MedStudy Peds Boards review

what are the rules for # teeth eruption

"7 + 4" by 7 months, they should have their first teeth by 11 months (7 + 4), they should have 4 teeth by 15 months (11 + 4), they should have 8 teeth (4 +4) and so on

FeNa

( U Na x S Cr ) / ( U Cr x S Na ) x 100

what are the five MINOR manifestations of rheumatic fever

1. arthralgia 2. increased ESR 3. prolonged PR interval 4. fever 5. increased crp

mIVF equation

10 kg = 100 cc/kg (aka 1 L) >10 kg - 20 kg = 1L + 50 cc/kg >10 >20 kg = 1.5L + 20 cc/kg >20

how long does it take after exposure for a tuberculin skin test to turn positive

10 weeks

what is the common age for males to develop gynecomastia

10-16 13 years... SMR 2, 3, 4

how close together can you give kids 9-23 months who are high risk menactra vaccine

12 weeks important for eculizumab and complement deficiency

how much degree is allowed before closed reduction is required in a greenstick fracture

15 degrees

at what age is it ok to start kids watching digital media

18 months

IVH grades

1: germinal matrix 2: intra ventricular 3: intra ventricular with enlargement 4: brain parenchyma

what are the 4 types of hypersensitivity reactions

1a: immediate IgE mediated (classical allergy, mast cell degranulation with histamine, within 1 hr) 1b: latent response (3-12 hrs later, eos/cytokines cause erythematous induration, burning, itching) 2: cytotoxic IgG or IgM mediated 3: immune complex mediated 4: delayed cell mediated (T cell) *5. stimulation effect of autoantibody (ex: graves)

what is the earliest age you can vaccinate sickle cell patients against encapsulated organisms

2 months of age, including meningococcal

at what age should kids run well and climb stairs with 2 feet on each step

2 years

normal timeframe for infantile acne

2-4 months of age usually boys if > 6 months, then should consider hyperandrogens measure 17 OH progesterone

after breast buds develop, when do you expect menarche

2-5 years later

how long after ingestion do iron levels peak

2-6 hours

at what age should screening for lipid disorders occur in patients with family history of early cardiovascular event

2-8 AND 12-16

how much does the anion gap fall for hypoalbuminemia

2.5 for every 1 drop in albumin

at what bilirubin level should you consider exchange transfusion in a 48 hr old infant

25

in a child < 13 years of age, how long must they wait between doses of varicella for catch up vaccination

3 months

when does ear cartilage develop during gestation

32 weeks

at what gestational age do sole creases develop

32-33 weeks

what percentage of children allergic to peanuts are also allergic to tree nuts

33% (1/3)

when does breast tissue develop during gestation

34 weeks +

what is the EEG finding with absence seizures

3Hz generalized spike and wave discharge

how long after measles does a kid stay out of daycare

4 days after the onset of the rash

when should the first Tylenol level be drawn after an overdose

4 hours

how long after MMR vaccination should you wait to test for TB

4-6 weeks because the measles vaccine may temporarily suppress reactivity

what factor levels can be helpful to distinguish synthetic liver failure vs DIC

5 and 8 5 is the poor man's liver biopsy 8 is made both in the liver and in other organs of the body so helps differtiate

when does birth length increase by 50%, double and triple

50% by 1 year doubles by 4 years triples by 13 years

what qualifies an ear infection for watch and wait

6 mo to 24 mo with unilateral AOM without otorrhea >2 years with unilateral or bilateral AOM without otorrhea

at what age do you need to refer to urology for undescended testes

6 months

at what age should an infant with cyrptochidism be referred for orchiopexy

6 months

how long do you treat patients with nocardia

6-12 weeks if immunocompetent 6-12 months if immunocomprimised

what cranial nerve may present with palsy in a patient with elevated ICP?

6th CN aka false localizing sign

what are the paradise criteria for tonsillectomy

7 in the last 1 year 5 in each of the last 2 years 3 in each of the last 3 years

what is the normal lifespan of a platelet

7-10 days

what are the blood pressure goals for shock reversal for children < 10 years

70 + age in years

what is the angle definitions for torticollis

70 degrees lateral neck flexion 110 degree passive rotation

how long after completing catch up PCV13 should you wait before giving PPSV23

8 weeks

at what bilirubin level should you consider phototherapy in a 24 year old infant

8-12 depending on gestational age and other risk factors

what is the definition of overweight based on BMI percentiles

85-95% obesity is > 95%

in a hep B positive mom, when do you need to test baby for first hep b serologies?

9 months of age

what is the first age a previously unimmunized child to influenza needs just one flu shot rather than 2?

9 years old 6 months to 8 years still needs two within one flu season x 1

at what age should universal screening for lipid disorders occur?

9-11 AND17-21 (pre puberty because puberty may create falsely lower readings)

who needs varicella vaccine for post exposure prophylaxis

>12 months and not previously immunized then follow regular vaccine schedule

Bernard soulier

AR absent functional platelet glycoprotein Ib inability to bind vwf giant platelet disorder

tay sachs disease

AR increased startle age 3-6 months loss of motor skills cherry red spot on macula hyperreflexia diffuse muscular hypotonia lysosomal storage disorder wit increased GM2 ganglioside

congenital microvillus inclusion disease

AR most common cause of congenital diarrhea sxs: severe watery diarrhea and failure to thrive, will have diarrhea despite taking no feeds by mouth dx: normal stool absorption studies, high stool electrolytes Rx: TPN, small intestine transplant

acute vs subacute endocarditis findings

Acute: heart murmur, new fever, splenomegaly Janeway lesions: non tender macules on palms/soles from septic emboli (vasc) *staph aureus subacute: weight loss, diaphoresis, prolonged fever Osler nodes: tender spots on palms/fingertips (IM) Splinter hemorrhages Roth Spots: pale retinal lesion surrounded by hemorrhage (IM) *viridans strep

primary hemostasis

Adhesion (Bernard Soulier) > activation > aggregation (Glanzmann) > thrombin

mechanism of action of dopamine

B: -increases cAMP production -releases norepi and decreases its degradation D: dilates peripheral vascular beds at low doses to provide enhanced coronary and renal perfusion A: vasoconstricts, increases afterload and decreases renal blood flow at hight doses

treatment for hereditary angioedema

C1 esterase inhibitors attenuated androgens (danazol) tranexamic acid

how do you vaccinate HIV + kids against MMR and Varicella

CANNOT give the combo MMRV vaccine give the first dose anytime after 12 months of age and follow up with a 2nd dose 28 days later to induce seroconversion as early as possible ONLY given if they are considered immunocompetent: children 1-13 years of age as a CD4+ T-lymphocyte percentage <15% and in adolescents ≥ 14 years of age as a CD4+ T-lymphocyte count < 200 lymphocytes/mm3

what is the only form of e coli colitis that you treat with antibiotics

ETEC treat with azithro or bactrim

"wall to wall heart" "boxed heart" on chest xray

Ebstein anomaly downwardly displaced tricuspid valve

PKU

FTT as a result of phenylketonuria hyperphenylalaninemia AR musty/pungent odor to urine usually in blonde hair/blue eyed/light skinned babies with eczema prioritize breast milk, Phe free formula, dietary restriction at risk for intellectual disability if not treated Dx: Newborn screen, serum amino acids, urine pterin analysis, dihydropteridine reductase activity

what are two metabolic causes of stroke

Fabrys (alpha galactosidase deficiency leading to thick ceramide accumulation) Homocysteinuria

what factor deficiency will still have a normal PT and PTT

Factor 13

what syndrome is associated with activated protein C mutation

Factor 5 Leiden mutation

what viruses are associated with DRESS

HHV6, HHV7, EBV, CMV

what is the most commonly identified cause of neonatal seizures

HIE

what is the most common thyroid condition in Trisomy 21

Hashimoto thyroiditis

what vaccines can be used as post exposure ppx

Hep A MMR Varicella

Gitelman syndrome

Hyperaldosteronism, Hyperrennin hypokalemia, metabolic alkalosis muscle cramps polyuria/polydipsia decreased urinary Ca and HYPOMAG NO HTNc

with which condition must you screen for breast cancer at the same rate as women

Klinefelters

"straight left heart border"

L- transposition of great arteries aka "corrected transposition"

juvenile myoclonic epilepsy

Myoclonic jerks of shoulders and arms that usually occur after awakening -PRESERVED consciousness usually combined with absence seizures and GTC's -Typical age of onset: 12-18 y.o. -Requires LIFELONG Tx w/AEDs (esp. levetiracetam or depakote) - triggered by sleep deprivation, EtOH, stress, or flashing lights - + FAM HX - dx: EEG with polyspike and wave at 4-6 Hz frequency

benign infantile myoclonic epilepsy

Myoclonic seizures begin around 6 months of age and are characterized by brief head nodding and eye rolling; sometimes they are associated with myoclonic jerks of the arms. both sleep and wakefulness. Electroencephalography will show epileptiform discharges associated with the clinical symptoms + Fam Hx

should you delay vaccines in the setting of a child with fever

NEVER!!!

what eye findings are specific for NF1 vs NF2

NF1: lisch nodules on iris (hamartomas), optic gliomas NF2: posterior subcapsular lens opacity

what tumors are associated with NF1 vs NF2

NF1: pheo, malignant peripheral nerve sheath tumor, optic glioma, acute myeloid leukemia NF2: bilateral acoustic neuromas (Schwanomas), hamartoma, meningioma

what syndrome are meningiomas associated with

NF2 *or prior XRT

do aminoglycosides penetrate the CNS

NO!

can hepatitis A be transplacentally transmitted

NO! and there is no carrier state

equation for NPV

NPV = TN/(TN + FN)

what treatments exist for PMS and PMDD

NSAIDS calcium supplementation Vit B6 OCP's herbal remedies SSRI

what is the initial treatment for reactive arthritis

NSAIDs then may consider systemic steroids

brugada syndrome

Na channelopathy RV conduction delay and ST elevation in V1-V3 at risk for arrhythmias

Langerhan's cell histiocytosis

Neoplastic proliferation of Langerhans cells with characteristic Birbeck (tennis racket) granules seen on electron microscopy cells are CD1a+ and S100+ by immunohistochemistry femurs, ribs, skull, humerus are all common locations - looks like osteolytic lesions may look like seborrheic lesions on the skin urine will show decreased osmolarity if pituitary is involved and leads to central DI

which three disease can be diagnosed with plasma amino acids

PKU tyrosinemia maple syrup urine disease

what teratogenic effects are associated with SSRI"s during pregnancy

PPHN

equation for PPV

PPV = TP/(TP + FP)

what is the treatment and post exposure PPX for corynebacterium diptheriae

Rx: equine antitoxin, erythromycin (makes not infectious and stops spread of toxin) PePX: oral erythro or IM PCN (No MATTER vaccination status)

noonan syndrome

Webbed neck Pectus excavatum Short stature Pulmonary stenosis, HOCM bleeding diasthesis (factor 11 deficiency) may have hypogonadism high frequency sensorineural hearing loss increased risk for ALL, CML, ASD

what are microdeletion syndromes

Williams Prader Willi/Angelman Beckwith Widemann Soto Rubenstein-Taybi Miller-Diecker CHARGE Alagille Rett

WAGR syndrome

Wilms tumor. Aniridia. Genitourinary abnormalities. Mental retardation.

can HIV kids get rotavirus

YES! no matter the CD4 count

which liver tumor is associated with OCP use

adenoma

what abx are used for neonatal meningitis

amp + gent + cefotaxime

how is juvenile psoriatic arthritis defines?

arthritis or psoriasis OR arthritis + 2: dactylics, nail findings, family hx in first degree relative

what is the formula for expected PCO2

bicarb + 15

how can you differentiate erythema multiforme and urticaria

both may occur after a viral infection urticaria will be migratory multiforme will not

what can rapid infusion of NaHCO3 to a preterm infant do?

cause intraventricular hemorrhage

which infection is associated with inclusion bodies

chlamydia

who gets group C strep

college students

CHARGE syndrome

coloboma heart defects choanal atresia retarded growth and development genital abnormalities ear abnormalities

what is the most serious complication from neonatal lupus

complete heart block

PPHN CXR findings

dark CXR hypoperfused lungs *should have a harsh systolic murmur as well

antidote to ethylene glycol poisoning

fomepizole

what GI manifestation are kids with ADPKD at risk for in late stages

hepatic fibrosis

Balanoposthitis

inflammation of the glans (head) and prepuce (foreskin)

subglottic stenosis

inspiratory stridor and resp distress in newborns to first few months A/w downs only treat if symptomatic

in which timeline for gbs sepsis is meningitis most common

late onset

what is the X-ray pattern with blastomycosis

mass like infiltrates or consolidation

how do young children present with bipolar

more rapid or short mood episodes, mixed states, severe irritability or disruptive behaviors

what is the most defining feature of SSRI overdose?

myoclonus

transverse myelitis

occurs after viral infection inflammation of the spinal cord pain at a specific level then progresses to progressive paralysis with sensory loss below the lesion, maybe burning sensation Dx: MRI with signal change 60% of people return to normal Rx: high dose steroids Risk Factors: herpes simplex, herpes zoster, CMV, EBV, Coxsackie virus, Mycoplasma pneumoniae, and Lyme disease

symptoms of osteoid osteoma

pain worse at night and improved with NSAID use Dx: lucency surrounded by sclerotic bone of the metaphysesis

side effects of didanosine

pancreatitis

what does bicarb gtt do in salicylate poisoning

protects the CNS from the salicylate

HyperIgE

recurrent lung infections, skin abscesses with elevated or normal levels of IgE delayed teeth erruption eczema Associated with coarse facial features, prominent forehead, wide spaced eyes, broad nasal bridge

what type of seizure does todd's paralysis usually follow

simple partial seizures

what is the common mechanism of injury in ACL tear

sudden deceleration and pivoting of knee

type I vs type II autoimmune hepatitis

type I: ages 10-20, positive ANA and anti smooth muscle Ab type II: younger kids, positive anti KLM, more severe

type 1 vs type 2 autoimmune hepatitis

type I: anti smooth muscle antibody and ana, broad range of severity, responds to treatment type II: antiLKM and antiliver cytosol 1 Ab, mosre severe sxs

benign neonatal convulsions

"Fifth day fits" lasting 1-3 minutes unilateral or multifocal tonic seizures normal labs and imaging, no fam hx no poor neurodevelopmental outcomes

how do you calculate a urine anion gap

(Una + Uk) - Ucl

free water deficit equation

(hyperNa - 140) + 4 x pts weight in kg = cc free water

likelihood ratio

(probability that individual with disease has a + test) / (probability that individual without disease has a + test) sensitivity / 1-specificity

equation for BMI

(weight in kg) / (height in m)^2

How do you determine if a newborn born to mom with HIV has HIV?

*make sure mom had a C/S to minimize risk of transfer need to test them with HIV PCR at 14-21 days, 1-2 months, 4-6 months If all 3 of these are negative, then the baby is considered uninfected if positive, then need a second confirmatory test

Stickler syndrome

-connective tissue disorder aka hereditary arthro-ophthalmopathy -flattened midface, depressed nasal bridge, short nose, anteverted nares, and micrognathia -Pierre Robin sequence -cleft lip, cleft palate, and/or bifid uvula -severe myopia, cataracts, retinal detachment, hearing loss, and joint hypermobility

Wolf-Hirschhorn Syndrome

-deletion of chromo 4p -Clinical features: facial anomalies, widely spaced eyes, prominent nose, abnormal iris (coloboma), cardiac anomalies, intellectual & developmental delay, face looks like "Roman Helmet"

why does physiologic jaundice occur in newborns

-higher numbers of RBC with shorter life span -deficiency of enzyme uridine DPGGT -increased enterohepatic circulation

who needs VariZIG for varicella

-immunocomprimized children with no history of natural infection -infants of moms with varicella 5 days prior to or 2 days after delivery -non-immune pregnant women exposed to varicella

what exam findings are common in sJIA

-migratory salmon colored rash -synovitis (oligo aka 4 or poly aka 5) -myalgias with normal CK but elevated aldolase -pericarditis and myocarditis -pleuritis -LAD -HSM -abd pain -weight loss and fatigue

what are the characteristics of macrophage activation syndrome

-persistent fever, HSM, high ferritin, cytopenias, liver dysfunction (LFT's, coags, fibrinogen, TG), neurological dysfunction

how does systemic JIA aka Stills disease differ from JIA?

-sJIA requires daily fever along with arthritis and other systemic findings -fever happens twice daily and may go away on its own (quotidian fever)

what is normal weight gain for 0-3 months 3-6 months 6-12 months

0-3 months = 30 g/day, 1 oz/day 3-6 months = 15 g/day, 1 oz q2 days 6-12 months = 10 g/day, 1 oz q3 days

what is the NRP code dose of epi

0.01 - 0.03 mg/kg of 1:10,000 solution

what dose of epinephrine is given for anaphylaxis

0.01 mg/kg with max dose of 0.5 mg

what is the rate of insulin infusion for DKA

0.1 U/kg/hr

anesthesia classes

1 = healthy 2 = mild systemic disease like mild asthma or DM 3 = severe systemic disease with definite functional limitation (obesity, PNA) 4 = severe systemic disease with constant threat to life (BPD, sepsis) 5 = very ill, not expected to survive without the operation 6 = brain dead organ harvesting

odds ratio

1 = no effect >1 = exposure is more likely to create outcome <1 = exposure is less likely to create outcome

how long do symptoms have to occur to diagnosed PTSD

1 month

how long do kids have to stay out of daycare after hepatitis A

1 week after onset of jaundice this minimizes how much of the virus is shed in stool

what are the 3 stages of ethylene glycol ingestion

1. CNS (drunk w vomiting, drowsy, slurred speech) 2. AKI, coma, tachypnea 3. renal failure

what are the 4 stages of iron ingestion at > 60 mg/kg

1. GI stage (erosive damage to mucosa) 2. relative stability 3. systemic toxicity (hemorrhagic or hypovolemic shock, severe metabolic acidosis, hepatic failure, coag Abnl, coma) 4. GI/pyloric scarring

4 stages of tylenol toxicity

1. asymptomatic vs malaise, vomiting 2. RUQ pain 3. peak transaminases and LFT failure 4. multisystem organ failure

arnold chiari I vs II

1. cerebellar tonsils and vermis below. syringomyelia 2. 4th ventricle and lower medulla below myelomeningocele

what three things are associated with rectal prolapse

1. constipation 2. infectious diarrhea (shigella, whipworm) 3. CF

what are the five differential diagnoses of a prolonged pt

1. early liver failure, check factor 5 (liver-only made factor) 2. vit k deficiency/warfarin, check 2/7/9/10 3. factor 7 deficiency, check 7 4. factor 7 inhibitor, check 1:1 mixing study 5. DIC, check d dimer and plt count

what are the five major criteria of acute rheumatic fever

1. fever 2. migratory polyarthritis of the large joints 3. chorea 4. carditis 5. subcutaneous nodules

what are the four phases of chronic hepatitis based on HepEAg and DNA levels

1. immune tolerant (Hep e Ag + and increased DNA) 2. immune active (Hep e Ag + and decreased DNA) 3. carrier state (Hep e Ag - and decreased DNA) 4. reactivation (Hep e Ag - and increased DNA)

very long-chain acyl-coenzyme A dehydrogenase (VLCAD) deficiency presentations

1. infants with severe early onset heart failure with hypotonia and hepatomegaly labs include hypoketotic hypoglycemia, hepatic dysfunction, elevated CD, increased dicarboxylic acids on urine organic acid analysis. Acylcarnitine analysis with elevated C14:1, C14:2, C14, and C12:1 metabolites 2. children with hepatic or hypoketotic hypoglycemic form without heart failure 3. older children with myopathy and intermittent rhabdomyolysis

11 criteria for lupus

1. malar rash 2. photosensitivity 3. discoid rash 4. oral ulcers 5. serositis (pleurites or pericarditis) 6. arthritis (non erosive or deforming and in >2 joints) 7. nephritis 8. hematologic (hemolytic anemia, leuko or lymphopenia, thrombocytopenia) 9. neurologic (seizures or psychosis) 10. ANA 11. immunologic (dsDNA, anti Smith, false positive syphillis test for 6 months)

what are the two different presentations of disseminated gonococcal infection

1. septic monoarticular arthritis 2. tenosynovitis and dermatitis with polyarthralgias, negative synovial cultures and positive blood cultures

what is the leading cause of death in adolescents age 21 - 24

1. unintentional injury 2. homicide 3. suicide 4. malignant neoplasms 5. heart disease

work up for reflux

1. upper GI (to diagnose anatomic or motility issue) 2. pH probe 3. upper endoscopy (diagnose esophagitis or EOE) 4. nuclear scintigraphy

how long do you treat acute bacterial sinusitis for

10-21 days

what is the risk of Downs if either parent has a 21:21 translocation

100%

what is the survival for a focal brainstem glioma

100%

what form of CAH presents with low levels of renin and aldosterone

11 B

at what age must ADHD symptoms be present by to consider this diagnosis

12 years

how sensitive is cfDNA testing for trisomy 13 vs 18

13 = 91% 18 = 97%

at what age should kids have 10-50 words

13-18 months

at what age should you not provide any additional doses of H flu if they received one older than this age

15 months

at what age do you test a newborn for hep C if their mother was chronically infected

18 months of age any earlier and it may be suggestive of mom's Ab's

HPV vaccine schedule

2 dose series if started younger than age 15 (6-12 months apart) 3 dose series if start older than age 15 (1-2 months, 3 months) if start and stop series, do not have to start over. 2nd and 3rd dose must be given 12 weeks apart it is not recommended to use in pregnancy but has no demonstrated teratogenic effects can be given as young as 9 years of age in child abuse

what is the treatment for neonatal HSV skin infection

2 weeks of IV acyclovir followed by 6 months of oral acyclovir to improve neurodevelopment outcomes

at what ages does lactase deficiency start

2 years

at what age do kids approach adult GFR

2 years of age by 3 months they have about 50% of adult GFR

what is the earliest age you can give PPSV23

2 years, after completion of PCV13 (preferably in a 4 dose series, esp for kids at risk. otherwise at least one additional dose if age > 2 years and incomplete series)

osmolarity

2(Na) + (glucose/18) + (BUN/28)

juvenile spinal muscular atrophy

2-17 years of age with proximal muscle weakness

how many meq/kg/day are recommended for Na and K

2-3 meq/kg/day Na 1-2 meq/kg/day K

what dose of penicillin is effective for syphillis

2.4 million units of penicillin benzathine G if neonatal: 50,000 aqueous pen G q8 or 50,000 procaine pen g daily

functional diarrhea aka chronic non specific diarrhea aka toddlers diarrhea

2/2 excessive fructose intake in form of fruits or juice, low fat or low fiber diets more than 3 stools daily for more than 4 weeks, with onset of symptoms between 6 and 60 months of age usually due to reduced intestinal transit time should NOT be associated with failure to thrive or malabsorption usually pass undigested food

serratus anterior palsy

2/2 excessive throwing motion causing long thoracic nerve palsy presents with winged scapula

Weil disease

2/2 leptospirosis hepatitis, renal failure, hemorrhagic complications, conjunctival suffusion with purulent discharge Rx: IV PCN if severe, doxy if mild

papular purpuric glove and stocking syndrome

2/2 parvovirus b19 sharply demarcated rash on. hands and feet with painful purpura, papules, petechiae a/w fevers, malaise, sore throat, arthralgias

exam for functional peripheral pulmonary artery stenosis

2/6 systolic murmur heard at LUSB, radiates to RUSB and back in a young infant 2/2 turbulent flow at acute angles of relatively small pulmonary arteries

what is the final volume a testicle should be at the end of puberty

20 mL

what is the risk of coronary artery aneurysm with kawasaki's disease

25% will get them if untreated if treated with IVIG/aspirin within 10 days of illness onset, this is reduced to 5% aspirin dosed 80-100 mg/kg/day for first 1-2 days, then decreased to 3-5 mg/kg/day x 6 weeks (timing of f/u echo) small aneurysms warrant aspirin which can be discontinued with aneurysmal regression giant aneurysms warrant aspirin + AC increased risk of aneurysms in < 1 and > 9, delayed time to treatment, inadequate response to treatment causing prolonged fever

what form of CAH can present with PCOS if discovered later in life

3 B salt wasting 2/2 build up of DHEAS may also see hypospadiasis, bifid scrotum or cryptorchidism in boys

kids with COME > ___ need hearing test

3 months

at what age can kids walk up and down on stairs one foot at a time

3 years

what is the youngest age you can offer radiation

3 years

pre-eclampsia

3+ protein or > 5 g protein in 24 hours sbp > 160 or DBP > 110 oliguria < 500 cc in 24 hours AMS or vision changes plt < 100K LFT abnormalities respiratory distress HELLP: hemolysis, elevated liver enzymes, low platelets, + dimer

when should the moro reflex disappear by

3-4 months

at what age should you be able to fully retract foreskin

3-4 years up until then just cleanse it without fully retracting it

at what age should infants have no head lag

4 months

how long after completing PCV13 should you wait before giving menactra

4 weeks

if MMR and varicella are not administered in the same day, how long much you wait between them

4 weeks

in a child > 13 years of age, how long must they wait between doses of varicella for catch up vaccination

4 weeks

for catch up DTAP vaccines, how long is the waiting period between doses

4 weeks (between doses 1-2, 2-3) 6 months (between doses 3-4, 4-5)

how far do you have to separate menactra and prevnar by

4 weeks to minimize risk of reducing effectiveness

at what age should kids hop on 1 foot

4 years

how long is refrigerated breast milk good for

48 hours if thawed after being in the freezer, then must use within 24 hours

how long after mumps does a kid stay out of daycare

5 days after onset of parotid swelling

how long does it take for a first order drug (most drugs) to reach steady state (>95%) if no loading dose is given

5 half lives

when should thumb sucking resolve by

5 years

what is the survival rate for DIPG

5% in < months from diagnosis

asthma staging

5-11 and > 12 years see chart 0-4: still < 2 sxs per week but 0 nighttime awakenings/month for intermittent and > 0/month for persistent

at what age do infants roll back to front

5-6 months front to back is 4-5 months (think trying to get out of tummy time)

at what degree of scoliometer reading should you refer for xrays

5-7 degrees

what age is the peak incidence for diabetes

5-7, and early pubertal

how long after CHD repair do they need PPX prior to dental procedures

6 months or indefinitely possibly if they had endocarditis or have a residual lesion *they also need PPX indefinitely for any prosthetic material

at what age does a raking grasp and ability to transfer objects between hands vs mature pincer grasp develop

6 months vs 9-12 months

what is a normal MCV

72 + child's age in years

what are the two most common presentations for burkitts lymphoma

90% start with abdominal mass (ileocecal junction), n/v 10% have adenoids/tonsils involved most common lymphoma in kids and most common malignant tumor of the small intestines

confidence interval

95% - < 0.05 p value 99% - < 0.01 p value a consistent result that falls outside of the 95% CI is statistically significant 0 = no correlation between data, no effect, not significant in graph form, to be significant, the whisker plot should not include the 0 line

what is the rate of correction for hyperNatremia

< 10 meq in 1-2 days avoid cerebral edema

what is the correction rate for hyponatremia

< 10 meq/24 hrs to prevent central pontine myelinosis (aka water rushes out of cells, likely in chronic hyponatremia)

which preterm infants need a plastic bag to minimize heat loss

< 29 weeks gestation

until what age is toe-walking a normal variant in kids

< 3 years

how to test for fetal pulmonary prematurity

< 32 weeks lecithin/sphingomyelin lamellar body count phosphatidylglycerol

at what temperature must varicella vaccine be stored at

< 5 degrees fahrenheit - 15 degrees celsius

what is the FEV1 of moderate persistent asthma

< 80% *< 60% is severe

what is the timeline for late vs early onset gbs sepsis

< or > 7 days

what is the most likely cause for neonatal eye redness <24 hrs after birth, 2-5 days after birth and 5-14 days after birth

<24 hrs: chemical irritant, no treatment 2-5 days: gonorrhea, topical erythromycin at birth readily prevents, CTX treats and should use if mom IS positive 4-14 days: chlamydia, oral erythromycin

cranial radiation less than what age places a patient at increased risk for neurocognitive impairment and makes them less likely to eventually achieve independent living

<3 years

what is a positive pulse ox screen for CHD

<95% or difference > 3% between right hand and foot

at what leg length discrepancy should you be referring to an orthopedic surgeon

> / = 2 cm

at what age would you only need PCV13/PCV23 in a one dose series in a child with sickle cell vs healthy child

> /=6 years for sickle cell >2 years for healthy child

what qualifies as recurrent ear infections

> 3 in 6 months >4 in 12 months

what are the criteria for metabolic syndrome

> 3 of the following: BMI > 97th %ile TG > 110 HDL < 40 SBP > 90th % ile fasting glucose > 110 OGT > 140 waist circumference > 90th %ile

at what lead level would you start chelation vs environmental search vs confirmatory venous puncture

> 45 > 10 > 5

what are the rules for a positive PPD

> 5 mm if in close contact with someone who was positive, immunosuppressed, X ray evidence of active TB > 10 mm if < 4 years, chronic medical condition, or increased risk for exposure (travel, homeless, incarcerated, healthcare) > 15 mm if > 4 years without risk factors *BCG vaccination should NOT impact PPD response. Will usually wane in response over time. Generally should be > 10 mm to be positive in a BCG vaccinated person

at what age do you stop vaccinating for HiB

> 5 years only need ONE dose between 15 and 60 months regardless of prior # of shots *kids who have a natural HiB infection still need the vaccination

necrotizing pancreatitis

> 50% mortality rate acutely ill, vomiting, fever, shock Cullen sign = blue discoloration around umbilicus Grey Turner sign = blue discoloration in flanks no need for antibiotics unless abscess formation

how many recurrences in a year may qualify someone for suppressive HSV therapy

> 6

what is the cut off measurement for genus valgum and at what age

> 7 years, > 8 cm

what is the O2 goal for TOF

> 75%

At what age can you start using doxycycline in kids

> 8 years of age

what kid can you start a statin in

> 8 years old LDL > 190 not responding to lifestyle changes after six months OR LDL 130-190 and other risk factors

at what a1c level do you consider insulin in addition to metformin for DM2 in children

> 8.5 OR a random glucose > 200 with classical symptoms

what size hypoechoic thyroid nodule with normal TFT's should get an FNA

>1 cm

what age group and staging has bad prognosis in neuroblastoma

>1 years of age with metastases

what is the hgb transfusion goal for patients with sickle cell undergoing surgery

>10

at what age do you refer to a Peds surgeon for stable gynecomastia

>17, otherwise likely normal variant of puberty

how long after shigella do daycare kids need to be excluded

>24 hours without diarrhea and stool culture is negative

chronic recurrent abdominal pain

>3 episodes of intermittent or continuous pain during >3 months

what arteriovenous oxygen saturation difference represents indicates cardiogenic shock

>40%

what is the diagnostic cut off for sweat chloride testing

>40-60 is positive for CF

how do you diagnose juvenile polyposis syndrome

>5 polyps characterized on biopsy sxs: painless rectal bleeding need yearly colonoscopy until no polyps are found, then can space out increased risk for colorectal and gastric cancers, in addition to vascular malformations

characteristics of low risk BRUE patients

>60 days >32 weeks >45 weeks post conception 1st event lasted < 1 minute no CPR required no concerning PE or history

what is the timeline for late late onset gbs sepsis

>90 days

venous hum

A benign continuous murmur heard on auscultation over the major veins at the base of the neck or around the umbilicus not normally heard heard best with the diaphragm of the stethoscope changes in intensity with movements of the head

androgen insensitivity syndrome

A condition caused by a congenital lack of functioning androgen receptors; in a person with XY sex chromosomes, causes the development of a female with testes but no internal sex organs will have elevated levels of circulating testosterone breasts may develop due to testosterone rx: remove testes given risk of seminoma

what is the murmur of pulmonic stenosis

A harsh crescendo-decrescendo systolic ejection murmur heard best at the left upper sternal border, with a systolic click heard immediately following the 1st heart sound during expiration may radiate to the left clavicle

sever disease

A traction-type injury, or osteochondrosis, of the calcaneal apophysis of the heel seen in young adolescents. worse with running, jumping Rx: RICE, NSAIDS, decreased participation until better

Crohns

A/w apthous ulcers, ashkenazi jews, peripheral non deforming arthritis, clubbing, gallbladder and renal stones, erythema nodosum screening: scope after 8 years of disease, q1-3 years

AC vs DC electrical burns

AC: alternating current, causes tetany and prolonged exposure DC: direct current, throws the person away from the source

what are contraindications to breast feeding

ACE-I, isotretinoin, statin, chemotherapeutics, HIV, glacatosemia, G6PD with certain foods consumed by mom, amphetamines, Tech - 99

what are the first line agents for treating HTN in children

ACE-I/ARB CCB Thiazide diuretics

an affected father has a 50% risk of passing on the gene mutation to offspring is consistent with what type of inheritance pattern

AD

what is the inheritance pattern for hereditary angioedema

AD

what is the inheritance pattern or marfan

AD

inheritance pattern for hereditary spherocytosis

AD but sometimes sporadic

what is the inheritance pattern for Sotos syndrome and what are some characteristics

AD overgrowth (usually LGA at birth, then growth rate slows down, then increases again: broad forehead, sparse frontotemporal hair, down slanting palpebral fissures, long/narrow face, malaria flushing macrocephaly

Alagille syndrome

AD triangular facies with pointed chin long nose with broad midnose bile duct paucity with cholestasis pulmonary stenosis vs TOF vs VSD ocular defects butterfly vertebrae dx: HIDA without biliary tracer excretion, liver bx with chronic colestasis Rx: cholestasis resolves on own *avoid kasai portoenterostomy (worsens underlying disease) *at risk for intracranial bleeding with with only minor head trauma

cyclic neutropenia

AD neutropenia q21 days values are ANC < 200 fever, aphthous ulcers, stomatitis, periodontal disease, pharyngitis, rectal and /or vaginal ulcers, and cervical lymphadenopathy susceptible to clostridium septicum species

what is the definition and treatment for tourettes

AD inheritance mostly males usually with motor tics > vocal, > 1 year usually regresses after 12 years of age but onset before age 18 no tic free period for > 3 months Rx: fluphenazine, pimozide, tetrabenzine, fluoxetine, clonidine, guanfacine

Cornelia de lange syndrome

AD, sporadic long eyelashes thin brushed on eyebrows (unibrow) short nose long philtrum hirsutism 5th digit clinodactyly cognitive impairment low set hairline

Charcot-Marie-Tooth

AD; common peroneal nerve palsy; inverted bottle appearance with "stork legs" aka distal muscle atrophy, high arched feet, ascending distal weakness usually develops in 1st or 2nd decade of life

SCID

ADA, RAG, IL2R defect or deficiency candidiasis, overwhelming sepsis, chronic lung infection, eczema, diarrhea, FTT, no thymic shadow, lymphopenia need irradiated blood products dx: decreased TRECS, < 300 T cells, decreased mitogen stim response rx: BM Tx

what is the antibody to in TTP

ADAMTS13 a protease that breaks down ultra large vWF multimers

osler-weber-rendu syndrome

AKA Hereditary Hemorrhagic Telangiectasias syndrome of pulmonary shunting hereditary hemorrhagic telangiectasis syndrome AVM's all over Sxs: R > L shunt, hypoxia, exercise intolerance, polycythemia Rx: surgical removal of sequestered lobe

what is the most common leukemia in children and what is the most common age range for leukemia

ALL 2-5

what is the most common childhood malignancy

ALL 4 P's: pyrexia, pallor, purpura, pain dx: BMbx with > 25% leukemic lymphoblasts good prognostic indicators: rapid response to treatment, female, trisomy bad prognostic indicators: < 1 yr or > 10 yr at diagnosis, 9:22 translocation, WBC > 50K, mature B or T cell leukemia

which antibodies are associated with type I autoimmune hepatitis

AMA anti SM Ab

which mutation is associated with familial adenomatous polyposis

APC

Friedreich ataxia

AR degenerative neurologic condition with ataxia, lower extremity weakness, cardiomyopathy, eye and ear difficulties, DM, hypoactive or absent DTR and bony abnormalities a/w HOCM death by age 30-40 2/2 cardiomyopathy

relative risk reduction

ARR / total reduction by experimental arm Remember that ARR is a subtraction between the two arms

what antibody is most associated with pharyngitis strep infection

ASO

what type of AKI does vanc cause

ATN

what two labs help determine recent streptococcal infection in patient suspected to have rheumatic fever?

Antistreptolysin O (ASO) Antideoxyribonuclease B (ADB)

what additional infection should you consider in a patient with symptoms of strep throat, including scarlatiform rash, who is not improvement with PCN treatment?

Arcanobacterium heamolyticum will grow on blood enriched medium so may be difficult to culture must treat with erythromycin

Smith-Lemli-Opitz Syndrome

Autosomal Recessive: Short stature, microcephaly, ptosis, anteverted nares, SYNDACTYLY of TOES 2,3; cryptochidism, hypospadias, mental retardation, congenital heart defects, cholesterol metabolism defect also micrognathia, posteriorly rotated and low set ears, hypotonia, poor feeding, CATARACTS death by age 18 months

which N. meningitis serotype is the most common cause of infection

B

what thalassemia is associated with Howell Jolly bodies

B thal major *can also see Heinz bodies which are more peripherally placed *also seen with post splenectomy patients

Beta thalassemia

B thal minor: Hgb A2 > 3.5% B thal intermedia: increased A2 and F B thal major: almost all F, transfusion dependent, erythroid hyperplasia of the bone marrow

what kids should get screened for DM2

BMI > 85 + - fam hx in 1st or 2nd degree relative - ethnic background - signs of insulin resistance on exam - maternal Dm or gDM while pregnant

what is the treatment for dystonic reactions

Benadryl

anticholinergic toxidrome

Blind as a bat (Mydriasis) Mad as a hatter (Altered mental status) Red as a beet (vasodilation, flushed) Hot as a hare (febrile) Dry as a bone (no secretions/diaphoresis) Bowel and bladder lose their tone Heart runs alone (tachycardia) 2/2 Atropine, antihistamines, scopalamine, antipsychotics Rx: ativan, physiostigmine

when should scoliosis patients be referred to a surgeon or bracing

Bracing: risser score (skeletal maturity of the bones) 0-3 and cobb angle > 25 if < 25 then just check in q6 months during rapid growth and yearly during stable growth Surgery: cobb angle > 50 *>90 is associated with cor pulmonale

what diseases are those with C2 complement deficiency vs terminal complement deficiency susceptible to?

C2 = pyogenic infections aka H flu, strep pneumo, N meningitidis Terminal = meningococcal

CD4 vs CD8

CD4: extracellular organisms; works with class 2 MHC HLA to activate B cells CD8: intracellular organisms, works with class 1 MHC HLA

what treatment is typically used for NHL

CHOP cyclophosphamide doxorubicin vincristine prednisone

what cranial nerve palsy is associated with TB meningitis and increased ICP

CN6 palsy causing double vision and strabismus

what manifestation of lupus does anti neuronal Ab indicate

CNS lupus

mycoplasma PNA

CXR looks worse than symptoms A/w hemolytic anemia (cold IgM agglutinin), splenomegaly, erythema multiforme, erythema nodosum, SJS, arthritis, pharyngitis, confusion make take 6 months to recover Rx: doxy, macrolide

delta delta

Change in anion gap / change in bicarb helps determine if mixed metabolic disorder < 1 = metabolic acidosis + non gap > 2 = metabolic acidosis + alkalosis 1 = no additional metabolic components

Lennox-Gastaut syndrome

Childhood onset seizures of multiple types often refractory to treatment. presents with daily multiple seizures, often nocturnal tonic seizures appearing b/w age 2-6 y/o. More common in males and strong association with mental retardation, behavior disorders, delayed psychomotor development. Abnormal interictal EEG characterized by slow spike and wave complexes.

small left colon syndrome

Complication of IDM decreased caliber of left colon -> abdominal distension and failure to pass meconium hypoglycemia, hypocalcemia, hypomagnesemia

Laryngomalacia

Congenital Soft, floppy laryngeal cartilages, esp epiglottis most common cause of stridor in a newborn Usually resolves by age 2-3 w/o TX INSPIRATORY stridor with lying, crying or feeding

12-15 month shots

DTAP, MMRV, Hib, prevnar

4 year shots

DTAP, polio, MMRV

MEN 1

Diamond pituitary hyperparathyroidism pancreatic endocrine tumors (Gastrinoma of zollinger ellison syndrome)

how is Macrophage activation syndrome diagnosed and treated?

Dx: bone marrow biopsy with hemophagycytosis by macrophages or labs (high ferritin cytopenias, Abnl liver studies) Rx: steroids, IL1 or IL6 inhibitors, cyclosporine

what teratogenic effects does Li have

Ebsteins anomaly hydrocephaly, neural tube defects, aqueductal stenosis, club foot, polydactyly, and polyhydramnios

Wiskott-Aldrich syndrome

Eczema, recurrent respiratory infections, thrombocytopenia (w tiny plt) all caused by X-linked B and T lymphocyte deficiency (Low IgM, elevated IgA and IgE) Caucasian when older, at risk for herpes and PJP may have prolonged bleeding from a circumcision or bloody diarrhea *Burkitts!

what two genetic conditions should you consider when spontaneous esophageal perforation occurs

Ehlers danlos marfans

trinucleotide repeat expansions to know

Expansion of CAG repeats in the HTT gene cause Huntington disease CTG repeats in the DMPK gene cause type 1 myotonic dystrophy GAA repeats in the FXN gene are associated with Friedreich ataxia. CGG repeats in FMR1 causes fragile x syndrome

what chains make up Hgb F vs A2

F: alpha 2, gamma 2 A2: alpha 2, delta 2

what physical exam maneuver is used to illicit pain and test for sacroiliac joint septic arthritis

FABER or Patrick test

Familial Mediterranean Fever

Fever, abdominal and testicular pain, and swelling of the knees and ankles ≥ 3 recurrent episodes lasting < 1 day, or up to 72-96 hours, which include ≥ 2 of the following: fever, abdominal pain, chest pain, arthritis, and a family history of the disorder. kids > 10 yrs rx: colchicine daily (to prevent amyloid)

what bacteria is responsible for Lemierre's syndrome

Fusobacterium necrophorum at risk for septic emboli to the lungs

how many daycare cases are required for H flu and N meningitidis to recommend ppx

H flu: 2 cases N meningitidis: 1 case

neonatal polycythemia

HCT > 65%

what infections are a contraindication to breast feeding

HIV, HTLV-1, HTLV-2, brucellosis mothers with active TB who have not received 2 weeks of treatment can only provide expressed breast milk HSV at site of feeding is a contraindication severe mastoiditis may necessitate a brief interruption in breast feeding

which routine vaccine is associated with in office syncope

HPV

what is the most common vasculitis of childhood

HSP

what adult conditions are associated with IUGR

HTN fatty liver metabolic syndrome

what other diseases are associated with selective IgA deficiency

Hashimoto thyroiditis SLE juvenile onset RA dermatomyositis nodular lymphoid hyperplasia DM I IBD celiac transfusion reactions

what will Hgb electrophoresis show in sickle cell trait and what is the most common complication

HbA and HbS but more A than S complication: renal papillary necrosis

How to differentiate the different fungal PNA by symptoms

Histo: hilar adenopathy and focal alveolar infiltrates Cocci: erythema nodosum and multiforme Blasto: mass-like infiltrates on cxr, spreads to bones ABPA: asthma with brown mucus plugs or peripheral eosinophilia, "fingers in glove" on CT *Use steroids

what are x-linked

Hunter/Hurler Menkes Kallman Rett Duchennes/Beckers Haemophilia Colorblindness OTC deficiency Fabry's Adrenoleukodystrophy

Hutchinson's triad

Hutchinson's teeth CN 8 deafness interstitial keratitis late findings of congenital syphillis

Job syndrome

Hyper IgE neutrophils fail to adhere to the endothelium causing cytokines to recruit more eosinophils causing increased stimulation of IgE sxs: severe atopic picture + abscess with s Aureus asymmetric face, 2 rows of teeth *cold abscess

what does it represent in a kid treated for tinea capitis with griseofulvin who errupts in an itchy papular rash with surrounding scales but his tinea capitis site looks better

ID reaction (autoeczematization) continue current treatment and add steroids

what bugs and risk factors should you think of in osteomyelitis

IV drug users: Pseudomonas neonates: s aureus, GBS, gram negative Acute: s aureus, group A strep, strep pneumo, kingella (esp if young) CGD: s aureus, serratia, candida sickle: salmonella Vertebral: s. aureus, MTb, bartonella

how long should you delay MMR vaccine after IVIG and steroid course lasting > 14 days

IVIG - delay for 3-11 months steroid course - delay by 28 days

celiac is associated with what other conditions

IgA deficiency Williams syndrome Downs syndrome DM1 Turners syndrome

what immunoglobulin subtypes are on the surface of B cells

IgG and IgD

kartagener's syndrome

Immotile cilia due to a dynein arm defect. male and female infertility (sperm immotile) bronchiectasis recurrent sinusitis associated w/ situs inversus. subgroup of primary ciliary dyskinesia

what is caffey disease

Infantile cortical hyperostosis that typically occurs in the first 6 months and is often confused for child abuse (however in abuse the periosteum would be involved). The prominent feature is swelling of the bone shafts only in cortical bone. usually resolves by 24-36 months of age think about this in a febrile and irritable infant with tender/swollen spots on bones usually involves the mandible, clavicle, scapula or ribs Rx: indocin, prednisone, self resolves

lymphocytic interstitial pneumonitis

Infiltration of pulmonary interstitium by mature lymphocytes. CT findings: Diffuse ground-glass opacity. Poorly defined centrilobular nodules. Thin-walled cysts. CXR with bibasilar infiltrate Associations: Sjogren syndrome. Hypogammaglobulinemia. Multicentric Castleman disease. AIDS. HIV in children most common ILD in kids

how do you differentiate between juvenile plantar dermatosis and tinea pedis on exam

JPD will spare the intertrigonous areas jpd will also have areas that appears shiny ("glazed" erythema)

what is the diagnosis of central precocious puberty

LH spike, brisk LH > FSH during GnRH stimulation test

Legionaires Disease vs Pontiac Fever

Legionaires: diarrhea, DNS symptoms, renal disease, PNA Pontiac fever: milder multi-system disorder but WITHOUT PNA

Duke Criteria

MAJOR: + blood cx x 2 with typical organism or 1 x c burnetti; + echo MINOR: predisposing condition; fever > 100.4; vascular phenomena (janeway lesions, mycotic aneurysm); immunologic phenomena (osler, roth, +RF); other positive blood cx need 2 major OR 1 major + 3 minor OR 5 minor

what disease should you suspect in a kid with severe hypoketotic hypoglycemia after a period of fasting or stress

MCAD (medium-chain-acyl-CoA dehydrogenase deficiency) a Fatty acid oxidation disorder Dx: acylcarnitine profile Rx: carnitine and carbohydrate feeds *otherwise could be seen in a normal 3-5 year old who skips dinner, sleeps all night, and awakens with seizure

mentzer index

MCV / RBC low in thalassemia since they have normal to increase RBC production and low MCV high in IDA since they have low RBC production and low MCV

mitochondrial encephalopathy with lactic acidosis and stroke-like episodes

MELAS presents with progressive decline in cognitive abilities, weakness, exercise intolerance, seizures, lactic acidosis, sensineural hearing loss inherited from mother to all children

what imaging is standard for staging neuroblastoma besides CT and MRI

MIBG scintigraphy to evaluate bone and soft tissue

which vaccines must be administered subcu rather than IM

MMR MMRV Varicella IPV

what is the initial best diagnostic test for PSC

MRCP will show beading along the gallbladder associated with Ulcerative colitis

what murmur is associated with anorexia?

MVP (late systolic murmur at the apex preceeded by a click) 2/2 myofibrillar atrophy

infant presentation with hypermagnesemia

Mag > 5 respiratory distress failure to pass meconium decreased bowel sounds abd distension lethargy flaccidity hyporeflexia difficulty feeding

precordial catch syndrome

Most common cause of chest pain in children lasting a few seconds and can normally be worsened by a deep inspiration, may recur throughout the day Sporadic sharp/knifelike acute onset pain no diagnostic work up necessary reassurance

Ornithine transcarbamylase deficiency

Most common urea cycle disorder X-linked recessive (other urea cycle enzyme deficiencies are autosomal recessive) First few days of life, may present later Excess carbamoyl phosphate converted to orotic acid HIGH orotic acid in blood and urine Low BUN HyperAMMONEMIA symptoms No megaloblastic anemia (vs orotic aciduria)

Hurler syndrome

Mucopolysacharide syndrome macrocephaly, coarse facial features, frequent URI, umbilical hernia, thickened valves, corneal clouding, intellectual disability, deafness

MRKH syndrome

Mullerian degeneration with primary amenorrhea no uterus, upper vagina, testes or elevated testosterone

superior mesenteric artery syndrome

Normally, the SMA and aorta form a 45 degree angle. If the angle diminishes to less than 20 degrees, the *transverse portion of the duodenum* can get trapped between SMA and aorta, and cause small bowel obstruction. Narrowing of the angle occurs with: - low body weight (anorexia) - recent excessive weight loss - severe burns - general catabolism - bed rest - scoliosis surgery Pain improves with knees to chest in side lying position

what is the treatment for acute rheumatic fever

PCN ASA glucocorticoids are not routinely recommended due to lack of evidence * will need PCN PPX for 5 years after to prevent recurrence

ibuprofen use within the past 24 hours causes a false positive result for which drugs?

PCP cannabis barbituates benzos

what cardiac abnormalities are associated with congenital rubella

PDA

what gene defect leads to hereditary pancreatitis

PRSS1

how does calcitonin work

PTH antagonist to slow down osteoclasts and decrease Ca resorption from bone and increase renal clearance of calcium

pseudohypothyroidism

PTH resistance 2/2 receptor issue tetany, short fingers, dimpling on dorsum of hands, intellectual disability decreased Ca, increased Phos, increased PTH

Fabry's

Pain in hands and feet exacerbated by heat angiokeratomas, corneal opacities, CVD, CAD, renal disease, autonomic dysfunction dx: maltese cross rx: enzyme replacement therapy x linked recessive

PANDAS

Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections may see worsening of OCD or tics

PFAPA

Periodic. Fever. Apthous stomatitis. Pharyngitis. Adenopathy (cervical). Occurring every 21 to 28 days. Rx: prednisone if missing school kids 6 mo - 7 yrs

what is asherman's syndrome?

Previous D&C →uterine scars and walls adhere to one another Dx: hysteroscopy

Omenn Syndrome

RAG protein has partial enzymatic activity from missense mutations causes rapid fatal immunodeficiency along with skin peeling and alopecia decreased immunoglobulins except for increased IgE and eos *Rare form of SCID

which BBB is more common in kids, esp after open heart procedures

RBBB look for RSR' in V1

what is + CCP Ab associated with

RF+ form of JIA

what is considered response to treatment with syphillis

RPR titer decrease by 4 fold

Romano-Ward vs Jervel and Lange-Nielsen syndromes

Romano Ward: AD, QT prolongation Jervel: AR, QT prolongation AND sensorineural hearing loss

S3 vs S4

S3 gallop - dilated ventricle filling, or volume overload S4 gallop - thick ventricle wall ("S4 stiff as a board")

what congenital defects are cocaine, pseudoephredrine and nicoteine associated with

SGA gastroschisis

serotonin syndrome

SHIVERS muscle rigidity, confusion/hallucinations, hyperreflexia, CNS depression, GI symptoms, tachycardia, fever If a result of MDMA or other drugs, try a benzo and if they don't respond, try cyproheptadine

side effects of nevirapine

SJS

which three nephritic syndromes have decreased C3

SLE membranoproliferative post strep glomerulonephritis

what should you suspect in a hypotonic baby with tongue fasciculations and muscle weakness that spares the face

SMA

what is the mnemonic for oculomotor cranial nerves

SO4 LR6 all the rest 3 superior oblique lets you look down while eye is adducted lateral rectus lets you look laterally the rest are self explanatory except for inferior oblique which helps you look up while eye is adducted CN3 also innervates the upper eyelid so is responsible for ptosis

Sturge-Weber syndrome

Skin and meningeal angiomatous lesions. Port-wine nevus: skin angioma in ophthalmic division of trigeminal nerve. Pial angiomas may result in chronic ischemia, gliosis, and gyral cortical calcifications in serpentine formation. Enlargement of deep and subependymal veins may mimic arteriovenous malformations. focal seizures or infantile spasms > refractory seizures at risk for glaucoma may also have cardiac rhabdomyosarcomas

tuberous sclerosis

Skin lesions: Adenoma sebaceum aka shagreen patch Ash-leaf spots. angiofibromas in malar pattern or periungual Brain lesions: Subependymal hamartomas. Cortical and periventricular tubers (calcific deposits). Subependymal giant cell astrocytomas located at foramina of Monro (May lead to hydrocephalus) Bone lesions: bone cysts Organ lesions: angiomyofibromas Cardiac Lesions: rhabomyosarcoma Neuro Lesion: infantile spasm

MEN 2A

Square medullary thyroid cancer hyperparathyroidism pheochromocytoma

what are sporadic disease

Sturge Weber VACTERL McCune Albright

superficial vs second vs third vs fourth degree burns

Superficial: red, dry, blanching second: superficial partial thickness (moist, blistering pink, normal blanching) or deep partial thickness (blistering, red or white, poor blanching) third: full thickness, dry, leathery, waxy, painless. needs skin grafting fourth: underlying fascia, muscle or bone involved

transverse myelitis

Symptoms include bilateral motor and sensory deficits. Bowel and bladder dysfunction and autonomic dysregulation may also occur dx: Spinal MRI demonstrates a longitudinal lesion traversing multiple contiguous segments involving both the white and gray matter of the spinal cord rx: steroids

what is the active form of thryoid hormone

T3

what is the difference between TAPVR and tetrology of Falot of CXR

TAPVR: given increased pulmonary venous pressures transmitted to entire pulmonary vasculature, get interstitial edema and ground glass appearance TOF: concave pulmonary artery, right sided aortic arch, normal sized heart but boot shaped, diminished pulmonary vascularity

what are four types of congenital thrombocytopenias

TAR Bernard soulier wiskott aldrich MYH9

what are some causes of constrictive pericarditis

TB, bacteria, mediastinal radiation will see kussmauls sign

specificity equation

TN/(TN+FP) high specificity means low false positives Therefore, a negative test is more likely to mean they do not have disease

what heart conditions are associated with DiGeorge syndrome

TOF, interrupted aortic arch or double aortic arch, truncus arteriosus, VSD, transposition of the great vessels

sensitivity

TP / (TP + FN) high sensitivity means low false negatives

true or false, the national collegiate athletic association requires a sickle cell screen prior to participation?

TRUE to prevent exertion rhabdomyolysis

equation for sensitivity

Test sensitivity= TP/(TP+FN) detects how often a positive test correctly identifies those with disease increased sensitivity = decreased false negatives

lissencephaly

The brain lacks sulci and gyri, and therefore appears smooth. sxs: seizures. blind. ftt. microcephaly a/w miller dieker syndrome (prominent forehead, bitemporal hollowing, antevertid nostrils, prominent upper lips, micrognathia)

what are AD diseases

Treacher Colins Waardenburg Stickler Crouzon Alpert Noonan syndrome Cornelia de Lange Beckwith Wiedemann Soto Rubenstein-Taybi Diamond Blackfan Holt-Oram CHARGE NF1/2 Alagille

MEN 2b

Triangle medullary thyroid cancer pheochromocytoma neuromas marfanoid body habitus

type 1 vs type 2 error

Type 1: rejecting the null hypothesis when it is actually true (assuming a difference when there is none) ex: you like girl, girl likes you, you don't ask girl out because you don't think she likes you *important for P value of a study *you're pregnant! (False positive) Type 2 : failing to reject the null hypothesis when it is actually false (assuming no difference when there is a difference) ex: you like girl, girl has boyfriend, you ask girl out and she rejects you cuz she had boyfriend *Decreased by strong power aka larger sample size *You're not pregnant. (false negative)

UMN vs LMN lesion

UMN: hypertonicity, + babisnki, hyperrelflexia, spasticity, atrophy LMN: hyporeflexia, atrophy, fasciculations, hypotonia

what is the most common cause of hydronephrosis in kids

UPJ obstruction sxs: pain, HTN, hematuria, UTI's dx: VCUG, US, diuretic renal scan

what are some causes of epididymitis in young boys

URI! myocplasma, enterovirus, adenovirus

what types of UV rays do tanning beds emit

UVA these cause premature aging, sun spots, immune suppression and increase risk for melanoma by 60% also risk for cataracts

Holliday-Segar Method

Used to calculate calories baseline requirements: First 10kg of body weight = 100mL/kg/d or 4/kg/hr Second 10kg of body weight= 50mL/kg/day or (2/kg/hr +40) Above 20kg of body weight = 20mL/kg/d or (1/kg/hr +60)

what paraneoplastic syndrome occurs with neuroblastoma

VIP syndrome intractable secretory diarrhea and abdominal dissension due to secretion of VIP which stops when the tumor is removed

what is the most common congenital heart disease lesion

VSD

what cardiac defect is associated with fetal alcohol syndrome

VSD and other septal defects septal defects are common

what vitamin is associated with peripheral neuritis, decreased deep tendon reflexes, loss of vibratory sense, muscle tenderness and cramping (especially in the lower extremities), heart failure, and psychiatric symptoms. FTT in infants as well

Vit B1 (thiamine) dry beriberi

what vitamin deficiencies are associated with hyperpigmentation

Vit B12 folate niacin

what vitamin deficiency is associated with nausea, vomiting, seizures, lethargy and failure to thrive

Vit B6 (pyridoxine)

what type of rickets has low levels of calcium

Vit D deficiency (low to normal) 1 alpha hydroxylase deficiency (Vit D Dependent type I) 1-25-hydroxylase2-D resistance (Vit D Dependent type II)

Incontinentia Pigmenti

X-linked dominant, lethal in males 4 overlapping skin phases 1. inflammatory vesicles and bullae on trunks and extremities 2. verruca on extremity hands and feet 3. thin bands of swirly plaques of blue-gray discoloration 4. hypopigmented atrophic streaks associated with scarring alopecia, delayed dentition, seizures

myotubular myopathy

X-linked recessive maturational arrest of fetal muscle Dx: muscle biopsy with small muscle fibers and abnormal nuclei polyhydramnios in utero

ewing sarcoma vs osteosarcoma

Xray: onion skinning or moth eaten vs sunburst pathology: undifferentiated vs spindle cells presentation: systemic symptoms vs painful mass location: diaphysis vs metaphysis

Carbamoyl phosphate synthetase deficiency

[ARe] Hyperammonemia; no orotic aciduria

what is a venous hum

a benign murmur of childhood A continuous 2/6 murmur heard over the anterior portion of the upper chest throughout systole and diastole and varies in intensity as the head is moved up and down and from side to side

Oppositional defiant disorder

a childhood disorder in which children are repeatedly argumentative and defiant, angry and irritable, and in some cases, vindictive usually only directed at authority figures lasting > 6 months

urethral prolapse

a condition in which the mucous membranes of the urethra protrude into the vagina and form a tiny, DONUT-shaped mass of tissue that bleeds easily sxs: difficulty urinating, dysuria, blood in underpants, no systemic signs of illness Rx: topical estrogen cream BID for 2 weeks

spermatocele

a cyst that develops in the epididymis and is filled with a milky fluid containing sperm painLESS does not change in size with valsalva +/- transilluminates

Nail-Patella Syndrome

a dominantly inherited disorder with malformations of the nails and the kneecap 50% have hematuria some progress to renal failure

when is it ok to use verapamil in kids

a fib but NOT in infants PSVT but NOT in infants

cellulitis adenitis syndrome

a form of late onset GBS facial cellulitis, pre-/postauricular lymphadenopathy, and/or submandibular cellulitis/lymphadenopathy usually have positive blood and CSF cultures Rx: amp, gent (or cefotaxime), vanc

tyrosinemia

a genetic disorder that results in increased blood levels of the amino acid tyrosine sxs: poor weight gain, hepatomegaly, FTT, liver failure, RTA presenting as Fanconi syndrome and rickets on xray imaging dx: urine succinyl acetone RX: NTBC

what is macrophage activation syndrome

a life-threatening condition that develops with severe sJIA -also caused by EBV, Parvo, varicella, drugs

what is a genetic syndrome

a recognizable pattern of structural defects due to a single genetic etiology with a predictable natural history

ulcerative colitis

a/w seronegative spondyloarthropathies, erythema nodosum, pyoderma gangrenosum, PSC, DVT/PE's Rx: 5ASA (sulfasalazine)- induce and maintain remission steroids - induce remission infliximab/adalimumab (remicaide or Humira) - induce and maintain remission, anti TNF aza/6MP - maintain remission, purine analogs MTX - maintain remission, impair DNA synthesis screening: after 8 years of disease, need a scope q2 years

classical triad of choledochal cysts

abd pain jaundice palpable RUQ mass may also have fever, nausea, vomiting and pancreatitis dx: radionuclide scan with accumulation of tracer in the cysts, ERCP/MRCP for anatomical mapping, US with intra/extrahepatic biliary duct dilation *increased risk of cholangiocarcinoma so must remove cysts/GB and reconstruct them

Acute Intermittent Porphyria

abd pain, N/V, peripheral neuropathy, progressive weakness +/- sz triggered by drugs (barbituates, sulfa, antiseizure, griseofulvin, synthetic estrogens), EtOH, steroids, low calorie diets dx: urine + for PBG Rx: IV glucose of heme

what are risk factors for development of metabolic syndrome in children

abdominal obesity genetic predisposition lack of exercise

what diarrheal disease are associated with chylomicron abnormalities

abetalipoproteinemia hypobetalipoproteinemia chylomicron retention disease

phocomelia

abnormal limb shortening associated with maternal use of thalidomide

swyer syndrome

absence of Sry gene congenital absence of testes in genotypical male; phenotypical picture similar to ovarian agenesis, because testes never develop, MIF not released and patients have both internal and external female genitalia. decreased levels of circulating testosterone streak testes need removal given high risk of malignancy

thrombocytopenia absent radii

absence of megakaryocytes in bone marrow with associated thrombocytopenia and absent radii BUT present thumbs AR *risk for fatal intracranial hemorrhage during the first 6 months of life if patient survives first six moths, then thrombocytopenia resolves by age 1-3

prune belly syndrome

absent abdominal musculature, b/l cryptorchidism, GU tract abnormalities leading to ESRD sometimes pulmonary hypoplasia which is incompatible with life

where is an ependymoma located and what is its survival rate

abutting the base of the brainstem or top of the spinal cord but not invading 50% if complete resection

which kidney stones do you want to acidify the urine and which do you want to alkalinize the urine

acidify: struvite (staghorn, proteus) stones alkalinize: cystine (hereditary, hexagonal crystals) and uric acid (myeloproliferative syndromes, Lesch-Nyhan)

paroxysmal nocturnal hemoglobinuria

acquired clonal stem cell d/o with surface protein deficiencies leading to complement mediated lysis coombs negative hemolytic anemia, pancytopenia, arterial/venous thromboembolism dx: flow cytometery with + GP1 linked proteins rx: eculizumab, BM tx

placenta acreta vs increta vs percreta

acreta = placenta villi attach to myometrium of uterine wall increta = invade uterine myometrium pancreta = penetrate the scar in placenta acreta, resulting in hemorrhage

what organism is associated with PID 2/2 IUD

actinomyces

ETEC

acute and chronic watery diarrhea in kids < 2 in low to middle income countries

what is the most common cause of childhood ataxia

acute cerebellar ataxia aka acute cerebellitis usually preceded by viral URI or viral GI illness also by group A strep and salmonella 90% recover by 6-8 weeks without therapy sxs: ataxia, hypotonia, dysarthria, tremors, nystagmus

reye syndrome

acute liver disease with hyperammonemic encephalopathy presents with vomiting and AST/ALT elevation eventual PT elevation

acute flacid myelitis

acute paralysis of 1 or more limbs, preserved sensation, and normal mental status, typically within a week of onset of viral symptoms dx: (MRI) of the spine will show extensive longitudinal centrally located inflammation affecting the gray matter of the cord. Cerebrospinal fluid analysis typically reveals a lymphocytic pleocytosis.

what glomerulonephritidies have decreased complement

acute post infectious (decreased C3, immune complexes) membranoproliferative (decreased C3) cryoglobulins SLE (immune complexes) anti GBM disease (goodpastures) (decreased C3, immune complexes)

acute and preventative treatment for migraines in kids

acute: almotriptan in ages 12-17, rizatriptan in ages 6-17 preventative: topamax in ages 12-17

what is the definition of mild, moderate, severe and profound intellectual disability

adaptive skills impairment and mild = 50-70 moderate = 35 - 55 severe - 20-40 profound - 20-25

what is the appropriate measles post exposure prophylaxis in infants < 12 months of age

administer MMR vaccine within 72 hours to those 6-11 months of age and then continue the regular vaccine schedule *if between 72 hours and 6 days, then do IVIG administer IVIG or IMIG within 6 days of exposure to those < 6 months of age -then delay MMR and other live vaccines by 5 months afterwards *if the infant is 12 months and has received first dose of MMR, need to wait 28 days from last dose before giving next dose

CONN syndrome

adrenal aldosteronoma

what is scrotal hematoma on a newborn exam indicative of

adrenal hemorrhage related to asphyxia from birth trauma

allgrove syndrome

adrenal insufficiency achalasia alacrimia

what classes of bacteria is aztreonam active against

aerobic and facultative gram negatives

what activities can children with stage 2 HTN participate in

aerobic exercise and low intensity conditioning should refrain from doing full contact sports *similar to Marfans

how long after pertussis does a kid stay out of daycare

after 5 days (entire rx) of abx

what are three indications for involuntary drug testing in teens

after a seizure after attempted suicide in an emergency such as an accident

at what age are ortolani and Barlow no longer useful?

after the newborn period so > 8 weeks

when does vision reach the adult threshold of 20/20 in a child

age 5 20/30 by age 4 20/40 by age 3

at what age do kids transition from getting DTap to Tdap

age 7

by what age should you be referring to orthopedics for internal tibial torsion aka in-toeing

age 8 years most common form of intoeing forward facing patella with in pointing toe and posterior malleoli

what are risk factors for cerebral edema in a DM1 patient presenting with DKA?

age < 5 years new onset diabetes low PCO2 at presentation high BUN treatment with bicarb lesser response in serum Na after treatment mortality is 20-25%

what side effects are associated with phenytoin

agranulocytosis rash gingival hypertrophy ataxia

what is the treatment of choice for intussusception

air enema for diagnostic and therapeutic unless peritonitis and then needs an ex lap

what type of precautions are needed for measles

airborne

benign rolandic epilepsy of childhood

aka benign epilepsy with centrotemporal spikes (BECTS) dx: eeg with centrotemporal sharp waves on left and right (spike and wave) nighttime only twitching, maybe shaking, drooling, usually have 1 GTC DURING sleep or immediately upon awakening SPEECH difficulty after most children grow out of it by puberty usually responds to monotherapy (oxcarbamazepine) but don't always have to treat may have mild intellectual delay

what hormone is the most important regulator of potassium

aldosterone (a mineralocorticoid) INCREASED aldosterone 2/2 volume depletion, decreased renal perfusion, increased renin, adrenal hyperplasia or cushings; leads to DECREASED K DECREASED aldosterone 2/2 K sparing diuretics, dysfunctional kidneys, ACE-I, NSAIDS, Addisons, heparin; leads to INCREASED K

what androgen problem would you suspect in hypo to normotension, hyPERkalemia and metabolic acidosis

aldosterone deficiency

what androgen problem would you suspect in HTN, hyPOkalemia and metabolic acidosis

aldosterone excess

what are the different presentations for alkaline vs acidic ingestions

alkaline: tasteless, causing liquefication necrosis, leads to scar tissues with strictures (lye, drain cleaner, bleach, antifreeze) acidic: bitter taste, causes superficial coagulation necrosis, leading to eschars (toilet cleaner, battery acid)

TAPVR

all 4 pulmonary veins with oxygenated blood return to the right atrium instead of the left and mix with non-oxygenated blood these kids NEED an ASD

what patterns are seen for estrogen, LH and FSH in female athlete triad

all are low

which kids with HIV need bactrim ppx for PJP

all kids < 1 year kids 1-5 years with CD4 < 500 kids 5+ with CD4 < 200

which kids with HIV need MAC PPX with azithro

all kids < 2 years kids 2-6 years with CD4 < 200 kids 6+ with CD4 < 100

what is the most common genetic cause of liver disease in kids

alpha 1 antitrypsin deficiency three different allele types, ZZ is most severely affected with both liver and lung involvement. liver first then lung in 4th or 5th decade of life

what type of medication is guanfacine

alpha agonist similar to clonidine AE: bradycardia, hypotension, sedation

why do we use amp and gent together for meningitis

althought gentamicin does not cross the blood brain barrier, it does act synergistically with amp to improve killing

what is the treatment for inhalation anthrax vs GI and cutaneous anthrax vs CNS anthrax

amoxicillin vs Cipro + clinda vs cipro + linezolid + meropenem

treatment for enterococci endocarditis

amp and gent

what is the empiric treatment for candidemia in neonates

ampho B

what is the cause of male limited gonadotropin independent precocious puberty

an activating mutation on the LH receptor

jersey finger

an injury to the finger resulting in tearing of the flexor tendon in the fingertip emergent evaluation by hand surgeon is necessary since flexor tendon injuries can contract if not properly treated inability to flex at DIP but preserved PIP flexion

ketamine uses and contraindications

analgesia, amnesia, immobilization, sedation, trance-like state intact airway tone and respiration side effects: laryngospasm, vomiting, hallucinations contra: increased ICP, psychosis, < 3 months of age, glaucoma, eye injury, porphyria, thyroid disorders, active pulmonary infection, cardiac disease

criteria for diagnosis of takayasu arteritis in children

angiographic abnormalities on imaging or aorta or main branches PLUS decreased peripheral pulses or claudication OR blood pressure difference > 10 between arms OR audible bruits OR HTN

what is a genetic association

anomalies seen together without single genetic or developmental etiology Ex: VACTERL

what condition presents in an infant with deep q waves on ekg and heart failure

anomalous coronary artery off pulmonary artery leads to heart failure 2/2 ischemia/infarction

parapharyngeal abscess

anterior compartment swelling of the parotid area

ankle pain after inversion injury

anterior tibiofibular ligament pain pain with palpation at insertion site on fibula

where do brachial cleft cysts occur relative to SCM

anterior to

what maternal antibodies are associated with neonatal lupus

anti Ro/ SSA anti La/ SSB

what antibody is most associated (highest sensitivity and specificity) with impetiginous strep infection

anti deoxyribonuclease B

which antibodies are associated with type II autoimmune hepatitis

anti liver kidney microsomal 1 anti liver cytosol antigen *usually have more severe disease

what broad class of drugs generally decrease effectiveness of OCP's

anti seizure meds *specifically phenytoin which is a CYP induce and decreases OCP effectiveness

which drugs are associated with development of DRESS

anti seizure meds allopurinol minocycline antidepressants NSAIDs

what are the typical Ab's of hypothyroidism

anti thyroid antibody anti TPO antibody

what autoantibody is characteristic of mixed connective tissue disease

anti u1RNP

which drugs are associated with development of toxic megacolon in a newborn

antidepressants and anticholinergics

midazolam uses and contraindication

anxiolysis, amnesia, muscle relaxation side effects: inconsolable crying, hyperactivity, aggressive behavior contra: myocardial depression

when do you need to add flagyl in PID coverage

any time there is a tuboovarian abscess present take for 14 days along with the doxy

congenital syphillis

appear by several weeks of age, no more than 3 months of life pseudoparalysis: painful osteochondritis in long bones, irregular bands of decreased mineralization and focal areas of bony destruction (wimberger sign) snuffles condoloma lata rash: red papulosquamous papules > copper colored crusted lesions hepatomegaly lymphadenopathy jaundice

what is the most common cause of testicular pain in boys age 7-14

appendix testis torsion a remnant of embryology Dx: blue dot sign, bluish hue at the top of the testicle Rx: rest, NSAIDS

at what age can you start using aripiprazole vs risperidone

aripiprazole : age 6 risperidone : age 5

when should HIV kids get menactra

as early as possible! may start dosing at 2 months depending on the vaccine formulation separate doses by 4 months

what drugs may induce G6PD

aspirin nitrofurantoin antimalarial medications chloramphenicol

spasmus nutans

asymmetric nystagmus with associated head nodding and torticollis in early childhood. usually resolves within months to years

what is the first presenting sign in alport syndrome

asymptomatic hematuria later on may develop recurrent gross hematuria associated with viral URI's may also see vision loss and hearing impairment

when do you vaccinate preemie's < 2 kg with Hep B Ag - moms?

at 1 month of age or hospital discharge, whichever comes first weight does not matter for their doses since by 1 month, all babies should be able to seroconvert

when should HIV kids get PCV23

at age 2 years at least 8 weeks after receiving PCV13

pap testing guidelines for teens with HIV

at diagnosis if >21 and then yearly until three normals then can space to q3 years

what are AR diseases

ataxia telangiectasia bloom syndrome fanconi anemia xeroderma pigmentosa dubowitz TAR Wilsons Zellweger Glutaric Acidemia type II Smith Lemli Opitz *all metabolic disorders

which ADHD med carries a black ox warning about increased risk of suicide?

atomexatine aka strattera

what is the treatment for babesiosis

atovaquone + azithromycin x 1-2 weeks If severe then clindamycin + quinine sxs: febrile, hemolytic anemia with hemoglobinuria *maltese cross

what two meds should you give to someone with organophosphate poisoning

atropine first to stabilize the patient and block Ach receptors pralidoxime second to prevent irreversible binding to cholinesterase enzymes

what is the cause of chronic benign neutropenia

autoantibodies to granulocytes diagnosed around 8 months of life and lasts about 2 years

what is the inheritance pattern in hereditary angioedema and what is a treatment for it

autosomal dominant danazole - this increases c1 esterase inhibitor

fanconi anemia

autosomal recessive poor DNA repair mechanisms dx: pancytopenia and hypo cellular bone marrow due to loss of pluripotent HSC, mitomycin c lymphocytic fragility sxs: short stature, microcephaly, low birth weight, horror hyper or hypo pigmented spots, hypoplasia of thumbs and radii, cafe au lait spots, strabismus, deafness *high risk for hematologic malignancies, esp AML Rx: SCT

kostman syndrome

autosomal recessive congenital neutropenia due to myeloid arrest in the bone marrow myeloid cells are insensitive to G-CSF leads to prolonged and dangerous neutropenia at risk for infection related to their own bacteria at future risk for AML

hyper IgD syndrome

autosomal recessive disease of French or dutch descent 2/2 excess IL1 production kids < 1 sxs: recurrent fevers monthly, lasting for 3-7 days can have and pain, N/V, arthritis as well migratory erythematous macular rash Dx: QIGS (IgD and IgA elevations) Rx: NSAIDs, anakinra, etanercept, prednisone, colchicine

osteochondritis dissecans

avascular necrosis of subchondral bone adjacent to articular cartilage usually occurs in males age 8 - 18 with vague knee pain that progresses Joint popping/locking exam: joint effusion or swelling, pain over lateral aspect of medial femoral condyle dx: get xray, may notice that part of the condyle is breaking away in extreme cases Rx: asymptomatic - nothing. symptomatic - rest until no further pain and referral to orthopedics especially if severe separation noted on xray

xray findings of Vit A toxicity

b/l cortical hyperostosis along the midshaft

VUR findings on imaging

b/l hydronephrosis without thickened bladder wall

PUV imaging findings

b/l hydronephrosis, dilated bladder, thickened bladder wall, dilated posterior urethra Dx: voiding cysturethrogram or direct visualization on cystoscopy

how long do you treat bacteremia, pneumonia, meningitis, osteomyelitis and endocarditis/ventriculitis in a neonate?

bacteremia: 10 days pneumonia: 10-14 days meningitis: 14-21 days osteomyelitis: 3-4 weeks endocarditis/ventriculitis: 4 weeks

what is the treatment for PJP PNA in infants

bactrim and steroids

what illicit is associated with nose bleeds

bath salts

why do you have to do b/l testicular fixation after testicular torsion

because the other testes is at increased future risk of torsion

what vasculitis can involve >1 blood vessel size and type

behcets

what causes a false negative PPD

being < 10 weeks post exposure from active TB severely disseminated TB aka miliary TB anergy

Ramsey-Hunt syndrome

bells palsy, vertigo, ataxia, hearing loss associated with VZV rash involving face and ear, specifically facial nerve need to treat promptly with acyclovir +/- steroids to decrease risk of hearing loss and permanent facial nerve palsy

thyroid binding globulin deficiency

benign condition leads to normal TSH, decreased total T4, normal free T4 (active hormone)

harlequin color change

benign dramatic color change due to imbalance of autonomic vascular regulatory mechanisms of cutaneous blood vessel tone occurs most commonly in preterm infants color returns to normal in a few minutes rare after 3 weeks of age

which CNS vascular malformation Is associated with APCKD

berry aneurysms in the circle of willis

what meds commonly trigger telogena fluvium

beta blockers amphetamines ACE-I OCP's retinoids Lithium

during which sexual maturity rating to girls achieve greatest height velocity

between 2 and 3

what characteristics about Loeys Dietz syndrome make it different from Marfan syndomre

bifid uvula craniosynostosis cleft palate generalized arterial tortuosity along with dissections throughout the entire arterial tree

Langherhans cell histiocytosis

birbeck granules (tennis racket inside cytoplasm of cells on biopsy) frequent diaper rashes with yellow crusting and petechia a/w facial edema, seizures, hearing loss, refractory otitis media, proptosis, diabetes insipidus, and/or cranial nerve palsies, spontaneous PTX Rx: oncologist

what is normal and abnormal for genu varum and vulgum

birth to 2 years: genu varum (bowing) 2 years to 4 years: genu valgum (knock knees) 4+ years: levels off

what blood smear result occurs with G6PD

bite cells Heinz bodies

what does crossing legs to avoid incontinence signify

bladder spasms

epidermolysis bullosa

blistering skin disease classified into SIMPLEX (with trauma), JUNCTIONAL (w/ or w/out trauma) and DYSTROPHIC (mitten finger deformities, SCC, esophageal strictures) based on level of epidermal skin separation dx: skin biopsy

hyphema

blood in the anterior chamber of the eye, usually appearing as murkiness 2/2 blunt trauma Rx: topical steroids, cycloplegia drops at risk for later development of glaucoma is repeat events

what are the side effects of linezolid

bone marrow suppression irreversible neuropathy serotonin syndrome

side effects of zidovudine

bone marrow suppression myopathy macrocytosis

what are complications of organic acidemias

bone marrow suppression pancreatitis

what are the LH and FSH levels in Turner's syndrome

both are elevated

how to differentiate 21 a OH vs 11 B deficiency in CAH based on exam

both will have virilization 11B will have HTN and hypokalemia (excess mineralocorticoid) 21 OH will have lethargy, hyperkalemia, hyponatremia (all testosterone)

what is a serious complication of unrepaired TOF

brain abscess

what dietary restrictions should be provided for maple syrup urine disease

branched chain amino acid restriction (leucine, isoleucine, valine)

how do breast milk and cows milk differ

breast milk has more iron and carbs, less protein and calcium, same amount of fat as cow's milk

what is premature thelarche and it's work up

breast tissue development <.2 years of age bone age: within 2 standard deviations from her chronological age estradiol levels: normal, < 10 LH/FSH: FSH predominant indicating prepubertal

what is a notable side effect of adenosine

bronchoconstriction resolves with albuterol

what tumor is associated with wiskott Aldrich and ataxia telangiectasia

burkitts! usually high grade and fast growing

by what age should you have identified infants with hearing loss

by 3 months so they can start receiving therapies by 6 months

when should infants have conjugate gaze?

by 6 months of life random b/l disconjugate gaze is normal between 2 and 4 months of life

which ANCA is positive in GPA

c-ANCA specific for PR3

medullary thyroid cancer hormone secretions

cacitonin (c-cells) ACTH melanocyte stimulating hormone histamine serotonin prostaglandin somatostatin

McCune Albright

cafe au lait spots with "Coast of Maine" borders precocious puberty polyostotic fibrous dysplasia of skeletal system complicated by bone pain, fracture or bony deformity or asymmetric growth hyperfuncitoning hormones leading to hyperthyroid and maybe a goiter dx: decreased LH/FSH, no response to GnRH, increased GH Rx: GnRH if dependence, octreotide for GH excess

what type of crystals form in ethylene glycol toxicity

calcium oxalate crystals

what is the most common infectious cause of bloody diarrhea in kids

campylobacter

PPI use within the past 24 hours causes a false positive result for which drugs?

cannabis

what can be treated with activated charcoal

carbamazepine TCA's tylenol amphetamines antihistamines ASA barbituates benzos beta blockers CCB's cocaine opioids theophylline pheny valproic acid

what are the potential energy from fats, proteins and carbohydrates?

carbs and proteins = 4 kcal/g fats = 9 kcal/g

what types of infection do chronic granulomatous disease kids get

catalase positive organisms serratia, s. aureus, candida recurrent fungal infections (aspergillis) Dx: neutrophil function test and DHR123 flow cytometry, or dihydroademine oxidation test

what eye manifestations are associated with congenital rubella

cataracts

hepatitis D

cause more severe or fulminant disease in hepatitis B patients increases likelihood of chronic disease immunity to hep B implies immunity to hep D since it is only acquired with hep B

internal femoral torsion

cause of intoeing medial location of patella and toes with walking with decreased external rotation of the hips "W" sitting position Rx: surgery if functional limitations or > 11 years

wolfram syndrome

central DI optic atrophy deafness diabetes mellitus

what is the etiology for low TSH and low free T4

central or secondary hypothyroid

ataxia telangiectasia

cerebellar ataxia and occulocutaneous telangiectasias accumulated DNA strand breaks resulting in cell death sinopulmonary disease, bronchiectasis dx: elevated afp in kids > 5, otherwise telangiectasias, decreased IgA and IgE

mercury poisoning symptoms

cerebral atrophy, seizures, developmental delay

which chiari malformation is associated with myelomeningocele

chiari type II have defect involving meninges, spinal cord, vertebral bodies. Low defects are better. Need urgent repair

what is window prophylaxis with TB testing and treatment

children < 4 exposed to active TB need to start latent TB treatment and get a CXR due to HIGH risk for disseminated disease. Get initial TST and follow up 10-12 weeks later. if follow up is negative, then stop latent TB treatment

painFUL lymphadenopathy painLESS genital ulcer

chlamydia trachomatis

what are evidence of chronic sinusitis

chronic cough worse with laying down and upon awakening halitosis occurring for > 12 weeks Rx: 2-4 weeks of abx

what is primary sclerosing cholangitis

chronic fibrosing inflammation of intra and extra hepatic bile ducts associated with IBD occasionally ANCA +

bronchiectasis

chronic wet cough and persistent wheezing irreversible dilation of bronchioles after damage dx: high res ct or cxr, pft's with obstructive pattern and air trapping a/w clubbing of fingers

chronic bacterial bronchitis

chronic wet non specific cough in a child for > 4 weeks without abnormalities on physical exam, cxr, spirometry rx: 2-4 weeks of augmentin

what drugs are responsible for gyneocmastia

cimetidine ACE-I -azoles CCB's atypical antipsychotics aldactone steroids marijuana amphetamines heroin/methadone INH TCA

what is the most common bacteria to cause intracranial abscesses

citrobacter

what sources of neonatal bacteremia have high likelihood of brain abscess

citrobacter, serratia, proteus

complement pathways

classical: activated by IgM or 2 x IgG lectin: binds mannose on pathogen surface Alternative: Bacterial cell wall cleaves C3 to activate *then C3b opsonizes the target and the MAC complex forms

what is the first treatment after a snake bite

cleansing the wound and immobilizing it

what antibiotics do you use in animal bite with pcn allergy

clindamycin + bactrim

crouzon syndrome

closure of bl coronal sutures have normal IQ, hands and feet

carpenter syndrome

closure of multiple sutures low IQ CHD orthopedic anomalies corneal opacifications

2nd most common plagiocephaly

closure of the coronal suture leading to asymmetric orbits and a "windblown" appearance aka anterior plagiocephaly

what heart condition is associated with rib notching on CXR

coarctation of the aorta 2/2 erosion of ribs from large collaterals will also see lower BP in lower extremities as compared to upper extremities

which kids do NOT need recurrent PCV23 q5 years

cochlear implants kids with chronic illness other than those at risk for encapsulated organisms (immunocompetent)

what are two ways to distinguish breath holding spells from seizures

color change (pale - trauma/vasovagal; cyanotic - shrill cry) and inciting event are indicative of breath holding spells

familial hematuria

common autosomal dominant cause of persistent microscopic hematuria without proteinuria

Paraphimosis

condition in which a retracted prepuce cannot be pulled forward to cover the glans, leading to swelling and discomfort

what type of hearing loss is associated with treacher collins?

conductive hearing loss 2/2 hypoplasia of the middle ear also have loss of lower eyelid lashes, choanal stenosis or atresia, cleft lip and palate

what bone condition is associated with NF1

congenital PSEUDOARTHROSIS aka false joints unilateral anterolateral angulation (bowing) of the tibia, progressing to thinning of the cortex and pathologic fracture along with SPHENOID DYSPLASIA

neonatal lupus

congenital heart block requiring pacemaker discoid rash that resolves at 6-9 months thrombocytopenia neutropenia elevated transaminases

moyamoya syndrome

constriction of certain cerebral arteries resulting in TIAs or hemorrhage, recurrent headaches and ams common in asian descent, sickle cell, nf1, trisomy 21 dx: stenotic or occlusive lesion in distal carotids and circle of willis on MRI

Hepatitis E

contaminated water after monsoon flooding in travelers high risk for fulminant hepatitis in 3rd trimester of pregnancy with 20% maternal fatality

what are contraindications for mefloquine?

contraindicated in patients with epilepsy, psychiatric disorders, arrhythmia, cardiac conduction defects is very useful for pregnant patients though

what are contraindications and precautions for rotavirus vaccine administration

contraindication: hx of intussusception precautions: bladder extrophy or spina bifida, moderate to severe gastroenteritis, other current GI issues, immunodeficiency condition

how does a child present with sinusitis

cough, nasal discharge, and halitosis an adult would more likely have facial pain, headache, tenderness to palpation of the sinuses

what are poor prognostic signs at presentation for IgA nephropathy

cr > 1.5 HTN persistent proteinuria > 500 mg/day extensive mesangial proliferation on renal biopsy

apert syndrome

craniosynostosis spectrum disorder closure of multiple sutures FGFR- related disorder cloverleaf skull intellectual disability mitten hands

failure to thrive definition

crossing 2 growth percentiles weight < 3rd %ile weight for heigh < 5th %ile

what skeletal anomaly is turner's associated with

cubitus valgus hyperconvex nails

what complications are common after supracondylar fracture

cubitus varus (gunstock deformity) brachial artery compression medial or radial nerve compression compartment syndrome leading to Volkmann contracture

Curling vs Cushing ulcer

curling: burn patient cushing: head injury or brain surgery patient

what is the treatment for anthrax in all its manifestations

cutaneous: 7-10 days amox inhalation or GI: cipro AND clinda CNS: cirpo + mero + linezolid *drain any pleural fluid to get rid of lethal toxin *add antitoxin to all treatments PePX: cirpo or doxy x 60 days

mastocytosis

cutaneous: urticaria pigmentosa from mast cells and melanocytes systemic: extracutaneous sites including bone marrow, abdominal symptoms, flushing, fatigue dx: tryptase > 20, skin bx, BM Bx rx: stay away from cold, heat, etoh, asa, opiates

what are the most common viral triggers for SJS

cytomegalovirus mycoplasma pneumonia (can form its own type of mucositis as well!)

what is the risk of Downs if mom vs dad has a 21:another chromosome translocation

dad: 1-2% mom 10-15%

how do you change insulin pump settings for an athlete

decrease basal infusion rate to lower the risk of hypoglycemia decrease it at least 1-2 hours prior to exercise and encourage them to eat a meal 3-4 hours before

5 alpha reductase deficiency

decreased DHT production leads to ambiguous external genitalia but normal testosterone (small penis, bifid scrotum, blind vaginal pouch, testes in inguinal canal) will develop more male appearance at puberty

what teratogenic effects do ACE-I have

decreased GFR and fetal urine = oligohydramnios limb and hand anomalies; widely separated eyes; small, low-set, and posteriorly rotated ears; and facial deformities pulmonary hypoplasia leading to death myelomeningocele and CNS malformations CHD (PDA, VSD) *Do not even BREASTFEED

secondary hypogonadism

decreased LH/FSH, decreased gonadal androgens (problem with the pituitary) dx: 8 am testosterone < 20, maybe other deficient pituitary hormones

sexual development in Kallman syndrome

decreased gonadotropin development (LH/FSH, GnRH) leading to small testicular size normal adrenal androgen development leading to normal pubic hair *also associated with cleft lip/palate, renal and cardiac anomalies, and color blindness, mirror movements (opposite hand mirrors active hand), hearing loss, GU anomalies

common variable immunodeficiency

decreased levels of IgG, IgA, IgM along with poor function 2/2 inability of B cells to transition to plasma cells recurrent sinopulmonary infxn with encapsulated organisms sarcoid like disease a/w ITP, pernicious anemia, hemolytic anemia, malabsorption, pancytopenia, polyarthritis rx: IVIG

X-linked agammaglobulinemia

decreased levels of all Ig subclasses 2/2 arrest of B cell pre-cursors many immature B cells in circulation small or absent lymph nodes and tonsils Rx: IVIG

6 sigma

decreased variation in order to increase quality by making the process more predicable and effective adapted from manufacturing

bloom syndrome

defect in DNA repair mechanism causing T cell deficiency small stature, telangiectasias, CNS abnormalities, immunodeficiencies high association with leukemias

Legg-Calve-Perthes Disease

degeneration of femoral head due to idiopathic avascular necrosis. presents with pain, decreased ROM, antalgic gait, positive Trendelenburg sign. primary treatment focus is to relieve pain and maintain femoral head in proper position.

what developmental delay occurs in achondroplasia

delayed gross motor these resolve by age 2-3

williams syndrome

deletion on chromosome 7 intellectual disability cocktail personality elfin-like facial features supravalvular aortic stenosis hypercalcemia

WPW

delta wave of pre-excitation through bundle of Kent a/w ebstein anomaly rx: procainamide, radiofrequency ablation AVOID BB, CCB, digoxin as may induce fatal arrhythmia

subacute sclerosing panencephalitis

dementia, myoclonus, seizures by age 10 death follows

xray findings of chronic lead intoxication

dense bands at the metaphysis of long bones

staph scalded skin syndrome

desquamating dermatitis 2/2 toxin presenting with fever and + Nikolsky sign at risk for superinfection

Ewing sarcoma on xray

destructive lesion described as moth eaten sis: insidious pain over weeks or months

ARPKD

detected after 24 weeks gestation on prenatal US US shows large echogenic kidneys ("salt and pepper kidneyds) and corticomedullary differentiation develop liver dysfunction (hepatic fibrosis) and failure later on a/w potters sequence 2/2 oligohydramnios

2 step dexamethasone suppression test

determine if ACTH dependent process 2 doses with the larger dose suppressing urinary free cortisol and serum cortisol

thyroid scan

determines how much technetium localizes to the thyroid determines hot vs cold nodule gives a picture

what does incontinence a/w bouncing up and down on the soles of feet signify

detrusor instability

essential fatty acid deficiency

developmental delay poor growth eczematous skin lesions poor wound healing increased susceptibility to infection

what is the treatment for methanol or ethylene glycol ingestion

dialysis + fomepizole + Na Bicarb

how long after shiga toxin E coli does a kid have to stay out of daycare

diarrhea has resolved and 2 negative stool cultures

what causes warfarin and diclofenac drug drug interaction

diclofenac displaces albumin bound warfarin leading to warfarin toxicity and supratherapeutic INR

what is one cause during infant delivery for vocal cord paralysis

difficulty delivery with injury to recurrent laryngeal nerve from stretching on the. neck and brachial plexus other causes: arnold chiari malformation, posterior fossa tumors, hydrocephalus sxs: absent or weak cry, aspiration with foods

RDS CXR

diffuse hazy/granular/ground glass pattern air bronchograms decreased lung volumes

congenital biotin deficiency

diffuse, pink to red, scaly patches with or without erosions alopecia

what is the most common type of cardiomyopathy in kids

dilated

what conditions will cause direct vs indirect coombs +

direct: AIHA *may have false negatives due to IgM or IgA) indirect: erythroblastosis fetalis (mom has Ab's to Rh), incompatible transfusions

guideline for tetanus re-vaccination pending wound status

dirty and incomplete vaccination: give IMMUNOGLOBULIN and vaccine UTD: NTD, no matter if dirty not UTD: give vaccine, > 5 years for dirty, > 10 years for clean

what disseminated virus may start with back pain and fever and then progress to a rash

disseminated varicella fever, liver involvement, pneumonitis, low platelets, coagulopathy, encephalitis, renal dysfunction

xray findings for vit C deficiency

distinct white lines at distal ends of tibia/fibula ground glass bones a/w irritability, generalized tenderness in lower extremities

alice in wonderland syndrome

distorted perception of size relative to size around may occur with mono!

what two neurotransmitters to ADHD meds act on

dopamine and norepinephrine

russell sign

dorsal hand calluses from induced vomiting

what is the most common vascular ring cause of stridor with negative barium swallow

double aortic arch

when does birth weight double, triple and quadruple by

doubles by 4 months triples by 12 months quadruples by 2 years

what is the expected babinski in GBS and why?

down-going (normal) because this is a demyelinating disease of peripheral nerves aka Lower motor neurons rather than upper motor neurons

what is the treatment for brucellosis

doxy x 6 weeks + aminoglycoside x 2 weeks

what is the treatment for nasal septum hematoma

drainage, nasal packing and antibiotics

what is the best low to no progesterone option for PCOS

drospirenone avoid any -gestrels or norethindrone

what is the difference between becker and duchenne's muscular dystrophy

duchennes: more progressive, usually earlier onset, complete ABSENCE of dystrophin in muscles beckers: less progressive, later onset, PARTIALLY DYSFUNCTIONAL dystrophin in muscles both are x linked recessive

primary dysmenorrhea

due to excess endometrial prostaglandins leading to increased smooth muscle contractility and causing vasodilation and platelet dysfunction may activate the GI tract and cause pain most common cause of abdominal pain related to menstrual cycle in teens

what minerals/vitamins are absorbed in the duodenum, jejunum and ileum

duodenum: iron, folate, calcium jejunum: nothing ileum: vitamin b12, bile salts, ADEK vitamins

restrictive lung disease

dx: decreased TLC, decreased RV

what type of CP is associated with kernicterus

dystonic CP a more severe form of dyskinetic (random mvmts)

which infection is galactosemia associated with

e coli sepsis

how do you calculate the age of a child based on the type of person they draw?

each feature (nose, mouth, 2 arms, 2 eyes, 2 legs) counts for 1/4 points add this onto a base of 3 and gives you the age of the child in years.

what characteristics of a suspected cholesteatoma should prompt referral to ENT for immediate evaluation

ear drainage > 2 weeks white-gray cystic mass behind the TM new onset hearing loss in a previously operational ear

late vs early vs variable decels

early - 2/2 head compression during uterine contraction late - 2/2 uterine placental insufficiency variable - 2/2 cord compression, get an acceleration first and then a deceleration *sinusoidal - severe fetal anemia likely 2/2 hypoxia or Rh incompatibility

late vs early onset hypocalcemic seizures

early onset (within first few days, stuff with birth or mom primarily): asphyxia, prematurity, IGUR, IDM, severe maternal vit d deficiency late onset (end of the first week, stuff with baby or mom): hypoparathyroidism maybe with DiGeorge, hypomagnesemia, rotavirus, phototherapy, maternal hyperparathyroidism, renal failure

presentations for late vs early onset listeria in newborns

early onset = < 1 week: sepsis or PNA, sometimes as granulomatosis infantiseptica (graunolomas all over the baby) late onset = > 1 week: meningitis

what screening is required for Trisomy 21 patients

echo as newborn eye exam by 6 months and 4 years hearing screen at birth and at 6 months thyroid screen at birth, 3, 6, and 12 months, then annually spine xray prior to sports participation

what screening is required in turners syndrome

echo q5-10 years for aortic dissection thyroid screening yearly renal ultrasound at diagnosis for horseshoe kidney

what does normal intentional voiding but continuous leakage signify

ectopic ureter

"Arkansas" "tick bite" "pancytopenia"

ehrlichiosis south central, south east and East Coast of the US, while Anaplasma infections (usually present similarly) are in upper Midwest and the northeast US

what neurotransmitter level trends are associated with aggressive behavior

elevated dopamine depressed serotonin depressed GABA

what is the treatment for TTP

emergent plasmapheresis and steroids

characteristics of enthesitis related arthropathy (ERA)

enthesitis and oligoarthritis OR either with 2: sacroiliac joint tenderness or inflammatory lumbosacral pain, HLA B27, onset in male > 6, acute asymptomatic uveitis, 1degree relative with ERA or IBD or sacroilitis or reactive arthritis

at what gestational age does lanugo cover the entire body vs absent on face vs only on shoulders vs none

entire body - 22-32 weeks absent on the face - 33-37 weeks shoulders only - 38-41 weeks none - > 42 weeks

what cbc finding is associated with chlamydia pneumonia

eosinophilia

clinical presentation of epidural vs subdural hematoma

epidural: has a lucid interval and then develops progressively decreasing levels of consciousness, develop a biconvex pattern on CT, 2/2 shearing forces on bridging veins (middle meningeal artery) rx: always surgery subdural: slower to increase in size, 2/2 compression forces, crescent shaped (BANANA) rx: surgery depending on size

how to distinguish epithelial vs epstein's pearl

epithelial are found along the gums epstein are found on the roof of the mouth

what additional findings are associated with erbs and klumpke's palsy

erb's: diaphragmatic paralysis, intact palmar grasp Rx: abduction and external rotation x 3 month klumpke's: horner's syndrome, no palmar grasp Rx: wrist/arm splint x 3 months

lymphogranuloma venereum

erosive painless ulcers on shaft of penis afterwards get large collection of inguinal nodes at risk for proctocolitis in MSM Rx: doxy x 3 weeks Due to chlamydia species

what antidepressants are approved for teen depression

escitalopram fluoxetine

what is the most common form of TEF

esophageal atresia (proximal pouch) with distal TEF

what happens with ingestion of drain cleaner

esophageal burns and later strictures

when do you need to treat with antibiotics for a cat or dog bite

essentially treat all cat bites since they create a puncture wound otherwise treat any hand/foot/face/genitals bites, any crush injuries, and anyone immunocomprimised *always treat human bites!

what is the treatment for absence seizures

ethosuximide

conductive vs evaporative vs convective vs radiant heat loss

evaporative: loss due to constantly being wet radiant: loss to surrounding air conductive: loss by transfer to another object convective: loss by moving air around

who do you need to provide rifampin ppx for in a daycare with > 2 cases of H flu?

everyone at the daycare and any household contact! regardless of age or immunization status provide rifampin 20 mg/kg daily x 4 days

what would be the quad screen results for trisomy 18

everything decreased

Tay-Sachs disease

exaggerated startle reflex in infancy a disorder that produces blindness and muscle degeneration prior to death; there is no treatment cherry red spot on macula

what are signs of infantile glaucoma

excessive tearing, photophobia, blepharospasm corneal edema, corneal and ocular enlargement, conjunctival injection, visual impairment

what are requirements for bariatric surgery

failure to lose weight after 6 months of attempts late SMR 4 or 5 BMI > 40 with serous comorbidities OR BMI > 50 with less severe comorbidities

what are the criteria for gastric bypass surgery in pediatrics

failure to lose weight after ≥ 6 months of organized attempts at weight loss physiologic maturity (late SMR 4, SMR 5) BMI ≥ 120% of the 95th percentile of BMI for age with a severe comorbidity (e.g., poorly controlled Type 2 diabetes, hypertension, obstructive sleep apnea, hypoventilation syndrome, nonalcoholic fatty liver disease, pseudotumor cerebri) OR BMI ≥ 140% of the 95th percentile for age with less severe comorbidities.

Hirschsprungs

failure to pass meconium within 48 hrs of birth dx: absence of ganglion cells on suction rectal biopsy

what is the most important factor of predicting sexual assault

family structure

what are the tests to diagnose diabetes

fasting glucose > 126 classic symptoms and random glucose > 200 2 hr plasma glucose > 200 during OGT A1C > 6.5 repeat any abnormal value on a subsequent day for DM2, should screen those with RF at 10 years or at puberty if < 10 years of age

what labs must be checked prior to starting isotretinoin

fasting serum triglycerides LFT's and urine pregnancy test risk of reversible hypertriglyceridemia

side effects of protease inhibitors

fat redistribution and lipid abnormalities *do not treat with simvastatin or lovastatin

what is the test of choice for protein losing enteropathy

fecal alpha 1 anti trypsin level this acts as a creatinine surrogate for the intestines

what causes a leftward shift (increased hemoglobin affinity for oxygen) on oxyhemoglobin dissociation curve

fetal hemoglobin, carbon monoxide, methemoglobin

Staph toxic shock syndrome

fever > 102 diffuse macular sunburn-like erythema hypotension >3 organ systems involved

presentation of polyarteritis nodosa

fever, malaise, night sweats, weakness erythematous nodules on skin if kidneys are affected then HTN, hematuria NO lung involvement mononeuritis multiplex (foot drop) *associated with chronic hepatitis B Dx: P-ANCA positive, angiography

what are the symptoms of serum sickness

fever, purple urticaria, arthralgias, proteinuria, lymphadenopathy, malaise

what are the first permanent teeth to errupt

first molars

mycobacterium marium

fish tank bacillus non healing skin ulcers Rx: ethambutol + rifampin

which antibiotics are not ok to breastfeed while taking

flagyl, tetracyclines, macrobid, primaquine

at what gestational age is the ear flat vs incurving vs fully curved

flat - 24-33 weeks incurving - 34-35 weeks upper 2/3 incurving - 36-38 weeks fully curved - > 39 weeks

TTN CXR findings

flattened diaphragm prominent pulmonary vascular markings fluid in the fissures mild cardiomegaly

how does listeria present in pregnant women

flu like illness in 2-3rd trimester usually deliver before 35 weeks gestation usually have green or brown tinged amniotic fluid infants have respiratory distress and cxr shows streaky or miliary pattern

what anti fungal should be added to ampho B when treating neonatal candidal meningitis

flucytosine

what is the most appropriate imaging modality for suspected diaphragmatic paralysis?

fluoroscopy with "sniff test"

how long should a child remain in rear facing car seat?

for as long as possible whenever they are too tall or weigh too much, then they can be front facing most car seats can accommodate up to 40 lbs or >2 years of age still rear facing.

calcium correction for albumin

for each 1 decrease in albumin, Ca increases by 0.8

anion gap correction for albumin

for each 1 decrease in albumin, add 2.5 to anion gap for each 1 increase in albumin (above 4), subtract 2.5

how do you calculate mid parental height

for girls: average of (dad's height - 5 inches or 13 cm) + mom's for boys: average of (mom's height + 5 inches or 13 cm) + dad's

why do we use amp + gent or amp + vanc in treating enterococcus in neonates

for its synergistic killing of the organism rather than the inhibitory mechanism when used solo

when would you use hepatitis A IMIG

for pre exposure PPX for kids < 6 months 0.1 mL/kg protects for 1 month 0.2 mL/kg protects for 2 months IVIG is not recommended Also for post exposure PPX < 12 months

what is the common mechanism of injury in patellar subluxation and dislocation

forceful quadriceps contractions during a cutting motion

what are appropriate second line agents for status epilepticus

fosphenytoin valproic acid levitiracetam

Hemoglobin Barts

four gamma globin chains Alpha thalassaemia tested in newborn screening.

what are the symptoms of copper deficiency

fragile or kinky hair depigmented skin myeloneuropathy edema HSM osteoporosis ataxia neuropathy

what condition is associated with FMR1 gene defects

fragile x

how are hookworms contracted

from penetration through the skin while walking barefoot in soil

ambylopia

functional decrease in visual acuity 2/2 disuse or misuse of visual pathways most common cause of decreased vision in those < 45 Rx: patch the good eye

what test must be used for DDH after 8 weeks of age

galeazzi look for asymmetric height between the two knees

what GI finding is associated with Kawasaki's

gallbladder hydrops aka acute GB distention self-limited, resolves on own

what congenital anomaly is associated with a vascular event to the right umbilical vein

gastroschisis

what type of infection is cellulitis adenitis associated with

gbs sepsis

congenital adrenal hyperplasia

genetic disease in which the adrenal gland is overdeveloped Increased ACTH stimulation > malfunctioning 21 OH > increased transition to testosterone and DHEA production female virilization at birth male adrenal salt crisis at 1-2 weeks (FTT, vomiting, hypotension, hyponatremia) labs: elevated testosterone, decreased cortisol, elevated ACTH, elevated 17 Oh progesterone

what is gitelman syndrome

genetic mutation leading to renal magnesium wasting hypokalemia metabolic alkalosis hypocalciuria

what do you need to do next in a patient < 24 months of age with febrile UTI and evidence of hydronephrosis or scarring on imaging?

get a VCUG

radioactive iodine uptake

give a dose of radioactive iodine and 24 hours later scan to determine how much was taken up by thyroid evaluates hyPERthyroidism increased in Graves and hot nodule (multinodular goiter, toxic solitary nodules, HCG secreting tumor) decreased in self-limited thyroiditis, amiodarone gives a #

what should you do if a partially immunized kid has a significant exposure to measles, mumps or rubella

give the second dose of vaccine within 72 hours of exposure

how do you protect newborns exposed to pertussis

given them prophylactic treatment with azithromycin x 5 days

what is the most common primary CNS tumor in children

gliomas -astrocytoma, DIPG, diencephalic, GBM

Gaucher disease

glucocerebrosidase (β-glucosidase) def. → ↑ glucocerebroside most common lysosomal storage disease BONY deformities *aseptic necrosis of femur* aka erlenmeyer flask deformity, Gaucher cells (lipid-laden Mφ's, crumpled tissue paper within bone), hepatosplenomegaly, seizures, blood manifestations NON-CNS, only visceral involvement

what disorder should you suspect in a neonate with the odor of sweaty feet

glutamic acidemia type 2 AR severe hypoglycemia, metabolic acidosis, hyperNH3 as well cardiomyopathy, facial dysmorphism and severe renal cystic dysplasia

what tumor causes peripheral precocious puberty and is associated with Turner's syndrome

gonadoblastoma more common in mosaic turners leads to excessive linear growth, acne, clitoral enlargement, pubic and axillary hair Increased levels of testosterone Rx: remove dysgenetic gonad

how do testes vs ovaries form

gonads respond to SRY gene (on Y chromosome) or absence to differentiate into testes or ovaries respectively testes therefore make mullerian inhibitory signal (Sertoli cells) which inhibit ovaries formation testes also make testosterone (from Leydig cells) which helps form Wolffian ducts (epididymis, vas deferens, seminal vesicle)

elevated PR3 ANCA Ab in cytoplasmic distrubution

granulomatosis with polyangitis vasculitis (wegeners)

Amsel criteria of bacterial vaginosis

grayish vaginal discharge that coats pH > 4.5 fishy odor before or after KOH test clue cells

what is ambivalent attachment style

great distress when removed from parents and difficult to console

what infection is associated with a diffuse fine maculopapular rash most prominent in inguinal, antecubital, axillary areas and noted to have circumoral pallor

group A strep

which bacteria is highly associated with necrotizing fasciitis after varicella infection

group A strep

what infections are associated with erythema nodosum

group b strep mycoplasma EBV yersinia coccidioides mycobacterium

what adverse effects can occur with tacrolimus

gum hypertrophy

what adverse effects can occur with cyclosporine

gum hypertrophy hirsutism HTN tremors

what is the usual cause for abdominal pain that wakes kids up from sleep

h pylori

Chancroid

haemophilus ducreyi Painful, unilateral, inguinal LAD following tender papule on genitals papule > erodes > ulcerates > gray fibrinous membrane Dx: gram stain with "schools of fish" Risk factor for HIV transmission Rx: azithro or CTX

what murmur is associated with AV canal defect

harsh holosystolic murmur at LLSB and mid diastolic rumble with apical pansystolic murmur

etomidate uses and contraindication

head trauma (reduces ICP) maintains hemodynamics sedation contra: adrenal insufficiency

propofol uses and contraindications

head trauma (since it reduces ICP) no analgesia contra: egg allergy, soy allergy, porphyria

what is the difference between heat exhaustion and heat stroke

heat exhaustion = temperature instability heat stroke = heat exhaustion + CNS abnormalities (altered mental status, seizures)

what is the difference between heat stroke and heat exhaustion

heat stroke: mental status changes, temp > 40 heat exhaustion: < 40, muscle cramps

which liver tumor is associated with a normal AFP and potentially multiple liver lesions

hemangioendothelioma

Kassabach-Merrit Syndrome

hemangioma thrombocytopenia syndrome platelet destruction within the vascular tumor Rx: propranolol, steroids, laser therapy

what is HBH

hemoglobin H disease 3 affected loci in alpha globin genes leading to moderate anemia of alpha thalassemia Hgb electrophoresis starts to have increased levels of hemoglobin bart

hypophosphatemia of refeeding syndrome

hemolytic anemia arrhythmias heart failure mental status changes coma sudden death

vibrio

hemorrhagic bullae, LAD, sepsis *especially in liver disease

what screening should be done prior to starting biologic agents in JIA

hep b hep c latent TB after starting: avoid live vaccines

what is the treatment for babies born to Hep B S Ag + moms

hep b vaccine within 12 hours of life concurrent HBIG complete the three dose vaccination series if baby is < 2 g at birth then the first dose doesn't count in the series test baby for Hep b between 9 and 18 months

what lab abnormalities are associated with steroids

hepatitis increased risk of HCC fluid retention growth retardation

what infection is associated with porphyria cutanea tarda, small vessel vasculitis, cryoglobulinemia, leukocytoclastic vasculitis

hepatitis C

what is the most common primary malignancy of the liver in childhood and who is at risk

hepatoblastoma kids < 3 who were preemies or have beckwith wiedemann

which kids are more likely to get hepatoblastoma vs HCC

hepatoblastoma: kids < age 3, ex preemies HCC: kids > age 3 both will have AFP elevations

who needs VZIG for post varicella exposure prophylaxis

high risk immunocomprimised patients (pregnant women included) newborns who's mom's had symptoms within 5 days prior or 48 hrs post delivery (along with IV acyclovir)

what is reverse differential cyanosis of a neonate mean and what does it represent

higher oxygen sat in lower extremities (pink) vs the right upper extremity (blue) signifies D-transposition of the great arteries with a PDA with pHTN OR aortic arch interruption *most common congenital heart lesion within the first few days of life

what is the X-ray pattern with histoplasmosis

hilar adenopathy and focal alveolar infiltrates

what disease should you think about in marfanoid body habitus but developmental delays and aggression

homocysteinuria

auditory brainstem response

how CN8 responds to clicks played in headphones with electrodes on the forehead

how do human's milk and cows milk differ?

human milk: less protein, less calcium and other electrolytes, same amount of fat, more carbs and iron

what calcium derangement can occur with rhabdo

hyPOcalcemia 2/2 decreased ability for vit D in kidney to activate *do NOT treat this hypocalcemia because they are at risk for natural hyPERcalcemia during recovery periods

Bartter syndrome

hyperaldosteronism, hyperrennin hypokalemia, metabolic alkalosis growth restriction polyuria/polydipsia nephrocalcinosis in neonatal period may develop deafness later in life NO HTN

what side effects are associated with valproic acid

hyperammonemia alopecia dizziness tremor weight gain

subcutaneous fat necrosis is at risk for what electrolyte derangement

hypercalcemia, usually during first 6 months will have flesh colored papules and nodules at birth through 6-8 weeks

what are exam findings of complex regional pain syndrome

hyperesthesia (pain to light texture) vasomotor disturbances (bluish discoloration or mottling) absence of fracture

dawn phenomenon of diabetes

hyperglycemia between 4 and 7 am without preceeding hypoglycemia due to transient mild insulin resistance 2/2 nocturnal GH secretion

what does the chest xray look like for chlamydia

hyperinflation with minimal interstitial and alveolar infiltrates

what lab findings are associated with a decreased anion gap metabolic acidosis

hyperkalemia hypercalcemia hypermagnesemia hypoalbuminemia

what electrolyte findings are associated with rhabdo

hyperkalemia, hyperphosphatemia, hypocalcemia (Ca deposits in injured muscles), hyperuricemia, and hypoalbuminemia

which electrolyte disturbance is associated with doughy skin

hypernatremia

congenital adrenal hyPOplasia

hyperpigmentation (ACTH breaking down to malnocyte stimulating hormone), salt WASTING (no aldosterone), cryptorchidism (no androgems) may have muscular dystrophy

what are two methods of improving nursemaid's elbow

hyperpronation Or Supination/flexion method

side effects of abacavir

hypersensitivity with HLA B5701

what may provoke absence seizures

hyperventilation

what calcium abnormality is associated with DiGeorge syndrome

hypocalcemia

how do Male infants with growth hormone deficiency present

hypoglcyemia direct hyperbilirubinemia micropenis

beckwith-wiedemann syndrome. (overgrowth syndrome)

hypoglycemia in infancy related to hyperinsulinism from pancreatic islet cell hyperplasia LGA, macroglossia, hum, omphalocele at risk for wilms tumor, hepatoblastoma, rhabdomyosarcoma need AFP, abd US and renal US q3 months in early childhood

u wave

hypokalemia bradycardia amiodarone if predominant then at risk for torsades

what are the lab findings for Rocky Mountain spotted fever

hyponatremia anemia thrombocytopenia left shift leukocytosis elevated serum transaminases

what are the lab findings with rocky mountain spotted fever

hyponatremia thrombocytopenia anemia leukopenia elevated transaminases

what are the adverse effects of long term use of topical steroids

hypopigmentation atrophy telangiectasias

gelastic seizures

hypothalamic hamartoma inappropriate laughter

what tumor may cause central precocious puberty

hypothalamic hamartoma optic glioma of NF1 (no growth acceleration with this type) germ cell tumors (secrete HCG which shares a receptor with LH)

risk factors for scfe

hypothyroidism obesity prader willi hypopituitarism low growth hormone down syndrome

what happens to neonates exposed to prolonged maternal mag?

hypotonia decreased bowel sounds abd distension meconium plug lethargy and trouble feeding treat with calcium if severe

hypohydrotic ectodermal dysplasia

hypotrichosis hypohydrosis hypodontia

what is the treatment for iron ingestion/overdose

identify any pills in stomach or intestines if severe symptoms and pills seen, consider lavage and start defuroxamine

transient erythroblastopenia of childhood

idiopathic and self limited anemia occurs in kids ages 1-4 years of age follow serial CBC with retic until it normalizes

what would prompt you to order FSH/LH as work up for premature puberty in a boy?

if his testicles were enlarged otherwise, if just pubic hair/body odor, testosterone, DHEAS, bone age, androstenedione

at what age should you refer for persistent strabismus?

if it persists past 4 months of age *most common form is esotropia (temporal displacement of light reflex)

what is the catch up schedule for TDAP

if never administered and child is > 7, then give a dose of Tdap with Td x2 4 and 6 months later *need just one dose of Tdap at age 11-12 to provide lifelong immunity to pertussis

when is primaquine appropriate for antimalarial PPX

if patient is NOT pregnant if patient has had negative G6PD testing for people traveling to places with primarily plasmodium vivax

when should you close a VSD

if there is > 2 x normal pulmonary blood flow (VQ: VS)

which carbapenem lowers the seizure threshold

imipenem

what is the treatment for priapism in sickle cell disease

immediate aspiration and irrigation of the corpus callosum exchange transfusion may be necessary but significant side effects are possible

when can Hep B S Ag + mom's breast feed

immediately low rates of transmission in breast milk

what is ADEM

immune-mediated acute demyelinating disease that causes multiple white-matter lesions of the brain and spinal cord signs: rapid fever, multifocal neurologic signs, MS like neurological findings can proceed quickly to brain death

at what age is HiB vaccine no longer indicated?

immunocompetent kids > age 5

who needs treatment for campylobacter infections and what do you treat with

immunocompromised or chronically ill those with severe or prolonged symptoms > 1 week 5 days azithromycin

Adrenoleukodystrophy

impaired addition of CoA to long chain FA very long chain FA accumulates damaging adrenal gland & white matter kid with ADHD like symptoms who transitions to gait instability, slurring words, agitation, dementia also has hyperpigmentation in his armpits (2/2 ACTH release from adrenal insufficiency from accumulation in adrenals) also seizures may need bone marrow transplant death within years

Chediak-Higashi syndrome

impaired phagolysosome formation leading to recurrent infections in mucus membranes, skin, lungs. a/w ataxia (2/2 progressive neurodegenerative), albinism with silvery hair, photosensitivity dx: large granules in neutrophils or granulocytes or eosinophils, prolonged bleeding time, DHR oxidation may develop a lymphoma like picture later on that only responds to SCT may develop apthous ulcers in the mouth Rx: BM Tx *EBV infection may produce an accelerated phase

what are symptoms of zinc deficiency

impaired smell and taste night blindness depressed immunity alopecia diarrhea angular chelosis

what does head tilt signify in a child and was is it due to

impending cerebellar herniation due to 4th cranial nerve dysfunction

commission

incorrect action taken leading to a medical error

what will the labs show with sickle beta thal

increased HbA2, HbF, HbS, normal MCHC, microcytosis will have a different mutation in each of the B globin genes (HgS and B thal)

what is kussmaul's sign associated with tamponade

increased JVP with inspiration

what causes a rightward shift (decreased hemoglobin affinity for oxygen) on oxyhemoglobin dissociation curve

increased temperature, acidic conditions

what lab abnormalities are associated with PCOS

increased testosterone increased LH to FSH ratio (> 2:1)

how do you diagnose wilson's disease

increased urinary copper decreased serum copper decreased ceruloplasmin

what findings are on a CBC of patient with whooping cough

increased wbc lymphocyte predominance

mechanism of action of dobutamine

increases myocardial contractility and provides mild vasodilation increases cardiac output and small increase in heart rate

how does estrogen effect thyroid

increases thyroid binding globulin and as a result increases total T4 with decreased free thyroxine

what is the most common cause of SVT in an infant vs an adolescent

infant - AVRT adolescent - AVNRT

what is the management for an infant with a hydrocele vs a 2 year old with a hydrocele

infant - observation, will likely go down on its own with time 2 year old - surgical referral since likely has a communicating tract between the inguinal canal and the scrotum

how can you differentiate infantile spams vs benign infantile sleep myoclonus

infantile spams occur during sleep or awake eyes will be OPEN sleep myoclonus only occurs during sleep eyes will be CLOSED

Hypsarrhythmia

infantile spasms high voltage, irregular slow waves that occur all over randomly, intermixed with spikes with multiple foci associated with flexor spasms "jackknifing" that occur in clusters and involve the trunk and neck and arms, head drops poor neurodevelopmental outcomes go on to develop severe intellectual disability or lennox gastaut rx: ACTH and vigabatrin, resolve over time even without therapy

what is considered tachycardia in infants vs children

infants > 160 children > 120

how can you differentiate between infectious and reactive lymphadenopathy

infectious will be exquisitely tender whereas reactive will be just mildly tender

enthesitis

inflammation and tenderness of area where tendon or ligament inserts into bone

balanitis

inflammation of the glans penis (head)

what is silk glove sign

inguinal hernia sac in an infant/toddler feels like a finger of a silk glove when rolled under the examining finger 2/2 thickened processus vaginalis

what drug of abuse is associated with excessive lacrimation, siallhorea and rhinorrhea

inhalants

what illicit drugs are associated with PTX and pneumomediastinum

inhalants cocaine inhaled marijuana inhaled

what is the treatment for HHS

initiate fluid boluses until perfusion is improved then may need to start maintenance fluids with potassium start insulin infusion at 0.025-0.05 u/kg/hr once glucose stops falling by more than 50/hr

etiologies for inspiratory vs biphasic stridor

inspiratory: variable extrathoracic obstruction such as laryngomalacia or croup (RVS, parainflu, flu, measles), subglottic stenosis or vocal cord dysfunction biphasic: fixed obstruction such as congenital tracheal stenosis or compression from a mass or vascular structure *tracheal stenosis may get better on its own with growth over time

non ketotic hyperglycemia

intractable seizures hiccups in utero hypotonia intellectual disability Rx: dextromorphan, sodium benzoate

what is the diagnostic tool of choice for biliary atresia

intraoperative cholangiogram Rx: *perform Kasai portoenterostomy immediately await eventual transplant

how does neonatal toxoplasmosis present (torch infection)

intrauterine meningoencephalitis microcephaly, hydrocephalus, microphthalmia, and chorioretinitis. Seizures hepatosplenomegaly, rash, and/or generalized lymphadenopathy generalized cerebral calcifications

what are some treatments for TCA ingestion

intubate 1 g/kg charcoal serum alkalization with bicarb (to increase protein binding and normalize QRS)

when do you start chelation therapy in someone with iron toxicity/overdose

iron level > 500 or moderate to severe symptoms

brain death criteria

irreversible cessation of all brain activity including brainstem no motor response to stimuli, noxious or otherwise absent dolls eyes no pupillary, corneal or gag reflex no response to cold caloric stimuli (if look towards the ear then brainstem is still intact) may choose to confirm with EEG or cerebral angiography or negative apnea test should have two measures, separated by a period of observation (12 hours 30 days to 18 years; 24 hrs birth to 30 days)

why shouldn't kids < 2 years have bactrim

it displaces bilirubin from albumin

how does kidney transplant affect growth in kids with CKD?

it improves their growth but often they do not reach full adult height potential if not assisted by growth hormone

if an ASD produces a murmur, why is this a systolic murmur

it is related to increased blood flow out of the RVOT considered a slight pulmonic stenosis murmur

what happens to a drug's half life during an overdose

it lengthens 2/2 zero order kinetics and oversaturation of metabolic pathways

why do we wait 24 hours to collect surface swabs on babies born to moms with + HSV outbreak that are otherwise well appearing?

it takes 24 hours for the virus to replicate so collection prior to this may be false positive from mom's virus must collect from mouth, nasopharynx, conjunctivae, anus may present with skin/eye/mouth dz, disseminated dz, or CNS dz

polymorphous light erruption

itchy rash occurring 1-2 days after sun exposure papulovesicular, urticarial or eczematous lesions responds to topical steroids

what is the first sign in baby of biliary atresia

jaundice get destruction of bile ducts leading to extra then intrahepatic fibrosis, eventual biliary cirrhosis and liver failure dx: elevated direct/conjugated bilirubin, elevated transaminases, HIDA without drainage, US with small or absent gallbladder, liver biopsy with bile duct proliferation or fibrosis Rx: kasai portoenterostomy. if done < 60 days of life then increased chance of success (drainage). Will need eventual liver transplant

five common symptoms of TTP

jaundice petechia lethargy headaches hematuria

what neonatal intestinal pathology is associated with maternal cigarette or cocaine use?

jejunal or duodenal atresia * cocaine can also cause agenesis of corpus callosum

what conditions can cause hydrops of the gallbladder

kawasaki strep pharyngitis prolonged fasting HSP TPN

which anti seizures meds are safe to use with OCP's

keppra depakote ethosuximide gabapentin

side effects of indinavir

kidney stones

kids on a ketogenic diet are at risk for what side effects

kidney stones constipation emesis abdominal pain

who needs treatment for flu

kids < 2 years of age CKD long term ASA morbit obesity long term care residents hospitalized kids severe or progressive disease *ideally within 48 hours

palivizumab indications

kids < 24 months with chronic lung disease of prematurity requiring medical therapy infants < 29 weeks gestation at birth in their first winter kids < 24 months with hemodynamically significant cyanotic or acyanotic CHD (not including ASD, small VSD, PS, or PDA) PPX: give monthly, up to 5 doses in wintertime

how does the definition of adult vs kids migraine differ?

kids: 2-72 hrs in duration, location bifrontal/bitemporal OR unilateral, characteristics inferred from child's behavior *usually NO aura *must have had 5 attacks in lifetime adult: 4-72 hrs in duration, usually unilateral

what is menke's disease

kinky hair disease x linked disorder mutation in transport protein for copper symptoms of copper deficiency in infancy

which syndrome is sprengel deformity associated with

klippel-Feil syndrome elevation, hypoplasia, and rotation of scapula associated with failure of scapula to descend to its normal position inability to raise arms above the head associated with fused cervical vertebrae, scoliosis , deafness, GU and cardiovascular findings No contact sports

what is the cause of anovulatory bleeding from dysfunctional bleeding

lack of LH surge and absence of corpus luteum it is normal to have anovulatory cycles up to 2 years after starting to menstruate

myositis ossificans

late complication from muscle contusion occurs 2-3 weeks after an injury calcifies over the area of the injury and may reabsorb the calcium over time

in what scenario is rotarix contraindicated, preferring rotateq

latex allergy 2/2 the tip for rotarix has latex in it

what blood tests are elevated in lead poisoning

lead level zinc protoporphyrin erythrocyte protoporphyrin

which metabolic disease is hyperuricemia associated with

lesch-nyhan syndrome

Koebner phenomenon

lesions induced by trauma may see vitiligo or psoriasis or salmon pink rash of JIA

what are common lab findings in sJIA

leukemoid reaction (>40K WBC) thrombocytosis (>1 million) high CRP/ESR elevated ferritin negative RF and ANA

what are the side effects of carbamazepine

leukopenia SIADH (hypoNa) hepatotoxicity

leydig cell aplasia

leydig cells don't differentiate within the testes phenotypically female but no uterus or fallopian tubes low levels of circulating testosterone

neonatal purpura fulminans

life-threatening thromboses 2/2 protein C/S deficiency

CVID

like XLA but variably decreased Ig's, normal B cell number Rx: Monthly IVIG with goal IgG > 800 recurrent sinopulmonary infections and enteral infections, splenomegaly, unexplained bronchiectasis, nodular lymphoid hyperplasia B cells recognize antigen and multiply but fail to progress to plasma cells

what are the complications of neonatal varicella

limb abnormalities (hypoplasia, scarring, cicatrix) microphthalmia, cataracts, chorioretinitis, and/or optic atrophy rash microcephaly, hydrocephalus

Pastice's lines

lines in flexural surfaces associated with scarlett fever

which vaccine is contraindicated to members sharing the same house as an immunocomprimised person under strict precautions

live attenuated flu vaccine

hereditary fructose intolerance

liver and kidney failure with hypoglycemia in setting of fructose consumption (juice or cereal) occurs later than galactosemia will find fructose in the urine

what is the gold standard diagnosis for Wilsons disease

liver biopsy a low ceruloplasmin level is suggestive

what is the treatment for crigler-najjar syndrome type 1

liver transplant also phototherapy

how long do you treat different types of lyme disease

localized disease (erythema migrans, bells): 14-21 days doxy disseminated disease (arthritis): 28 days doxy PPX: single dose of doxy if a tick has been in contact > 72 hours in an endemic area

what is the survival for a medulloblastoma that is metastatic and standard risk and where is it located

located along the midline of the cerebellum 30% if metastatic 85% if standard risk

what are the most common sites of metastases for neuroblastoma

long bones skull liver bone marrow lymph nodes orbits

how can you determine allergic contact dermatitis

looked for a papular rash in linear shapes and plaques

achalasia

loss of ganglion cells in the esophagus and dorsal motor nuclei reduction of vagus nerve signal may have antibodies to auerbach plexus dx: bird beak on barium swallow

lab findings in hereditary angioedema

low C1 inhibitor protein (low C1 INH functional assay) decreased C4

low birth weight vs very low birth weight vs extremely low birth weight

low birth weight < 2500 very low birth weight < 1500 extremely low birth weight < 1000

what would you expect for glucose and ketones in glycogen storage disorder

low glucose and high ketones

what would you expect for glucose and ketones in fatty acid oxidation defect

low glucose and ketones along with hepatomegaly

what would the value for ketones be in hyperinsulinism or fatty acid oxidation disorder with hypoglycemia

low to zero since insulin will suppress these

a mass along where in the spinal cord would cause loss of DTR

lumbar spine

what are the most common sites of metastases for wilms tumor

lung

glutaric acidemia type 1

macrocephaly at birth with normal development until a febrile illness then hypotonia Dx: retinal hemorrhages, SDH, frontal cortical atrophy Rx: L-carnitine, riboflavin, IVF with glucose

what abx is used to treat campylobacter in a neonate

macrolides

which drugs decrease warfarin metabolism? (bleeding risk)

macrolides fluoroquinolone azoles flagyl bactrim

what is the proper sequence of erruption of teeth

madibular central incisors maxillary central incisors maxillary lateral incisors *remember the 7+4 rule

blue diaper syndrome

malabsorption of tryptophan leading to blue-urine stained diapers

midparental height equation for males and females

males: ((maternal height + 5" or 13 cm) + paternal height)/ 2 females: ((paternal height - 5" or 13 cm) + maternal height)/2

teacher collins syndrome

mandibular and zygomatic hypoplasia coloboma of lower eyelids absent lower lashes external ear abnormalities pre-auricular hair displacement on to the cheekbones

which metabolic disease is elevated serum alloisoleucine and leucine associated with

maple syrup urine disease

heterotypic ossification on xray

mass located away from a joint rounded mature peripheral ossification with distinct margin surrounding a radiolucent center of immature osteoid

what uncontrolled maternal condition causes caudal regression syndrome in infants?

maternal diabetes

which moms are more likely to transmit HSV to their infants

maternal first infection with HSV

what facial features may accompany b thal

maxillary hyperplasia frontal bossing nasal bridge flattening

why should you not intubate a patient with a large primarily right sided mediastinal mass?

may lose the negative intrathroacic pressure and cause resultant collapse of the right atrium leading to cardiac failure

what exam maneuvers help diagnose meniscal tears

mcmurrays test duck walk

prevalence

measuring amount or rate at a set point cross sectional studies

incidence

measuring trends on amounts or rate of disease

which GI issues is associated with CF at birth

meconium ileus

what disease is associated with "soap bubble" appearance on xray

meconium ileus, and CF with pancreatic insufficiency

men 2a

medullary thyroid cancer pheochromocytoma parathyroid hyperplasia

men 2b

medullary thyroid cancer pheochromocytoma marnoid body habitus mucosal neuromas intestinal ganglioneuromas

what is the most common malignant CNS tumor in children

medulloblastoma *associated with head tilt 2/2 CN4 dysfunction

what is the most common brain tumor in pediatrics and where is it found?

medulloblastoma found in posterior fossa and can spread through the CSF may obstruct the 4th ventricle leading to increased ICP Sxs: morning headaches and vomiting

what does a peripheral blood smear show in ITP

megathrombocytes aka large platelets

which nephrotic syndrome is associated with Hepatitis B

membranous nephropathy

when can you give menactra vs menveo vs menHibrix

menactra: as early as 9 months, in a two dose series. *must wait until 2 years in kids at risk for invasive pneumococcal disease menveo: as early as 2 months, in a four dose series menhibrix: as early as 6 weeks, in a four dose series

how can you distinguish IDA from B thal

mentzner index : low in b thal (normal RBC production aka the denominator), high in IDA (reduced RBC production aka the denominator) Hgb electrophoresis : B thal has elevated A2 and F hemoglobin while IDA may be normal or low A2

what is the acid base status with Tylenol ingestion

metabolic acidosis

homocysteinuria

metabolic disorder increased risk of vascular disease and thrombus formation marfanoid body habitus Rx: pyridoxine (B6) to enhance the activity of the deficient enzyme (measure homocystein levels for to track efficacy) then add in folate if not working use ASA for stroke PPX monitor dietary intake of protein

which conditions cause both a anion gap metabolic acidosis and an elevated serum osmol gap

methanol and ethylene glycol ingestions

what anti-graves drug is ok to use in kids

methimazole PTU had increased risk of hepatotoxicity and death

which ADHD medications may lead to hypertension

methyphenidate amphetamine atomoxetine

what are signs of iron deficiency

microcytic anemia spooning nail deformity glossitis conjunctival pallor palmar creases decreased appetite fatigue

what dysmorphic features are noted with TAR

micrognathia low set and posteriorly rotated ears hypertelorism broad forehead

elevated anti MPO ANCA Ab

microscopic polyangitis vasculitis

austin flint murmur

middiastolic murmur 2/2 MV being impinged by a jet of blood from aortic insufficiency

PPS murmur

midsystolic ejection murmur at RUSB that radiates to the back 2/2 turbulent flow from blood hitting small and relatively stiff pulmonary vessels heard shortly after birth innocent

what condition are those with cyclical vomiting syndrome likely to develop in adolescence

migraine headaches Rx: cyproheptadine, propranolol, amitryptilline

NO uses and contraindications

mild analgesia, sedation, amnesia, anxiolysis side effect: vomiting, dysphoria contraindications: psychotropic meds, PTX, eustachian tube dysfunction, bowel obstruction, pregnancy, COPD

dubin johnson

mild conjugated hyperbilirubinemia 2/2 a defect in the transporter

what disease is associated with anti U1 RNP

mixed connective tissue disease *has many overlap symptoms with DM, JIA, Scl

how long is the treatment for whipple disease

months of antibiotics

characteristics of infants with metabolic disorders

most are born at term most have normal prenatal growth most have no abnormalities detectable on prenatal ultrasound

multiple dysplastic kidney

most common cystic renal disease in kids usually involves the whole kidney which that kidney is then nonfunctioning and may spontaneously involute small risk of malignant transformation

metatarsus adductus

most common deformity of the foot marked by the middle bones of the foot pointing in toward the body draw an imaginary line from the hind foot that goes between the 4th and 5th toe vs usually the 2nd and 3rd toe 2/2 intrauterine crowding a cause of in-toe walking but not the most common cause

congenital hepatic fibrosis

most common ductal plate abnormality sxs: portal HTN and associated sxs, HSM dx: liver bx rx: portosystemic shunt for pHTN and abx for any cholangitis

scrofula

most common form of extrapulmonary tb 2/2 m bovis related to consumption of unpasteurized cows milk causing swollen nodes DX: FNA, PPD is < 2 year, quant gold if > 2 years Rx: 4 drug regimen for 6-9 months

Still's murmur

most common innocent murmur in children. Functional low-frequency, vibratory or buzzing or musical (kazoo) noise that occurs in midsystole and heard best at lower left sternal border disappears with valsalva

myeloperoxidase deficiency

most common neutrophil disorder recurrent mild infections due to variable expression of the defect

Porphyria Cutanea Tarda

most common porphyria blistering skin lesions, photosensitivity, hyperpigmentation, liver damage predisposes to HCC a/w hepatitis C may be triggered by EtOH, Fe or estrogen dx: increased porphyrin rx: therapeutic phlebotomy, plaquenil

idiopathic generalized epilepsy

most common type in kids sz: abrupt onset with LOC or AMS, b/l symmetric motor activity, no aura *tonic-clonic (grand mal) - sustained contractions followed by rhythmic jerks

twin-twin transfusion syndrome

most likely with monozygotic monochorionic twins the larger twin has greater risk for hyperbili and polyhydramnios (larger blood volume to kidneys to more output) The smaller twin has greater risk for oligohydramnios both twins are at risk for hydrops 2/2 vascular anastamosis between placentas

where does neuroblastoma typically present

mostly adrenals some in the paravertebral ganglia or pelvic ganglia some at organ of Zuckerkandl within aorta

motor stereotypies

movements such as shuddering attacks, which look like a sudden shiver or 'chill', limb stiffening or trembling, or hand flapping. occur when the child is awake, and often when the child is excited or overstimulated benign

Puetz-Jeghers syndrome

mucocutaneoux macules, polyps throughout the GI tract at risk for intussusception and FAP increased risk for extra intestinal malignancies and colorectal cancers

fixed drug erruption

mucosal and or skin rxn to drugs that reappears at the same site after administration of any antigen, usually medication (specifically bactrim). usually on hands, trunk, genital regions central blistering > desquamation > crusting > residual hyperpigmentation rx: dc drug, antihistamines, topical or oral steroids

what does pathology for celiacs disease look like microscopically

mucosal lesions with increased lymphocyte, plasma cells and macrophages in the lamina propria and increased numbers of intraepithelial lymphocytes grossly: increased fat, cobblestoning, fissures and thick intestinal wall

ADEM (acute disseminated encephalomyelitis)

multifocal periventricular inflammation and demyelination after infection or vaccination dx: Brain MRI will demonstrate evidence of demyelination, with focal or multifocal lesions of the white matter without evidence of prior white matter lesions. sxs: supportive care, steroids

what is a genetic complex

multiple anomalies of several different structures that are near each other during embryologic development

papular acrodermatitis aka Giannotti Crosti

multiple edematous papules on face, buttocks and extremities which spare the trunk persist for weeks to months follow a viral illness or some immunizations

acute radiation syndrome

multiple phases Symptoms include nausea, vomiting, diarrhea, fatigue, fever, headache, hematopoietic disturbances The long-term symptoms are dose-related and are hematopoietic and gastrointestinal.

Whipple disease

multisystem disorder due to tropheryma whippeli sxs:severe malabsorption, arthritis, CNS symptoms, polyserositis dx: PAS positive granules in lamina propria of gut Rx: abx x 6 months

murasmus vs kwashikor

murasmus = protein calorie malnutrition WITHOUT edema kwashikor = protein calorie malnutrition WITH edema

what symptoms develop after black widow spider bites

muscle rigidity/cramping and hemodynamic instability (HTN) abdominal pain if these develop in young children, then consider antivenom otherwise no need for antivenom, esp in older children can consider benadryl or muscle relaxants

testotoxicosis

mutation on LH receptor causing activation of Leydig cells leads to increased testosterone and advanced bone age rx: ketonconazole, oral arimotase inhibitors

progressive myoclonic epilepsy

myoconic seizures, GTC's and progressive neurodegeneration very rare cognitive decline, ataxia, neuropathies, myopathies Rx: supportive treatment, aimed at seizure control

what is the antidote for fentanyl induced rigid chest

naloxone, succinylcholine or rocuronium

what imaging findings will present on MRI for discitis

narrowed disc space

fetal warfarin syndrome

nasal hypoplasia and stippled epiphyses

how is chalmydia conjunctivitis in a newborn diagnosed?

need scrapings from everted eyelid with epithelial cells since chlamydia is an obligate intracellular organism

how do you classify severe BPD

needing > 30% FiO2 beyond 36 weeks gestational age associated with poor neurodevelopmental outcomes associated with pulmonary HTN which is diagnosed on echo

congenital hypopituitarism

neonatal hypoglycemia micropenis (hypogonadism) (< 2 cm at full term birth) breech presentation post dates gestation poor feeding hypotonia jaundice midline defects CAH and hypothyroidism

what does erythromycin at birth of neonates prevent

neonatal ophthalmia and gonorrhea related ophthalmia

how do nephritis vs nephrotic syndromes differ

nephrotic: proteinuria, hypoalbuminemia, edema, hypercholesterolemia, hypogammaglobulinemia, increased risk for thrombosis nephritic: hematuria, variable proteinuria, HTN, edema, +/- decreased complements

side effects of tenofovir

nephrotoxic

if an infant presents with raccoon eyes (periorbital ecchymosis), what cancer should you suspect

neuroblastoma

what is the most commonly diagnosed malignancy of infancy

neuroblastoma

what tumor responsible for ataxia leads to opsoclonus myoclonus

neuroblastoma

how can you tell the difference between neuroblastoma and nephroblastoma on imaging?

neuroblastoma: compression on the kidney nephroblastoma: splay's open the kidney

which categories of drugs are most commonly associated with dystonic reaction

neuroleptics (antipsychotics) antiemetics

what are the symptoms of methotrexate toxicity

neurotoxicity nephrotoxicity chronic hepatitis

how can you differentiate a nevus simplex from a port wine stain

nevus simplex will blanch

what is the recommended needle length for vaccinations based on size

newborn: 5/8 " 2-12 months: 1 " > 12 months: 1.5 "

which age group has the highest protein requirement

newborns!

what is pellegra

niacin deficiency 3 Ds: dermatitis, dementia, and diarrhea

what is the difference between when night terrors and nightmares occur

night terrors = in the beginning of the night, during non - REM sleep night mares = in last 1/3 of the night, during REM sleep

secondary enuresis

nighttime urinary incontinence in child > 5 years who had achieved at least 6 months period of consistently dry nights

what is the treatment for cyrptosporidium

nitazoxanide in those with symptoms for > 2 weeks or immunocomprimised

at what gestational age does scrotum have few rugae vs anterior rugae vs entire scrotum vs pendulous

no rugae = 28-35 weeks few rugae = 36-39 weeks entire scrotum = 40-41 weeks pendulous = > 42 weeks

how often do you screen for cervical cancer in patients with HIV

no sooner than age 21 screen once every year for 3 years, then can space to q3 years

do you need to get a renal US for an infant with isolate periauricular pit

no! only if they have a family hx of deafness or other focal findings on exam

XYY syndrome

nodulocystic acne behavioral problems tall developmental delay

lymphoma

non hodgkins is most common type overall hodgkins is 3rd most common childhood cancer lymphomas present with more B symptoms than leukemias stage 3: lymph nodes on both sides of the diaphragm stage 4: disease outside of the lymph nodes

who needs post exposure immunization for varicella with the vaccine

non immune children > 12 months of age within 5 days of exposure

at what gestational age do soles have no crease vs 1-2 creases vs 2-3 creases vs heel crease

none - 32-33 weeks 1-2 - 34-35 weeks 2-3 - 36-37 weeks heel crease - 38-41 weeks

constitutional growth delay

normal growth during first year of life, then slows down then picks back up delayed bone age with normal GH titers

growth hormone deficiency

normal height/weight at birth falls off the growth chart delayed bone age delayed tooth eruption and loss presents by age 18 - 24 months since prior nutrition and thyroid hormone were responsible for growth continue gaining weight so have cherubic appearance may have dysmorphic facial features dx: usually bone age < chronological age, decreased IGF-1, decreased GH even when stimulated

rhinitis sicca

nose bleeding related to kids who have to use oxygen delivery devices AND live in cold climates present with desiccation of nasal mucosa

what is the treatment for femoral anteversion

nothing usually resolves on its own if causing pain or disturbing cosmetically then can consider referral to orthopedics refer if persists past age 11

granuloma annulare treatment

nothing at first, usually resolves on own over time then may consider steroids topically

what would be the next imaging modality for a incidentally diagnosed thyroid nodule with a suppressed TSH

nuclear thyroid scintigraphy

Laurence-Moon-Bardet-Biedl syndrome

obesity intellectual delay hypogonadism retinitis pigmentosa polydactyly

what is the treatment for varicocele

observation surgical correction only if severe pain; testicular growth retardation or arrest over a 6-12 month period of observation; a volume disparity of > 2 mm between the testes; or if the varicocele is present in a solitary testis; infertility

what are different treatment options for itp

observation steroids ivig (if plt < 20K) anti-Rh Ab

neonatal gonococcal conjunctivitis

occurs 2-5 days after birth more fulminant presentation rx: erythromycin ointment prevents, single dose of IM CTX treats

neonatal chlamydial conjunctivitis

occurs 5-14 dyas after birth get watery discharge that becomes purulent Rx: oral erythromycin (watch out for pyloric stenosis)

what is the definition of chronic daily headache

occurs > 15 days a month for > 3 months and is NOT due to organic pathology

juvenile idiopathic arthritis definition

occurs before age 16 persistent synovitis in >/= 1 joint synovitis for at least 6 weeks *most common rheumatic disease diagnosed in kids

transient myeloproliferative disorder

occurs in 10% of all Downs infants sxs: peripheral blood with blasts, vesiculopustular skin lesions Rx: spontaneous resolution by age 3 months *presents 10-20 fold higher risk of developing acute megakaryocytic leukemia as they grow older

benign paroxysmal vertigo of childhood

occurs in kids 1-4 years of age ataxia, nystagmus, pallor, emesis but retained consciousness precursor to migraines

what is a provisional tic disorder

occurs in patients < 18 years of age lasts for > 1 year has facial tics multiple times per day

rufous albinism

oculocutaneous albinism found in african americans red hair, pale skin, freckles, nystagmus, photophobia, decreased visual acuity

granuloma anulare

often misdiagnosed as tinea corporis chronic (years) rash with smooth, raised borders and no scaling Rx: topical steroid cream

when are kids considered not infectious with parvovirus

once the slapped cheek rash appears, they are no longer infectious

how long do you need to give antibiotics for after sinus fractures?

one week

hyperimmunoglobulin D

oral and vaginal ulcers, fever periods for 3-5 days, headache, nausea and vomiting, large joint arthritis, macular rashes autosomal recessive

what is the treatment for chlamydia conjunctivitis in a newborn

oral erythromycin to eradicate the organisms from the nasopharynx, conjunctiva and decrease risk of PNA

what is the treatment regimen for first, first recurrence and second recurrence of c diff in kids

oral metronidazole oral metronidazole oral vanco

why is oral naloxone less effective than IV naloxone

oral naloxone goes through first pass effect metabolism in the liver so does not get directly to the brain whereas iv naloxone will get there while bypassing the liver without any diminished effects

what is the treatment for perianal dermatitis

oral penicillin

what is one treatment for calcium related kidney stones

oral potassium citrate since it helps the kidney chelate calcium to prevent precipitation of stones

what is the treatment for symptomatic congenital CMV

oral valganciclovir for 6 months must be started by 4 weeks of life

orbital (post septal) vs periorbital (pre septal) cellulitis

orbital occurs when other bacterial infection spread to ethmoid sinus has proptosis, limited EOM 2/2 pain, systemic symptoms, pupillary defects need emergent ophtho exam, IV unasyn and vanc x 3-5 days, then oral x 2-3 weeks and CT orbits periorbital cellulitis occurs skin surrounding the eye is penetrated and infected with bacteria treat with PO abx x 7-10 days *s aureus and group A strep

what is the most common type of ASD

ostium secundum

what methods can help determine conductive hearing loss

otoacoustic emissions tests - outer hair cells of the cochlea pneumotoscopy tympanogram testing - status of ear pressure

which antiepileptic drug is associated with hyponatremia

oxcarbazepine

where does pain occur with peroneous brevis tendon injuries

pain just inferior/posterior to lateral malleolus

where does pain occur with anterior talofibular ligament injuries

pain over lateral and anterior part of the ankle

what is the purpose of joint injections in JIA

pain relief prevention of leg length discrepancy (affected joint may be longer)

what is the prehn sign

pain relief with testis elevation; absent in testicular torsion and present in epididymitis

tennis elbow

painful inflammation of the tendon at the outer border of the elbow (lateral) resulting from overuse of lower arm muscles (as in twisting of the hand)

acute febrile neutrophilic dermatosis (Sweet Syndrome)

painful nodules/papules/plaques asymmetrically distributed on face/neck/limbs that are elevated or indurated with violaceous hue may also see fever, conjunctival injection, arthralgia usually preceeded by a URI Dx: bx with dense neutrophillic perivascular infiltrate on dermis rx: systemic steroids

what is the side effect of asparaginase

pancreatitis

immune reconstitution inflammatory syndrome

paradoxical worsening of HIV after initiation of CART which may exacerbate pre-existing subclinical conditions rx: steroids

which maternal infection is most associated with hydrops fetalis

parvovirus B19

conduct disorder

patterns of behavior in which the rights of others or basic social rules are violated physical altercations > 6 months ODD + antisocial

Acute Lymphoblastic Leukemia

peak age 2-5, < 1 and > 10 are poor prognostic indicators a/w downs, ataxia telangiectasia, NF, bloom syndrome and fanconi anemia pancytopenic and often profoundly neutropenic at diagnosis other poor prognostic indicators: WBC > 50,000, philadelphia translocation, African American or hispanic ethnicity

how can you identify colic

peaks in 2nd month of life fades by 3rd to 4th month > 3 hrs a day of crying for > 3 days of the week, for >3 weeks can try probiotics for treatment

what is the distribution of calcifications in neonatal cmv (torch infection)

peri ventricular

what eye conditions are associated with alport syndrome

perimacular pigment changes lenticonous - conical projection of the anterior or posterior surface of the lens of the eye

what is berliner sign

periorbital and palpebral edema associated with roseola infection

what are adverse effects of an athlete taking growth hormone

peripheral edema HTN elevated fasting blood sugars and a1c cardiomegaly muscle weakness joint pain

what is the black box warning for vigabatrin

permanent visual field defects

Zellweger syndrome

peroxisomal disorder Dx: increased very long chain fatty acids Sxs: neurologic degeneration with dysmorphic facial features and liver cysts, along with renal failure, worsening over the first year of life, then eventual death

what are criteria for valvular surgery in endocarditis

persistent vegetation or vegetation > 10 mm or vegetation on anterior mitral leaflet valve dysfunction perivalvular extension staph epi on prosthetic valve

disruptive dysregulation mood disorder

persistently irritable mood with severe temper outbursts occurring on average 3 or more times per week

what ekg finding is characteristic of transposition of the great arteries

persistently positive T wave in V1

what is the size difference between petechia vs purpura; papule vs plaque

petechia = < 2 mm purpura = 2-10 mmg papule/macule = < 1 cm plaque/patch = > 1 cm

what is treatment for Crigler Najar type II

phenobarbital

what condition is associated with SRY gene defects

phenotypically female in chromosomal male patient

what is the treatment for x linked hypophosphatemic rickets

phosphorus AND calcitriol aka Vit D resistant rickets characterized by renal phosphate wasting AND impaired stimulation of 1α-hydroxylase that normally occurs in response to low phosphorus levels most common form of rickets in north america only form with NORMAL PTH

Auspitz sign

pinpoint bleeding after a scale is removed from psoriasis

what complication is common with S Aureus pneumonia

pneumatoceles

what two vaccines can be given IM or SQ

polio pneumococcal

gardners syndrome

polyposis + soft tissue tumors + extra teeth + osteomas + congenital hypertrophy of retinal pigment epithelium 100% cancer risk if colon is not removed need yearly screening colonoscopy between 10-12 years of age

What does a HIDA scan show in acute cholecystitis

poor or no visualization in presence of inflamed gallbladder

what illicit substances are associated with methemoglobinemia

poppers inhalents *chocolate brown blood

what location of polydactyly is LEAST likely to be associated with other conditions

post axial

PHACES syndrome

posterior fossa brain malformations hemangiomas (may confuse with port wine stain) arterial anomalies cardiac defects eye abnormalities increased risk of strokes, hearing/vision loss (2/2 large hemangiomas) Rx: oral propranolol Think Dandy Walker and an echo!

what is the most common cause of obstructive uropathy in kids leading to kidney failure

posterior uretrhal valves dx: prenatal b/l hydronephrosis on US or postnatally with VCUG sxs: lower abdominal mass, weak urinary stream needs immediate urology consult and bladder drainage

how do the ears appear in posterior plagiocephaly vs positional plagiocephaly

posterior: has posterior ear displacement positional: has anterior ear displacement

what are the FDA approved uses for growth hormone

prader willi severe idiopathic short stature turner syndrome noonan syndrome CKD kids prior to transplant hx of SGA with lack of catch up by 2 years of age SHOX haploinsufficiency

growth hormone is associated with sudden death in which patient population

prader-willi interaction with obstructive sleep apnea

preHTN vs Stage 1 vs Stage 2

pre = sbp and dbp > 90th%ile but < 95th%ile or BP > 120/80 stage 1 = 95th%ile to 5 mmHg above 99th%ile on 3 occasions (130-139/80-89) stage 2 = > 99th%ile + 5 mmHg (>140/90) *measured on 3 elevated readings

what are contraindications to live flu vaccines

pregnancy reactive airway disease or asthma chronic heart disease diabetes chronic renal disease immunosuppressive disoreders daily aspirin therapy severe allergy to the live flu vaccine NOT egg allergy

what do natal teeth represent

premature eruption of primary deciduous teeth some are supernumerary and some are not confirm with X-ray that they are supernumerary before removing to minimize crowding or gaps

what is the most common type of craniosynostosis

premature fusion of the midline sagittal suture results in a long narrow skull aka scaphocephaly

cross-sectional study

presence of a risk factor or association and the presence of an outcome or disease are reviewed simultaneously in a population. This is the weakest type of study.

what does an elevated osmolar gap indicate with a high anion gap metabolic acoidosis

presence of exogenous organic acids

lactose intolerance

presents by > 2 years of life Dx: endoscopic bx, breath hydrogen test, stool reducing sugars

central vein thrombosis in neonates

presents in neonates with seizures RF = dehydration and infection Dx: MRI venogram Rx: anticoagulation, decrease ICP and possible surgical thrombectomy

renal vein thrombosis in the newborn

presents with hematuria and flank mass may also have HTN, oliguria and microangiopathic hemolytic anemia RF: sepsis, shock, dehydration, congenital hypercoagulable states, maternal diabetes

linear Iga bullous dermatosis

presents within 1st decade of life with spontaneous remission prior to puberty Rx: DAPSONE eruption of widespread, large, tense, clear or hemorrhagic bullae may also have "string of pearl" bullae with a central crust (ring of blisters)

at what age do term and preterm infants need to start getting iron supplements no matter if they are breast or formula fed

preterm - 2 weeks to 2 months term - 4 months this is when iron stores in their body deplete

Addisons

primary adrenal insufficiency 2/2 autoimmune disease decreased cortisol and aldosterone leads to decreased Na and increased K hyperpigmentation, hypotension, salt cravings, anorexia, weight loss

men 1

primary hyperparathyroidism pituitary tumors pancreatic tumors

which gallbladder disease is UC associated with

primary sclerosing cholangitis

how long is the treatment duration for primary/secondary vs tertiary vs neurosyphillis

primary/secondary: single dose of IM PCN tertiary: weekly for 3 weeks vs doxy BID x 3 weeks Neurosyphilis: q4 for 10-14 days

rubella panencephalitis

progressive neurologic deterioration starting in 2nd decade of life with mental decline, myoclonus, ataxia, seizures, and death

EKG findings suggestive of TCA ingestion

prolonged PR, QRS, QT

what coag abnormality would lupus anticoagulant present with

prolonged PTT

what increases risk of retinopathy of prematurity

prolonged exposure to 100% FiO2 low birth weight, low gestation age, surfactant therapy, prolonged ventilation must screen those infants < 30 weeks or < 1500 g

congenital hypothyroidism manifestations

prolonged jaundice large tongue eyelid swelling hypotonia extremity edema umbilical hernia large protuberant belly open fontanelles mottled skin constipation myxedema

pentobarbital uses and contraindications

prolonged sleepiness, hypotension, increased heart rate contra: cardiac, renal or hepatic insufficiency; porphyria

cavernous sinus thrombosis

proptosis, chemosis, uni- or bilateral ophthalmoplegia 2/2 infection of facial sinus, nose, mouth

what is the first clotting factor to vanish in warfarin therapy

protein C can lead to warfarin necrosis aka purpura fulminans can prevent this by treating with heparin in the first 24-48 hours

DRESS

pruritic rash that may be morbiliform with erythrodermic eruption, papules, pustules and bullae fever, LAD, generalized facial edema eosinophilia may occur 2-12 weeks after exposure to the drug Rx: steroids, stop the drug

what is a ranula

pseudocyst on floor of the mouth that appears similar to the belly of a frog congenital formation 2/2 anomalous drainage from a gland. may also be acquired after trauma Rx: surgical removal along with the gland

Liddle syndrome

pseudohyPERaldosteronism abnormal renal tubules causing Na retention leading to HTN increased Na absorption and K secretion (HTN, hypokalemia) metabolic alkalosis decreased renin/aldosterone

Gordon syndrome

pseudohyPOaldosteronism HTN Hyperkalemia metabolic acidosis

what three bacteria can form ring abscesses

pseudomonas proteus bacillus

what manifestation of lupus does anti-ribosome P Ab indicate

psychosis

what vascular ring is associated with indentation of the esophagus on barium swallow and presents with stridor

pulmonary sling

what does differential cyanosis in a newborn mean and what cardiac diseases does it signify

pulse ox readings higher in the right hand (pink) vs the lower extremity (blue) signifies pulmonary HTN with a PDA maybe seen in hypoplastic left heart, pulmonary atresia, ebsteins anomaly, interrupted or obstructed aortic arch,

how is keratitis diagnosed on exam

pus discharge pinpoint pupils on pen light exam corneal opacity positive fluorescein stain inflammation of the cornea pain, photophobia, reduced visual acuity

what is the treatment for MG

pyridostigmine IVIG steroids

osteosarcomas on xray

radio dense and radiolucent areas with periosteal new bone formation lifing the cortex and forming "Codman's triangle" soft tissue mass has associated sunburst pattern

papular purpuric gloves/stocking syndrome

rapid swelling/erythema of hands and feet related to parvovirus B19

what are characteristics of mixed connective tissue disease

raynauds fever arthritis dorsal hand edema rash myositis

idiopathic pulmonary hemosiderosis

recurrent cough, IDA, restrictive pattern on PFT's and eventual pulmonary fibrosis dx: sputum culture with hemosiderin laden macrophages rx: steroids

what infections are likely with selective IgA deficiency

recurrent sinopulmonary, GI, urogenital infections, recurrent giardiasis

what happens with ingestion of bleach

relatively nothing

2 vessel umbilical cord is associated with what underlying anomalies

renal

what cancer are patients with sickle cell at increased risk for

renal medullary carcinoma

what is the treatment of congenital hypothyroidism

replacement hormone 10-15 ug/kg/day as TABLETS (liquid is not stable)

what is the acid base status with aspirin ingestion

respiratory alkalosis with metabolic acidosis compensation 2/2 tachypnea

what happens with ingestion of lamp oil

respiratory distress due to pulmonary aspiration

what is the main difference in treatment from dilated to restrictive cardiomyopathy

restrictive needs AC since there is increased risk for thromboembolic events compared to dilated

what happens to reticulocytes count and RDW in IDA

reticulocyte count is normal to decreased RDW is increased

what is the most effective treatment for comedomal acne

retinoids

Eisenmenger syndrome

reversal of L-R shunt to a R-L shunt presents with cyanosis and clubbing, hemoptysis, shortness of breath, decreased exercise tolerance, syncope, dysrhythmias rx: heart and lung transplant

what is the number 1 cause of aortic regurg worldwide

rheumatic fever

which antimicrobial is the only one proven to decreased effectiveness of OCP's

rifampin

which drugs increase warfarin metabolism? (stroke risk)

rifampin phenytoin carbamazepine alcohol

what is the PPX for those exposed in close setting to meningococcal meningitis

rifampin BID x 2 days OR CTX x single dose (pregnant ladies) OR Ciprofloxacin x single dose (kids > 18)

right atrial vs left atrial isomerism

right atrial: b/l right sidedness, asplenic left atrial: b/l left sidedness, polyspenia with combined mass equal to normal spleen both have bowel malrotations, complex congenital heart disease

what is the screening recommendation for an isolated juvenile polyp

routine screening at age 50 if benign

what is the best way to diagnosed primary ciliary dyskinesia

rule out CF (2 negative sweat chloride tests or no mutations) and then utilize exhaled NO test

what are the most common microbes to cause joint infection

s aureus n gonorrhea parvovirus b19 HBV less common: lyme, strep, GI infection including c diff and giardiasis

what is the causative agent in most cases of discitis

s. aureus

salt wasting vs non salt wasting CAH

salt wasting = loss of cortisol AND aldosterone 21 OH, 3B, lipoid non salt wasting = 11B OH, 17 A OH

at what age can kids scribble vs vertical stroke vs draw a circle, cross, square, triangle, and diamond

scribble - 15-18 months vertical stroke - 2 years circle - 3 years cross - 3-4 years square - 4 years triangle - 5 years diamond - 6 years

how do you distinguish primary vs secondary raynauds?

secondary has abnormal nailfold capillaries on exam

side effect of etoposide

secondary malignancies

precedex uses and contraindication

sedation + some analgesia minimal respiratory depression or recovery agitation contra: bradycardia, other arrhythmias, does have dose related hypotension

post pericardiotomy syndrome

seen after ASD repair inflammatory process 1-4 weeks post op causing acute pericarditis, pericardial effusions, fever, pleural effusions and elevated ESR rx: asa with wean over 6 weeks, +/- steroids

bone within bone

seen in osteoporosis and sickle cell disease due to repeated infarction to bone and joint destruction

what is the most common presentation for perinatal stroke

seizure

how does shcizencephaly present and what does it look like

seizures, developmental delay, spasticity, focal neurological deficits cortical malformation causing a split or cleft in the brain

what immunodeficiency is associated with anaphylaxis after blood transfusion without any clear cause from blood bank

selective IgA deficiency

atomexetine (strattera)

selective presynaptic norepinephrine reuptake inhibitor approved for ADHD treatment in kids > 6 useful if unable to tolerate stimulants black box warning of suicide htn ae

what are 3 deadly syndromes associated with candida

septic peripheral thrombophlebitis septic thrombosis of great central veins hepatosplenic candidiasis

which three deadly syndromes is candidemia involved with in neonates

septic peripheral thrombophlebitis septic thrombosis of the great centra veins hepatosplenic candidiasis

persistent mullerian duct syndrome

sertoli cells don't make MIS found when patient is getting surgery or cryptorchidism or inguinal hernia and noted to have presence of uterus/fallopian tubes rx: remove mullerian structures will have normal testicular function

what is the metabolic work up for microcephaly

serum amino acids urine organic acids NH3

What infant populations need peanut allergy testing prior to introduction of peanuts and at what age should this be done

severe eczema egg allergy between 4-6 months, as shows is developmentally appropriate

familial lipoprotein lipase deficiency

severe hypertriglyceridemia with intermittent abdominal pain, recurrent acute pancreatitis, HSM, cutaneous xanthomata AR so not always a family hx Rx: total dietary restriction of fat, goal TG < 1000 *fish oil makes this worse

glutaric acidemia type 2

severe hypoglycemia, metabolic acidosis, hyperammonemia sweaty feet odor cardiomyopathy, facial dysmorphism, renal cystic dysplasia dx: no ketones, decreased carnitine rx: continuous gastric feeds, NO MCT!, carnitine

myxedema coma

severe hypothyroidism leading to slowing of multiple organs decreased mental status, bradycardia, hypothermia, hypoglycemia, hypoNatremia increased TSH, decreased T4, normal cortisol

what are risk factors for post concussive syndrome

severe initial symptom burden or delayed onset loss of consciousness for more than 1 minute at the time of injury vestibular symptoms preinjury history of psychiatric disorders, migraine headache, learning disorder, or repeated concussions

what bacterial infection can cause colitis or bloody vaginal discharge?

shigella

diamond blackfan anemia

short webbed neck severe MACROCYTIC anemia with RETICULOCYTOPENIA and absence of RBC precursors in bone marrow thumb abnormalities small snubbed nose urogenital abnormalities cardiac abnormalities usually presents with anemia < 1 year of age

what condition is associated with SHOX defects

short stature

case control study

similar subjects are divided into groups based on the presence or absence of the outcome of interest. Then comparisons are made regarding the frequency of risk factors in each group. Associations can be made but never proven in these types of studies. useful for rare conditions

treatments for strep throat

single dose IM PCN G daily amox x 10 days daily cefdinir x 5 days daily keflex x 10 days TID clinda x 10 days (for type 1 PCN hypersensitivity) daily azithro x 10 days

what is chemoprophylaxis for meningococcal exposure in close contacts?

single dose of ciprofloxacin 500 mg orally CTX 125 vs 250 mg as single dose Rifampin 600 mg BID x 2 days

symptoms of TCA ingestion

sinus tachycardia heart block QRS widening QT prolongation PVC's life threatening ventricular arrhythmias peripheral dilation and hypotension respiratory depression seizures coma

at what age can infants sit with and without support

sit with support by 6 months sit without support by 7 months

medullary cystic kidney disease

slowly progressive with onset in teenage years ESRD between ages 20-70

bronchiolitis obliterans

small bronchioles/bronchi are obstructed by masses or fibrinous material 2/2 adenovirus serotypes Rx: diuretics, O2, airway clearance Dx: bx

what type of cells make up neuroblastoma

small, round, blue cell tumors

which lifestyle factor is associated with follicular ovarian cyst

smoking

what are treatments for nitroprusside related cyanide toxicity after the drug has been removed

sodium thiosulfate hydroxocobalamin

at what gestational age is the pinna soft vs slowly returns from folding vs springs back from folding vs is a firm pinna

soft pinna - 24-31 weeks slowly returns from folding - 32-35 weeks springs back from folding - 36-39 weeks firm pinna - > 40 weeks

what back injury are gymnasts, ballet dancers and wrestlers at risk for?

spondylolysis with progression to spondylolisthesis 2/2 repetitive microtrauma from lumbar extension Dx: xray with scottie dog fracture, or PET exam: hamstring tightness and point tenderness, worse with backwards bending, NO lordosis

what is meant by stage I, II, III, IV in cancers

stage 1: single node/lesion above the diaphragm stage II: >2 nodes or lesions above the diaphragm stage III: on both sides of diaphragm or within the mediastinum, unresectable abdomen, paraspinal area, epidural stage IV: bone marrow or CNS is involved

between what stages of puberty do girls have the most heigh velocity

stage 2 and 3

what is the most common bacterial cause of chronic rhinosinusitis

staph aureus

what is the most common cause of breast abscesses in newborns

staph aureus

bullous impetigo

staph aureus causing flaccid coalescent bullae on previously normal skin

what are the most common bugs for septic arthritis in kids by age group

staph aureus is most common in all age groups < 3 months : strep agalactiae, GNR 2-3 years: kingella, strep pyogenes, strep pneumo > 5 years: strep pyogenes menstruating females: neisseria sickle cell: salmonella

what is the most common organism in VPS infection

staph epi

staph toxic shock syndrome vs strep toxic shock syndrome

staph: generalized red skin, fluid resistant hypotension, fever, diarrhea, multiorgan failure, desquamation Rx: vanc + clinda (decrease toxin production) +/- IVIG strep: similar symptoms but with POSITIVE BLOOD CX Rx: nafcillin + clinda

how does 1-25-(OH)2-D affect calcium absorption

stimulates Ca and PO4 absorption from the gut (small intestine) and renal tubules, may increase Ca re-absorption from bone if the levels are low this is the active form of Vit D

what immunologic mechanism causes contrast allergies?

stimulation of mast cells and basophils

what is the treatment for propofol related infusion syndrome

stop the drug + hemodialysis IF AKI and severe acidosis

what is the treatment for tularemia

streptomycin and gentamicin x 10 days cipro if mild disease sxs: ulceroglandular vs oculoglandular disease

severe apophysitis

stress reaction to multiple pulling ligaments common in kids 2/2 their skeletal immaturity leads to heel pain, worse with activity, improved with rest RF: recent increase in high impact sports a clinical diagnosis, no imaging needed Rx: heel cushion, icing, OTC anti-inflammatories

what is the only parasitic infection that you can find larvae in the stool

strongyloides

which neurological disorder is associated with glaucoma

sturge weber syndrome (port wine stain, might affect brain blood vessels on the same side)

cohort study

subjects are divided into groups based on the presence or absence of a presumed risk factor and are followed prospectively. These are powerful studies but are often costly and take a long time to complete. useful to study things in sequence

guttate psoriasis

sudden small scaly papules and plaques preceeded by strep throat or perianal strep will also have auspitz sign may appear on face, trunk or diaper region

what are the age cut offs for the triptans

sumatriptan > 18 yrs old almotriptan 12-17 yrs old rizatriptan >6 yrs old

what is the treatment for IgA nephropathy or HSP

supportive care consider steroids if they have significant proteinuria or Cr elevation, pulmonary hemorrhage, GI bleeding or severe abdominal pain

complete pyloric atresia

sxs: polyhydramnios, non bilious emesis, enlarged gas-filled stomach with rest of abdomen gasless on xray a/w epidermolysis bullosae Dx: upper gi

at what age do kids have symbolic vs parallel vs fantasy vs cooperative play

symbolic - 12 months parallel - 18 - 24 months fantasy - 3 years cooperative - 3-4 years

what are the reasons for symmetric and asymmetric IUGR

symmetric: congenital anomalies, chronic alcohol use, in utero infections, maternal SLE, cigarette smoking, maternal anemia asymmetric: multiple gestations, chronic maternal HTN, uteroplacental insufficiency, high altitude

clonidine toxicity

symptoms similar to opioid toxicity CNS depression respiratory depression pinpoint pupils bradycardia hypotension

painLESS lymphadenopathy painLESS genital ulcer

syphillis

what is the preferred device for dosing administration of meds

syringe! medication cups have more error

systemic loxoscelism

systemic toxicity following a bite from a brown recluse hemolysis, fever, chills, N/V, myalgia and rash

mechanism of action of milrinone

systemically and pulmonary vasodilates (PDE) inotrope and provides afterload reduction

what is the presentation for large cell non-hodgkins lymphoma

tender lymphadenopathy constitutional symptoms manifests in abdominen or mediastinum

side effects of efavirenz

teratogenicity cns toxicity

which drugs are associated with idiopathic intracranial hypertension

tetracyclines excessive vitamin A growth hormone

boot shaped heart on chest xray

tetralogy of Fallot anterior deviation of infundibular septum

ascaris lumbricoides

the LARGEST intestinal nematode and the cause of roundworm disease 2/2 ingestion of soil contaminated food ingest the eggs then larvae travel to the lung which you cough up and swallow *Loeffler syndrome: fleeting migratory infiltrates and atelectasis with eosinophilia found in tropics

what happens during the late phase response in type 1 hypersensitivities

the initial IgE reaction stimulates synthesis of cytokines basophils and eosinophils get involved typically occurs 3-12 hours after the immediate reaction responsible for airways hypersensitivity of asthma

idiopathic hypercalciuria

the most common metabolic condition that predisposes to the formation of kidney stones given persistent excretion of calcium into the urine, may cause dysuria Urinary Ca/Cr ratio > 0.2 isolated hematuria

why must you wait 4 weeks between live virus vaccines if not administered at the same time?

there is increased risk of seizure and decreased immunogenicity

why do kids with oligoarthritis JIA get frequent eye exams

they are at higher risk for asymptomatic anterior uveitis -especially if ANA positive (q3 mo eye exams) -especially the closer they are to initial diagnosis (q6 mo if ANA -, q12 mo if > 7 years post dx) -especially the younger they are when diagnosed

which vitamin deficiency is responsible for berberi and wernicke's encephalopathy

thiamine berberi: dry = paraethesias, foot and wrist drop; wet = heart failure and edema wernicke's: ophthalmoplegia, ataxia and confusion

what vitamin deficiency is associated with an infant crying but unable to make a sound

thiamine, B1

at what gestational age is vernix thick vs scant vs absent

thick - 24-38 weeks scant - 40-41 weeks absent - 42 weeks

cryptogenic organizing PNA

thickened alveoli 2/2 chronic inflammatory cell infiltrate and type II cell hyperplasia recurrent bronchitis that responds to abx but recurs Dx: lung bx Rx steroids

who needs an additional dose of the polio vaccine

those traveling to endemic countries who are fully vaccinated and intend to stay for >4 weeks

shwachman-diamond syndrome

thumb duplication neutropenia exocrine pancreatic insufficiency skeletal anomalies rib dystrophies osteopenia Autosomal recessive

what is a reliable tumor marker after thyroidectomy for cancer

thyroglobulin level

what is the most common cause of crhonic urticaria

thyroid disease

what is the most common cause of congenital hypothyroidism in the US

thyroid dysgenesis

window period with hepatitis B

time between when Hep B s Ag is negative and Hep B s Ab has not risen yet measure Hep B c IgM to confirm acute hepatitis

what treatments for GBS have shown benefit

tincture of time! plasmapheresis and IVIG which may improve recovery time do IVIG first and reserve plasmapharesis for severely ill people steroids have NOT shown benefit anticipate recovery in 3-4 weeks

what symptoms are seen in NF2, not NF1

tinnitus unsteady gait facial weakness hearing loss

otoacoustic emissions

tiny probe in the ear canal measures echos when clicks are played

what medication is approved for migraine ppx in kids 12-17

topamax

which anti seizure medication is associated with acute angle closure glaucoma

topamax

what is the treatment for pityriasis alba and what underlying skin condition is it associated with

topical emolient atopic dermatitis

what is the initial treatment for vaginal adhesions

topical estrogen therapy

what is the treatment for mild to moderate rosacea?

topical metronidazole and avoidance of triggers

what is the first line therapy for warts

topical salicylic acid

what is the management of hyphema (blood in the anterior chamber of the eye?)

topical steroids and cycloplegic drops +/- zofran to make sure it doesn't get worse with vomiting short term risk for rebleeding long term risk for glaucoma

retropharyngeal abscess examination and age

torticolis febrile appear ill drooling widened retropharyngeal space on CXR common in kids 2-4 years complication from sore throat

sciatic nerve injury 2/2 gluteal injections

total foot paralysis with loss of leg flexion sensory loss of leg below the knee

torch infection with parenchymal calcifications vs periventricular calcificaitons

toxo = perenchymal CMV = periventricular

benign acute childhood myositis

transient severe muscle pain and weakness usually affected the calves and occuring 1-2 days after URI from influenza A and B and occasionally parainfluenza and adenovirus usually in early school/pre-school kids

Pseudotumor cerebri

transient visual obscuration: episodes of blurry/gray vision associated with pseudotumor cerebri RF (meds): doxycycline, excess Vit A, isotretinoin, growth hormone RX: weight loss, topamax, acetozolamide sxs: diploplia (with optic nerve edema), migraines, pulse synchronous tinnitis, transient visual obscuration

"egg on a string" sign on newborn chest xray

transposition of the great vessels

which STI is responsible for strawberry cervix

trichomonas

the 5T's of cyanotic congenital heart defects

tricuspid atresia (LAD, dilated RA, LVH, decreased pulmonary vasculature) transposition of the great vessels (RAD, RVH) truncus arteriosus (RVH, LVH, dilated LA) tetrology of fallot (RVH, RAD) TAPVR (RAD, dilated RA, RVH) also think of critical pulmonary stenosis or pulmonary atresia

what cranial nerves are involved with marcus gunn pupil

trigeminal and occulomotor jaw winking phenomenon - simultaneous winking along with jaw-sucking motion

fragile x syndrome

trinucleotide repeat (> 200) X-linked dominant elongated facies, prominent and wide foreheads, large ears, prominent jaws, macro-orchidism, and attention-deficit/hyperactivity disorder (ADHD) females: premature ovarian failure, emotional disturbances

what is HIV post exposure PPX

truvada (emtricitabine-tenofovir) + raltegravir

what lab can be checked to determine if an allergic reaction has occurrec

tryptase

what is the preferred method for TB screening in kids < 2

tuberculin skin test

what is the most common cause of cord compression in pediatrics

tumors

what is the method of manual detorsion for testicular torsion

twist laterally left testicle gets clockwise rotation right testicle gets counterclockwise rotation

toddler's fracture

twisting distal mid tibial fracture 2/2 the child's foot getting stuck in something and them attempting to twist it free spiral fracture

what is the common mechanism of injury in meniscal tear

twisting motion of planted knee

what is represented by a normal serum anion gap with a positive urine anion gap and hyPOkalemia, Nephrocalcinosis and nephrolithiasis

type 1 distal RTA cannot excrete H+ ions will have elevated urine pH

von Gierke's

type 1 glycogen storage disease absent G6P fasting induced hypoglycemia, FTT, sz, hepatomegaly recurrent bacterial infections 2/2 neutropenia delayed puberty with gout afterwards 2/2 increased uric acid dx: decreased glucose, lactic acidosis, increased uric acid, HLD *lactic acidosis gets worse after glucagon but glucose does not increase rx: continuous gastric feeds, oral cornstarch

when do you start screening type 1 vs type 2 DM for proteinuria or microalbuminuria

type 1: 3-5 years after diagnosis type 2: yearly after diagnosis

progressive familial intrahepatic cholestasis

type 1: ADEK deficiencies, poor growth, cirrhosis, diarrhea, pancreatitis type 2: liver disease and normal GGT type 3: pruritus and increased GGT presents age 3-6 months with severe conjugated hyperbilirubinemia

vWF

type 1: quantitative deficiency *low vWF Ag, Factor 8, ristocetin cofactor assay activity type 2: qualitative problem type 3: undetectable vWF and decreased Factor 8 Rx: desmopressin, factor 8 concentrates, vWF dx: normal PT, +/- normal PTT, prolonged bleeding time

what is represented by a normal serum anion gap metabolic acidosis with a negative urine anion gap with urine pH > 5.5 and normal to low K, and Fanconi syndrome

type 2 (proximal) RTA cannot reabsorb bicarb will have decreased urine pH

Pompe's

type 2 glycogen storage disease and lysosomal storage disease because glycogen accumulates in the lysosome infantile = most severe and death by age 1 juvenile = death in 20's, no cardiac involvement adult: progressive myopathy without cardiac involvement that involves the pelvic girdle and diaphragm alpha glucosidase deficiency dx: muscle bx with vacuoles full of glycogen Rx: enzyme replacement therapy

serum sickness

type 3 hypersensitivity IgG + foreign Ag form immune complexes 6-12 days after exposure sxs: fever, rash, joint pain, LAD, myalgias, proteinuria, itching, urticaria, angioedema rx: sxs resolve spontaneously

what is serum sickness

type 3 hypersentitivity erythema marginatum arthritis glomerulonephritis LAD

what is represented by a normal serum anion gap with a negative urine anion gap with urine pH < 5.5 and hyPERkalemia, adrenal failure, CAH and DM

type 4 RTA cannot secrete K ions

McArdles

type 5 glycogen storage disease exercise induced muscle cramps with rhabdo after exercise - "2nd wind phenomenon" dx: increased NH3 with exercise rx: avoid strenuous exercise and eat glucose prior

how long after salmonella typhoid and non typhoid does a kid have to stay out of daycare

typhi: diarrhea has resolved, 3 negative stool cultures, 48 hours after antibiotics are done non typhoid: diarrhea has resolved

night terrors

typically occur in non-REM sleep during the first 1/3 of nocturnal sleep. ages 3 - 7 usually with family history worse with sleep deprivation The child awakens abruptly from sleep with a loud scream, appearing agitated, frightened, and confused. Facial flushing, diffuse sweating, tachycardia, and other signs of autonomic arousal are common. The child usually does not respond to parental efforts at calming. Amnesia for the episode the following morning is typical

lab studies for galactosemia

ua without glucose but with reducing substances GALT deficiency in RBC increased G1P

Blount disease

unilateral growth disturbance of the medial tibial epiphysis leading to severe varus deformities Requires bracing or surgical osteotomy. Presents in overweight African American females metaphyseal BEAK on xray

hemiplegic migraine

unilateral sided weakness that persists for days without aphasia + fam hx

peritonsillar abscess oral examination and age

unilateral tonsillar swelling associated with soft palate edema and deviation of the uvula common in teens hot potato voice Rx: drain the abscess, then may start steroids. if pus then abscess, if none then just cellulitis. can also differentiate abscess vs cellulitis on CT scan

how long can an up going babinski reflex be present until?

up to 12 months of life! if it is still present by 2-3 years of life then it likely represents neurodevelopmental abnormality

what is the gold standard for diagnosing volvulus and malrotation

upper GI series Volvulus = corkscrew sign malrotation = not crossing the midline

sxs of sphincter of Oddi dysfunction

upper abdominal pain like IBS of the bile ducts most common after cholecsytectomy

what restrictions in EOM occur with orbital floor blowout fracture

upward gaze restrictions

what are the two most common types of kidney stones in kids

uric acid or struvite stones

Darrier sign

urticaria 2/2 rubbing the skin

excess vitamin A consumption

usually in athletes hyperostosis leads to long bone and muscle pains, headaches, dry eyes and mouth, HSM, seborrheic skin changes and signs of pseudotumor cerebri

left anomalous coronary artery

usually presents in infancy or with kids who get chest pain with exercise may lead to sudden death

how do you differentiate between diarrhea and RTA in determining NAGMA

utilize urine anion gap a negative urine anion gap is more indicative of diarrhea (neGUTive) a positive urine anion gap is more indicative of RTA

what seizure medication is associated with hyperammonemic encephalopathy

valproic acid

what abx are used for a baby with skin, soft tissue, bone or joint infections

vanc + gent

what abx are used for late onset sepsis in a baby hospitalized since birth

vanc + gent

what are empiric abx for kids > 3 months old with osteomyelitis or septic arthritis

vanc OR clindamycin

what is empiric antibiotic treatment for epidural abscess

vanc and CTX

what are empiric abx for kids < 3 months old with osteomyelitis or septic arthritis

vanc and cefotaxime

mechanism of action of epinephrine

vasoconstricts (a) inotropic and slight increase in HR (b)

what medication is ppx treatment for cluster headaches?

verapamil to be started once the cluster begins and then tapered off

basilar artery migraine

vertigo, syncope, dysarthria, migraine, and sometimes loss of consciousness

Factor 12 deficiency

very prolonged PTT WITHOUT risk of bleeding with surgery

what is the Ag associated with acute early onset of EBV

viral capsid antigen *nuclear antigen means the patient is convalescing from EBV

most common bacteria in endocarditis in kids

viridans strep staph aureus

what vitamin deficiency is associated with glossitis, chelosis, seborrheic dermatitis, and conjunctivitis

vit B2 (riboflavin)

decreasing consumption of what vitamin can help decrease formation of calcium derived kidney stones

vit C and Vit D Other things to avoid: spinach, rhubarb, peanuts, cashews, almonds, salt Things to try and eat: fruits, vegetables, calcium, potassium, increased fluids, HCTZ

what vitamin deficiency is responsible for edema, thrombocytosis, hemolysis and anemia in an infant also ataxia, upward gaze restriction with visual field defects, hyporeflexia

vit E

what vitamin deficiency is associated with resistant neonatal seizures

vitamin B6 deficiency this is used to help with synthesis of GABA neurotransmitter

what is the drug of choice for invasive aspergillosis

voriconazole

how to differentiate central vs nephrogenic DI

water restriction test with restriction, in central DI the ADH remains inappropriately low and urine remains dilute but after desmopressin will become concentrated in nephrogenic DI, ADH becomes elevated but urine remains dilute since it cannot respond to it. after desmopressin, urine is STILL dilute

cryptosporidium parvum

watery diarrheal illness from water outbreaks sxs: abd pain, emesis, fever, weight loss, lack of appetite Rx: self limited, usually resolves by 2-3 weeks

what are the average birth weight, head circumference and length

weight 3.32 kg head circumference 35 cm length 50.8 cm

what is the parkland formula for calculating burns related replacement fluids

weight in kg x 3 x % of body surface burned *divide this rate in two and give first half over 8 hours, 2nd half over next 16 hours remember rule of 9's: 18 for each leg, 9 for arms, 18 for front and back of torso, 9 for head/neck, 1 for genitals *only applies for > 14 years of age if < 14 years, child's palm equates 1% of burned BSA Include miVF if child weighs less than or =30 kg

which four allergies do most kids outgrow

wheat egg milk soy

when can kids immunized against varicella who get varicella return to daycare?

when they have no new lesions appear in a 24 hour period

how does magnesium deficiency present

widened QRS peaked t waves wide PR arrhythmias tremor tetany muscle spasms convulsions weakness

xray finding for ricketts

widening of the growth plate associated with metaphysial fraying and cupping of the distal radius/ulna may also see scoliosis

how does hydroxyurea impact hemoglobin electrophoresis and CBC in sickle cell disease

will increase the amount of HbF and cause macrocytosis

what congenital heart disease is associated with left axis deviation on ekg with and without a murmur

with a murmur: VSD, or other AV canal defect such as ASD without a murmur: tricuspid atresia, total AV canal defect

lean production

work team defines the problems and finds solutions to enhance customer values by eliminating waste and incorporating defect alert system

what is the number 1 cause of MR worldwide and in the US

worldwide: rheumatic fever US: MVP

at what visual deficit should children be referred to opthalmologist

worse than 20/30

what vision testing makes athletes considered as functionally 1 eyed

worse than 20/40 when corrected

inheritance pattern of G6PD

x linked recessive

Hunter syndrome

x linked recessive mucopolysaccharide disorder where this accumulates in brain, spleen, liver, heart, lung, bones progressive neurological decline, hydrocephalus coarse facial features, hypertelorism, at risk for cardiac valvular leaflet dysfunction *differentiate from Hurler because of no corneal clouding

Hyper IgM

x-linked high IgM, all other immunoglobulins are low (compare to brutons*) + NEUTROPENIA T cell problem limiting ability to switch subclasses ~ recurrent sinopulmonary infections, PCP pneumonia , poor response to immunizations

what diabetes screening is needed in CF

yearly OGTT after age 10 to screen for CF related diabetes

how does nasolacrimal duct obstruction present

yellow discharge from the eye without corneal or red reflex abnormalities resolves by first year of life treat with daily gentle massage over duct

what is the most common malignant childhood germ cell tumor

yolk sac tumor will have elevated AFP

first order vs zero order kinetics relationship to drug concentration

zero order: rate of drug elimination is constant no matter the drug concentration first order: rate of drug clearance increases as drug concentration increases; reaches steady state by 4-5 half lives

what should MAP be for a neonate

~ gestational age in the first week of life