep, S.pneumo >> - Naficillin, Clinda, Cefazolin, Vanco Empiric antibiotics >Only after arthrocentesis

A 5-month-old B, presents d/t fussiness & fever for 2 days. PMHx is sig for previous episodes of otitis media, oral candidiasis, & gastroenteritis d/t to rotavirus. The pt is at the 3rd percentile for wt. His temp 102.8F, pulse is 120/min, RR is 28/min, & BP is 80/60. PE shows an erythematous, bulging right tympanic membrane w/ poor mobility on pneumatic otoscopy. The LN are not palpable, & the tonsils are not visualized. Labs show: - Plts--------------240K - Lympho's--------5% - IgG--------------210 - IgA--------------35 - IgM--------------14 -CD4+-------------80/mm3 - CD8+------------70/mm3 Which of the following is the most likely etiology of this patients condition? (CVID OR SCID)

SCID -A) Key deficiency ------1) B-cells dysfunction d/t to absent T-cells -B) Signs/Symptoms ---1) Recurrent, severe Viral, Fungal, or opportunistic (pneumocystis) infections ---2) Failure to thrive ---3) Chronic diarrhea CVID -A) Key deficiency ----1) Autosomal disorder affecting B-cell differentiation that results in LOW IMMUNOGLOBULIN levels (most or all types) -B) Signs & symptoms -----1) Pt would present w/ recurrent sinopulmonary or GI bacterial infections - However, key differences include: ---1) ALMOST ALWAYS presents in older pts (20-30) ---2) Pt would have > Usually presents in older pts (20-30) -------[pt has NORMAL IgG]

A 6-month-old B, comes in for eval of a progressive facial rash that started 3 wks ago near his eyebrows & spread to areas of the face & scalp. The rash does not appear to be painful or pruritic. The mom recently started using a mild, unscented shampoo that slightly improved the scalp rash. PE shows plaque located at the scalp that appears yellow and oily. In addition, erythematous, scaly plaques are on the eyebrows, nasolabial folds, & behind the ears. Which of the following is the most likely Dx? (Atopic dermatitis OR Contact dermatitis OR Seborrheic dermatitis OR Psoriasis)

Seborrheic dermatitis > Peaks in the *1st year of life* and agin in adulthood >> associated w/ *Malassezia* >> *primarily affects ares w/ numerous sebaceous glands* >> Characteristics include: 1- Peak in infancy & adulthood 2- *Erythematous plaques &/or yellow*, greasy scales 3- Located on *scalp - cradle cap*,face (*eyebrows*/ eyelids, posterior ears, *nasolabial folds*), umbilicus, diaper area TMT > usually spontaneous resolution OR 2- Gentle emollients & non-mediated shampoos If widespread or recalcitrant SD 1- Low-potency glucocorticoid creams 2- Topical ketoconazole Atopic dermatitis > Recurrent rash that can affect cheeks, scalp, trunk, & extensor surfaces >> In contrast to SD >> atopic dermatitis is associated w/ *severe pruritus* and most pts will have a *FHx of atopic disorders (asthma, rhinitis)* Contact dermatitis > Symptoms include *severe pruritus* & are confined to specific exposed areas Psoriasis > Chronic inflammatory condition that affects the extensors of elbows & knees but can involve scalp >> rash is described *red, scaly, sharply demarcated, scaling lesions that coalesce to form round or oval plaques* > *onset is typically seen in adults*

A 3 AA boy presents to the ED w/ fever. Since returning from a family trip to Nigeria last week, he has been lethargic w/ constant nausea & headache. He has had several episodes of vomiting & watery diarrhea each day & high spiking fevers, chills, & drenching sweats EOD. The boy carries sickle cell trait & has no other sig PMHx. He takes no meds & his vaccinations are up to date. Temp is 104F, BP is 90/60, Pulse is 132/min, RR is 24/min. Exam shows a tired-appearing boy w/ jaundice. Lungs are clear to auscultation. The abd is soft & non-tender. Hepatosplenomegaly is present. ROM in his neck & extremities is normal. Labs shows mild anemia & thrombocytopenia. Which of the following factors is associated w/ decreased morbidity from this illness? (Sickle cell trait OR Young age)

Sickle cell trait - Pt has *malaria* → presents w/: 1- Periodic febrile paroxysms *Cyclic fevers* 2- Non-specific malaise (*Headache*, *Nausea*, *Vomiting*, *Diarrhea*, Abd pain, myalgia, pallor, *jaundice*, petechiae, *hepatosplenomegaly* Young age - younger pts have ↑ susceptible to cerebral malaria

A 5B, presents for a mild fever up to 101F & a day of left-sided neck swelling. Mom says, "he was fine yesterday, but now it's really bothering him". The boy has had no trauma to the area, has not been ill recently, & has no known sick contacts. All of his vaccinations are up to date. Current temp is 100F. PE shows a red, tender, & fluctuant anterior cervical mass approximately 2 cm in diameter in the left anterior LN chain. His dentition & oropharynx are normal. There are no other areas of lymphadenopathy. Which of the following is the most likely infectious agent? (Bartonella henselae OR Staph OR Prevotella buccae)

Staph > Pt presents w/ acute cervical adenitis >> which presents w/ 1- *acute onset* matter of days (compared to subacute/chronic which can take wks: caused by *bartonella* 1- *unilateral neck swelling* 2- Enlarged LN (enlarged, tender, warm, & erythematous) >> If untreated can progress to suppuration & abscess >> most common organisms *in children <5 are Staph & strep* Bartonella > Would be correct if child had subacute/chronic LAD (Acute lymphadenitis) >> pt would also have a Hx of scratch w/ papule & ipsilateral lymphadenitis Prevotella > Oral anaerobe >> Would be correct if pt had *poor dentition & caries*

A 4B, presents for a routine visit. He is growing well & talking in short sentences. The pt can hop & copy a square. He has started to play board games w/ his brothers & sister but occasionally hits his siblings when he loses a game. His parents are concerned that he cannot yet tie his shoelaces & ask if it is normal for him to occasionally fondle his genitals in public or try to touch his brother's genitals while bathing. The mom says. "he has shown me dolls kissing each other's private parts a few times & he asked where do babies come from. I haven't even had that talk w/ his 6-yr-old brother. Is at a normal question for a 4 yr-old to ask?" The parents are also concerned that the pt still sucks his thumb when anxious & occasionally wets his bed at night. He will be starting school soon. Which of the following behavior is the most concerning in this pt? (Stimulating oral sex using dolls OR Thumb sucking)

Stimulate oral sex using dolls - Concerning b/c he is 4 & it shows *precocious sexual knowledge* → also concerning would be: 1- Preoccupation w/ masturbation 2- Excessive or aggressive talk about sexuality 3- Stimulating in genital-genital, oral-genital, anal-genital contact Thumb sucking - Most kids engage in some form of rhythmic activity for soothing purposes (thumb sucking, rocking, nail biting) → this tends to decrease w/ age

A 6B, presents for refusal to walk. Today he has been asking his father to carry him & cries when he attempts to move his right leg. 1 wk ago, he was Dx'd w/ cellulitis from an insect bite. He completed a 7-day course of cephalexin today. his 9-yr old bro was treated for ALL & is currently in remission. The pt's temp is 102F, BP is 96/60, Pulse is 118/min, RR are 18/min. On exam, he keeps his right hip externally rotated & screams w/any mvmt involving the right hip. He refuses to stand or walk. The remainder of the exam is normal. Labs and ultra-sound guided joint aspiration reveal: - Neutros---------92% - Lymphos-------4% - Bands-----------3% - Hb---------------13 - Plts--------------280K - ESR--------------52 Blood culture is pending. Synovial aspirate shows: - Leukos----------110K - Neutros---------95% Which of the following TMT options is most beneficial in preventing long-term morbidity? (Physical therapy OR Surgical drainage of the hip)

Surgical drainage of hip > Pt presents w/ 1- Fever 2- Acute joint pain, 3- inability to bear wt 4- Marked leukocytosis & elevated ESR >> all signs for *septic arthritis* >> which can cause permanent joint destruction >> usually preceded by a seemingly mild infection *cellulitis* >> Most common organisms are *Staph and Strep* TMT 1- Surgical drainage (most important) 2- Empiric antibiotics (vancomycin) Physical therapy > Correct if pt has legge-Calve-Perthes disease ----(joint aspirate shows 95% neutrophils, which points toward infection)

A 12B, presents b/c of right groin pain, knee pain, & limping. He has had these symptoms for the past 2 weeks. He is at the 90th percentile for wt & 60th percentile for ht. He is afebrile, & his other vitals are normal. Exam shows that the ROM of the right knee joint is w/in normal limits but hip mvmts are restricted & the right foot points outward. There is external rotation of the right thigh on flexion of the hip. After confirming the Dx, what is the most appropriate next step in mgmt? (Conservative mgmt w/ rest & analgesics OR Surgical pinning of the femoral head)

Surgical pinning of the femoral head > Pt presents w/ *Slipped capita femoral epiphysis* Common characteristics include: --1) commonly seen in obese adolescent boys --2) Thigh & groin pain (most common presenting symptom) ----Can also present as Knee pain ----Bilateral involvement seen in 20% of patients --3) PE findings ----a) Trendelenburg gait ----b) External rotation during passive hip flexion ----c) Limited hip internal rotation & abduction -----(pts hip point outward - indicating internal rotation is limited) TMT > Prompt tmt w/ surgical pinning of the slipped epiphysis where it lies (to lessen the risk of avascular necrosis) Conservative mgmt - This would be the TMT for Legge-Calve-Perthes disease -Presentation includes: --1) Children 4-8 w/ M>F ----(Obesity is NOT a factor) --2) RF's ----a) FMHx ----b) low-birth weight ----c) Second-hand smoke ----d) Asian, Inuit, Central European descent ----e) Associated w/ ADHD in 33% of cases ---------(pt has NONE of these RF's) --3) Insidious/progressive onset -> gradual painless limp followed by intermittent knee, hip, thigh, groin pain ----(Pts pain is not progressive; he does not complain that the pain has worsened) --4) PE findings ----a) Hip stiffness w/ loss of internal rotation & abduction ----b) Gait disturbance ----c) Limb-length discrepancy -----(pt does not have this) --5) XR findings ----a) Joint space widening (earliest finding) ----b) Small femoral head (d/t irregularity of femoral head ossification) ----c) Subchondral fracture -------(pt does not present w/ these PE findings) TMT --1) Surgery/bracing -> to contain hip w/in acetabulum --2) Supportive (observation, PT, restricted activity)

A newborn is brought to the nursery for eval after delivery. The mom received no prenatal care but reports that the pregnancy was uncomplicated & she was healthy. The infant was born via spontaneous delivery & required no resuscitation. Apgar scores were 8 & 9 at 1 & 5 mins. On exam, the infant is below the 3rd percentile for wt, 25th percentile for length, and 50th percentile for head. Hepatosplenomegaly is present on exam. Over the next 48 hrs, the infant develops jaundice, clear rhinorrhea, & a maculopapular rash on the feet & buttocks that later desquamates. Which of the following is the most likely in this pt? ( Rubella OR Syphilis OR Toxo)

Syphilis > Specific to Congenital syphilis presents w/: 1- Rhinorrhea 2- Abnormal long-bone radiographs 3- Desquamating or bullies rash Rubella 1- Cataracts 2- Cardiac defects Toxo 1- Diffuse intracerebral calcifications 2- Severe chorioretinitis

A 2F, presents for left knee pain & fever. The knee pain began 2 months ago. At the time of onset, the pt & other several children at day care had cough & rhinorrhea, but no one else developed joint pain. Mvmt is limited by pain in the morning but seems to improve in the afternoon. For the past month, the pt has had a daily fever, & an erythematous macular rash over her chest around bedtime. Temp is 97F, BP is 90/60, Pulse is 114/min. PE shows swelling, erythema, & warmth of the left knee, w/ pain elicited on passive & active motion. Pain is also elicited w/ mvmt of the left hip. The pt can walk but has slight limp. Labs show: - Hb------------------10 - Plts------------------520K - Leuko's--------------21K - ESR-----------------100 Which of the following is the most likely Dx? (ALL OR Systemic juvenile idiopathic arthritis)

Systemic juvenile idiopathic arthritis -A) Signs/symptoms presents 1- Chronic Oligoarthritis -------(worse in morning) 2- Daily fever -----(always starts w/ ≥2 wks of fever) occurring once a day (Quotidian) 3- Maculopapular rash -----(that worsens during fever) 4- ±Lymphadenopathy & hepatosplenomegaly Labs 1- *Leukocytosis* 2- *Thrombocytosis* 3- ↑↑↑ ESR or CRP 4- Anemia (from chronic inflammation and/or iron deficiency ALL - Can also present w/ Fever, joint pain, & leukocytosis - However, key differences are: ---1) Presents w/ thrombocytopenia ------[pt has thrombocytosis] ---2) DOES NOT present w/ maculopapular rash

A 17F, present to the ER by her mom after an episode of loss of consciousness. The girl was taking a shower when her mom heard her fall. By the time the mom unlocked the bathroom door, the girl was lying on the bathroom floor & not responding to questions. The pt recently broke up w/ her bf & has been under a lot of stress at school. She has been sleeping poorly. BP is 120/70, Pulse is 90/min, RR are 13/min. After 20 mins in the ED, the to begins to wake up & respond to external stimuli. She gives her correct name but is disoriented to time & place. Pupils are symmetric & reactive to light. Fingerstick glucose level is 100mg/dL. Which of the following exam or lab findings is most likely to be seen in this pt? (Systolic murmur that increases w/ standing OR Tongue lacerations OR Vasovagal syncope)

Tongue lacerations > Sudden loss of consciousness, Loss of postural tone, & delayed return to baseline mental status should raise suspicion for seizure *generalized tonic-clonic* >> may also present w/: 1- aura (Olfactory hallucination) >> prior to event 2- +/- *lateral tongue lacerations* *Vasovagal syncope* > results in mental status that immediately return to baseline after event ---(pt is disoriented after event) Systolic murmur that increases w/ standing > This is Hypertrophic obstructive cardiomyopathy

A 4-day-old F, presents w/ bilateral eye discharge that began 2 days ago & is worsening. The pt's mom is a 20F, G2P2m which had limited prenatal care. During the 1st trimester, the mom was treated for a STD. During the 3rd trimester, GBS screening was positive. The mom had no additional testing or TMT during pregnancy. Delivery occurred vaginally w/ no known complications at home under the care of a midwife. Temp is 98.1F. The pt has bilateral chemosis, severe eyelid swelling, & sig purulent discharge. The face & chest have mild jaundice. The rest if the exam is normal. Which of the following would most likely have prevented this infant's clinical findings? (Intrapartum ampicillin for the mom OR Topical erythromycin for infant)

Topical Erythromycin for infant - Pt presents w/ *gonococcal conjunctivitis* → which presents w/: 1- Symptoms begin at *2-5 days & include ---Purulent discharge --- Eyelid swelling & Chemosis (Conjunctival edema) TMT - For moms who have not been screened → Topical erythromycin prophylaxis Intrapartum ampicillin for mom - would be the best tmt if pt had chlamydial trachomatis → which presents: 1- In 2nd week of life 2- Watery, serosanguinous, or mildly purulent discharge

A 4B, presents to the volunteer medical center of a large refugee camp in Egypt for eval of eye redness. For 10 days the boy has had mild rhinorrhea & cough that are now resolving. He began having mild eye redness 2 days ago w/ scant, watery discharge. He has not had ocular pain or itching. The pt's 6-yrs-old bro has similar symptoms. The family fled their home in Sudan d/t regional conflict & arrived in the refugee camp approximately 2 months ago. The boy's grandfather & 2 dogs remained at their home. Vitals are stable. Exam of the chest & abd is unremarkable. Exam of the eyes shows several pale follicles & inflammatory changes in the tarsal conjunctivae bilaterally. The conjunctivae appear mildly thickened. Visual acuity 20/20 bilaterally. Which of the following is the most likely Dx? (Chlamydial conjunctivitis OR HSV keratitis OR Gonococcal conjunctivitis OR Trachoma)

Trachoma - D/t Chlamydia trachomatis -A) Etiology ------1) Usually spreads in unsanitary/crowded conditions -----2) Often a concomitant nasopharyngeal infection ----------(Rhinorrhea, pharyngitis) -B) Signs/Symptoms ------1) Bilateral Conjunctival injection ------2) Tarsal inflammation ------3)Pale follicles -C) Eye exam findings ---------Follicular conjunctivitis & pannus (neovascularization) formation in the cornea Gonococcal Conjunctivitis - Usually presents 2-5 days after birth Chlamydial conjunctivitis - Usually presents 5-14 days after birth - Both present w/ Conjunctival injection, eye discharge, & Swollen eyelids HSV keratitis - Also Presents as pain, photophobia, & ↓ vision - However, key differences include: ---1) Presents w/ VESICULAR lesions that are on the eye and face (in trigeminal line) ---2) Not associated w/ unsanitary conditions

An 18-month-old B, presents to the ED d/t sleepiness. He has been tired & difficult to arouse for about one hour. For the past 6 months, the boy has had frequent episodes of crying inconsolably for 10-15 mins at a time. However, he seems "totally normal" btwn episodes. His appetite is decreased & he has had multiple episodes of emesis. Just prior to arrival, the boy passed a loose stool that looked dark & "sticky" & contained streaks of blood. He has had no other BM today. The pt has no sig PMHx and takes no meds. Temp is 98F, BP is 100/62, Pulse is 128/min, and RR is 18 min. On PE, he is lethargic. There is no rhinorrhea, oral mucous membranes are moist, the pharynx in not erythematous, & tympanic membranes are clear. S1 & S2 are normal w/out murmurs. Lungs are clear without wheezes or rales. The abd is soft 7 & non-distended w/ mild LLQ tenderness, hypoactive bowel sounds, & no hepatosplenomegaly. Which of the following is the most important next step in MGMT? (Abd CT scan w/contrast OR Technetium-99 scan OR US guided air contrast enema)

US guided air contrast enema > Pt presets w/ episodic crying, emesis, bloody stool, & lethargy all of which suggest *intussusception* -A) Signs/Symptoms ------1) Sudden, intermittent abd pain ------2) Currant jelly stools ------3) Sausage-Shaped abd mass ------4) Lethargy/ altered mental status ------5) Intermittent episodes of pain -----------(episodic inconsolable crying) -B) Diagnostic -------1) US -> (DIAGNOSTIC) -----------a) Target sign -----------b) Pseudo-kidney sign -------2) Abd CT scan w/ contrast ------------b) May reveal target sign >> but not study of choice b/c time consuming & radiation -C) TMT -------1) Pneumatic/hydrostatic enemas -> (non-operative reduction) Technetium-99 scan > Used to Dx Meckels which can lead to intussusception - However, key differences include: ---1) Meckles typically presents w/ PAINLESS rectal bleeding ------[pt has episodes of INTENSE PAIN] ---2) Meckles usually presents in children ~ 2 years old

A 15-day-old presents to the ED for evaluation of 2 episodes of "green" vomit that began this morning. The boy has been exclusively breastfed since birth but has shown little interest in feeding over the past 24 hrs. He was born full-term by uncomplicated vaginal delivery & had an uneventful nursery course. He passed meconium on the first day of life & has had normal daily stools. Temp is 98F, BP is 85/50, pulse is 142/min, RR is 46/min. Exam shows an irritable boy w/ dry mucous membranes. The abd is soft & mildly distended. A nasogastric tube is placed for decompression & IV fluids are started. Abd XR shows a gasless abdomen. Which of the following is the most appropriate next step in MGMT? (Abd US OR Serial Abd x-rays OR upper GI series)

Upper GI series - Used to Dx *Midgut volvulus* → which classically presents in a *neonate <1month w/ bilious vomiting* - Signs of ischemia (shock) are an indication for emergent laparotomy Diagnostic tests 1- XR → to rule out pneumoperitoneum (shows gasless abdomen) 2- Upper GI series (barium swallow) → *fastest & most accurate way of Dx-ing malrotation w/ midgut volvulus Abd US - Preferred for Pyloric stenosis but not sensitive enough for midgut malrotation Serial Abd X-rays - used in infants to follow the progression of non-surgical conditions (such as ileus or mild necrotizing enterocolitis) - Not used in surgical conditions

A 3F, presents w/ dysuria. Her parents say that she has been crying w/ urination, even though a "small amount comes out at a time." She has no vomiting, nausea, or fever. In the past year, the girl had 3 bladder infections that were treated w/ antibiotics; the last infection was 2 months ago. She also has a Hx of constipation since starting cow's milk at age 1 & takes a laxative as needed to help w/ bowel mvmts. Temp is 99F, BP is 80/50, Pulse is 110/min. Exam shows suprapubic tenderness & small anal fissures at 12 o'clock & 2 o'clock. Rectal exam shows normal anal wink & tone; hard stool is palpated in the rectal vault. Urinalysis shows positive leukocyte esterase, positive nitrites, & WBC's 60/hpf. Urine culture shows 100K colonies of E.coli. Renal US & voiding cystoureterogram are normal. Which of the following is the most likely mechanism for this pt's infection? (Urinary Reflux OR Urinary stasis)

Urinary stasis - Pt presents w/ Pediatric constipation - Pediatric constipation can cause recurrent cystitis -A) Signs/Symptoms (pediatric constipation) ------1) *Painful/hard BM's* ------2) Stool with-holding ------3) Encopresis (soiling of underwear) ------4) Recurrent cystitis -> Signs/Symptoms -----------a) Suprapubic pain, -----------b) dysuria, -----------c) pyuria & bacteriuria -C) RF include (pediatric constipation) ------1) Initiation of solid food & *cows milk* ------2) Toilet training ------3) School entry -D) Complications (pediatric constipation) ------1) *Anal fissures* ------2) Hemorrhoids ------3) Enuresis/ *UTI's* Urinary reflux - Also present w/ UTI symptoms - However. key differences include: ---1) Most commonly commonly associated w/ boys & PUV ---2) US findings -> hydronephrosis --------[pts is normal US] ---3) Cysturethrogram -> abnormal voiding --------[pt has NORMAL cysturethrogram]

A 1-month-old B, presents d/t poor feeding. When nursing, he becomes diaphoretic, & for the past week he has fed for only 5 mins at a time before falling asleep. The pt was born full term via spontaneous vaginal delivery. Today, his Wt is 1 oz below birth Wt. Temp is 98.6F. BP is normal. Exam shows a thin infant who is seeping but easily arousable. Cardiac exam reveals tachycardia & a I/VI holosystolic murmur over the left lower sternal border w/ a diastolic rumble over the cardiac apex. The S2 is prominent. The pt is tachypneic, but the lung fields are clear to auscultation. There is no clubbing, cyanosis, or peripheral edema. Which of the following is the most likely cause of this pt's symptoms? (Tetralogy of fallot OR VSD)

VSD -A) Signs/Symptoms ---1) Murmur -------a) Characteristic murmur is *holosystolic* & heard at *left lower sternal* border --------b) apical diastolic rumble may he heard over the cardiac apex --------c) Prominent S2 ---2) NO Cyanosis at birth ---------However cyanosis can occur if a large VSD presents & pt has Eisenmenger syndrome (cyanosis from Rt-to-Lt shunting) Tetralogy of fallot - Also presents w/ a murmur at birth - However, key differences are: ---1) Pt would have cyanosis AT BIRTH ---2) Murmur -------a) Harsh, systolic ejection murmur at the left upper sternal border (d/t pulmonary stenosis) ----------[pt has a holosystolic murmur heard the left lower sternal border]

Explain these findings in a hypochloremic, hypokalemic metabolic alkalosis? In what condition would you see this ABG? (dehydration, vomiting, DKA, etc) -pH-------Elevated - PaCO2--Elevated - HCO3--Elevated - K+-------decreased - Cl-------decreased

pH > Pt is vomiting K+ > Pt is vomiting Cl > pt is vomiting PaCO2 > *breathing compensation* pt is in alkalosis so body keeps CO2 (acid/ hypoventilation) to try and balance HCO3 > *kidney compensation* >> elevated b/c it is reabsorbed to try and keep more CO2 in body