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
