Peds Uworld Flashcards
cyanosis and single S2 (most common cardiac defect of neonatal period)
in contrast, what is the most common after the neonatal period?
in contrast, what will present with shock due to impaired systemic perfusion?
transposition of the great vessels
after: ToF
shock: coarctation of aorta and hypoplastic left heart
recurrent skin/mucosal infections, periodontal disease, ^WBCs with neutrophil predominance, think?
Leukocyte adhesion deficiency
also: lack of umbilical cord separation and no pus
acute edema of face, extremities, genitals, trachea, abdominal organs without urticaria, think?
hereditary angioedemia
C1 inhibitor deficiency
leads to ^bradykinin and C2b (edema-producing factors)
most common cause of acquired form is from using ACE-i
recurrent infections with S. aureus, Serratia, Burkholderia, Aspergillus, think ? what test to get?
Chronic Granulomatous Disease: impaired oxidative burst, NADPH oxidase deficiency, get inf. with catalase+ organisms
think Cats Need PLACESS to Belch their Hairballs
Nocardia, Pseudomonas, Aspergillus, Candida, E. coli, Staph, Serratia, Burkholderia, H. pylori
get dihydrorhodamine 123 test (old: nitroblue)
partial albinism and recurrent cutaneous infections with S. aureus and S. pyogenes, think?
Chediak-Higashi
eczema, thrombocytopenia, recurrent infections, think?
Wiskott-Aldrich
impaired cytoskeleton changes in WBCs/plts
tx: SCT
fever, lethargy and signs of heart failure after a viral prodrome, think?
how to manage?
viral myocarditis
ICU due to risk of acute decompensation and fatal arrhythmia
+/- cardiomegaly and pulmonary edema on CXR
failure to thrive, recurrent infections of all types, low lymphocyte and T cell concentrations, think?
tx?
SCID
tx: stem cell transplant
recent Giardia infection and lobar pneumonia, consider ?
Bruton’s X-linked agammaglobulinemia: abnormal B lymphocyte maturation, at risk for sinopulmonary infections with encapsulated orgs (H. flu, S. pyogenes) due to imp. humoral response, and GI infections i.e. Giardia due to absence of IgA
contraindications to DTaP
anaphylaxis to vaccine ingredients
additional for pertussis part: progressive neurological disorder or encephalopathy within wk of vaccine
tx for long QT syndrome
B-blockers (propranolol) with pacemakers (except sotalol which blocks K+ channels)
electrolyte derangements that can cause prolonged QT
low Ca2+, low K+, low Mg2+
meds that can cause prolonged QT
macrolides, FQs, antipsychs, TCAs, SSRIs, methadone, oxy, zofran, quinidine, procainanmide, flecainide, amiodarone, sotalol
failure to thrive and recurrent respiratory and GI infections, chronic lung disease especially with Giardia, encapsulated bacteria, and enterovirus, think?
common variable immunodeficiency: B-cell differentiation impaired leading to decreased of all Igs
wheezing, coughing, dysphagia, biphasic stridor that improves with neck extension, think?
what improves with prone positioning?
vascular rings
prone: laryngomalacia (collapse of supraglottic structures during inspiration)
should you tx rheumatic fever with abx?
yes, PCN, as they are at risk of recurrent episodes and progression of rheumatic HD with repeated infection with GABS pharyngitis
what is never normal on neonatal EKG?
left axis deviation
if present + decreased pulmonary markings on CXR, think tricuspid atresia (hypoplasia of RV and pulm. outflow tract)
AOM orgs
S. pneumo, H. flu (not Hib), Moraxella
tx: amoxicillin, then augmentin
how to visualize laryngomalacia
direct laryngoscopy
tinea capitis tx
oral terbinafine, griseofulvin, itraconazole, fluconazole
bruise looking rash on minority kiddo
Mongolian spots, self-resolve during childhood
tinea corporis treatment
topical clotrimazole, terbinafine
2nd line: oral terbinafine, griseofulvin
hemangiomas in kiddos
strawberry (superficial)
vs cherry in adults
proptosis, ophthalmoplegia (pain with moving eyes), diplopia, think?
most common predisposing factor?
orbital cellulitis
bacterial sinusitis
neonate with blanching erythematous papules and pustules, think ?
erythema toxic neonatorum, benign and resolves within 2 weeks
bedwetting is normal before age ? and does not warrant treatment before this time
age 5
conjunctival injection, tarsal inflammation, pale follicles, think ?
trachoma: C. trachomatis A, B, C infection, leading cause of blindness worldwide
tx: oral azithromycin, may need eyelid sx
macrosomia, hypoglycemia, macroglossia, umbilical hernia/omphalocele, hemihyperplasia (1 side bigger than other) think ?
what to do next?
Beckwith-Wiedemann syndrome
get abdominal U/S to check for development of Wilms tumor or hepatoblastoma
in contrast, hypothyroidism may have macroglossia and umbilical hernia but not the other features
conjugated hyperbili + hepatomegaly in a newborn, think?
what to do next?
treatment?
biliary atresia
dx: abdominal U/S
tx: Kasai procedure
Reye syndrome:
presentation
labs
biopsy
n/v, encephalopathy, acute liver failure
^LFTs, ^PT, INR, PTT, ^NH3
microvesicular steatosis
in contrast: macrovesicular fatty changes are seen in alcoholic hepatitis and in NAFLD
1 month old with painless blood-streaked stools, eczema, regurgitating feedings, think?
milk- or soy-protein proctocolitis (enterocolitis/allergy)
intussusception dx? tx?
dx: U/S: “target sign”
tx: air or water-soluble contrast enema
see microcolon on contrast enema, think?
meconium ileus (at the level of the ileum), will also have thick "inspissated" meconium in contrast, Hirschsprung will have a "transition point" on contrast enema (typically rectosigmoid)
infant supplement timeline
all exclusively breastfed infants: 400 IU vitamin D 1st mo
breastfed + preterm infants: iron at birth-1 yr
can introduce pureed foods at 6 mos
can introduce cow’s milk at 1 yr
vitamin B6 (pyridoxine) deficiency presents how?
cheilosis, stomatitis, glossitis + irritability, confusion, depression
in contrast, riboflavin (B2) def. presents with cheilosis, stomatitis, glossitis + normocytic anemia, seborrheic dermatitis
15 day old with bilious vomiting and no gas seen on abdominal XR, think ? what to do next?
treatment?
malrotation with volvulus (both meconium ileus and Hirschsprung will have some gas, those are dx with contrast enema)
dx: upper GI series (barium swallow), will see Ligament of Treitz on right side of abdomen (malrotation), and “corkscrew” pattern (volvulus)
tx: Ladd procedure (fix bowel)
why do newborns get jaundiced?
at what levels to do exchange transfusion?
^hgb turnover/bili production
decreased UGT activity (conjugating enzyme)
sterile gut cannot break down bili to urobilinogen
if total bilirubin exceeds 20-25 mg/dL, otherwise can use phototherapy
where it is worrisome for batteries to get lodged
esophagus: needs immediate endoscopic removal to prevent mucosal damage and esophageal ulceration
if distal, 90% pass uneventfully
8 yo: RUQ pain, dark urine, jaundiced, ^bili (D, T), ^amylase, ^lipase, +extrahepatic cyst on u/s, think?
biliary cyst: cause cholestasis, palpable mass, +/- pancreatitis
tx: surgical resection
f/u: cholangiocarcinoma
“lead points” in intussusception
most common: hypertrophy of Peyer patches from recent illness
others: Meckel’s, HSP, celiac, tumor, polyps
other risk factors: rotavirus vaccine
intussusception tx
U/S guided air contrast enema or saline enema
barium enema not done due to risk of peritonitis from barium leakage if intestine perforates
rehydration for dehydrated kiddos
mild-mod: oral
mod-severe: IV NS, (dextrose-containing fluids may be used in maintenance, but not resuscitation)
weight loss, iron-def anemia, vesicular skin rash, T1 DM, think?
celiac
also associated with IgA deficiency, Downs, AI thyroiditis
constitutional growth delay
normal birth weight/height
then drop to 5th/10th % btw 6mo-3yrs, remains low until growth spurt, should reach normal adult height
giving GH will NOT help, they are not deficient
3 mo old: hypoglycemic, lactic acidosis, hyperuricemia, hyperlipidemia
doll-like face, thin extremities, hepatomegaly
Glucose-6-phosphatase deficiency (type 1 glycogen storage disease, von Gierke disease)
cannot convert glycogen to glucose
in contrast, glucocerebrosidase deficiency (Gaucher) presents with bone pain, cytopenias, hepatosplenomegaly, but NOT hypoglycemia and lactic acidosis
Turners hormone levels
low estrogen, progesterone (“streak ovaries”)
^FSH, LH (disinhibited feedback)
most common cause of congenital hypothyroidism ww
thyroid dysgenesis
in contrast, transplacental passage of TSH-rec Abs leads to hyperthyroidism
when to remove testes in AIS
after puberty so chica gets to full height but reduces risk of malignancy
lead poisoning tx
moderate 45-69: DMSA
severe 70+: DMSA + EDTA
precocious puberty with low LH levels that do not ^after GnRH stim test
nonclassic CAH (21-hydroxylase deficiency)
primary amenorrhea w/u
FSH if no breast development
if FSH decreased: pituitary MRI (prolactinoma)
if FSH increased: karyotype
abdominal pain, shock, hematemesis, metabolic acidosis + radio opaque areas on XR think ?
tx?
iron poisoning, tx with deferoxamine
airborne vs droplet vs contact diseases
airborne: measles
droplet: influenza, RSV
contact: MRSA, rotavirus
most common causes of acute, u/l LAD in kids
S. aureus, S. pyogenes
Lyme tx for kids <8
amoxicillin PO or cefuroxime
impetigo tx
topical mupirocin
posterior oropharyngeal vesicles/ulcerations, fever, pharyngitis, think?
herpangina (coxsackie virus)
tx: supportive
all nonimmune, asymptomatic pts older than 1 yr with varicella exposure should be treated how?
post-exposure ppx with varicella vaccine
if immunocompromised get VZIG
Cat-scratch vs cat bite abx
Cat-scratch: azithromycin
Cat bite: augmentin (Pasteurella)
osteomyelitis tx
nafcillin/oxacillin or cefazolin
if suspect MRSA: vancomycin or clindamycin
if SCD: same as above + ceftriaxone/cefotaxime
most common predisposing factor for acute bacterial sinusitis
viral URI
prophylaxis for close contacts of pertussis?
macrolides for all, regardless of age, immunization status, or symptoms
most common cause of sepsis in SCD pt
S. pneumo (encapsulated, N. men and Hib are less common due to vaccinations)
Salmonella in osteomyelitis, not sepsis
hydroxyurea SE
myelosuppression
ALL markers
Tdt+, PAS+, no peroxidase + granules
pure red cell aplasia + webbed neck, short stature, cleft lip, shield chest, thumb anomalies, think?
diamond-blackfan anemia
medulloblastoma location/symptoms
posterior fossa, from cerebellar vermis
truncal/gait ataxia
obstructive hydrocephalus and s/s of ^ICP (near 4th ventricle)
kid with a stroke, think ? what to do next?
SCD, get Hgb electrophoresis
most common cause ped. stroke
labs in splenic sequestration
low Hgb, ^retics, low plts
in contrast, aplastic crisis will have normal plts but low retic count
limitation of upward gaze, think?
Parinaud syndrome, occurs with pinealoma
in contrast, craniopharyngiomas (suprasellar) can cause visual field deficits
s/s of polycythemia in neonate
respiratory distress, hypoglycemia, neurological manifestations
craniopharyngioma vs pituitary tumor
both can cause visual field deficits and hormone derangements
craniophayngiomas have calcification and light up on CT/MRI
anemia of prematurity
low EPO, low Hbg, HCT, and retic count
poor growth, macrocytic anemia, congenital marrow failure, short stature, other physical anomalies (skin, digits), think?
Fanconi anemia (AR) DNA repair defect causing chromosomal breaks
aplastic anemia vs aplastic crisis
anemia: pancytopenia due to BM failure (congenital or acquired)
crisis: just anemia, typically in SCD pt exposed to Parvovirus B19 (arrest of EPO, low retic)
kiddo develops isolated thrombocytopenia (low plts) and petechia after viral infection, think ? how to manage?
immune thrombocytopenia (ITP)
kids typically recover spontaneously in 6 mos, only need observation (regardless of platelet count)
BUT if bleeding should receive IVIG or steroids
in contrast, adults with plts less than 30,000 or bleeding need IVIG/steroids
nuclear remnants within RBCs in asplenic pts
ribosomal precipitates with thals or lead
small inclusions seen in G6PD def.
Howell-Jolly bodies
Basophilic stippling
Heinz bodies
iron def labs
low MCV, ^RDW, low iron, low transferrin sat, ^TIBC, low RBC count
in contrast, thalassemia has a normal RDW and RBC cell count
sepsis in a SCD kid, think?
pneumococcal, ppx with conjugate capsular polysaccharide vaccine and PCN
SCD can lead to what vitamin deficiency?
folate (^reticulocytosis)
what is hemophilic arthropathy seen in hemophiliacs caused by?
iron/hemosiderin deposition leading to synovitis and fibrosis in the joint
(prolonged PTT)
dx of ALL
+25% lymphoblasts on bone marrow biopsy
most common complications of bacterial meningitis
hearing loss!
others: intellectual injury, CP, learning disabilities, seizure disorder
absence seizure disorder is associated with
ADHD
cherry red macula, hepsplenmeg, areflexia, feeding difficulties (2-6 mos), think?
sphingomyelinase deficiency: Niemann-Pick
IN CONTRAST, Tay-Sachs has same presentation but HYPERREFLEXIA and NO hepslenmeg
Krabbe disease?
Gaucher?
Hurler?
Krabbe: galactocerebrosidase deficiency (hypotonia, areflexia, regression)
Gaucher: GLUCocerebrosidase deficiency (anemia, low plts, hepsplenmeg)
Hurler: lysosomal hydrolase deficiency (coarse features, hernias, corneal cloud, hepsplenmeg)
Marfan vs Homocystinuria
Marfan: AD, NORMAL intellect, aortic root dilation, UPWARD lens dislocation, Fibrillin-1 gene
Homo: AR, intellectual disability, +thrombosis, DOWNWARD lens dislocation, meg. anemia, fair skin
fever, HA, FNDs (classic triad)
+/- seizure in kiddo with congenital HD and recurrent sinusitis, think?
brain abscess
^risk for direct spread: OM, mastoiditis, sinusitis, dental infection
^risk blood spread: cyanotic HD (bypass lung), other infected sites (seed GW-J)
CGG repeats, think?
Fragile X: MCC inherited intellectual disability
prominent jaw, large ears, macroorchidism
stroke s/s after fall with toothbrush in mouth, think?
carotid artery dissection or thrombus formation
FTT, b/l cataracts, jaundice, hypoglycemia, think?
galactosemia: galactose-1-phosphate uridyl transferase deficiency
may lead to MR, cirrhosis, ^risk E. coli sepsis
tx: eliminate galactose
mild (only cataracts) if galactokinase deficiency
kids with Downs are at ^risk what malignancy
ALL
LE weakness more than UE
Werdnig Hoffman (spinal muscular atrophy)
infant botulism is from ?
how to tx?
in contrast to food-borne
C. botulinum spores (environment)
tx: human-derived botulism Ig
IN CONTRAST, food-borne is from ingestion of preformed toxin, tx: equine-derived antitoxin
BOTH have descending flaccid paralysis
LOC, post-octal state, transient hemiplegia (1-sided paralysis), think?
Todd paralysis
transient, focal weakness after focal/generalized seizure
r/o other causes with CT/MRI if no clear hx of preceding seizure
most common brain tumor in kids
astrocytoma
brain tumor presentations supretentorial: posterior fossa: brainstem: SC:
supretentorial (astrocytoma, craniopharyngioma): ^ICP, seizures, sensory changes
posterior fossa (medulloblastoma, ependymoma): ^ICP, ataxia, clumsiness
brainstem: ataxia, clumsiness, CN palsies
SC: back pain, weakness, abnormal gait
risk factors for IVH
premies, LBW
if brief loss of consciousness, HA, and vomiting after head injury, what to do?
get head CT without contrast as it is a mild TBI
cavernous hemangioma along trigeminal nerve distribution + intra-cranial calcifications that resemble a train-track
Sturge-Weber
Uncoordinated and limited voluntary movements, think? What are the most common risk factors?
Cerebral palsy
Prematurity, intrauterine growth restriction, alcohol consumption, multiple gestation, tobacco use
Comorbidities: epilepsy strabismus scoliosis
A two-year-old should have how many words in their vocabulary?
More than 50 and be able to combine them into two word phrases
Delayed muscle relaxation, facial weakness, foot drop, dysphasia, cardiac conduction anomalies, cataracts, testicular atrophy, baldness, think?
Myotonic muscular dystrophy, autosomal dominant expansion of the CTG repeat
Later onset than other dystrophies, age 12 to 30
Wilms tumor versus neuroblastoma origins
Wilms tumor: metanephros, precursor of the renal parenchyma
Neuroblastoma: neural crest cells, precursor of the sympathetic chains and adrenal medulla
Wide-based, unsteady gait, weakness and lower limbs, decreased vibratory in position sense in the extremities, atrophy of the cervical spine accord, T-wave inversion’s on EKG, think?
Friedreich ataxia, autosomal recessive, GAA nucleotide repeats, leads to myocarditis and cardio myopathy, arrhythmias, CHF
Genetic counseling is recommended
Amenorrhea, short stature, anosmia, delayed development, low FSH and LH, normal uterus and ovaries, think?
Kallmann syndrome
46, XX
Tourette’s is associated with increased risk of?
Tourette’s treatment?
ADHD and OCD
Treatment: antipsychotics: Risperidone, alpha 2 adrenergic receptor agonist: clonidine, Guanfacine
Gold standard for muscular dystrophy diagnosis
Genetic studies
Supportive diagnostic tests: elevated CK, aldolase; fibrosis and fatty infiltration on calf muscle biopsy
Precocious puberty, pigmentation, polyostotic fibrous dysplasia think?
McCune Albright syndrome
Pigmentation: café au lait spots
Edema in Turner’s syndrome is from?
Abnormal development of the lymphatic system
Hypotonia, hyperphagia, obesity, think? What genetic abnormality?
Prader Willi syndrome, loss of paternal copy of 15q11-q13
Loss of maternal copy results in Angelman syndrome, (paternal uni parental disomy): short stature, intellectual disability, abnormal smiling/laughter, hand flapping, ataxia, seizures
Ataxia, scoliosis, cardio myopathy, Think? Most common cause of death?
Friedrich ataxia, most common cause of death is cardiomyopathy or respiratory complications
Risk factors for RDS
Prematurity, maternal diabetes, C-section
Hi levels of insulin antagonize cortisol and block surfactant production
Diagnosis of minimal change disease
Not required, highly responsive to steroids
Toddler with the firm, smooth, unilateral abdominal mass and hemateria, think?
Wilms tumor, the most common pediatric renal malignancy, peaks age 2 to 5, Does not cross midline
In contrast, neuroblastoma happens more commonly in the first year of life, affects the adrenal gland, crosses the midline and has systemic symptoms
African-American boy with asymptomatic hemateria think?
Renal papillary necrosis due to Sickle cell trait
If subcutaneous emphysema, get? To rule out?
Get chest x-ray to rule out pneumothorax
May occur secondary to severe coughing
2 to 24 month old with febrile UTI, get? To evaluate?
If recurrent UTIs also get? To evaluate for?
Get Renal ultrasound to evaluate for anatomic abnormalities
If recurrent, get a VCUG to evaluate for vesicoureteral reflux
First line medical treatment for enuresis if behavioral an alarm therapy fails
Desmopressin
TCAs: imipramine, are just as effective but have more serious side effects
First steps If newborn with respiratory compromise and suspected diaphragmatic hernia
Endotracheal intubation, gastric tube to decompress the stomach and bowel
Bag mask ventilation can exacerbate respiratory decline
Get CXR when stable
Nephrotic syndrome in kid with hepatitis B think?
Membranous nephropathy
Complications of bronchiolitis
Apnea and respiratory failure
CF kid with pneumonia, treated with Tamiflu, cefepime and ? for ?
Vancomycin for S. aureus, most common cause of bacterial pneumonia in young kids with cystic fibrosis especially with coexisting influenza
In contrast, Cipro would be appropriate for adults with CF to treat Pseudomonas
Upper motor neuron findings in a kid with down syndrome, think?
Atlantoaxial instability
Kawasaki disease is a vasculitis characterized by fever for at least five days and 4/5 of the following:
Conjunctivitis, extremity changes, cervical LAD, oral mucosal changes (strawberry tongue), rash
Migratory Polyarthritis, pink rash with sharp edges,fever, elevated ESR, think?
Acute rheumatic fever
Transient Cinnabay this versus Legg-Calve-Perthes disease
Transient synovitis should self resolve in 1 to 4 weeks
Solitary, painful,lytic bone lesion plus Hypercalcemia in a kid, think?
Think neoplastic process: Langerhans cell histiocytosis
In contrast, hyperparathyroidism typically occurs in patients over 50
Fever, rash, joint pain 1 to 2 weeks after exposure to penicillins or sulfa drugs, think?
Serum sickness like reaction
Type 3 hypersensitivity
Treatment: remove offending agent, supportive care, steroids if severe
In contrast, penicillin in the setting of EBV causes a rash that spares the extremities and does not have joint pain
think compartment syndrome if?
Six P’s:
Pain, pallor, poikilothermia, paresthesias, and late findings of pulselessness, paralysis
Isolated polyarthralgia in a kid, think?
Lab values?
Juvenile idiopathic arthritis
Elevated ESR, CRP, elevated ferritin, elevated IgG, elevated platelets, anemia
