• Girls: thelarche (breast buds)→ pubarche (pubic hair) → growth spurt → menarche
• Boys: gonadarche (testicles enlarge) → pubarche → adrenarche (axillary & facial hair, voice changes) → growth spurt

• Boys: 11.5 years
• Girls: 10.5 years is average age of puberty. Average age of menarche is 12.5 years.

• normal variant of growth
• growth curve lags behind peers, but is consistent
• there's often a + FHx
• children catch up and ultimately achieve target height potential.

• systemic disease (ex. IBD)
• malnutrition
• gonadal dysgenesis (ex. Klinefelter's or Turner's)
• endocrine abnormalities (ex. hypopituitarism, hypothyroidism, Kallman's syndrome, androgen insensitivity, Prader-Willi)

• Hib
• abrupt onset of fever, drooling, sore throat, dysphagia; patient may keep neck hyperextended to maximize airway diameter 
• potential complication is airway obstruction - "muffled, hot potato voice"
• may require nasotracheal intubation

• parainfluenzae type I
• presents with fever, stridor, and barking cough (drooling is uncommon)
• tx - corticosteroids, nebulized epinephrine

• 1 month old with projectile vomiting, fair skin, blue eyes, eczematous rash, and mousy odor
• Test phenylalanine levels in blood or Guthrie test = qualitative coloration test that detects phenylalanine metabolites in urine

• cluster of findings of porphyrias
• what do you test for diagnosis?

• neurologic sx, photosensitivity, hyperpigmentation
• test for aminolevulinic acid and porphobilinogen

• brain tumors (gliomas (includes GBM) and medulloblastomas) associated with FAP or HNPCC
• autosomal recessive

• autosomal dominant
• intestinal hamartomatous polyps and mucocutaneous melalocytic macules

• also known as multiple hamartoma syndrome
• GI hamartomas
• breast cancer
• thyroid cancer
• nodular gingival hyperplasia

• juvenile polyps
• ectodermal abnormalities: alopecia, hyperpigmentation, nail loss (onycholysis)

• usually preceded by diarrheal illness caused by E. Coli 0157:H7, Shigella, Salmonella, Yersinia, & Campylobacter
• Sx: GI bleed, purpura, HTN, microangiopathic hemolytic anemia
• labs: blood smear with schistocytes and giant platelets, elevated LDH, elevated retic count, elevated indirect bili, elevated BUN, and elevated creatinine; thrombocytopenia
• kidneys have greatest M&M 

• PE: hepatosplenomegaly
• microcytic hypochromic anemia
• blood smear shows target cells

• macrocytic anemia
• hypersegmented neutrophils on blood smear

Serum sickness-like reaction -

what is it? presentation/ sx (4), tx

• adverse reaction to certain drugs: penicillin amoxicillin, TMP-SMX, cefaclor
• sx occur 1-2 wks after administration of drug and are similar to sx seen in serum sickness: polyarthralgia, urticarial rash, fever, lymphadenopathy
• resolves with withdrawal of drug (not a true allergy)

• systemic IgA-mediated vasculitis that occurs after URI
• sx: arthralgias, purpura on lower extremities, abdominal pain, renal disease (may also have scrotal swelling)
• sx can develop in any order over a period of days to weeks
• diagnosis confirmed by evidence mesangial IgA deposition in kidney or IgA deposits in skin (detected with immunofluorscence microscopy)
• intussusception is a potential complication - may present with blood in stool; tx = emergent surgical repair (can use barium or air enema)

• infant has macrosomia (4000 g), macroglossia, visceromegaly (kidneys, liver), omphalocele, hypoglycemia, hyperinsulinemia, microcephaly, prominent eyes and occiput
• usually sporadic; can be assoc with chr 11 duplication (IGF-II gene)
• increased risk of Wilm's tumor, hepatoblastoma, gonadoblastoma

• Wilm's tumor
• Aniridia (absent iris)
• GU anomalies
• mental Retardation
• deletion on chr 11 involving WT1 gene and PAX6 (aniridia) gene

• increased risk of Wilm's tumor
• male pseudohermaphroditism
• early onset renal failure with mesangial sclerosis

• deficiency of glucose-6-phosphatase
• affects kidneys and liver
• severe hypoglycemia with fasting (liver does not release glucose)

• Patients usually less than 7-8 yo
• mutliple seizure types (intractable epilepsy) & mental retardation
• interictal EEG shows diffuse slow spike wave discharges

• classic EEG: generalized, symmetrical 3-Hz spike and wave activity on a normal background; can be provoked by hyperventilation during EEG
• tx: ethosuximide, valproic acid 

• screening test = finger stick (capillary blood). If it is > 10 μg/dL, do confirmatory test (to r/o FP)
• confirmatory test = serum venous test
• If serum test is mildly elevated (<20): counsel re:remove child from house to reduce exposure and re-check in 1 mo
• If serum test is very elevated (>45): chelation tx with dimercaprol or DMSA, succimer.

• extravascular hemolytic anemia
• lab findings: reticulocytosis, elevated indirect bili, elevated LDH, low serum haptoglobin, Hct of 20-30%

• X-linked recessive d/o
• Atopic dermatitis, thrombocytopenic purpura, increased susceptibility to infxns
• small defective platelets
• survival beyond teens is rare (death d/t infections, bleeding, sometimes malignancy)

• + anti-Smith Ab
• + anti-dsDNA Ab

• autosomal recessive congenital QT prolongation syndrome d/t defect in ion channels
• patients also have congenital hearing impairment
• prolonged QT predisposes to torsades de pointes which can lead to syncope or death
• tx: BB (DDD pacemaker also placed it pt has sx, i.e. syncope)

• sx: swollen/painful eye, pain with eye movemment, proptosis, decreased visual acuity. 
• tx: broad spectrum IV abx
• note: CT scan determines extent of infection/presence of abscess

• autosomal recessive
• >90% are 21-hydroxylase deficiency
• Complete 21-hydroxylase defiency: presents at birth with virilization, decreased glucocorticoids and mineralocorticoids, and salt wasting
• Partial deficiency (non-classical CAH) - presents at puberty (or later) with hyper-androgenism, usually not associated with salt wasting
• Dx: increased levels of 17-alpha-hydroxyprogesterone; dx confirmed with ACTH stimulation test

• fatty liver with encephalopathy
• children <15 who were given aspirin for virally-induced fever (varicella, flu)
• common presentation: vomiting and mental status changes. Can lead to fulminant hepatic failure (elevated LFTs, elevated ammonia, hypoglycemia)
• tx: administer glucose with FFP and mannitol (to reduce cerebral edema)

• 30 min to 6 hrs post ingestion: N/V, hematemesis (coffee grounds), melena, abdominal pain
• 6-24 hrs: latent phase
• 6-72 hrs: shock & metabolic acidosis
• 12-96 hrs: hepatotoxicity
• weeks later: bowel obstruction secondary to mucosal scarring
• Dx: serum iron level of 350 mcg/dL or more

• Caused by chlamydia trachomatis serotypes A-C
• Major cause of blindness globally
• Presents with follicular conjunctivitis and pannus (neovascularization) formation in cornea; also have infection of nasopharynx that causes nasal discharge
• Dx: Giemsa stain of conjunctival scrapings
• Tx: topical tetracycline or oral azithromycin 

• pain, photophobia, decreased vision, and dendritic ulcer
• on exam: small, clear vesicles in corneal epithelium

• anti-gliadin IgA Ab
• anti-endomysial IgA Ab
• anti-transglutaminase Ab

• irrigate with warm fluid to flush the foreign body out
• if that fails, remove with child under sedation or general anesthesia
• note: this is common in prepubertal children (don't jump to calling CPS)

• 6 sx of inattention or hyperactivity/impulsivity
• onset before age 7
• sx present for at least 6 mo
• sx present in 2 or more settings

• lethargy
• altered mental status
• irritability
• seizures
• muscle cramps and weakness
• decreased DTRs
• Note: must not lower Na too rapidly - can cause cerebral edema (do not resuscitate with hypotonic solutions)

• Stroke - due to either internal cartoid artery dissection or compression of the artery with secondary thromboembolism.

• can be autosomal recessive or x-linked
• involves genes that affect DNA repair
• most patients are diagnosed by age 16, avg age at dx is 8
• diagnosis: chromosomal breaks on genetic analysis combined with clinical findings 

• progressive BM failure → aplastic anemia (s/sx of anemia), macrocytosis
• short stature, microcephaly, lowset ears, abnormal thumbs, hypogonadism, microophthalmia, horseshoe kidney
• hypo/hyper-pigmented areas, cafe au lait spots, large freckles
• strabismus, middle ear abnormalities (hemorrhage, deafness, chronic infxns, incomplete development)
• increased risk of cancer

• drugs (NSAIDs, sulfonamides)
• toxic chemicals (benzene, glue)
• idiopathic
• viral infxn (HIV, EBV)
• immune d/o
• thymoma

• port wine stain in distribution of trigeminal nerve 
• mental retardation
• seizures
• glaucoma 
• may have hemianopsia, hemiparesis, hemisensory disturbance
• skull XR after 2 yo shows gyriform ("tramline") intracranial calcifications 

• fever, nonproductive cough/cold sx, nonpurulent conjunctivitis
• Koplik spots (pathognomonic) = bluish white lesions (sometimes appear like table salt crystals) on erythematous buccal mucosa opposite the molars, sometimes on inner conjunctivae and vaginal mucosa. 
• rash - maculopapular, starts on face and spreads to trunk and extremities
• lab findings: leukopenia, lymphopenia, +/- proteinuria, 4-fold increase in hemagglutination inhibition Ab titer
• vitamin A can be of benefit during tx

• HHV-6
• also called 6th disease and exanthema subitum
• seen mainly in infants: high fever for 3-4 days, rash that spares the face and presents after the fever
• MC cause of infantile seizures

• adenovirus
• pharyngitis and usually unilateral, nonpurulent conjunctivitis, fever
• note: no rash!

• mild fever
• malaise
• posterior cervical lymphadenopathy
• fine, pink maculopapular rash that progresses from face to trunk in 3 days
• +/- leukopenia

• frontal bossing
• Hutchison teeth
• saddle nose
• high-arched palate
• perioral fissures 
• interstitial keratitis

• systolic ejection murmur
• grade of II/VI or less
• murmur changes with position
• no other extra heart sounds (abnormal s2, clicks)
• no symptoms
• normal pulses

• patient is symptomatic 
• intensity of III/VI or greater
• abnormal s2
• pansystolic murmur
• murmur loudest at upper left sternal border
• absent or diminished femoral pulses
• first step in work-up is usually echo

• autosomal recessive
• excess number of trinucleotide repeats results in abnormal tocopherol transfer protein
• cardiac and neuro sx (myocarditis, myocardial fibrosis, CHF, gait disturbance, hearing/vision/speech impariment, weakness)
• high plantar arches, skeletal deformities (hammer toes, scoliosis), DM
• sx onset usually between 5 - 15 yo (there is late onset form)
• progressive d/o with poor px - usually death by 30-35 yrs from cardiomyopathy or respiratory complications

• pericardial effusions (reactive pericarditis) that may occur in patients with congenital heart disease 1-6 weeks after they undergo cardiac surgery.
• thought to be autoimmune-mediated
• sx are usually mild and self-limited.

• can be congenital or acquired
• congenital type usually found in patient < 5 yo
• acquired is usually secondary to chronic otitis media
• presents with continued ear drainage despite appropriate abx tx and/or new-onset hearing loss
• PE: retraction pocket in TM filled with granulation tissue and skin debris
• complications: hearing loss, vertigo, CN palsies, life-threatening infxns like brain abscess or meningitis
• mgmnt: refer to ENT

• >90% form in the cerebellar vermis
• manifests as posterior vermis syndrome = truncal ataxia
• note: cerebellar astrocytomas form in cerebellar hemispheres and cause appendicular ataxia

A patient has fever, fatigue, exudative pharyngitis, and lymphadenopathy. They are given amoxicillin and develop a polymorphous, maculopapular rash.

What's the dx?

Infectious mono (EBV)

note: rash is not a true drug allergy

• hand-foot-mouth disease
• herpangina = fever, severe sore throat, and vesicles on palate, tonsils, and pharynx
• myocarditis
• aseptic meningitis 

• eyes
• skin
• immune system

• profound to normal IQ
• learning and language disabilities
• hyperactivity/inattention
• autism
• large head and jaw
• low-set ears
• macro-orchidism

• autosomal dominant mutation of fibrillin 1 gene (up to 30% of cases are sporadic)
• 6 major features: tall/thin stature, long/thin extremities, arachnodactyly, upwpard dislocation of lens (ectopia lentis), joint hypermobility, aortic root dilation (may hear diastolic murmur)
• also: crowded teeth, prominent sternum, hypotonia, iridodonesis 

• autosomal recessive
• cystathionine synthase deficiency (involved in methionine metabolism)
• some common features with Marfan's: tall/thin stature, long limbs, lens dislocation (but downward in homocystinuria)
• also: thromboembolic events, fair complexion, osteoporosis

• disorder of collagen structure
• hypermobile joints, hyperelastic skin, easy bruising, poor wound healing
• complications: organ rupture, hemorrhage

• 5p deletion
• cat-like cry, microcephaly, short stature, hypotonia
• additional sx: protruding metopic suture, moon-like face, hypertelorism, MR, wide and flat nasal bridge, high-arched palate

• screening: CK levels elevated
• confirmation: muscle biopsy (confirms dx in most cases)
• gold standard: genetic studies; may be required in atypical cases

• 2 types: 1) vesiculo-pustular lesions on exposed areas of faces/extremities that rupture and develop golden crust 2) bullous type (Staph only)
• cause: GAS, S. aureus
• tx: topical mupirocin or oral erythromycin

• Neisseria gonorrhea and neisseria meningitidis
• Strep pneumo
• H. influenzae

• Cavernous = flat, non-blanching
• Capillary = raised, blanches

• strep pneumoniae

• H. influenzae (gram neg rod)
• Pseudomonas (gram neg rod)
• Staph (gram pos cocci in clusters)

• autosomal dominant
• involves all muscle types (skeletal, cardiac, and smooth)
• patient develops muscles weakness/wasting (adolescence)
• myotonia = delayed muscle relaxation
• DM, testicular atrophy, frontal baldness, hypothyroidism

• can occur with recurrent UTIs secondary to anatomical reflux
• IVP shows focal parenchymal scarring and blunting of calyces

• Meconium ileus - diagnostic of CF
• presents with bilious vomiting and failure to pass meconium within first 24-48 hrs of life
• h/o polyhydramnios in mom, FHx of CF
• abdominal XR: dilated smal bowel & granular, ground-glass appearance of lower abdomen
• complication = intestinal perforation (pneumoperiotenum on XR if it occurs after birth)
• meconium plug syndrome has similar presentation but occurs in non-CF babies and is not complicated by perforation.

• strep pneumo - 40%
• H. influenzae - 25-30%
• viruses (esp RSV and rhino) - 30%
• Moraxella catarrhalis - 10-15%
• first line tx is amoxicillin x10 days
• second line tx (if first line fails) = augmentin, cefuroxime axetil, IM ceftriaxone

• age between 6 mo and 36 mo
• low SES
• smoke exposure
• formula-fed
• craniofacial anomalies
• chronic midde ear effusion
• respiratory allergy

• disseminated infection
• CNS infection
• skin, eyes, mouth
• acquired during transit thru birth canal (HSV2 MC)

• inspiratory stridor that is exacerbated by exertion (MC cause of chronic inspiratory noise in infants)
• stridor is worse when infant is supine
• [cyanosis usually not present]
• sx appear in first 2 weeks - 2 months 
• dx: on laryngoscopy the epiglottis "rolls from side to side"
• sx disappear by 2 yo - usually ~18 mo
• Mom should hold child upright for 30 min after feeding and never feed lying down

• Stage of I of lyme disease = erythema migrans (can also be asymptomatic)
• Children < 9: tx is amoxicillin x 21 days
• Children > 9 : doxycycline x 21 days

• Disseminated disease with neuro and/or cardiac manifestations + joint involvement (esp knees)
• Tx: ceftriaxone or penicillin G

• definition: Hct > 65%
• sx: respiratory distress, poor feeding, neuro sx (lethargy, irritability, seizure)
• MC cause = delayed clamping of the umbilical cord
• assoc finding: hypoglycemia, hypocalcemia
• tx: hydration; partial exchange transfusion if symptomatic
• Hct in newborn peaks at 12 hours

• It's a hemolytic anemia
• Very high reticulocyte count (up to 9%)

• #1 = Staph aureus
• #2 = group A strep
• Francisella tularensis (tularemia) - contact with infected rabbit, hamster, arthropod
• Peptostreptococcus (anerobe) - older child with h/o periodontal disease

• direct laryngoscopy and *rigid* bronchoscopy

• autosomal recessive inheritance of adenosine deaminase deficiency
• MC infxns: pneumocystis pneumonia, candida, parainfluenza, herpes
• lymphopenia

• They are prone to get pneumonia, suppurative adenitis, and developing abscesses secondary to catalase-producing organisms, ex. Aspergillus and staph

• autosomal domintant mutation of gene encoding type I collagen
• blue sclera
• recurrent fractures
• hyperlaxity of joints, hypotonia, wormian bones, early hearing loss, dentinogenesis imperfecta (discolored, translucent, weak teeth)

• most severe type = type 2 (still autosomal dominant)
• they have multiple intrauterine and perinatal fractures and usually die in utero or perinatally

• deficiency of fat soluble vitamins → vitamin K deficiency
• deficiency of vitamin K-dependent clotting factors: II, VII, IX, X & protein C & protein S

• they last 2-10 weeks
• can cause pneumothorax, epistaxis, and/or rectal prolapse

• Fever (with constitutional sx)
• centripetal rash (starts on extermities and goes inward)
• caused by Rickettsia rickettsii
• can develop encephalitis

•  "thumb sign" due to swollen epiglottis 
• thickened aryepiglottic folds
• obliteration of the vallecula

• Relative to birth weight, the weight should double in 5 months and triple by 1 year

• compared to length at birth, height should increase by 50% in first year, double by 4 yo, and triple by 13 yo

• congenital pure red cell aplasia 
• presents in first 3 months - pallor, poor feeding
• normocytic or macrocytic anemia with reticulocytopenia and normal WBC and platelet counts.

• suspect in term and post-term infants with cyanosis and hypoxia that does not respond to 100% oxygen.
• clinical picture similar to HMD, but lacks specific CXR seen in HMD (fine reticular granularity, esp in LLs)

• Marfan's features, MR, thromboembolic events, downward lens discolation
• fair complexion, skeletal/bone problems (scoliosis, osteoporosis), can have FTT
• cystathionine synthase deficiency
• body fluids have elevated homocysteine and methionine levels
• tx: high dose vitamin B6

• Niemann-Pick dz: sphingomyelinase deficiency, cherry red macula, protruding abdomen, hepatosplenomegaly, cervical lymphadenopathy, regression of milestones during infancy.   
• Krabbe's disease: galactocerebrosidase deficiency, MR, blindness, deafness, paralysis peripheral sensorimotor neuropathy, seizures
• Tay Sach's: beta hexosaminidase A deficiency, MR, blindness, cherry red macula, weakness, seizures
• Fabry's dz: alpha galactosidase A deficiency. Angiokeratomas, peripheral neuorpathy, asymptomatic corneal dystrophy, may develop kidney & heart failure, thromboembolic events

• common in school-age children
• nocturnal perianal or vaginal itching
• dx: scotch tape test
• tx: empiric tx w/ mebendazole

• duodenal atresia presents in first 24-48 hrs with bilious vomiting
• pyloric stenosis presents in first 3-6 weeks with nonbilious vomiting

• self limiting episodes/periods of vomiting in children with no apparent cause (no other sx)
• commonly there is FHx of migraines

• sx: seizure, hypotension, prolonged QRS
• management: ABCs, IV sodium bicarb (corrects acidosis, narrow QRS, corrects hypotension), benzos (diazepam) if needed for seizures

• Minimal change disease
• generally benign course, but relapses after steroid discontinuation are common
• empirically treated with steroids; may add an alkylating agent (cyclophosphamide) for resistant cases 

• empiric treatment with abx
• use IV abx if systemic sx are present
• all children between 2-24 months with their first febrile UTI should get renal US
• after acute illness is over, consider doing voiding cystourethrogram to look for anatomical abnormality (kids should not get UTIs; do cystourethrogram after 2nd febrile UTI)

• inherited d/o involving degeneration of anterior horn cells and CN motor nuclei
• SMA type 1 (Werdnig-Hoffman dz, autosomal recessive) = most severe form; presents in early infancy
• SMA type 2 = less severe, presents between 6-12 mo
• features (both): generalized hypotonia/weakness, normal cognitive function, tongue fibrillation. 
• dx: characteristis changes on EMG and muscle biopsy; confirmed by specific gene tests
• death d/t repeated aspiration or lung infxns

• chemical conjunctivitis - presents within 24 hrs of birth ad resolves within 24 hrs
• gonococcal conjunctivitis - presents btwn days 2-5
• chlamydial conjunctivitis - presents btwn days 5-14+

• highly purulent
• most destructive type of neonatal eye infxn - can result in corneal perforation and permanent blindness if untreated
• tx = ceftriaxone

• congestion and discharge that may be scant, mucoid, or purulent
• not prevented by prophylactic silver nitrate drops
• tx = oral erythromycin - must use oral to reduce risk of chlamydial pneumonia [oral tetracyclines can be used in kids 8 and up]

• medial deviation of the eye
• MC type of strabismus

• Strabismus (MC), errors of refraction, opacity along visual axis
• tx: occlusion of the normal eye 
• if there's error of refraction, first get lens for affected eye
• if there's opacity along visual axis, first remove the opacity before occlusion therapy

• leukocoria
• sensitivity to light
• excessive lacrimation
• increased intraocular pressure

• healthy infants < 1 yo require 120 kcal/kg/day
• after 1 yo, 100 kcal/kg/day
• FTT require an additional 50-100% for adequate catch-up growth

• learning difficulties
• behavior problems
• impaired family functioning 

• acquired red cell aplasia in otherwise healthy children
• usually kids between 6 mo - 5 yo
• gradual sx of pallor and decreased activity
• normocytic, normochromic anemia
• Hgb levels between 3-8, low retic count

• DTaP is contraindicated if patient previously had an anaphylactic reaction or an encephalopathy/CNS event within 7 days after getting the vaccine
• These patients should get DT instead. 

• active drug abuse (does not include alcohol or tobacco)
• HIV
• TB
• certain inborn errors of metabolism in the infant (i.e. galactosemia, PKU, urea cycle defects)

• 90% also have VSD; 30% also have TGA [VSD, ASD, PDA are needed to survive)
• EKG: Left axis deviation, LVH
• CXR: normal heart size, decreased pulmonary vascularity
• tx: PGE1 to keep ductus patent and balloon atrial septostomy

• CXR: cardiomegaly, increased pulmonary vascular markings, right aortic arch
• EKG: biventricular hypertrophy

• Usually occurs in full-term, uncomplicated neonates 
• More common post c/s
• Sx: tachypnea, mild retractions, nasal flaring
• thought to be d/t slow absorption of fetal lung fluid
• CXR: perhilar streaking and fluid in fissures; lungs are overaerated, flattened diaphragm.
• self-resovles within 24-48 hours
• management is supportive (+/- supplemental oxygen)

• CXR with hyperinflation, coarse streaking, and patchy inflitrates; may also have areas of air trapping d/t plugs
• presents within first hour of birth
• can lead to pneumothorax
• note: congenital diaphragmatic hernia can also cause pneumothorax in newborn

Dry, silver-gray plaques on the bulbar conjunctiva.

Seen in vitamin A deficiency.

• presents between 2-3 mo of age
• fulminant cardiac syndrome with cardiomegaly, tachycardia, cyanosis, dyspnea, and vomiting

• systemic: anorexia, hepatosplenomegaly
• CNS: irritability, increased ICP
• bone swelling/tenderness and limited motion
• ectoderm: alopecia, seborrhea, pruritis, perioral fissures

• severe pain, patient refuses to walk, keeps limb externally rotated (hip joints = MC joint affected)
• labs: leukocytosis, elevated ESR. Synovial fluid aspirate may have > 100K WBCs/uL. 
• XR often is normal 
• surgical emergency: immediate I&D as well as IV abx
• delay in tx of 4-6 hrs can lead to avascular necrosis

• Iron deficiency anemia (common in children > 6 mo)
• Children with hypochromic, microcytic anemia should be empirically treated with oral iron and recheck Hgb in 4 wks.

• IV abx with coverage of pseudomonas - penicillin/cephalosporin AND aminoglycoside (ex. amakacin or gentamicin)

• a raised, sharply demarcated, indurated patch with advancing margin; often on the cheek
• 80% of cases are caused by strep. Often there's h/o trauma or pharyngitis
• tx = penicillin

• viral URI = MC predisposing factor
• Sx of persisent or purulent nasal drainage (at least 3 days), persistent congestion, cough, high fever (>102.2 or 39); patient appears ill
• most cases are diagnosed clinically (CT not necessary for dx!)
• first line tx: amoxicillin + clavulanic acid

• cystic dilation of the 4th ventricle and cerebellar agenesis with malformation of the vermis
• can present in infancy, childhood, or older
• common complications: hydrocephalus, seizures

• isolated displacement of cerebellar tonsils into cervical canal
• can be congenital or acquired secondary to low ICP
• most cases are asymptomatic; can have HA, tinnitus, ataxia
• associated with syrinx, kinked cervical spinal cord, hydrocephalus
• severe cases are treated with surgical decompression

• entire cerebellum, medulla, and 4th ventricle are displaced into cervical canal
• common complication = obstructive hydrocephalus
• all patients have myelomeningocele and 96% have cortical malformations
• usually presents in neonate with neuro deterioration, respiratory distress, stridor, and hydrocephalus
• Tx: surgical decompression and shunts. 
• Severe neuro sequelae often persist

• If a kid swallows a battery, first step is XR to see where it is.
• If it's in the esophagus: immediately remove endoscopically to avoid damage to and ulceration of esophagus
• If distal to the esophagus: observe patient for 24-48 hrs for spontaneous expulsion and confirm expulsion with stool or f/u XR)

• infatile botulism is caused by ingestion of the organism, which then grows in the gut and produces the toxin (vs ingestion of toxin itself in adults)
• sx: first poor feeding & constipation, then progressive hypotonia/weakness, loss of DTRs, CN problems, respiratory difficulty, sometimes autonomic dysfunction

• Treatment = macrolide (azithromycin, erythromycin, clarithromycin)
• Treat anyone with suspected OR confirmed infection regardless of age, stage of disease, or immunization status.
• Tx shortens evolution of the disease and decreases spread of infection.
• Hospitalization is indicated for infants <3 mo, those 3-6 mo with severe paroxysms, and for complications

• It's a developmental disorder with forward slip of  a vertebra (usually L5 over S1)
• onset in pre-adolesence
• sx: back pain, neurologic dysfunction (ex. incontinence)
• PE: palpable, "step off" lesion in lumbosacral area may be present in severe cases

• hospitalized preemies and LBW infants
• usually pr3sents in 2nd/3rd month
• causes can include: decreased RBC production, blood loss, diminished fetoplacental transfusion, and shorter RBC life span
• Labs: normochromic normocytic anemia with decreased retic count and decreased RBC precursors in BM; WBC, Plt, Bili are normal
• Tx: iron supplement (even though iron deficiency is not cause), Hgb checks, blood transfusions if necessary

• Adolescent male with epistaxis, nasal obstruction, visible nasal mass on exam, +/or bony erosion (CT). [can also be in upper pharynx vs nasal]
• requires specialist to manage - surgery may be needed if it's enlarging, obstructing the airway, or causing chronic bleeds
• Benign, but locally invasive and recurrence after removal is common

• Criteria: fever > 39 x5 days and 4 out of 5:
• cervical lymphadenopathy (usually unilateral)
• rash
• extremity changes
• conjunctivitis (usually nonpurulent and bilateral)
• oral changes
• Supporting labs: thrombocytosis (after few weeks), WBC > 12K, sterile pyuria, elevated ALT, hypoalbuminemia (<3), ESR>40 , CRP>3

• Cradle cap = sebborheic dermatitis in infants - often starts on scalp and also affects eyebrows and nasolabial folds
• papular, scaly lesions
• tx: moisturizers, antifungals, topical steroids

• Always give for cat or human bites
• Give for dog bites only if wound is deep, on hands/face, severe crush, compromised host, etc
• 1st choice = augmentin
• 2nd choice = first generation cephalosporin (cefazolin, cephalexin)
• PCN allergic: doxycycline or clinda plus TMP/SMX

• Pellagra = 3 D's: diarrhea, dementia, dermatitis (symmetric sunburn-like rash on extremities)
• beefy red tongue (glossitis)

• cheilosis
• glossitis
• seborrheic dermatitis - often affecting genitals
• oral findings: pharyngitis, oral edema/erythema

• irritability, depression
• polyneuritis
• generalized seizured in infants
• sideroblastic anemia (hypochromic, microcytic and increased iron)
• dermatitis and stomatitis
• increased risk of thromboemoblism d/t increased homocysteine concentrations
• can be a side effect of isoniazid

• Occurs in infants of unimmunized mother often following umbilical stump infection
• Presents in first 2 weeks
• Sx: poor suckling/fatigue followed by rigidity, spasm, and opisthotonus (arching)

• increased mobility btwn the atlas (C1) and axis (C2), usually due to excessive laxity of the posterior transverse ligament
• affects 10-15% of Down Syndrome patients
• only 1-2% have sx, which are secondary to spinal cord compression and can include behavior changes, torticollis, urinary incontinence, and vertebrobasilar sx (vertigo, dizziness, diplopia)
• PE: UMN symptoms - spasticity, hypertonia, + Babinkski
• Dx: lateral radiographs of cervical spine in flexed, extended, and neutral positions
• Tx: surgical fusion of C1 & C2

• 5 areas - each given 0, 1, or 2 points
• Color: body & extremities blue or pale < body pink, extremities blue < all pink
• HR: no cardiac activity < HR<100 < HR>100
• Respirations: absent < slow, irregular < good respiratory effort
• Response to nasal stimulation: none < grimace < cough
• Tone/activity: limp < some flexion of extremities < active flexion of extremities

• Must have 2 major criteria OR 1 major and 2 minor plus evidence of strep infection
• Major criteria: Carditis, polyArthritis, subcutaneous Nodules, Chorea, ERythema marginatum
• Minor criteria: ↑ CRP, arthralgia, fever, ↑ ESR, prolonged PR interval, leukocytosis

• paramyxovirus
• classic sx: parotitis, orchitis (NO rash!)
• complications: encephalitis, pancreatitis

• For healthy (nonpregnant) adults and children: give if exposed in the past 3-5 days (not given after this period d/t decreased efficacy)
• Susceptible, high risk (immunocompromised, pregnant) individuals: give postexposure prophylaxis of VZIG with in 96 hours of exposure. Note: may not prevent infection but will decrease severity.

• a common cause of FTT in infants
• all 3 types involve defects in the ability of the renal tubules to reabsorb bicarb or excrete H+, leading to a normal anion gap, hyperchloremic metabolic acidosis
• all three types: low bicarb, high chloride, normal AG

• Type I RTA - distal tubule has impaired H+ secretion  - hypokalemia, elevated urine pH (>5.5), nephrolithiasis
• Type II RTA - proximal tubules has impaired bicarb reabsorption - association=Fanconi syndrome
• Type IV RTA - distal tubule defect in Na/K exhange - hyperkalemia

• verotoxin produced by E Coli
• sudden onset of bloody diarrhea, abdominal pain, and triad of anemia, thrombocytopenia, and renal failure
• supportive tx - may include plasmapheresis, dialysis, steroids

• Increased risk in Asians and Africans
• Dx is suggested by (1) improvement with abstaining from dairy, (2) acidic pH of stool, (3) presence of positive reducing sugars
• Dx is confirmed by positive hydrogen breath test

• fever, malaise, erythematous rash on abdomen (rose spots), diarrhea x several weeks
• Dx: stool culture
• can spread to brain, bone, kidney, and joints

• solid foods (veggies, eggs, meat): introduced around 4 months
• fruit juices introduced around 5-7 months