PEDS UWORLD

homocystinuria vs marfan

(h) *AR intellectual disability thrombosis downward lens dislocation* megaloblastic anemia fair complexion thrombosis (m) *AD normal intellect aortic root dilation upward lens dislocation*

synovial fluid aspiration with what values should prompt for surgical drainage of joint space?

+100k/uL leukocytes, +90% neutrophils and purulent fluid otherwise > femoral head necrosis > hip dislocation or leg length discrepancy MC: staph aureus and streptococci

SCD complications

- renal disease/papillary necrosis - chronic RBC transfusions > iron overload > hemosiderosis-related cirrhosis and cardiomyopathy; iron chelation therapy - osteonecrosis at femoral heads; progressive hip pain on weight-bearing - increased stroke risk

management of sickle cell anemia and acute pain crisis

- vaccination - penicillin until 5 yo - folic acid supplementation - hydroxyurea for patients with recurrent faso-occlusive crises ( myelosuppression is dose limiting side effect) for acute pain crisis: - hydration, analgesia, +/- transfusion

Kawasaki dz

1. fever for 5 days 2. cervical lymph node + 1.5 cm 3. rash 4. swelling and/or erythema of palms/soles 5. bilateral nonexudative conjunctivitis 6. mucositis 7. coronary artery aneurysm txt: aspirin + IVIG

evaluation of bilious emesis

1. stop feeds, NG tube decompression, IV fluids 2. Abd xray a. free air, hematemesis or unstable vital signs: surgery b. dilated loops of bowel: contrast enema > if microcolon: *meconium ileus* if rectosigmoid transition zone: *hirschsprung dz* c. NG tube in misplaced duodenum: upper GI series> ligament of treitz on right side of abdomen: *malrotation* if corkscrew pattern: *volvulus* d. double bubble sign: *duodenal atresia*

infantile hypertrophic pyloric stenosis

1st born boy erythromycin bottle feeding non bilious emesis poor weight gain dehydration olive-shaped mass hypochloremic metabolic alkalosis thickened pylorus tx: IVF and pyloromyotomy

Myotonic Muscular Dystrophy

AD expansion of a CTG trinucelotide repeat in DMPK gene on chromosome 19 12-30 yo facial weakness, hand grip myotonia, dysphagia arrhythmias, cataracts, balding, testicular atrophy/infertility death from respiratory or heart failure depending on age of onset

Fanconi's anemia

AR progressive pancytopenia and macrocytosis cafe-au-alit spots microcephaly micropthalmia short stature horseshoe kidneys absent thumbs

krabbe disease

AR lysosomal storage disorder galactocerebrosidase deficiency intellectual disability blindness deafness paralysis neuropathy seizures

systolic ejection murmur at left upper sternal border

ASD

Burr cells

CKD, low EPO

ascending weakness + feet tingling + neuropathic pain > flaccid paralysis with absent DTRs and nerve conduction velocities h/o recent URI or GI infection (C. jejuni)

GBS - demyelination of peripheral motor nerves

posterior fossa tumor

ICP ataxia clumsiness

Edward's Syndrome

IUGR microcephaly *VSD - holosystolic murmur: left lower sternal border* closed fists with overlapping fingers micrognathia prominent occiput rocker-bottom feet severe intellectual disability 95% die in year 1 due to cardiac failure or respiratory failure

6 yo h/o CF; multiple hospitalizations of respiratory infections pt: SOB PE: fever, nasal flaring, grunting, bibasalr crackles and expiratory wheezing, flu + s/p cefepime and oseltamivir empiric abx?

IV vancomycin S. aureus is most common pathogenic organism in young children with CF especially w/ influenza cefepime: methicillin sensitive s. aureus and Pseudomonas MRSA covered with IV vanc

naficillin

MSSA and strep pneumo

acetaminophen toxicity

N-acetylcysteine

continuous flow murmur in left subclaviccular murmur

PDA

impetigo

S. aureus or Strep pyogenes painful, erythematous lesions --> honey-crusted

holosystolic murmur at left lower sternal border

VSD

Duchenne

X linked deletion dystrophin 2-3 yo progressive weakness, gower maneuver, calf pseudohypertorphy scoliosis and cardiomyopathy wheelchair by adolescence death by 20-30 from respiratory or heart failure

breast feeding C/I

active/untreated TB maternal HIV infection herpetic breast lesions varicella infection 5 days prior to or within 2 days of delivery chemo street drugs or alcohol galactosemia

lyme dz tx

adults: doxycycline children under 8 and pregnant women: amoxicillin or cefuroxime lyme meningitis or heart block: IV ceftriaxone

varicella infection

aerosolized transmission with 2 week incubation, prodrome, maculopapular rash followed by successive "crops" of vesicles that are pruritic and crust over prevention: 2 doses of VZV vaccine ages 1 and 4

fabry disease

alpha-galactosidase deficiency angiokeratomas peripheral neuropathy asymptomatic corneal dystrophy

C/I to Diptheria/tetanus vaccine

anaphylaxis

C/I to pertussis vaccine

anaphylaxis to vaccine ingredients progressive neurologic disorder (uncontrolled epilepsy, infantile spasms) encephalopathy w/i week of previous vaccine dose

SCD - blood smear

asplenia - S. pneumo, H. flu and N. men infection - Howell Jolly bodies

brainstem tumor

ataxia clumsiness cranial nerve palsies

spherocytes

autoimmune hemolytic anemia (abx destruction of RBCs)

atypical pneumonia tx

azithromycin

spinal cord tumor

back pain weakness abnormal gait

midgut volvulus

bilious emesis < 1 month corkscrew pattern on upper GI Ladd procedure

SGA infants

bw below 10th percentile for gestational age maternal factors: preeclampsia, malnutrition, placental insufficiency, multiparty, drug use complications: hypoxia, perinatal asphyxia, polycythemia, meconium aspiration, hypothermia, hypoglycemia, hypocalcemia and polycythemia

moderate to severe lead poisoning tx

calcium EDTA

hyperkalemia - cardioprotection?

calcium gluconate

tuberous sclerosis

cardiac rhabdomyomas renal angiomyolipomas eventually all get epilepsy

radial head subluxation

child pulled, lifted or swung by arm (axial traction on forearm with elbow extended) pt: arm held extended and pronated no swelling, deformity or focal tenderness tx: hyperpronation of forearm or supination of forearm and elbow flexion

eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

chronic rhinosinusitis nasal polyps asthma prominent eosinophilia

P. aeruginosa is MCC of pneumonia in adults tx

ciprofloxacin

ehler's danlos

collagen scoliosis joint laxity skin hyper elasticity

Rubella (german measles)

congenital: sensorineural hearing loss, cataracts, PDA children: fever, cephalocaudal spread of maculopapular rash, post auricular and sub occipital adenopathy (v measles with cervical lymphadenopathy) adults: same as kids plus or minus arthritis dx: serology prevention: live attenuated vaccine

pediatric viral myocarditis

coxsackie b virus adenovirus viral prodrome heart failure: dyspnea, syncope, tachycardia, nausea, vomiting, hepatomegaly CXR: cardiomegaly and pulmonary edema ECG: sinus tachycardia Echocardiogram: decreased ejection fraction, diffuse hypokinesis Endomyocardial biopsy: inflammatory infiltrate of myocardium with myocyte necrosis tx: diuretics and inotropes

trisomy 13 patau

cutis aplasia and micropthalmia

maternal lithium use displacement of malformed tricuspid valve into RV severe tricuspid regurgitation and right atrial enlargement

ebstein's anomaly

Wiskott-Aldrich syndrome (3)

eczema, thrombocytopenia, hypogammaglobulinemia

clubfoot (talipes equinovarus)

equines and various of calcaneumand talus, , mid foot virus and adduction of forefoot stretching and manipulation followed by serial casting

foreign body ingestion

esophageal coins in asymptomatic patients can be observed for up to 24 hours if pt is symptomatic or time of ingestion is unknown, remove via flex endoscopy

neonatal displaced clavicular fracture

fetal macrosomia (maternal DM, hyperglycemia, post-term pregnancy), instrumental delivery, shoulder dystocia crying/pain with passive motion of affected extremity crepitus over clavicle asymmetric moro x-ray confirm tx: reassurance, gentle handling, analgesics, can pin with elbow flexed

iron poisoning

free radical production and lipid per oxidation > abdominal pain, hematemesis, hypovolemic shock and metabolic acidosis radiopaque tablets seen on abdominal X-ray deferoxamine

SCID

gene defect leading to failure of T cell development B cell dysfunction due to absent T cells X-linked recessive and autosomal recessive recurrent, severe viral, fungal or opportunistic infections, FTT, chronic diarrhea (pneumocystis jirovecii) lymphopenia and hypogamma globulinemia tx: stem cell transplant

neonatal polycythemia

hematocrit +65% - increased erythropoiesis from intrauterine hypoxia: maternal DM, hypertension or smoking; IUGR - erythrocyte transfusion: delayed cord clamping; twin twin transfusion - genetic/metabolic disease: hypo or hyperthyroidism; genetic trisomy (13, 18, 21) asymptomatic (MC); ruddy skin, hypoglycemia, hyperbilirubinemia, respiratory distress, cyanosis, apnea, irritability, jitteriness, abd distension IVF glucose partial exchange transfusion

severe lithium tox tx

hemodialysis

herpangina vs. herpetic gingivostomatits

herpangina - coxsackie A - gray vesicles on tonsillar pillars and posterior oropharynx HSV - herpes - vesicles on anterior oropharynx and lips

eczema herpeticum

herpes virus infection h/o atopic dermatitis painful vesicular rash, fever, and viral dissemination

OP tx of community acquired pneumonia due to s. pneuma

high dose oral amoxicillin

HIV infancy

high maternal viral load; breastfeeding by infected mother failure to thrive, chronic diarrhea, lymphadenopathy, pneumocystis pneumonia dx: DNA PCR testing; persistence of HIV antibody after age 18 months tx: HAART

LGA infants complications

hip subluxation talipes calcaneovalgus

serum sickness-like reaction

immune complex formation abx (betalactams: penicillin, amoxicillin, cefaclor or sulfa: TMP-SMX) acute hep B 1-2 weeks s/p exposure fever, skin rash and polyarthralgia supportive care steroids or plasmapheresis if severe

supratentorial tumor

increased ICP, seizures

when IV access cannot be obtained in emergency cases, what kind of access should be attempted?

intraosseous MC site: proximal tibia C/I: infection over access site, fracture or previous IO attempts in chosen extremity or bone fragility

intermittent abdominal pain draws legs up non bloody bilious vomiting currant jelly stool ileocolic junction

intussusception ultrasound > target sign air enema

chronic pulmonary aspergillosis

itraconazole and voriconazole

hand foot and mouth dz

lesions in hands, feet and in mouth, vesicular but rarely painful of pruritic

11b hydroxylase

low cortisol and aldosterone high testosterone and 11 deoxycorticosterone (mineralocorticoid) and 11 deoxycortisol ambitious genitalia in girls fluid and salt retention, hypertension

21 hydroxylase deficiency

low cortisol and aldosterone high testosterone and 17-hydroxyprogesterone ambigious genitalia in girls salt wasting (vomiting, hypotension, low sodium and high K)

17a hydroxylase

low cortisol and testosterone high mineralocorticoids high corticosterone female looking fluid and salt retention, hypertension

Diamond-Blackman syndrome

macrocytic anemia (w/o hyper segmentation of neutrophils unlike megaloblastic anemia) low reticulocyte count webbed neck, cleft lip, shielded chest, triphalangeal thumbs defect in erythroid progenitor cells > increased apoptosis > programmed cell death

torsades de pointes tx

magnesium sulfate

1 yo girl 1 day hx of fever and drowsiness hypothermic, lethargic, nuchal rigidity flexes hips when neck is flexed septic looking large petechial and purpuric lesions on body ED -- suddenly hypotensive COD?

meningococcemia > Waterhouse-friderichsen syndrome sudden vasomotor collapse and skin rash due to adrenal hemorrhage

schistocytes

microangiopathic hemolytic anemia HUS

5 yo boy 1 week h/o edema, fatigue, abd pain labs: proteinuria

minimal change dz renal biopsy would show no changes EM: effacement of foot processes tx: corticosteroids

precautions to diptheria/tetanus vaccine

moderate or severe acute illness w/wo fever GBS within 6 weeks of tetanus toxoid containing vaccine arthrus type hypersensitivity reaction following diphtheria or tetanus toxoid containing vaccine

precautions to pertussis vaccine

moderate or severe acute illness w/wo fever seizure within 3 days temperature +40.5c/105f wi 2 days hypotonic-hyporesponsive episode within 2 days inconsolable, persistent crying within 2 days macrolides for post exposure prophylaxis

breastfeeding benefits

more rapid uterine involution and decreased postpartum bleeding faster return to prepartum weight improved child spacing improved maternal-infant bonding reduced risk of breast and ovarian cx improved immunity, gi function prevention of OM, gastroenteritis, respiratory illness, UTIs decreased risk of childhood cx, DM1 and nec

SCFE

obese adolescents displacement of femoral head on femoral neck due to disruption of proximal femoral growth pain hip or knee pain of insidious onset that causes limping loss of abduction and internal rotation of hip as well as external rotation of thigh when hip is being flexed frog-leg, lateral view xr is dx tx: surgical pinning of slipped epiphysis

Meckel's diverticulum

painless hematochezia heterotypic gastric tissue technetium-99 m pertechnetate scan lead point for intussusception (abd us > diagnostic and therapeutic air enema)

limited upward gaze upper eyelid retraction pupils non-reactive to light, reactive to accommodation non bloody, non bilious emesis

parinaud syndrome (dorsal midbrain) pineal gland masse

diagnostic features of acute bacterial rhinosinusitis

persistent symptoms for + 10 days w/o improvement OR severe symptoms, fever + 39 (102), purulent nasal discharge or face pain +3 days OR worsening symptoms +5 days after initially improving viral URI S. pneumo (30%) H. influenzae (30%) M. cat (10%) tx: amox-clav predisposing: viral URI

MCC brain tumor in children

pilocytic astrocytoma (low grade)

nocturnal perianal pruritis eggs on tape test albendazole or pyrantel pamoate for patient and all household contacts

pinworm (enterobius vermicularis)

ependymomas

posterior fossa; 4th ventricle glial cell tumors arise from ependymal cell lining of ventricles and spinal cord obstruction of CSF and increased ICP

ALL

predominant leukemia ages 2-10 30-50% present with infections, half with lymphadenopathy and splenomegaly dx: presence of more than 25% lymphoblasts in bone marrow; lymphoblasts stain PAS +; immunostaining for TdT is + in more than 95% of patients

Measles (rubeola)

prodrome: fever, malaise, conjunctivitis, coryza, cough Kopek spots exanthema: blanching, reddish-brown, maculopapular or morbilliform rash with cephalocaudal and centrifugal spread *(no palms/soles)* dx: PCR or anti measles IgM or IgG prevention: live attenuated measles vaccine tx: vitamin A complications: OM, pneumonia, encephalitis, SSPE

Transient erythroblastopenia of childhood

pure red cell aplasia without macrocytosis

LAD

recurrent skin and mucosal bacterial infections (omphalitis periodontitis) - no pus - poor wound healing delayed umbilical cord separation (+21 days) marked peripheral leukocytosis with neutrophilic

lead poisoning managemnt

screening capillary lead level +5 ug/dL > confirmatory venous lead measurement if lead level is: 5-44 mcg/dL (mild): no medication, repeat in less than 1 month 45-69 (moderate): DMSA +70 (severe): dimercaprol + EDTA

medulla blastoma

second most common tumor of posterior fossa vomiting headaches ataxia

anaphylaxis during blood transfusions

selective IgA deficiency

cranipharyngiomas

sella turcica (supratentorial) cystic structures with calcifications visual field defects and hormone probs (compression of optic chasm and pituitary stalk)

TCA and aspirin OD

sodium bicarbonate

absence seizures

staring spells preserved muscle tone unresponsive to tactile/verbal stimulation short duration (<20 seconds) simple automatisms provoked by hyperventilation no post-octal phase dx: 3 hz spike wave discharges during episodes cm: ADHD and anxiety tx: ethosuxamide

scarlet fever

strep progenies (group A) fever and pharyngitis tonsillar erythema and exudates strawberry tongue tender anterior cervical nodes sandpaper rash rapid streptococcal antigen test throat culture penicillin

mild to moderate lead poisoning tx

succimer

glioblastomas

supratentorial high-grade astrocytic tumors that arise in cerebral hemispheres and present with seizures

Becker

x-linked deletion dystrophin 5-15 yo milder weakness compared to duchenne cardiomyopathy death by 40-50 from heart failure

target cells

thalassemia

cyanotic infant left axis deviation and small/absent R waves in precordial leads hypo plastic Right ventricle and diminished R ventricular forces on ECG pulmonary under circulation > decreased pulmonary markings

tricuspid valve atresia