NBME PEDS

What antibodies are associated w/ Graves? What HLA's is graves associated w/?

1- Thyroid-stimulating Ig's (TSI) → activate TSH receptor (mimic TSH) 2- Anti-thyroglobulin & Anti-thyroid peroxidase ---(NOTE- THESE ARE ALSO SEEN IN HASHIMOTOS) HLA-DR3 (MHC-II) & HLA-B8(MCH=I)

What is the TMT for bronchiectasis?

1- Treat underlying disease process 2- Lobectomy (localized disease) 3- Lung transplant (severe diffuse)

A mother of a 5-lbs 5-oz baby received no prenatal care during pregnancy & has tested HIV positive. Exam of the newborn shows no abnormalities. Which of the following is the most appropriate next step in MGMT in the newborn? (A 6 wks course of oral AZT w/in 12 hours after delivery OR A 6 wks course of triple HARRT w/in 24 hrs after delivery)

A 6 wks course of oral AZT w/in 12 hours after delivery - Do not use triple HARRT therapy in neonates → only in mom

A previously healthy 4B, presents b/c of a 1-wk Hx of intermittent fever. During the past 3 days, he has had decreased activity & joint pain. Yesterday he developed a red rash on his chest. He attends day care. He appears pale & is not in acute distress. Temp is 101.1F, pulse is 140/min, RR are 24/min. Exam shows a petechial rash over the chest. The conjunctivae are pale. There is diffuse Adenopathy in the cervical chain. A grade 2/6 systolic murmur is heard. The spleen is palpated 1-cm below the left costal margin. There is no swelling or redness of joints. Labs show a Hb of 6.4, leukocyte ct of 50K, & platelet ct of 10K. What is the most likely Dx? (ALL OR Autoimmune hemolytic anemia)

ALL > Most commonly presents in *child (B>G) 2-5 yrs old* >> symptoms include: 1- Symptoms of anemia (*tiredness*, breathlessness, ↑risk of excessive bleeding* (petechial rash), Pale conjunctivae 2- *Fever* 3- Pale skin 4- *Bone & joint pain* 5- Unexplained wt loss 6- Purpura/ petechiae 7- Swollen LN/ glands 8- *splenomegaly* 9- *↓RBC's & ↓Plts* w/ ↑ leukocytes > Associations *Down syndrome → 2/6 murmur* Autoimmune hemolytic anemia > pt would have: 1- IgM or IgG antibodies > Warm hemolytic anemia (IgG) 1- Preceding viral infection 2-Underlying Autoimmune disorder 3- Lymphoproliferative disorder *CLL* 4- Immuno deficiency *CVID* PBS findings 1- Normocytic anemia 2- Spherocytes 3- ↑Reticulocytes 4- ↑LDH 5- ↓ Haptoglobin

A previously healthy 5-month-old F, presents to the ED by her mom's BF 30 mins after she stopped breathing. He says that prior to giving her a bath, she suddenly cried, became limp, & stopped breathing. She is unresponsive, flaccid, & cyanotic. Temp is 99.7F, pulse is 160/min, RR is 5/min, & SBP is 45. The pupils are dilated. Fundoscopic exam shows bilateral retinal hemorrhages. After securing an airway, a CT scan of the head shows bilateral cerebral edema & acute interhemispheric subdural hemorrhage. Which of the following is the most likely cause of these findings? (Acceleration-deceleration head injury OR Suffocation)

Acceleration-Deceleration head injury - Pt presents w/ *baby shaking syndrome* → which presents w/: 1- Bilateral retinal hemorrhages 2- Possible bilateral cerebral edema & acute interhemispheric subdural hemorrhage Suffocation - usually present w/ vague, non-specific, apparently life-threatening symptoms such as: 1- Limpness 2- Pallor 3- Cyanosis 4- Apnea

A 3F, presents to the ED mins after an episode of syncope followed by generalized tonic-clonic mvmts lasting 30 sec. Two mins after the episode, she is fully alert. On arrival, she stops talking, closes her eyes, & has 3-4 rhythmic jerks of the RUE. During the episode, an ECG shows P waves at 80/min w/ no QRS complexes; normal sinus rhythm resumes in 20 sec. One min later, she is fully alert. Which of the following is the most likely Dx? (Vasovagal syncope OR Adam stokes attack)

Adam stokes attack - Sudden loss of consciousness *periodic fainting spell* (no stressor needed) → d/t disorder of heart rhythm in which there is a slow or absent pulse resulting in syncope ± convulsions - Lasts around *30 sec* - Jerking *not seizures* - Attacks caused by temporary lack of cardiac output leading to abnormal pulse & abnormal ECG Vasovagal syncope - Syncope defined as *Sudden loss of consciousness & postural tone followed by spontaneous complete recovery* - Usually d/t to some stressor -(Not associated w/ slowed/abnormal pulse) - (Not associated w/ rhythmic jerking)

A 2-wk-old presents to the ED b/c of a 30-minute Hx of her chest fluttering. 30 mins ago, she vomited after breast-feeding. She was born at 40-wks following an uncomplicated pregnancy & delivery. She weighed 7lbs 8oz at birth. On arrival, she is awake & appears alert. Temp is 98.2F, Pulse is too rapid to count, RR 48/min, BP is 74/84. The extremities are pink & warm; capillary refill time is less than 2 seconds. The lungs are clear to auscultation. No murmurs are heard on cardiac exam. An ECG shows a regular heart rate of 240/min, QRS complex duration of 0.05 secs, & no discernible P-waves. Application of an ice bag to the face for 5 secs does not change the heart rate. Which of the following is the most appropriate next step in MGMT? (Adenosine OR Verapamil OR Cardioversion)

Adenosine - Pt presents w/ paroxysmal supraventricular tachycardia -A) ECG characteristics -----1)Short QRS complexes* (<120ms) -----2) ↑ HR (>100bpm) -----3) No P-waves -B) MGMT -> Based on hemodynamic status -----a) TMT -> BASED ON HEMODYNAMIC STATUS ---------AA) HEMODYNMICALLY stable --------------1) Carotid massage/vagal maneuvers --------------2) Adenosine → PREFERRED --------------3) Verapamil ----------BB) HEMODYNAMICALLY UNSTABLE --------------1) Immediate cardioverison -------------------a) This pt has a LOW BP → However, she is only 2-wks old (so this BP is ok for her) --------------------She has no ADDITIONAL signs of shock → so cardioversion is not indicated

An 11B, presents to the physician b/c of a 1-day Hx of lethargy, mild, abd pain, & dark urine. He had a sore throat & an URTI 10 days ago that resolved spontaneously. Pulse is 90/min, RR is 20/min, BP is 140/90. Exam shows slight swelling around the eyes. Urinalysis shows light brown urine w/ RBC & granular casts. Which of the following is the most likely mechanism for these findings? (Ag-Ab immune Complex OR Destruction of tubular epithelial cells)

Ag-Ab immune complex deposition - *Most common form of acute glomerulonephritis in school aged children *5-12yrs* - Preceded by strep infection *prior 2-4 wks* - Pt presents w/ *PSGN* → usually Presents w/: 1- Gross hematuria Cola (dark) colored urine 2- HTN 3- Edema (often generalized, *periorbital* Urinalysis 1- Hematuria -----Granular casts -> "Granular" signifies the presence of blood, they are not specific to only ATN 2- Pyuria 3- Proteinuria Pathophys *d/t IC complex deposition* - Also includes (Lupus, Henoch-Shonlein purpura, IgA nephropathy) Destruction of tubular epithelial cells - Seen in Acute tubular necrosis - The Pateint present after a URTI infection -----(This presentation does not fit ATN) - The Pateint present after a URTI infection & has nephrotic symptoms -----(ATN does not present w/ nephrotic symptoms) -----(ATN usually presents in the setting of renal ischemia or with ingestion of a Toxin/med) - Would present w/ muddy-brown OR granular casts

An 18-month-old B, presents to the ED 4 hours after the onset of lethargy. He has had intermittent abd pain for the past 24 hrs. He has not had vomiting or diarrhea. He appears lethargic. Exam shows a soft, non-tender abd; a mass is palpated in the RLQ. Bowel sounds are decreased. The stool is red, & test for occult blood is positive. An AB-XR shows no air in the ascending or transverse colon. What is the most appropriate next step in Dx? (Air contrast enema OR Upper GI series)

Air contrast enema - Pt presents w/ *intussusception* → which presents w/: 1- *intermittent/episodic colicky abd pain (15-20 mins intervals) → pts may draw their legs up to their abdomen 2- *Episodic lethargy* or altered consciousness → that is disproportionate to abd signs is common in LATER bouts of abd pain [pt has had abd pain for 24 hrs] 2- ± vomiting → initially non-bilious but then becomes bilious (d/t progressive intestinal obstruction) 3- Palpable, sausage shaped abd mass 4- Decreased bowel sounds 5- Currant jelly stools (positive for occult blood) -Diagnostics- 1- US → ----Target (Bull's eye) sign ----Pseudo-kidney sign TMT 1- Non-operative reduction w/ either pneumatic or hydrostatic *enema* (preferred in stable pts w/out bowel perforation) Upper GI series - Used to detect esophageal disorders

A 7F, w/ juvenile RA presents b/c of a 1-year Hx of progressive fatigue. She has had occasional fever & swelling & pain of the knees during the past month. She appears ill. Temp is 102F, pulse is 104/min, RR are 20/min. Exam shows pale conjunctivae. The spleen is palpated 2-cm below the left costal margin. The right knee is warm, erythematous, & mildly swollen; ROM is limited. Labs show: - Hb-------------9.5 - MCV-----------75 - Leuko's---------11K - RC's------------0.5% - Plts-------------400K What is the most likely Dx? (ALL OR Anemia of chronic disease)

Anemia of chronic disease > Occurs in the setting of *Chronic infectious or inflammatory disease* [[Pt has RA]] Labs > usually normochromic normocytic but can be hypo chromic microcytic > RC's usually decreased (N 0.5%-1%) ALL > most common in boy ages 2-5 >> symptoms include: 1- Fatigue, fever, bone and joint pain, splenomegaly, but key to differentiate is *LABS* - ↓RBC's & *↓plts* [[pt has increased plts]] - ↑leukocytes [[pt has normal leuko's]]

A 3B, presents for a follow-up exam 2 wks after being treated for an ear infection. He has had recurrent ear infections, sinusitis, & two episodes of bacterial pneumonia since birth. Over the past year, he has undergone placement of two sets of tympanostomy tubes for otitis media. His mother is HIV negative. He currently takes no meds. He is at the 25th percentile for ht & wt. Exam shows no abnormalities except for small anterior cervical LN. Which of the following is the most likely mechanism of disease? (Antibody deficiency OR T-lymphocyte dysfunction)

Antibody Disease > Child *> 6 months* that presents w/: 1- Recurrent bacterial infections *Classically Recurrent otitis media, sinusitis, pneumonia* (w/ increased susceptibility to pyogenic encapsulated bacteria) -- S.pneumo -- H.influenzae -- Pseudomonas 2- *Decreased in size/ absent tonsils* & spleen (pt has small LN) LABs - Absent mature B-cells in peripheral blood - *↓Ig's of all classes* T-lymphocyte dysfunction > *Hyper IgM* → failure of CD40L to class switch > Pt would have severe pyogenic infections w/ 1- Pneumocystis 2- Crypto 3- Histo 4- CMV Labs 1- ↔/↑ IgM 2- ↓↓ IgG, IgA, IgE 3- No germinal centers

A 2B, presents to the ED b/c of fever for 3 days & right elbow pain for 1 day. He had a GBS meningitis at the age of 5 months & septic arthritis caused by H.flu type B at the age of 9 months. A brother & three uncles died in infancy of septicemia. Immunizations are up to date. Temp is 104.5F. Exam shows redness & swelling of the right elbow; ROM is limited. The remainder of the exam shows no abnormalities. Labs show: - Leuko's-----------34K - Neutro's----------60% - Bands-------------15% - Lympho's---------25% Blood culture grows Strep pneumo. The most likely cause of these findings is impairment of which of the following? (B-lymphocyte function OR Complment function)

B-lymphocyte function - Descries Brutons -> presentation includes: --1) X-linked (Would affect only males) --2) Would be present > 6 months & be susceptible to recurrent infections w/: -----1- Bacteria ------------*H.flu* ------------*S.pneumo* (pt also has GBS) -------------S.aureus -----2- Enteroviral -------------Coxsackie --------------Polio -----3- Giardia Complement function - Seen in *terminal complement deficiency* → which presents w/: 1- *Recurrent infections w/ *Nesseria*

A 2-hr-old newborn has a large fluctuant area of swelling in the left parietal region. She was born at term following a difficult forceps delivery. Exam of the area shows margins limited by suture lines. Which of the following is the most Likely cause go the swelling? (Blood under the galea aponeurotica OR Blood under the periosteum of the parietal bone)

Blood under the periosteum of the parietal bone > Pt presents w/ *Cephalohematoma* - Presentation includes: ---1) likely to occur in setting of *prolonged, difficult second stage of labor *particularly forceps delivery* → d/t rupture of the blood vessels crossing the periosteum ---2) Subperiosteal* → symptoms -------*appear 2-3 days after birth* (or hrs as in this pt) -------*blood would be limited by suture lines* ----3) Symptoms include 1- Jaundice 2- Anemia 3- Hypotension 4- Osteomyelitis or meningitis Blood under the galea aponeurotica > *Subaponeurotic hemorrhage* -----blood would be between the scalp and skull & NOT LIMITED by suture lines

A 5F, w/ a 1 year Hx of chronic non-productive cough is brought for a follow-up exam. The cough occurs during the day & night & is not exacerbated by exercise. She has not had chills, diarrhea, or Wt loss. Inhaled β- adrenergic agonists, two courses of oral antibiotics, & a 10-day course of oral prednisone have not relieved her symptoms. She is at the 50th percentile for Ht & Wt. She appears well. Temp is 99.5F, pulse is 96/min, RR is 18/min, BP is 82/60. Exam shows no abnormalities. A CXR shows no abnormalities except for a linear consolidation in the right middle lobe. Which of the following is the most likely Dx? (Bronchiectasis OR Chronic Bronchitis)

Bronchiectasis - Symptoms include 1- Chronic cough w/ yellow/green sputum (but can be *nonproductive*) 2- XR → Tram lines *Linear consolidation* Chronic Bronchitis - usually associated to COPD, usually obese, & have peripheral edema or an enlarged liver d/t RHF - Dx criteria → *A productive cough for greater than 3 months out of the year for more than 2 consecutive years* [Pt does not meet criteria]

How can you differentiate bronchiectasis from chronic bronchitis using sputum production?

Bronchiectasis → >100mL sputum/day → Purulent sputum Chronic bronchitis → <100mL/day → non-purulent

A 4-month-old infant presents by her mom for a routine exam. The pt was born at 27 wks to a 26F, Multiparous. The pt's birth Wt was 2-lbs 10-oz, & she spent the first 3 months of her life in the hospital. During hospitalization, she was treated for sepsis & RDS that required intubation & mechanical ventilation. She is currently receiving O2 & diuretic therapy at home. She is at the 5th percentile for length & Wt. Temp is 99F, pulse is 104/min, RR is 32/min, BP is 115/67 in the right arm & 105/67 in the left leg. Breath sounds are decreased bilaterally, & wheezing is heard occasionally. Cardiac exam shows a pronounced S2 & a precordial heave. Abd exam shows hepatomegaly. An ECG shows a right axis deviation & RVH. US shows normal-sized kidneys. Which of the following os the most likely cause of this pt's increased BP? (Bronchopulmonary dysplasia OR Coarctation of the Aorta)

Bronchopulmonary dysplasia *Any infant that requires O2 supplementation for at least 28 days postnatally is considered to have BD* - RF's include 1- *Prematurity* 2- *Mechanical ventilation* 3- O2 toxicity 4- Infection (post-natal or Antenatal) -Symptoms include: 1- *Tachypnea* 2- *Pulmonary edema* 3- Audible rales -TMT- 1- Mechanical ventilation 2- Continuous positive airway pressure ventilation 3- Supplemental O2 alone -Complications- 1- Pulm HTN Coarctation of Aorta - *Preductal coarctation* Usually associated w/ *Turners syndrome* → would present w/: 1- *Differential cyanosis* (well perfused UE & cyanotic LE) [pt does not have cyanosis in LE, only decreased systolic BP *Post-ductal coarctation* → usually seen in adulthood w/ bicuspid aortic valve

A 3B, presents to the ED by his dad b/c of rapid breathing for 1 hour. The pt states that he cannot catch his breath. His temp is 98.6F, Pulse is 100/min, RR is 30/min, & BP is 120/80. The lungs are clear to auscultation. There is light hyper resonance on the right side of the chest. Decreased breath sounds on the right. An CXR shows slight over-expansion of the right lung compared with the left; there are no infiltrates or effusions. O2 is administered via nasal cannula. Which of the following is the most appropriate next step in MGMT? (Bronchoscopy OR Thoracotomy OR Tube Thoracostomy)

Bronchoscopy - pt most likely swallowed a foreign object which is causing air trapping in the right lung, leading to: ---1) An over-expanded right lung ---2) Light hyper-resonance on right side ---3) Decreased breath sounds on right - Allows exam of the lungs & bronchi → can be used to asses for: 1- Tumor 2- Infection 3- Collapsed lung 4- Foreign object Thoracotomy - Small incision in chest wall for drainage Tube Thoracostomy - Tube placed in pleural cavity to drain blood, fluid, air, bile, pus

An 8G, presents b/c of a 3-day fever, progressive cough, & sputum production. Exam at birth showed no abnormalities, but she has a Hx of respiratory problems since infancy. Temp is 99.5F, Pulse is 100/min, RR is 32/min, pulse is 100/min. Pulse Ox on room air is 84%. Exam shows clubbing, circumoral cyanosis, & a hyperextended chest. There are diffuse rhonchi bilaterally w/ end-expiratory wheezing. A CXR shows hyperinflation, scattered atelecatsis, & chronic interstitial changes. What is the most likely Dx? (Bronchopulmonary dysplasia OR Cystic fibrosis)

CF - Pt most likely has CF -A) Signs/Symptoms ----1) Hx of re-current respiratory problems since infancy ----2) Persistent productive cough & wheezing -B)PE findings ---1) Digital Clubbing ---2) Circumoral cyanosis ---3) Increased anterior posterior diameter ------(All Specific to CF) -C) MGMT -----a) Dx tests ---------1) CXR -------------a) Hyperinflation* -------------b) Bronchiectasis* -------------c) Cyst formation -------------d) Flattening of the diaphragms -------------e) scattered atelectasis (mucus plugs) -------------f) chronic interstitial changes Bronchopulmonary dysplasia - Also presents w/ Tachypnea in children - However, key differences include, ---1) Usually associated w/ several RF that MUST be present in order to consider this Dx, including: ------a) Mechanical ventilation & prematurity -----------Any child requiring supplemental O2 for at least 28 days postnatal is considered to have BD -------------[pt DOES NOT have this RF] ------b) Infection (postnatal/antenatal) --2) BD presents w/ KEY SYMPTOMS including: ------a) Pulmonary edema ------b) Rales -------------[pt has NONE of these symptoms]

What would be seen on CXR for bronchiectasis? What is the gold standard for Dx & what would be seen?

CXR → Increased bronchovascular markings & tram lines (parallel *Linear* lines outlining bronchi & consolidation) Gold standard → High-resolution CT (HRCT) → shows *marked dilation of bronchioles*

A 12-hour-old F, becomes irritable, has difficulty breathing, & then has a 2-min generalized tonic-clonic seizure. She was born at 36 wks by CS for a breech presentation & weighed 8-lbs 13-oz. Her mom had GDM poorly controlled w/ diet; she refused insulin therapy. Apgar scores were 7 & 8 at 1 & 5. Exam shows decreased tone & lethargy. Pulse OX shows an O2 sat of 99%. An ECG shows a prolonged QT interval. In addition to measurement of serum glucose conc, the most appropriate next step is measurement of which of the following serum conc's? (Ca2+ OR Mg+ OR TSH)

Ca2+ - Signs symptoms include: 1- *Prolonged QT interval*, Bradycardia 2- Seizures (severe) 3- Laryngospasm *difficulty breathing* 4- Confusion *Peri & Post-natal complications of GDM 1- *Hypocalcemia* 2- Resp distress 3- Delayed neurologic maturity 4- Polycythemia & neonatal jaundice 5- Traumatic delivery & brachial plexus injury 6- *Hypoglycemia* Mg+ - Not associated w/ GDM → presents w/: 1- Lethargy 2- Confusion 3- Tremor 4- Ataxia 5- Nystagmus 6- Tetany 7- Seizures 8- Arrhythmias [Associated w/ Hypocalcemia & hypokalemia but not a direct effect of GDM] TSH *Hypothyroid* 1- Poor brain development 2- Pot-bellied 3- Pale 4- Puffy-faced 5- Protruding umbilicus 6- Protuberant tongue 7- Prolonged jaundice *Hyperthyroid* - Would occur in the setting of Graves disease by mom

What can bronchiectasis be a cause of? When should you suspect it?

Can be a cause of *secondary amyloidosis* → suspect it in a pt w/ bronchiectasis who develops chronic renal failure & splenomegaly & ↑ urine protein

A 12-month-old AAF, w/ sickle cell disease presents to ED b/c of the sudden onset of fever, irritability, & tachypnea 6 hrs ago. Her diet consists of iron-fortified formula & pureed fruits & veg. Her mother states that she only misses an occasional dose of her penicillin prophylaxis. She appears ill. Temp is 102.2F, Pulse is 180/min, RR is 48/min. Exam shows conjunctivae pallor. A grade 2/6 systolic murmur is best heard at the left sternal border. Labs show: - Hct------------------23% - Leuko's--------------23K - Plts------------------250K The most appropriate initial step in MGMT is administration of which of the following? (IV penicillin OR IV cefotaxime)

Cefotaxime - Frequently prescribed in pediatric pts with SCD characterized by: 1- Vaso-occlusive crisis 2- Chronic hemolytic anemia 3- Defective immune function leading to severe infections (Recommended for septic episodes) IV penicillin - Used as prophylaxis but not in acute setting

An 8G, presents b/c of pallor & easy fatiguability for the past 2 months. Her symptoms began after an URTI. She was adopted, & her FHx is unknown. She appears pale. The spleen tip is palpated 2-cm below the left costal margin. Labs show: - Hct------------------28% - Reticulo's-----------4% - Spherocytes--------3+ Splenectomy is most likely to prevent which of the following complications? (Cholelithiasis OR Overwhelming sepsis)

Cholelithiasis - Pt has *hereditary spherocytosis* → Pigment *bilirubin* gallstones commonly occur in adolescents and adults (d/t increase breakdown of spherocytes in spleen) Overwhelming sepsis - Not a complication of hereditary spherocytosis

How would a pt w/ bronchiectasis present? What are physical exam findings?

Chronic cough w/ yellow/green sputum & dyspnea ± hemoptysis PE findings 1- Rales 2- Wheezes 3- Ronchi Depending on the cause of the bronchial inflammation

A 1-week-old newborn presents b/c of a 1-day Hx of difficulty breathing & discoloration of the extremities. He was born at term following an uncomplicated delivery. Apgar scores are 8 & 9, at 1 & 5 mins. He appears ill. His rectal temp is 97.5F, pulse is 160/min, RR is 52/min, BP is 60/36 in the UE & unobtainable in the LE. The skin, mucous membranes, & nail beds are dusky, & there is mottled discoloration of the extremities. Exam shows moderate intercostal retractions & grunting. The lungs are clear to auscultation. A grade 3/6 holosystolic murmur is heard along the left sternal border. The liver edge is palpable 4-cm below the right costal margin. ABG analysis on room air shows: -pH----------------------7.15 - PCO2------------------28 - PO2--------------------98 Intubation, mechanical ventilation, & an IV fluid bolus of saline are initiated. One hour later, there is no sig improvement. A CXR shows cardiomegaly & pulm congestion. Which of the following is the most Likely explanation for this newborn's condition? (Closure of the ductus arteriosus OR Increased pulm vascular resistance)

Closure of a patent ductus arteriosus - Pt presents w/ cyanosis → most likely has congenital disorder that requires the patency of a PDA to get oxygen → if this closes the pt will have increasing cyanosis Increased pulm vascular resistance - Seen in tetralogy of fallot but would not be the cause of cyanosis

A 13F, w/ CF presents b/c of a 6-month Hx of progressive difficulty breathing. Her mom says that her daughter's hands & feet have appeared swollen during this period. Temp is 98.6F, Pulse is 98/min, RR is 30/min, BP is 124/84. Exam shows dusky nail beds & clubbing of the fingers & toes. There is nasal flaring & moderate intercostal retractions. Bilateral crackles and bronchi are heard on auscultation. S2 is loud & split in the second left intercostal space. The liver edge is palpable 5-cm below the right costal margin, & the spleen tip is palpated 1-cm below the left costal margin. There is bilateral splitting edema over the dorsal of the hands & feet. Labs show: - Na+---------------126 - Cl-----------------84 - k+-----------------4.1 - HCO3------------36 - Albumin----------2.8 - Urine protein ----+1 Pulmonary function tests show a decreased in FVC from 75%-30% of predicted value over the past year. A CXR shows marked hyperinflation w/ coarse nodular infiltrates bilaterally. Which of the following is the most likely explanation for this pt's edema? (Lymphedema OR Cor pulmonale)

Cor Pulmonale > Long standing CF causes a type or "interstitial lung disease" - Over the long term, this causes an increase Pulmonary HTN - Plumonary HTN can then lead to RHF (Cor Pulmonale) Lymphedema - associated w/ blockage of lymph drainage - Also presents w/ edema - However, the key difference is ---1) This would ONLY EXPLAIN THE EDEMA -> the pt has additional symptoms that would not be explained my lymphedema including: -----a) Hepatosplenomegaly, -----b) a loud S2, -----c) hyperinflation on lungs, ------d) coarse nodular infiltrates on CXR ---------[these signs are characteristic of interstitial lung disease in CF]

An asymptomatic 10B, presents for a health exam. He has no Hx of serious illness & takes no meds. His parents both have essential HTN & hyperlipidemia. He has an active lifestyle & plays several sports. He consumes a wide variety of foods, including meat, fruit, & veggies, & drinks 2-3 glasses each of whole milk & fruit juice daily. He is currently at the 90th percentile for Ht & above the 97th percentile for wt & BMI. Vitals are normal. The remainder of the exam shows no abnormalities. Which of the following is the most appropriate dietary recommendation at this time? (Decrease his intake of milk & fruit juice OR Increase his intake of fruit & vegetables)

Decrease his intake of milk and fruit juice - The pt is above the 97th percentile for Wt - We must reduce his weight; the best way would be to reduce his intake of: ---1) Whole milk -> Contains a lot of calories & fat ---2) Fruit -> contains sugars that can lead to weight gain Increase his intake of fruit and veg - No b/c do not want to increase his fruit (contains sugars that can lead to weight gain)

An otherwise asymptomatic 4-wk-old presents b/c of increasing jaundice for 1 wk. He was born at term following a prolonged labor. One day after birth, he received phototherapy for jaundice & was discharged on the 3rd day. He is exclusively breast-fed. Exam shows no abnormalities except for jaundice. Labs show a Hb of 12, w/ normal red cell morphology, & a total serum bilirubin conc of 12 mg/dL w/ a direct component of 8mg/dL. Which of the following mechanism is the most likely underlying mechanism of this pt's jaundice? (Decreased biliary excretion OR Increased serum bilirubin production OR Decreased hepatic GGT activity)

Decreased biliary excretion - Pt most likely presents w/ physiologic jaundice -A) Signs/Symptoms -----1) Jaundice ---------but NO OTHER SYMPTOMS -----2) Usually peaks at 3-5 days postnatal -----------a) However, It can last for a up to 4 weeks -----------b) Never present before 24 hrs of age -----3) Total bilirubin > 5 indicates jaundice -B) Pathophysio of physiological jaundice includes: -----1) ↑ production (d/t high RBC turnover) -----2) ↓ Clearance (decreased biliary excretion) ----------a) d/t latent deficiency of UGT1A1 -----3)↑ in enterohepatic circulation Increased serum Bilirubin production - This is associated w/ other defects, such as: 1- congenital defect (like biliary atresia) 2- hepatitis 3- infection 4- Inborn error of metabolism ----(Pt has no PMHx of these) Decreased hepatic GGT activity - This describes the pathophysiology of Galactosemia -Also presents w/ jaundice -However, key differences include: ---1) Other KEY SYMPTOMS would be present including: --------a) Poor growth --------b) Cataracts --------c) Hepatic dysfunction (Jaundice, Hepatomegaly, ascites) --------d) Intellectual disability --------e) Increased E.coli septicemia ---------------[pt does not have any of these symptoms]

A previously healthy 3-week old newborn presents by his mother b/c of his skin becoming progressively yellow over the past 6 days. She says that her son's stools have become lighter in color over the past 3 days. He was born at term after an uncomplicated pregnancy & vaginal delivery. He has been fed formula since birth. Exam shows jaundice. Labs shows - Total bilirubin----------14.0 - Direct------------------6.0 Which of the following is the most likely mechanism for this infant's condition? (Decreased excretion of bilirubin OR Hepatic enzyme deficiency OR Decreased conjugation of bilirubin)

Decreased hepatic excretion of bilirubin -Describes biliary atresia -> presentation includes: --1) Pt is normal at birth, presents at ~4-8 wks of life --2) Jaundice --3) Acholic stools --4) Darkening urine --5) +/- hepatosplenomegaly Diagnostic 1) Gold standard -> Intraoperative cholangiogram TMT 1) Hepatoportoenterostomsy (Kasaii procedure) Decreased conjugation of bilirubin -This describes Gilbert syndrome -Most pts are asymptomatic; will present later in life w/ increased unconjugated bilirubin during times of increased stress Defective enzyme deficiency -Describes Crigler-Najjar -> presents w/ severely increased unconjugated bilirubin -Key symptoms is neurological (Kernicterus)

A 5B, presents for a well-child exam. He is active & has been healthy except for an episode of otitis media 3 wks ago treated w/ amox. His parents emigrated from Vietnam. His diet consists mainly of fruits and veggies; he occasionally eats fish & chicken but no red meat. Exam shows no abnormalities. Labs show: - Hb-------------10 - MCV----------64 - Leuko's--------6100 - Plts------------225 - RBC DW------10% (N 13-15) A blood smear shows target cells. Which of the following is the most likely explanation for this pt's anemia? (Antibiotic induced autoimmune hemolysis OR Decreased synthesis of globin chains OR Transient post-infectious BM suppression)

Decreased synthesis of alpha-globin chains > pt has *thalassemia* which presents w/: 1- Hypochromic Microcytic anemia 2- Profound microcytosis *MCV < 75) 3- *Target cells* on PBS Antibiotic induced autoimmune hemolysis - PBS would show schistocytes Transient pot-infectious BM suppression > Everything would be decreased Platelets, & leuko's *pt's are normal* > pt is described as healthy and active

A 18-month-old F, presents to the ED 1 hour after the onset of vomiting & drooling. She had been left alone in the bathroom of her home for a few mins before the onset of the symptoms. The family keeps nasal decongestants, acetaminophen, ibuprofen, diphenhydramine, drain cleaner, & insecticide spray in the bathroom. During the past 5 days she has had an URTI treated w/ a nasal decongestant containing pseudoephedrine & acetaminophen. She is agitated & drooling. Temp is 98.6F, pulse is 120/min, RR is 26/min, BP is 80/60. Exam shows two small superficial ulcers on the lips & tongue. The remainder of the exam shows no abnormalities. The most likely cause of these findings is ingestion of which of the following? (Drain cleaner OR Organophosphate)

Drain cleaner - Signs of intox include: 1- *Drooling* 2- Burns *Ulcers* of mouth & throat 3- Severe abd pain 4- Throat swelling (diff breathing) 5- Chest pain 6- Collapse 7- Diarrhea 8- Loss of vision (if poison touched eyes) Organophosphate - Signs on intox include *DUMBELLS* & *SLUDGE* *S* → salivation *L* → lacrimation *U* → urination *D* → diarrhea *G* → GI upset *E* → emesis [pt is only agitated & drooling]

A 9-month-old B, presents 2 months after his parents noticed that he seemed to be ignoring them. On questioning, they note that he does not appear to be startled by loud noises. Growth & development have been otherwise normal. He was born at term to a 28F, G2P1. His mom is A-, and he is A+. He had a serum total bilirubin conc of 25mg/dL at the age of 2 days & received phototherapy for 2 days. He continued to be mildly jaundiced until age of 1 month. Serum studies since then have shown no abnormalities. Which of the following is the most likely to have prevented this pt's current condition? (Exchange transfusion OR Antibiotic therapy)

Exchange transfusion > PT presents w/ *hemolytic disease of the newborn* & *Hydrops fetalis or Kernicterus* *Hydrops fetalis* 1- Unconjugated hyperbilirubinemia postnatally >> *jaundice w/in the first 24 hrs of life* *Kernicterus* > may present w/: 1- Cerebral palsy 2- *Auditory neuropathy* 3- Gaze abnormalities 4- Dental enamel hypoplasia Antibiotic therapy > Not used in hydrops fetalis or kernicterus

A 2-month-old B, presents for a well-child exam. He was born at 39 wks following an uncomplicated pregnancy, labor, & delivery. He is at the 50th percentile for length & Wt & 75th percentile for head circumference. Temp is 99F, pulse is 130/min, RR is 45/min. Pulse OX on room air shows an O2 sat of 97% at the right thumb & 98% at the left thumb & right great toe. The apical impulse is prominent, & there is a thrill in the second left intercostal space. There is a continuous "machinery-like" murmur increasing in intensity during systole & waning during diastole; it is loudest in the 2nd left intercostal space & radiates down the left sternal border. Peripheral pulses are 4+ in all extremities. The remainder of the exam shows no abnormalities. Which of the following is the most likely explanation for these findings? (Extracardiac left-to-right shunting OR Intracardiac atrial right-to-left shunt)

Extracardiac left-to-right shunt - Pt has PDA - PDA presents w/ a "machine-like, continuous heart murmur - A PDA is a Patent ductus arteriosus (remnant of aorta & pulmonary structures) and thus it is Extracardiac (outside the heart) Intracardiac atrial left-to-right shunt - Correct direction but *intracardiac shunting is associated w/ conditions that cause cyanosis* - An example is a VSD or ASD

A 7-day-old presents to the ED 8 hrs after the onset of vomiting bilious material. He has passed one bloody stool during this period. He has refused to feed for 12 hrs. He was born at 38 wks following an uncomplicated pregnancy & delivery. Apgar scores were 8 & 9 at 1 & 5 mins, respectively. He was initially well except for occasional wet burps after breast-feeding. He appears lethargic. He weighs 7-lbs 3-oz. Temp is 100F, Pulse is 170/min, RR is 28/min, BP is 65/35. Exam shows mottled skin & a supple neck. The lungs are clear to auscultation. Abd exam shows mild distention & diffuse tenderness w/ guarding. Bowel sounds are decreased. On DRE, there is gross blood. Which of the following events in utero is the most likely cause of the findings? (Failure of cannulization of the proximal bowel OR Failure of the normal bowel rotation)

Failure of bowel rotation - Pt presents w/ malrotation - Presentation includes: --1) Most commonly presents in first few days of life w/: --2) Abd distention w/ acute onset of bilious vomiting --3) Constipation --4) *Hematochezia* (10-15% → suggests bowel ischemia) [This is the key difference between malrotation & duodenal atresia] XR findings - Coffee bean sign (sigmoid) Failure of the cannulization of the proximal bowel - This is *duodenal atresia* → Presentation also includes bile stained vomiting includes & abd distension --1) Presents in the first few days of life & usually following first feed -----(Pt has had a feed and only had "wet burps") --2) (Not associated w/ hematochezia) -----(pt has hematochezia) -Associated w/: ---1- Trisomy 21, ---2- Congenital rubella ---3- Prader-Willi syndrome ----(pt has no signs of these syndromes) 1- XR → would show double bubble sign *dilated proximal duodenum*

What is acute otitis media with effusion?

Fluid in the middle ear space w/out infection → distinguished by: 1- Absence of inflammation (AOM w/out effusion → otalgia, otorrhea, systemic symptoms) 2- Retracted or neutral tympanic membrane (AOM w/out effusion bulging tympanic membrane)

A 6-week-old F, presents b/c of a 2-week Hx of persistent irritability & passing stools streaked w/ mucus & blood. She has not had vomiting. Two weeks ago, she was switched from a cow's milk-based formula to a soy milk-based formula. She is at the 25th percentile for length, Wt, & head circumference. Her Wt remains unchanged from 2 weeks ago. Exam shows no other abnormalities. The most appropriate next step in MGMT is to begin which of the following? (Formula w/ evaporated milk, water, & corn syrup OR Formula w/ hydrolyzed casein)

Formula w/ hydrolyzed casein - Hydrolyzed casein breaks the protein molecules in to smaller molecules that are more easily absorbed → they are a good alternative in pts w/ milk allergies Formula with evaporated milk, water, & corn syrup - Above would the best answer for pt wi possible milk allergies

A 9-month-old B, has pneumonia for the 4th time. He was born at 28 wks w/ a grade 4 intraventricular hemorrhage & generalized neurologic devastation. Barium swallow shows a grade 4 gastroesophageal reflux. Which of the following operative procedures is most likely to decrease the frequency of recurrent pneumonia? (Esophagostomy OR Fundoplication OR Vagatomy & pyloroplasty)

Fundoplication - pt presents w/ GERD associated recurrent pneumonia -A) MGMT -----a) Fundoplication ----------a) PREFERRED TMT for GERD associated recurrent pneumonia Esophagostomy - reserved for extremely ill pts that provides long-term tubal alimentation for chronically ill or debilitated pts Vagatomy & pyloroplasty - used as TMT for diseased (narrowed or thickened) pylorus - Destroys the pyloric sphincter & drains stomach into duodenum

A 6F, in remission of ALL has had pain in her right shoulder for 2 days. She is receiving maintenance chemo & TMP-SMX prophylaxis against P.jiroveci pneumonia. She has a 4x1-cm erythematous patch beneath the right scapula w/ 6-8 superimposed, small, clear fluid-filled vesicles. Which of the following is the most likely explanation for this rash? (HSV OR Herpes zoster)

Herpes Zoster (VZV) > pt has shingles that present b/c of the immunodeficiency from chemo >> rash presents as *Erythematous papules that eventually evolve into vesicular lesions* & * macules and papules w/ central vesicles* HSV > Would present as genital infection or on lips but not on shoulder

A 6-day-old girl presents b/c of vomiting for 2 days. She has not had any fever. She was born at term following an uncomplicated pregnancy, labor, & delivery. Results of the newborn screening are pending. She was discharged at the age of 1 day. She is exclusively breast fed & is feeding every 3 hrs. She is at the 50th percentile length & head circumference & 5th percentile for wt. Exam shows generalized jaundice. The liver is enlarged & firm. Total serum bilirubin is conc is 14mg/dL, w/ a direct component of 8mg/dL. Test of the urine for reducing substances is positive. Which of the following Dx is most likely? (Galactosemia OR Type I glycogen storage disease)

Galactosemia > Pt would present w/: 1- Poor growth *failure to thrive* 2- *Vomiting* 3- Hepatic dysfunction (*Jaundice*, *hepatomegaly*, ascites) 4- Cataracts 5- Intellectual disability Type I (Von Gierke) > presents w/: 1- Severe fasting hypoglycemia ----Pt shows no signs of hypoglycemia 2- Elevated blood lactate 3- Elevated triglycerides ----Would cause hyperlipidemia manifesting as xanthomas, ----Would see a ↑VLDL on Labs 4- Elevated uric acid ----Would cause gout 5- Hepatomegaly

A previously healthy 3F, presents b/c of leg pain during the past 3 months. The pain is predominantly in her calves; it only occurs at night & often awakens her from sleep. The pain is exacerbated when she has been particularly active during the day & resolves w/ acetaminophen use & massage. She appears well. Temp is 99F, pulse is 108/min, RR is 36/min, BP is 88/50. Exam shows no abnormalities. What is the most likely Dx? (Growing pains OR Patellofemoral compression)

Growing pains - Neurological pains *usually in lower limbs* → *Usually develop in the night* → which occur in the Limbs of some young children *ages 3-12* thought to the caused by rapid growing & stretching of body, nn's, muscles, & bones - Pain can be intense/ cramp-like & in both legs → Most often effects the shins, *calves*, or ankles but can affect thighs Patellofemoral compression - AKA *Runner's knee* → characterized by *anterior knee pain* ranging from mild to severe discomfort originating from contact of the posterior surface of the patella w/ femur bone

A previously healthy 17B, presents b/c of fever & enlarged LN for 3 wks. He has had a 20-lbs wt loss over the past 2 month. He has had 6-8 loose stools daily for 4 weeks. Temp is 102.2F, pulse is 80/min, RR are 30/min. Exam shows dry skin. There are bilateral 2-cm LN's in the neck, axillae, & groin. The lungs are clear to auscultation. The liver edge is palpated 1cm below the right coastal margin; the spleen tip is palpated 7-cm below the left coastal margin. Labs show: - Hb-------------10 - Neutro's-------80% - Lymphs-------18% - Mono's--------2% - IgA-----------340 - IgG----------2300 - IgM-----------390 Which of the following is the most likely Dx? (HIV OR SCID)

HIV >pt presents w/ acute HIV infection which presents w/: 1- Myalgias 2- *Fever* 3- Fatigue 4- Mucocutaneous ulceration (anus, penis, oral mucosa, esophagus) 5- Generalized rash following fever 6- *Nontender axillary, cervical, occipital lymphadenopathy* 7- *Nausea, diarrhea, wt loss* 8- *hepatosplenomegaly* SCID > Usually presents in younger child w/: 1- Failure to thrive 2- Chronic diarrhea 3- Thrush 4- Recurrent viral, bacterial, fungal, & protozoal infections *No lymphadenopathy* *No hepatosplenomegaly* *No diarrhea w/ wt loss* Labs - Low T-cells - No germinal centers - Absent thymic shadow (CXR)

A 6B, has progressive AML despite numerous therapeutic interventions. During a meeting w/ his parents to discuss the prognosis & the futility of further TMT, his parents request that the pt not be told his prognosis. During an exam when his parents are not present, the pt asks if he is going to die soon. Which of the following is the most appropriate response? (Have you talked to your parents about this? OR Most children w/ your condition live a long time)

Have you talked to your parents about this

An 18-hour-old female is being evaluated b/c of jaundice. She was born at term following an uncomplicated pregnancy & delivery to a 31F, G2P1; she weighed 8-lbs 6-oz at birth. The mom's blood group is O+. The newborn has urinated twice but has had no bowel mvmts. Her urine is dark yellow. She has had difficulty latching onto the breast correctly. She is alert. Exam shows jaundice of the face & chest. The abd is soft. The liver edge is palpable 1cm below the right coastal margin, & the spleen tip is palpated 1-cm below the left costal margin. Labs show: - Total bili-------------11.1 - Direct---------------0.1 Which of the following is the most likely Dx? (Hemolytic disease of the newborn OR Physiological jaundice of the newborn)

Hemolytic disease of newborn (AKA hydrops fetalis) - Caused by: 1- Rh incompatibility 2- ABO incompatibility *most common* 3- other blood system antigens -Presentation- 1- Jaundice w/in the first 24 hours of life 2- Hepatosplenomegaly 3- Hydrops fetalis ----fluid in two or more compartments (i.e., Ascites/polyhydraminos) 4- Kernicterus (complication) 5- Associated tests ---Postive Direct Coombs test Diagnostic techniques - Middle cerebral artery velocimetry TMT 1- Resuscitation 2- Transfusion 3- Phototherapy Physiological jaundice 1- Usually presents in first 3-5 days of life & NEVER before 24 hours ---(Pt is 18 hours old - this eliminates physiological jaundice) -Pathology-Decreased enteral intake & increased enterohepatic circulation of bilirubin → *no associated increase in bilirubin production*

What are the histological findings in Graves?

Histology 1- Diffuse hypertrophy & hyperplasia of thyroid follicular cells → *Abundant tall columnar cells lining the follicles* 2- Colloid appears pale w/ scalloping *moth-eaten* margins 3- *Lymphocytic infiltrate* leads to germinal centers in the thyroid (Germinal centers should be in LN's not thyroid)

A 16F, presents b/c of a 2-month Hx of a mass on the right side of the neck, progressive axillary & inguinal lymphadenopathy, & generalized pruritus. She has had a 15-lbs Wt loss during this period. Exam shows no other abnormalities. A CXR shows a mediastinal mass. Labs shows anemia w/ a normal leukocyte ct & differential, increased ESR, & increased serum ferritin conc. A node biopsy is likely to show which of the following? (Hodgkin's OR Thymoma)

Hodgkin - Bi-modal age distribution (<20 & > 65) - May present w/: 1- Most common → *Lymphadenopathy of the neck, axilla, inguinal* 2 - Fever 2- *Wt loss* 3- Night sweats 4- *Persistent itching* Labs 1- Anemia 2- ↑ ferritin (d/t increase in iron stores to fend off infection) 3- ↑ ESR Thymoma - Typically presents at age 30-40 - Associated w/ Myasthenia gravis - Other symptoms caused by compression of surrounding organs, such as: 1- SVC syndrome 2- Dysphagia 3- Cough 4- Chest pain

One day after undergoing appendectomy for perforated appendicitis, a 16F, w/ DM I has an ileus. During the operation, she found to have diffuse peritonitis & received 3.5L of lactated Ringer sol'n. Her initial post-op course was uncomplicated. Current meds include cefepime, morphine, & insulin. Temp is 100.4F, pulse is 100/min, RR is 18/min, BP is 110/70. Abd exam shows moderate distention & diffuse tenderness. The surgical incisions are clean & dry. Labs show: - Hb-------------------11 - Hct-------------------32% - Leuko's---------------23,4K - Plts-------------------357K - Na+-------------------128 - K+---------------------3.9 - Cl---------------------96 - HCO3----------------22 - BUN------------------14 - Glucose--------------642 - Creatinine------------0.9 Which of the following is the most likely cause of this pt's serum sodium conc? (Excessive admin of free water during the operation OR Hyperglycemia)

Hyperglycemia - Hyperglycemia caused water to move out of cells leading to subsequent reduction in serum sodium level *dilutional hyponatremia* Excess admin of free water during the operation - Pt would not present so quickly with this - Her glucose levels are extremely high making hyperglycemia more likely - The Pateint was given lactated ringers solution which is isotonic ----(not free water)

Six days after undergoing an operation for a truncus arteriosus defect, an 8-day-old newborn appears jittery. Temp is 98.9F, Pulse is 130/min, RR is 44/min, & BP is 70/58. Exam shows micrognathia & hypertelorism. His serum Ca2+ conc is 7mg/dL. An CXR shows cardiomegaly w/out a thymic shadow. Which of the following is the most likely Dx? (HypoPTH OR PseudohypoPTH)

HypoPTH - Pt has an absent thymic shadow -> this implies he has no parathyroid gland -> which means he most likely has a PRIMARY low PTH Presentation includes: - Usually as a result of complication of thyroidectomy - DiGeorge's is also a common cause *familial hypocalcemia* - Signs from Hypocalcemia PseudohypoPTH -Pt would present w/ a normal thymic shadow - Group of disorders resulting from *end-organ resistance to PTH* → caused by defects in G-protein second messenger system - Classic variant is *Albright's hereditary osteodystrophy* → which presents w/: 1- ID 2- Short 4th & 5th metacarpals (knuckle-knuckle-dimple-dimple sign) 3- short stature Labs 1- Hypocalcemia 2- Hyperphosphatemia 3- ≥ PTH → Key diagnostic feature *Admin of PTH will not effect Ca2+ levels*

A 9F, w/ DM I presents to the ED b/c of lethargy, labored breathing, & seven episodes of vomiting over the past 6 hours. Her skin is flushed & warm & appears severely dehydrated. There is a fruity odor to her breath. She is at the 75th percentile for Ht & 25th percentile for Wt. Temp is 99F, pulse is 110/min, RR is 24/min, BP is 100/60. Capillary refill time is 4 sec & capillary BG conc is > 500mg/dL. Which of the following is the most appropriate initial step in mgmt? (IV 20mL/kg of 0.9% saline OR IV 20mL of lactated ringer solution w/ 40 mEg/L of KCl

IV 20mL/kg of 0.9% saline - An IV bolus is the first initial step in treating *DKA* - Lactated ringer solution would be correct on its own, but it also has KCl which is not given in an IV bolus

A previously healthy 9-month-old B, presents b/c of temps to 104F, fussiness, & decreased oral intake over the past 5 days. No close contacts have been ill. Temp is 103.3F. Exam shows cracked fissured lips, redness of the oral mucosa & conjunctivae, & a single enlarged cervical LN. There is a maculopapular rash over the trunk & extremities & dorsal edema of the hands. Labs show: - Hct----------------32% - Leuko's-----------15K - Plts---------------550K What is the next best step in MGMT? (Acetaminophen OR IVIG)

IVIG - Pt presents w/ *Kawasaki* → which presents w/*CRASH* & *Burn*: *C* → Conjunctivitis (bilateral, non-exudative) *R* → Rash (polymorphic & usually on the trunk but can involve *palms & soles* *A* → Adenopathy (cervical lymphadenopathy that is most often *unilateral*) *S* → Strawberry tongue [or other mucocutaneous findings (erythema of oral cavity, *dry, cracked lips*)] *H* → *Hand & foot edema* or erythema often w/ desquamation of the finger/toes *Burn* → Fever ≥ 101.4 lasting ≥ 5 days *STEAM* *S* → Sterile pyuria *T* → *Thrombocytosis* *E* → ↑ESR & ↑CRP *A & M* → Aseptic meningitis TMT 1- *IVIG* → mainstay; reduce risk of coronary aneurysms 2- Aspirin → reduce risk of coronary thrombosis 3- TNF-α (second line) 4- Glucocorticoids (2nd line)

A 2-hr-old infant was born to a mom w/ poorly controlled DM I. He is at the 70th percentile for length & 90th percentile for wt. Exam shows plethora. Which of the following is the most likely primary mechanism of the blood glucose concentration? (Decreased insulin secretion OR Increased insulin secretion)

Increased insulin secretion - Mom Has poorly controlled DM I so Hyperglycemia would expected - B/c of the hyperglycemia, the baby will have Increased secretion of insulin w/ accompanying low glucose levels (after birth) Decreased insulin secretion - The pathophysiological response to hyperglycemia is INCREASED insulin secretion - Decreased insulin secretion Would be seen in certain conditions only, such as: ----1) Iatrogenic (too rapid infusions of dextrose) ----2) Physiologic stress (sepsis, hypoxia, RDS, surgery)

A 4B, is hospitalized b/c of fever, shallow breathing, & unresponsiveness for 3 hrs. Temp is 104F, Pulse is 160/min, RR 60/min, BP is 60/35. Exam shows purpura over the LE. He is intubated & mechanically ventilated. Blood cultures grow nesseria meningitidis. two days later, a CXR shows bilateral patchy infiltrates. Which of the following is the most likely mechanism for these findings? (Enzymatic deficiency OR Increased vascular permeability)

Increased vascular permeability > Pt presents most likely w/ Sepsis → pathophysiology is described as above //Enzymatic decificiency// > Pt would have to have a disease where he can not fight off encapsulated organisms → would be a chronic type setting (the vignette clearly describes an *acute picture* → more likely toward sepsis)

A 14B, presents b/c of a 3-month Hx of dragging sensation of his left scrotum. He was born at 30 wks & had a left scrotal hydrocele during infancy that resolved spontaneously. Both testes are well descended. When the pt stands up. the left scrotum hangs lower than the right, & there is a soft tissue mass near the top of the left testis that feels like a bag of worms. In the supine position, the mass mostly disappears. If untreated, this pt is at increased risk for which of the following? (Infertility OR Testicular torsion)

Infertility - Pt presents w/ *varicocele* → which presents w/: 1- Dilation of the pampiniform plexus of veins *bag of worms* that surrounds the spermatic cord 2- Scrotal swelling 3- Aching scrotal pain 4- Sense of fullness in the scrotum 5- Testicular atrophy 6- *Symptoms exacerbated by standing & relieved by lying down* 7- Does not transilluminate 8- Increased size w/ increased intra-abd pressure Complication *Infertility* → d/t testicular atrophy & hypogonadism secondary to increased scrotal temp Testicular torsion - usually d/t inadequate anchoring of the testis to the posterior scrotal wall d/t congenital defects of the processus vaginalis → which allows the testis to hang freely - Usually presents in puberty *12-18 yrs* following *intense exercise*

A newborn is examined after 12 hrs after birth for excessive oral secretions & coughing following the first feeding. The symptoms resolve after suctioning. Polyhydraminos was Dx'd by US at 35 wks. The pregnancy was otherwise uncomplicated. He was delivered at term w/ no complications. Apgar scores were 7 & 9 at 1 & 5 mins. He weighs 6 lbs 10 oz. Exam shows no abnormalities. Which of the following is the most appropriate next step in MGMT? (US of abdomen OR Insertion of nasogastric tube)

Insertion of nasogastric tube - Pt is having trouble breathing → *must secure airway*

What is the TMT for a pt that has a Positive PPD (anyone >15mm induration) test but is asymptomatic? (Isoniazid OR Rifampin)

Isoniazid (INH) - This pt is positive for latent Tb -> MGMT includes ---1) Prophylaxis TMT is self administered isoniazid for 9 months OR ---2) INH & rifapentine for 3 months administered by direct observation

A previously healthy 6F, presents by her parents b/c of intermittent knee pain for 2 months. The pain typically occurs in the morning & after naps. She has not had fever, rash, or symptoms in any other knee joint. She plays soccer twice weekly but has not had any injuries. Her parents recall that she had a sore throat for 2 days prior to the onset of knee pain. There is no FHx of osteoarthritis. She is at the 25th percentile for Ht, and 70th percentile for wt, & above the 97th percentile for BMI. Temp is 98.6F. Exams shows slight bowing of the LE. The right knee is larger than the left knee. There is tenderness of the right knee to palpation. ROM of the right knee is decreased. Her ESR is 45mm/h. X-ray of the knees show no abnormalities. Which of the following is the most likely Dx? (Juvenile arthritis OR Tibia vara)

Juvenile arthritis > Most common rheumatic disease in children >> Subtypes include: 1- Oligoarthritis *≤ 4 joints* 2- Polyarthritis *≥ 5 joints* 3- Systemic *arthritis w/ rash & fever* >> Affects *girls > boys* Symptoms include: 1- Joint pain & swelling 2- *Joint stiffness in the morning or after a period of immobility* 3- *Fatigue in early afternoon* Labs show: 1- CBC: ↑ WBC, ↑ platelets 2- Microcytic anemia (d/t inflammation) 3- *↑ESR & ↑CRP* (d/t inflammation) 4- RF presents (polyarticular) 5- ANA (oligoarticular) Tibia Vara (Blount's disease) > Characterized by varus deformity of the upper epiphysis & metaphysis >> child would have rickets (or any other ailment that prevents ossification of bones) *Not associated w/ any other symptoms presented by the pt*

A previously healthy 6-month-old B, presents to the ED b/c of difficulty breathing for 2 hours. He also has had a low-grade fever, nasal congestion, & a runny nose for 2 days. He is in mild resp distress. Temps is 101F, pulse is 140/min, RR is 48/min. Generalized harsh breath sounds & inspiratory stridor are heard in auscultation; inspiratory stridor can be heard w/out the use of the stethoscope. Which of the following is the most likely site of pathology in this pt? (Bronchi OR Bronchioles OR Larynx)

Larynx - Pt presents w/ croup → *Larynx* is the most common site of infection in croup (also called Laryngotracheobronchitis) - Most often Caused by Parainfluenza virus - Presents in kids *6 months - 3 yrs* w/: 1- *1-3 days* Viral prodromal symptoms 2- Hoarseness 3- Barking cough 4- *stridor* (usually exacerbated at night & w/ agitation) Pathophysio - Larynx, bronchi, & trachea may all be affected - However, the larynx is most common site Bronchioles - this is *Bronchiolitis* - Presents in kinds *<2* & B>G - signs include: 1- URTI symptoms (Rhinorrhea, nasal congestion, cough, fever) -Gradual progression to LRTI, which presents w/: 1- Tachypnea 2- Fine rales 3- Wheezing 4- Hepatomegaly 5- Hyper-inflated lungs ---(Pt has inspiratory stridor as main symptom)

A 5B, presents b/c of a painful limp for 3 wks. He has no Hx of serious illness or trauma & has not had any other symptoms. Developmental milestones are appropriate for age. On exam, he is unable to bear his full wt on the right & winces when he is asked to stand on on his right foot. Flexion & internal rotation of the right hip are decreased. Muscle strength is 4/5 on abduction of the right hip. AP XR of the pelvis show a dense, contracted right femoral capital epiphysis. The left femoral capital epiphysis appears normal. Which of the following is the most likely Dx? (Legg Calve Perthes disease OR Slipped capital epiphysis OR Osgood Schlatter disease

Legg Calve Perthes - Idiopathic avascular necrosis of the proximal femur epiphysis - Most often in kids *ages 4-8, M>F* - Associated w/ ADHD -PE findings- 1- Hip stiffness w/ *loss of internal rotation & abduction* 2- Gait disturbance 3- Limb length discrepancy (late finding) -XR findings- 1- Medial joint space widening (early) 2- Small femoral head d/t irregularity of the femoral head ossification center (dense contracted) 3- Subchondral fracture Slipped capital epiphysis - Usually present in obese pts → Presents w/: 1- Thigh & groin pain (but sometimes knee pain) XR findings - Epiphysis remaining w/in the acetabulum while the neck displaces anteriorly & externally rotates leaving the head posterior to the neck - Growth plate may be widened or show increased lucency -[Would not show irregular calcification (dense contraction)] Osgood schlatter - (AKA osteochondritis of the of the tibial tubercle) → Caused by overuse & associated w/ sports → presents w/: 1- Adolescents ages 9-14 who have undergone a growth spurt 2- Anterior *knee pain* that gradually increases -TMT- Conservative - ICE - Analgesics/ NSAID - PT

What chronic condition should be excluded in HIV pts that present w/ AOM w/ effusion?

Lymphoma in HIV pts b/c they are more susceptible to regional lymphoid tumors in this region

A 10-day-old male newborn is admitted for TMT of indirect hyperbilirubinemia d/t isoimmune hemolysis. He is placed in a room w/ a 2-month-old female infant w/ dehydration secondary to rotavirus gastro. Two days later, he develops vomiting & diarrhea, & a stool specimen is positive for rotavirus antigen. This nosocomial infection is most Likely caused by failure of the caregivers to do which of the following? (Observe gown & glove precautions OR Observe hand washing techniques)

Observe hand washing techniques > Rota (Reo) is transmitted "Right-Out-The-Anus" *fecal-oral*

A 5B presents b/c of a 4-day Hx of increasing difficulty breathing. Temp is 99.5F, Pulse is 116/min, RR is 36/min, BP is 116/80. Exam shows JVD & mild swelling of the face & upper extremities. There are moderate intercostal retractions. Inspiratory & Expiratory wheezes are heard bilaterally. A CXR shows a mass in the superior mediastinum & large pleural effusion in the right hemothorax. Thoracentesis yields 400mL of reddish brown serosanguineous fluid containing numerous RBC's. Which of the following is the most likely cause of the pleural effusion? (Malignant pleural effusion OR TB pleural effusion OR Traumatic pleural effusion)

Malignant pleural effusion - Common causes of malignant pleural effusion are BCC & *Lymphoma* - Thoracentesis reveals reddish brown serosanguineous fluid w/ RBC's - This would be *exudative fluid* b/c ---It is cloudy (not clear) ---Fluid protein:Serum protein is >0.5 (<.5 in transudate) - Exudative fluid caused by: 1- Trauma 2- Malignancy 3- Infection (TB, pneumonia) Traumatic hemothorax - Pt has no Hx of trauma TB pleural effusion - Pt would have to have Hx of TB (not seen in this pt)

A previously healthy 7B, presents b/c of progressively worsening headaches & difficulty walking over the past 6 wks. Exam shows bilateral papilledema, right abducens palsy, & gait ataxia. Which is the most likely Dx? (Dandy-walker syndrome OR Pinealoma OR Medulloblastoma)

Medulloblastoma > Most common malignant tumor in children (~20%) > frequently cerebellar → compress the 4th ventricle → non-communicating hydrocephalus > Would have PNET tumor markers [[Presentation; secondary to increased cranial pressure]] 1- *Gait ataxia* (effects 4th ventricle which if near vermis) 2- *Abducens palsy* (CN VI) 3- *Bilateral papilledema*, diplopia 4- Listless + repeated vomiting 5- *Headache* Dandy walker > agenesis of the cerebellar vermis w/ cystic enlargement of the 4th ventricle >> Associated w/ hydrocephalus & spinal bifida >> would present w/ 1- Hydrocephalus 2- Headaches 3- Visual changes 4- Seizures 5- Confusion Pinealoma > Leads to parinaud's syndrome b/c compresses the superior colliculus causing: 1- Vertical gaze palsy 2- Eyelid retraction (Collier's sign) 3- Convergence-Retraction nystagmus

A previously healthy 10B, presents to the ED b/c of a 2-hrs Hx of increasing headaches, dizziness, & nausea. His father is a butcher and says his son ate a new batch of sausage shortly before becoming ill. Earlier today, the pt was given the task of cleaning out the garage. On arrival, the pt is alert & is not in respiratory distress. Pulse is 136/min, RR are 20/min, BP is 104/70. Pulse OX on room air shows an O2 situation of 73% that increases to 80% when 15L/min of O2 is administered via face mask. Exam shows profound central cyanosis. The pupils are equal & reactive to light. Cardiopulmonary exam shows no abnormalities. His methemoglobin is 40.5% of his total hemoglobin. ABG analysis shows: - pH----------------7.37 - PCO2------------42 - PO2--------------48 - HCO3------------24 Which of the following is the most appropriate next step in MGMT? (Methylene blue OR Hyperbaric oxygen chamber)

Methylene Blue - Pt most likely presents w/ Methemoglobinemia -A) Signs/Symptoms -----1) CNS dysfunction -----------(*Headache*, fatigue, coma, *dizziness*, *nausea*) -----2) Cyanosis -----3) Chocolate-colored blood -B) Labs -----1) elevated Methemoglobin levels -C) Etiology -----1) Anesthetics ----------(benzocaine) -----2) Antibiotics ----------(dapsone, chloroquine, sulfonamides) -----3) Benzenes -----4) Nitrites ----------(used as additives to prevent meat from spoiling) -D) MGMT -----a) Dx tests TMT ---------1) Methylene blue → PREFERRED ---------2) Vit C --------------(slower action, 2nd line) Hyperbaric O2 chamber - This is a TMT for Carbon monoxide poisoning - Also Presents w/ headache, cyanosis, altered mental status - However, key differences include ----1) Lab findings --------1) Would show INCREASED CARBOXYHEMOGLOBIN levels -------------[WOULD NOT show ELEVATED/ANY METHEMOGLOBIN levels] ----2) CO poisoning presents w/ other key symptoms including --------a) Cherry-red skin color

A 14B, presents b/c of 1-year Hx of learning disability & hyperactivity. His teacher reports that he has a short attention span. His mother says that he cannot concentrate on his homework. He had a generalized tonic-clonic seizure at 8 yrs. Exam shows approximately 9 lesions w/ a coffee-stain-like appearance on the chest and Abd. There are small areas of increased pigmentation in the axillae & many small skin tags over the chest, abdomen, & back. Which of the following is the most likely Dx? (NF-1 OR Tuberous sclerosis)

NF-1 - NF1 gene Ch.17q11 → presents w/*must have at least 2 to Dx*: 1- *Axillary/inguinal* Freckling 2- Cafe-au-lait spots *coffee stain appearance* 3- Lisch nodules *eye* 4- *optic glioma* 5- Neurofibromas 6- Bone abnormalities 7- Kyphoscoliosis & long bone dysplasia 8- Gait abnormalities 9- *seizures* (cognitive impairment) w/ increased brain volume Tuberous sclerosis - Most often presents in *infancy* → w/: 1- Infantile spasms *epilepsy* 2- *Autism* 3- Neonatal *cardiac rhabdomyoma* 4- Ash-leaf spots (hypopigmented on skin) 5- Shagreen patches (rough patches w/ orange peel consistency) 6- Angiofibromas (Reddish-brown papules) 7- Seizures 8- Intellectual disability 9- Retinal lesions Mulberry tumors/ phakomas (round, flat, gray lesions located in periphery of retina) 10- Angiolipomas of kidney 11- Subependymal astrocyte proliferation in brain (Candle guttering) 12- Hamartomas

An otherwise healthy 16F, girl is brought to the physician by her mom b/c of a 6-month Hx of increasingly severe pain w/ menses. Her mom reports that during this time, her daughter has missed 2 days of school during each menstrual period b/c of severe cramps associated w/ vomiting. Menses occur at regular 28-day intervals & last 4-5 days. Her last menstrual period was 7 days ago. She currently takes acetaminophen, which provides minimal relief of her symptoms. She has had three lifetime sexual partners, & they use condoms consistently. The pt is an honor roll student despite frequent absences from school. PE shows no abnormalities. PE shows a tender, normal-sized uterus & no masses. Which of the following is the most appropriate next step in MGMT? (Pregnancy test OR NSAID OR SSRI)

NSAID > Pt has signs of *primary dysmenorrhea* which presents w/: 1- Incapactiation for 1-3 days 2- Usually presents in women <20 (but decreases from 20-30) 3- Pain usually begins on first 1-2 days of Menses (endometriosis 1-2 days *before* menses) TMT 1- *NSAIDs* 2- Hormonal OCP (if no improvement w/ NSAIDs) SSRI > Would be correct if pt had PMS or PMDD (Premenstrual dysphoric disorder) >> symptoms begin *1-2 wks before menses & resolve a few days after* >> symptoms include: 1- Irritability 2- Mood swings 3- Anxiety 4- Depressed mood 5- Increased appetite/ food cravings 6- Diminished interest in activities Pregnancy test > Not correct b/c 1- She uses condoms consistently 2- Last period was 7 days ago 3- Symptoms started 6 months ago

A 12B, has recently begun to snore loudly. He has a Hx of chronic URTI's & steatorrhea. Which of the following most likely explains his symptoms? (Tonsillar hypertrophy OR Nasal polyps)

Nasal polyps - Pt presents w/ *CF* → *Nasal polyps are associated with CF in children* Tonsillar hypertrophy - Leading cause of obstructive sleep apnea on kids *ages 3-6* (pt is 12)

A 2G, presents b/c of a 2-wk Hx of irritability, poor appetite, occasional cough, & reluctance to walk. She has had a 4.4 lbs Wt loss since her last exam 6 months ago. There is no recurrent respiratory illness, constipation, vomiting, or diarrhea. She appears comfortable & quiet. She is at the 75th percentile for Ht & 25th percentile for Wt. Temp is 100.2F, pulse is 140/min, RR is 24/min, BP is 145/100. Exam shows bluish discoloration under both eyelids. Cardiac & abd exam show no abnormalities. Labs show: - Hb-----------------10.5 - Leuko's-------------8,3K - Neutro's-----------40% - Eosino's------------2% - Lympho's-----------55% - Mono's-------------3% - Plts-----------------240K A lateral CXR shows a mass in the posterior mediastinum. Which of the following is the most likely Dx? (Neuroblastoma OR Thymoma)

Neuroblastoma - presents w/: 1- Asymptomatic 2- *Abd fullness or distention* 3- Fatigue 4- Bone pain (METS) 5- Changes in bowel/bladder habits 6- Wt loss/*Failure to thrive* 7- Abdomen → *firm irregular mass hat crosses the midline* 8- Opsoclonus myoclonus syndrome (paraneoplastic) characterized by: --*ataxia* --horizontal & vertical saccades (opsoclonus* --Rhythmic jerking (myoclonus) Thymoma - Pt would present w/ myasthenia gravis symptoms

An 11G, presents for a well-child exam. Her mother is concerned about her daughter's early & rapid onset of sexual development. She says that her daughter began breast development at the age of 9, initial pubic hair growth at the age of 9.5, & menarche 2 days ago. Sexual development is Tanner stage 4. The remainder of the exam shows no abnormalities. Which of the following is the most likely Dx? (Idiopathic precocious puberty OR True precocious puberty OR Normal development)

Normal development - Puberty development is normal as long as patient had no development before age 8 ----(in boys it is before age 9) True precocious puberty - True precocious Puberty before age 8 in females and 9 in males Idiopathic - AKA GnRH-dependent precocious puberty -> Most commonly caused by early maturation of the HPA axis SIDE NOTE ---GnRH-independent precocious puberty -> caused by excess secretion of hormones (usually d/t tumor/cysts) ---Incomplete precocious puberty -> Only one presenting sign (premature thelarche or premature adrenarche)

A 5B, presents to the ED b/c of leg pain for 2 days & an associated limp for the past 4 hours. 1 weeks ago, he had watery stools & temp to 100F.; these symptoms resolved w/in 2 days. There is no Hx of trauma or recent immunizations. He is otherwise healthy, & his immunizations are up to date. He appears alert. Temp is 96.8F, pulse is 90/min, RR is 18/min, BP is 110/75. The left hip is not swollen or erythematous, but there is pain w/ mvmt & Wt bearing. His ESR is 28. US of the hip shows a small effusion. Which of the following is the best TMT? (IV vancomycin OR IV ceftriaxone OR Ibuprofen)

Oral Ibuprofen - Pt presents w/ *Juvenile idiopathic arthritis* → presents w/: 1- Limp & swelling 2- Systemic *Flu like* symptoms (rash, *fever*, diarrhea) 3- ↑ ESR, ↑ CRP 4- US findings → *soft tissue swelling* small effusion IV ceftriaxone - Would be correct Dx was *septic arthritis* →would present w/: 1- *knee pain* (most common affected joint 2- Fever 3- Pain during passive ROM Associated labs 1- ↑ Neutro's (50K) 2- WBC >30K 3- ESR >30 4- CRP >5 IV vancomycin - May be used in Septic arthritis resistant to 3rd gen cephalosporin

What is bronchiectasis? What are the RF's for bronchiectasis?

Permanent dilation of bronchi caused by recurrent cycles of infection/inflammation leading to fibrosis & remodeling RF's include - CF *50% of cases) - COPD - Airway hypersensitivity diseases - Primary ciliary dyskinesia - Immunodeficiency - Autoimmune disease - Localized airway Obstruction - Recurrent pulmonary infections or recurrent aspiration

A 16B, presents 2 months after discovering a painless lump in his right breast. He is otherwise healthy. Sexual development is Tanner stage 4. Exam shows a 1-cm, smooth, firm, mass under the right nipple; there is not nipple or skin retraction & no axillary lymphadenopathy. Which of the following is the most likely Dx? (Accessory breast tissue OR Physiologic pubertal development)

Physiologic pubertal development - *Small lump w/ tenderness beneath the nipple* is normal in puberty → about 50% of all boys develop gynecomastia during puberty - Can be unilateral or bilateral Accessory breast tissue - Presents anywhere along the embryologic mammary streak (axilla to inguinal

A 6-hr-old female newborn has had cyanosis of her arms & legs since birth. She was born at 38 wks following an uncomplicated pregnancy & CS delivery for breech presentation. She weighed 6 lbs 13 oz at birth. She has been breast-feeding well. She is alert & is not in acute distress. Her rectal temp is 97.2F, Pulse is 112/min, RR 36/min, BP is 80/45 Pulse Ox on room air shows on O2 sat of 96%. Exam shows cyanosis of the UE & LE but no circumoral cyanosis. Cardiopulmonary exam shows no abnormalities. Capillary refill time is mildly delayed. Which of the following is the most appropriate next step in MGMT? (Placement of the newborn under warming lights OR CXR)

Placement of newborn under warming lights > Pt has *peripheral cyanosis only* w/ Low temp → she has no cardiac defects → so must be from cold exposure CXR > no cardiac abnormalities are seen in PE

A 15B, presents to the ED b/c of a 2-day Hx of fever & increasingly severe headache & malaise. He has a Hx of recurrent pneumonia; the last episode was 2 years ago. His family recently immigrated to the US from Mexico. On arrival, he is somnolent but arousable. Temp is 102.8F, Pulse is 120/min, RR are 34/min, & BP is 146/94. Exam shows nuchal rigidity. Crackles are heard over the left lower lung field. On MSE, he is oriented to person but not to time or place. A lumbar puncture is performed. CSF analysis shows: - Opening pressure----340 (N 70-180) - Glucose---------------28 (N 40-70) - Total protein----------154 (N < 14) - Leuko's----------------8468 - Neutro's---------------85% - Lymphocytes----------15% - RBC's-----------------95/mm3 Which of the following is the most likely Dx? (Cryptococcal meningitis OR Pneumococcal meningitis)

Pneumococcal meningitis > CSF findings of bacterial meningitis are: 1- *Opening pressure > 200* 2- *Glucose <40* 3- *Protein > 200* 4- CSF:serum glucose ratio ≤0.4 5- [[↑ neutrophils]] //Cryptococcal meningitis// > Usually seen in the *setting of HIV* (pt does not have HIV → CSF findings are: 1- Opening pressure > 200 2- Glucose Low (<30) 3- Protein Elevated 20-500 (N ~100) 4- [[↑ Lymphocytes]]

A 12-hour-old male newborn has not urinated since birth. He was born at 36 wks to a 15F & weighed 5 lbs 2 oz. Apgar scores were 7 & 8 at 1 & 5 mins. His mom received no prenatal care. During her pregnancy, she smoked 1/2 pack of ciggs daily & occasional marijuana & drank approximately 2 beers on the weekends. Since birth, the newborn has received one ounce of cow's milk-based formula w/out vomiting & has passed one small meconium stool. He is alert & active. His temp is 98.2F, pulse is 135/min, RR is 35/min. Exam shows no dysmorphic features. The lungs are clear to auscultation. Heart sounds are normal. The lower abdomen is distended w/ a midline mass. Which of the following is the most likely Dx? (Wilms tumor [nephroblastoma] OR Posterior urethral valves)

Posterior urethral valves - Most common cause of obstructive hydronephrosis in *newborn & infant males* - Signs include: 1- *Incontinence* 2- Enlarged bladder that presents as *midline abd mass* 3- UTI most common complication 4- Painful urination or weak stream Severe cases 1- Resp distress 2- hydronephrosis 3- Vesicoureteral reflux 4- Bladder dysfunction 5- Renal impairment Wilms Tumor --1) usually presents in kids aged 3 years (not common >6) ----(pt is 12 HOURS old) --2) Abd mass & swelling +/- Abd pain --3) HTN --4) Hematuria ------(pt doesn't present w/ HTN, hematuria) --5) Fever --6) mass in kidney -----(pt has a MIDLINE mass)

A 2F, presents by her mom for a well-child exam. She is at the 50th percentile for length & wt. A grade 2/6 systolic, crescendo-decreshendo murmur w/ a musical quality is heard at the lower left sternal border w/ no radiation. S2 is normal. Precordial activity & peripheral pulses are normal. There were no previous notations of a murmur in her medical records. An ECG shows no abnormalities. Which of the following is the most appropriate next step in MGMT? (Reassurance that this is a benign murmur OR Echocardiograph)

Reassurance that this is a benign murmur > Heart murmurs are common in children and babies >> vast majority there is nothing to worry about > This heart murmur is described as having musical quality but: 1- No Radiation 2- S2 normal 3- EKG normal

While being evaluated for a cardiac murmur, a 12B, is noted to have bilateral gynecomastia. His sexual development is Tanner stage 3. Which of the following is the most appropriate next step? (Measure serum gonadotropin conc's OR Reassure him that this is normal)

Reassure him that this is normal > Gynecomastia in children is common b/c they may have more fat in the chest area >> *usually goes away by late teen years*

A newborn girl is noted to have a palpable clunk when the hip is abducted, flexed, & lifted forward. Which of the following is the most likely mechanism of disease? (Muscle imbalance resulting from spasticity OR Shallow, poorly developed acetabulum)

Shallow, poorly developed acetabulum - Pt presents w/ *hip dysplasia* → caused by abnormal development of the acetabulum, which leads to displacement of the femoral head → *Developmental dysplasia of the hip* is the most common orthopedic disorders in newborns

A previously healthy 11B, presents b/c of a 14-day Hx of fever, headache, & yellow-green nasal discharge. He has had a nocturnal cough during this period. His 8-year-old sis has a cold. He appears mildly ill. Temp is 102.2F, pulse is 100/min, RR is 18/min. Pulse OX is 96% on room air. The posterior pharyngeal wall is erythematous & covered w/ gray thin mucus. The lungs are clear to auscultation. An CXR shows no abnormalities. What is the most likely Dx? (Sinusitis OR Bronchiectasis)

Sinusitis - Most common cause is *Viral* but can present w/ viral → signs include: -Viral & bacterial- 1- Sinus congestion 2- Purulent nasal discharge 3- *Cough* 4- Sinus pain & pressure 5- *Mucosal edema & inflammation* 6- Pain upon palpation of sinuses -Bacterial specific signs- 1- Symptoms lasting > 10 days 2- Sever pain & Fever >102 3- Symptoms that improve & then worsen after several days Bronchiectasis - Pt would have Hx of underlying chronic disease (CF, COPD, Airway hypersensitivity disease, Primary ciliary dyskinesia, Immunodeficiency, Autoimmune, Localized airway obstruction, Recurrent pulm infections or recurrent aspiration) - Presents w/ 1-*chronic cough w/ yellow-green sputum* 2- Dyspnea 3- CXR → would show *increased bronchovascular markings & tram lines* (pt would not have fever)

A pt presents w/ Juvenile Idiopathic arthritis And ANA titers. In addition to naproxen, what is the most appropriate next step in MGMT? (Arthroscopy OR Slit-Lamp exam)

Slit lamp exam - Pts w/ JIA & ANA titers (signifying oligoarticular) [RF seen in polyarticular] → have a higher incidence of uveitis → *requires slit lamp exams at Dx & every 3-4 months thereafter* TMT includes 1- NSAIDs (mainstay) 2- Methotrexate (polyarticular & resistant oligoarticualr) 3- DMARDS (etanercept/infliximab) 4- *Slit lamp exams*

Two weeks after a camping trip in NE, a 5B, develops a 3-cm, red, circular lesion w/ a clear center, lymphadenopathy, conjunctivitis, myalgia, & nausea. Antibodies to Proteus Vulgaris OX-19 are negative. Which of the following is the most likely causal agent? (Rickettsia OR Spirochete)

Spirochete - Pt most likely presents w/ Lyme disease -A) Signs/Symptoms: ------1) Usually presents 2 weeks after tick bite ---------a) Most prevalent in the North East US ------2) Rash ----------a) Red, circular lesion w/ a clear center ------3) Flu like symptoms -----------a) Lymphadenopathy, Conjunctivitis, Myalgia, nausea ---4) Labs ------NEGATIVE Antibody to proteus vulgaris OX-19 SIDE NOTE *B.burgdorferi is cause of Lyme disease and is a spirochete* (Also Lepto & Treponema) Rickettsia - Cause of RMSF, - Also presents w/ flu like symptoms( Myalgias, arthralgias, nausea) ~2 weeks after a tick bite - However, key differences include: ---1) Most prevalent in the SE & SC states --------------[pt was in NE] ---2) Additional KEY SYMPTOMS include -------a) Acute onset fever & Headache ---3) Rash is a MACULAR-PAPULAR PETECHIAL rash that STARTS ON ANKLES & WRISTS THEN SPREADS CENTRALLY TO TRUK (w/in 72 hours) --------------[pts rash is described as a BULLS-EYE rash] ---4) Labs ---------POSITIVE Antigen to proteus vulgaris --------------[pt has a NEGATIVE antigen]

An 8B, presents b/c of a 9-month Hx of non-productive cough that is worse at night & SOB during physical activity. On questioning, his mother says that he has not had an unusual number of colds for his age, but she is concerned b/c the colds seem to go to his chest & linger. He is at the 75th percentile for Ht and Wt. Cardiopulmonary exam shows no abnormalities. A CXR shows mild hyperinflation. Which of the following is the most appropriate next step in Dx? (Sweat chloride test OR Spirometry)

Spirometry - Pt presents w/ *Asthma* → which presents w/: 1- Intermittent dyspnea, episodic cough, wheezing 2- Symptoms often worse at night or physical activity Sweat chloride test - Only if pt has suspected CF → pt has no history of recurrent infections - Bronchiectasis d/t CF → pt would present w/: 1- chronic cough w/ yellow/green sputum (>100mL of sputum) 2- Dyspnea 3- ± hemoptysis PE 1- Wheezing, rales, or bronchi

A 2F, presents for a follow-up exam 2 days after initiating amoxicillin therapy for a rt lower lobe pneumonia. She had been healthy prior to onset of pneumonia. She continues to have fevers & cough, & she now has right-sided chest pain. Her immunizations are up to date. She appears ill & is tachypneic. Temp is 101.3F, pulse is 140/min, RR are 45/min. Repeat CXR show a large dense consolidation in the right lower lung field & a fixed right sided-effusion. Which of the following is the most likely causal organism? (Enterobacter OR Pseudomonas OR Staph)

Staph aureus - Pt presents w/ a Lung abscess - The most common cause of lung abscess after pneumonia is staph SIDE NOTE -> Most common causes of bacterial abscesses are: 1- *Peptostrep → most common overall* 2- Prevotella 3- Fusobacterium 4- Bacteroides Aerobic causes 1- Klebsiella 2- Nocardia 3- *Staph* Enterobacter - Would be the cause of infective endocarditis Pseudomonas - Not associated w/ lung abscesses b/c it is aerobic

A previously healthy 2B, presents to the ED 2 hrs after the acute onset of fever & diff breathing. He has had rhinorrhea for the past 24 hrs. His mom says that he has noisy breathing that is most noticeable when he inhales. The symptoms seemed to improve when she took him outside to come to the ED. Immunizations are up to date. He is alert. Temp is 100.4F, pulse is 120/min, RR is 30/min. Exam shows supraclavicular retractions. Inspiratory stridor is heard. The remainder of the exam shows no abnormalities. Which of the following is the most likely mechanism of these findings? (Edema of the epiglottis OR Subglottic edema)

Subglottic edema - Croup is Most common cause of stridor in children (>90%) - pt presents w/ *Croup* (aka Laryngotracheobronchitis) → pathology is Narrowing of *subglottic pathway* - Caused by Parainfluenza-1 - Most common in children *6 months to 3 yrs* in late fall & early winter -Presentation- - 1-3 day viral prodrome -----(pt has had rhinorrhea for 24 hours) → followed by classic triad of: 1- Hoarseness 2- Barking cough 3- *Inspiratory stridor* ----Typically exacerbated at night & w/ agitation Edema of the epiglottis - Usually presents in pts 3-7 yrs old → caused by hiB [pt is 2 and has all vaccinations] -presentation- 1- Abrupt onset (<24hrs) high fever & soft stridor d/t rapidly progressing upper airway obstruction ----(w/out Viral prodrome symptoms) → *3 D's* 1D→ Dysphagia 2D→ Drooling 3D→ Distress (anxiety) 4- Tripod or sniffling posturing

A 14F, presents d/t progressive tiredness during the past month. She received the Dx of DM I 4 years ago & takes insulin twice daily; she has never missed a dose. Two weeks ago, her HbA1c was 6%. She has no other sig PMHx & takes no meds. She has had no changes in appetite or Wt. Menarche occurred at the age of 13. Menses occur at irregular 30-40 day intervals. For the past 3 months, they have lasted longer, & the flow has been heavier. Her LMP was 4 wks ago. Pulse is 60/min, BP is 120/75. The thyroid gland is easily palpated. The remainder of the PE shows no abnormalities. Breast & pubic hair development are tanner stage 4. Serum studies are most likely to show an increased conc of which of the following hormones? (DHEA sulfate OR Estrogen OR TSH)

TSH - Pt has DM I → Thyroid problems are more common in DM I pts b/c the antibodies can attack the thyroid gland and cause Hyperthyroidism or hypothyroidism - Pt most likely has hyperthyroidism, signs include: ---1) *Abnormal or absent menstruation* ---2) Pts thyroid gland may be enlarged (Easily palpable) DHEA - Pt would have virilization Estrogen - Pt would have associated symptoms besides menstruation → precocious puberty or mass

A 2-month old infant has a large posterior fontanelle, a large tongue, & hypotonia. He was born at 38 wks; pregnancy & delivery were uncomplicated. Apgar scores were 7 & 9 at 1 & 5 mins. Which of the following is the most likely underlying mechanism of disease? (Anterior horn degeneration OR Thyroid dysfunction)

Thyroid dysfunction > *hypothyroidism* >> presents w/ *6 p's* 1- Poor brain development 2- *Enlarged fontanelle* 2- Pot-bellied 3- Pale 4- *Puffy-face* broad nose, wide set eyes (coarse facial features) 5- Protruding umbilicus 6- *Protuberant tongue* 7- Myxedema Anterior horn degeneration > would cause hypotonia but would not cause any of the other symptoms

A 1-month-old infant has had a 12 x 8-cm, red, raised, well-defined lesion that is lobulated & compressible overlying the buttocks since birth. Exam also shows multiple petechiae & bruises. Which of the following is the most likely explanation for these findings? (Increased tissue growth d/t increased vascular supply OR Thrombocytopenia from plts sequestration)

Thrombocytopenia from plts sequestration - pt presents w/ kasabach-Merritt syndrome AKA *hemangioma w/ thrombocytopenia* 1- Lesion 2- Petechiae & bruises - Picture is of thrombocytopenia Increased tissue growth d/t increased vascular supply - May cause the growth but would not cause the thrombocytopenia or petechiae

A 6F, presents b/c of a scaly, pruritic rash in intertriginous areas of her elbows & knees that worsens in the spring & fall; It often appears in conjunction w/ episodes of respiratory distress & wheezing. TMT with which of the following is the most effective short-term TMT of the rash? (Clotrimazole OR Triamcinolone)

Triamcinolone > used to treat allergic rxn's of skin → topical steroid that reduces inflammation Clotrimazole > Anti-fungal → for yeast infections!!

A previously healthy 4B, presents b/c of a 2-day Hx of mild itching of his head & scattered areas of hair loss on his scalp. Exam shows several small oval areas of alopecia over the posterior scalp w/ numerous hairs broken off close to the scalp. Several small, occipital, non-tender LN's are palpated. The remainder of the exam shows no abnormalities. Which of the following is the most likely causal organism? (Epidermophyton floccosum OR Malassezia furfur OR Microsporum audouinii OR Tricophyton tonsurans)

Tricophyton tonsurans - Is most common infection for nail, beard, skin & *Scalp* → causes infections such as: ringworm, jock itch, athletes foot - Pt would present w/: ---1) lesion w/ a clear raised border -------(Small oval area/areas of alopecia) ---2) hair that breaks off & scalp & that is coated in a scaly layer --------)w/ remaining short hair stubs after hair breaks off) -------- Short hairs will look like *Black dots* ---3) +/- papules in area Epidermophyton floccosum - 4th Most common cause of dermatophytosis in North America - Causes superficial disease such as *tinea pedis* & *tinea cruris* & less commonly *tinea corporis* & *onychomycosis* Malassezia furfur - usually presents in summer d/t hot, humid, warm weather - RF → hyperhydrosis & topical oil use - Presents w/ hypopigmented/ Hyperpigmented/ salmon colored macules in the trunk & extremities Microsporum audouinii - primarily infects hair → typically causes *Tinea capitis* or *tinea corporis* - pt would have ring appearance on scalp ----(Pt has small oval areas of hair loss w/ hair that breaks -> more specific to trichophyton)

A 14F, presents b/c of lower abd cramps & intermittent pain in her knees & hips for 6 weeks. The cramps are relieved by bowel mvmts. During this period, she has had 6-10 urgent, bloody bowel mvmts daily. Exam shows a swollen, mildly tender left knee joint. Hb is 14.5, Leuko's are 16K, Plts 790K. Analysis of the knee joint aspirate shows straw-colored, slightly turbid fluid w/ a Leuko count of 2K (40% segmented neutrophils). Which of the following is the most likely Dx? (Henoch-Schonlein purpura OR UC)

UC - The presentation of UC includes: 1- Hematochezia ----Recurrent episodes of abd pain & Bloody diarrhea 2- Frequent small bowel mvmts 3- Rectal mucus discharge 4- Tenesmus ----(feeling of incomplete evacuation of stools) 5- Extra intestinal symptoms (Uveitis, pyoderma gangrenosum necrotic lesions on legs, Ankylosing spondylitis, Primary sclerosing cholangitis) 6- Inflammation -> of the skin, *joints*, eyes Henoch-Schonlein Purpura > AKA IgA vasculitis >> most common cause of vasculitis in children - Presentation includes:: ---1) Recent Hx of URTI (often viral or strep) ---Followed by *tetrad* --------a) Rash ----------------(symmetric maculopapular rash on extensor surfaces of arms, buttocks, legs) -----------------Palpable purpura in pts *w/out thrombocytopenia nor coagulopathy* --------------------[pt's plts are increased & has no coagulopathy] 3- Polyarticular arthritis (transient non-migratory) 4- Colicky Abd pain frequently w/ vomiting -----(Pt does not have) 5- Renal failure (IgA IC deposits) -----(Pts does not have signs of renal failure)

A 3B, presents for a well-child exam. His mom states that his belly seems big. His vitals are normal. Exam shows aniridia. There is a palpable mass in the right flank. The remainder of the exam shows no abnormalities. Which of the following is the most appropriate next step in Dx? (Abd XR OR US of abd)

US of abd - Pt presents w/ Wilm's tumor → *2nd most common malignant abd tumor* & *Most common primary renal malignancy* in childhood -Presents w/ *WAGR* *W* → Wilm's tumor *A* → Aniridia *G* → genitourinary abnormalities *R* → Intellectual disability (retardation) -Also associated w/ -----1) Denys-drash syndrome -----2) Beckwith-Wiedemann syndrome -B) MGMT -----a) Diagnostic tests ---------1) Abd US -> BEST INITIAL TEST ---------2) Doppler US + contrast CT/MRI ---------3) Surgical exploration -------------(if 2nd kidney is suspected to be affected) -----b) TMT ----------1) Involves surgery, radiation & Chemo therapy -------------a) Radical nephrectomy -------------b) Chemo -----------------(Vincristine, Dactinomycin, Adriamycin) Abd XR - Not used in the Diagnostic work-up for Wilms tumor - Additionally, US & MRI are used more frequently in children (and Women) -> b/c X-rays & CT's have radiation

A 2-month-old presents for a well-child exam. He is feeding well. Growth & development are appropriate for age. Immunizations are up to date. Vitals are w/in normal limits. The lungs are clear to auscultation. A grade 3/6 holosystolic murmur is heard ta the left lower sternal border w/ radiation throughout the precordium. A systolic thrill is palpated at the left lower sternal border. Abd exam shows no abnormalities. Femoral pulses are equal. An ECG shows no abnormalities. An CXR shows an enlarged left atrium. Which of the following is the most likely Dx? (Tetralogy of Fallot OR VSD)

VSD Cardiac auscultation findings - Holosystolic murmur at *lower left* sternal border - Loud pulmonic S2 - Systolic thrill - Signs of CHF - If large VSD → Apex diastolic rumble (d/t increased flow across mitral valve) Tetralogy of fallot - Pt would present w/cyanosis or cyanotic spells → especially when crying or feeding -----(Pt does not have cyanosis) Cardiac auscultation findings - Single S2 (inaudible P2) - Early systolic click (d/t flow thru dilated aorta) - Crescendo-Decrescendo systolic murmur at the left upper sternal border

A 4F, present by her mom b/c of vaginal bleeding for 12 hrs. She has a 3-month Hx of purulent vaginal discharge. Breast & pubic hair development is tanner stage 1. Exam shows a purulent bloody discharge on her underwear. Which of the following is the most likely Dx? (Vaginal foreign body OR Central lesion causing precocious puberty)

Vaginal foreign body > Purulent discharge is sign of foreign body > Foreign body would also cause bleeding Central lesion causing precocious puberty > Pt would have something like ↑ACTH which would be associated w/ electrolyte imbalances > Pt would have greater than Tanner stage 3

An 8-year-old is referred to the school nurse practitioner for eval of a murmur heard during an annual school physical exam. There is no Hx of cyanosis, decreased exercise tolerance, or rheumatic fever. Exam shows no cardiomegaly. The apical impulse is tapping & there are no thrills; the split S1 widens during inspiration. There is pronounced sinus bradycardia. A low-pitched vibratory murmur is heard throughout the cardiac cycle; it is more prominent at the left upper sternal border when the child is in the seated position. The murmur disappears when the child is in the supine position & when his neck is rotated in the sitting position. Which of the following is the most likely cause? (PDA OR Venus hum)

Venous hum - Heard throughout the cardiac cycle → often confused w/ a heart murmur but it is benign -> Presentation includes: --1) Low-pitched vibratory murmur --2) Best heard in upper chest near clavicle --3) Disappears when in supine position --4) S1 Widens during inspiration PDA - Continuous machine like murmur - Best hears at left infra-clavicular area - Loudest at S2 - S1 does not widen during inspiration