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163 Cards in this Set
- Front
- Back
heroin in utero
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IUGR, macrocephaly, SIDS, neonatal abstinence syndrome (irritability, high pitched cry, poor sleeping, tremors, seizures, sweating, sneezing, tachypnea, poor feeding, bomiting, diarrhea)
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valproic acid in utero
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neural tube defects, cardiac anomalies, dysmorphic facies, cleft lip, narrow bifrotnal diameter, midface hypoplasia, broad and depressed nasal bridge and long philtrum
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phenytoin in utero
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nail and digit hypoplasia, dysmorphic facies and MR, IUGR, hirustism, dev delay, cleft lip and palate
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alcohol in utero
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growth def, long smooth philtrum, small palpebral fissures and thin upper lip, MR
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cocaine in utero
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jitteriness, excessive sucking, hyperactive moro, behavior, attention and intelligence affected long term
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myocarditis
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dilated heart, increased end diastolic volume, CHF, fever, anorexia, lethary, irritability, resp dsieastress, holosystolic murmur due to dilation, functional mitral regurg, hepatomegaly, faituge, tachypnea, tachy, decreased perfusion, EKG low volt QRS and sinus tachy
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kawasaki
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med vasculitits,18-24m, fever >5d, extremity changes (erythema, edema, desquamation), conjunctivitits, rash on trunk, cervical lymphadenopathy, oral changes (erythema, fissured lips, strawberry tongue), increased CRP, ESP, ALT, thrombocytosis, leukocytosis, urethritis, orchitis, arthritis, hepatitis, induration of previous bcg site
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which rash spreads from extremities inward to trunk?
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rocky mountatin spotted fever
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tx of kawasaki
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IVIG and aspirin (high dose)
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serious complication of kawasaki
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coronary artery aneurysms, orchitis, hepatitis, myocarditis
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intraventricular hemorrhage in infant
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mainly due to prematurity, Low birth weight, cyanosis, pallor, focal neuro signs, seizures, hypotension, apnea, bradycardia
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dermatomyositis
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violaceous poikiloderma, periorbital edema, shawl sign, gottrons sign/ papules, internal malignancies, pul fibrosis
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inflamm diseases assoc with aortic aneurysm
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behcets, takayasu, ankylosing spondylitis, RA, psoriatic arthritis, relapsing polychronditis, and reiters
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measles
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leukopenia and thrombocytopenia (helped by vit A), high grade fever, rash, brick red maculopapular rash first on face
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obstructive/ noncommunicating hydrocephalus
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dandy walker- expanded 4th ventricle; chiari- protrusion of structures of posterior fossa through foramen magnum
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causes of neonatal hyperbili (conjugated/direct)
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biliary atresia, sepsis, endocrinopathy, nutritional hepatotox (galactosemia, tyrosinemia)
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when to give varicella vaccine if exposed?
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active immuzation first 3-5d after
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when to give VZIG
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immunocompromised, pregnant women, within 72-96 hrs
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beckwith wiedemann
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neonatal hypoglycemia, macroglossia, large size, visceromegaly, omphalocele and characteristic earlobe crease, prominent eyes, occiput, hyperplasia of pancreas, increased risk for wilms, hepatoblastoma, gonadoblastoma
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prader willi
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narrow bifrontal diameter, diamond shaped eyes, small downturned mouth, hypothalamic dysfunction, short statue, obesity, hypotonia, hyperphagia
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medulloblastoma
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usually in cerebellar vermis, mets through CSF tract, radiosensitive
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eczema herpeticum
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form of primary HSV infection, assoc with atopic dermatitis, need prompt acyclovir, umbilicated vesicles over area of healing atopic dermatitis
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SCIDs
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recurrent sinopul infections, oral candidiasis, persistent diarrhea, opportunistic infections, viral infections, absent lymph nodes, tonsils, lymphopenia, absent thyrmic shadow, abnl T and B and NK cell
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common varible immunodef (acquired hypogammaglobuliemia)
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decreased IgG,IgA,IgM, and IgE, no absence or decrease in B cells
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bruton's agammaglobulinemia (X linked agammaglobulinemia)
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male asympt 6-9m, recurrent pyogenic infections, decreased IgG,IgA,IgM,IgE, absent or markedly decreased Bcells
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wiskott aldrich
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XR, ezcema, thrombocytopenia, recurrent infections with encapsulated germs, petechiae, bruises, bleeding from circumscision, bloody stools, low IgM, high IgA IgE,
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chronic granulomatous disease
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defect of phagocytic cells, dysfunction of NADPH oxidase, catalase + infections, lymphadenitis, abscesses of skin and vicera, nitro blue tetrazolium test,unexplained aspergillus, serrate marcescens and burkholderia cepacia, hepatomegaly, splenomegaly, anemia of chronic disease, chronic diarrhea, short, gingivitis, dermatitis, decreased cytochrome C, tx bone marrow txplant
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transposition of great vessels
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diabetic mothers, no murmur, single loud S2, prostaglandin E1 can be given IV to stabilize
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tetralogy of fallot
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S2 and systolic murmur from pul stenosis, cyanosis, present from birth to few yrs
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apathy, weakness, hypotonia, large tongue, sluggish movement, and bloating ,umbilical hernia, jaundice, difficulty breathing ,noisy respiration, hypothermia, refractory macrocytic anemia
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congenital hypothyroidism, from dysgenesis, hypoplasia or ectopic, errors of synthesis, or blocking antibodies, tx levothyroxine
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what are the two causes of floppy baby
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botulism or werdnig hoffman
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rubella
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maculopapular rash, posterior cervical and psoterior auricular lymphadenopathies and polyarthralgia
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MENII
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medullar ca of thyroid, hyperparathryoidism, pheo, serum calcitonin elevated in pts with medullary thyroid ca, hyperparathyroidism
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SC patient with pain in hip, no fevers
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avascular necrosis (other non traumatic causes: chronic corticosteroid therapy, SLE, alcoholism, gaucher's disease), supplied by ascending arteries and foveal artery (inside ligamentum teres)
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schoolteacher, MCP,PIP, wrist and ankle jt pain, diarrhea, fatigue
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parovirus infection, test for anti B19 IgM
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acid base status in pyloric stenosis
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hypok alkalosis
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congenital deafness, QT prolongation
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jervell lange nielson, tx BB +/- DDD pacemaker
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risks of otitis media
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6-36m, low SE status, use of formula, caretaker smoking, reps allergy, craniofacial anomalies, chronic middle ear effusion
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bronchiolitis tx for high risk
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ribavirin
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previous seizure with DTaP, which part caused it
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pertussis, give DT
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craniopharyngioma
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from epi remnant of rathke's pouch, calcifications and cystic, growth failure, headaches, nests of squamous cells in loose stroma, DI
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HUS
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hemolytic anemia, acute renal failure, thrombocytopenia
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HBV tests
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HBsAg unresolved disease, HBeAg infectious, HBsAb ONLY if immune (NEVER in chronic disease)
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choanal atresia
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better with crying, can't pass NG through nose
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kernicteris
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unconjugated bili, seizures flaccidity, poor feeding, opisthotonos, apnea
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physiologic jaundice
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term <12 peaks 2-3d, over in 2wks; premie <15 peaks 3-5d over in 3wks
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tetrology of fallot
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VSD, overriding aorta, PS, RVH
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changes as infant is born
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decreased pul vascular resistance, increased left sided heart pressure, closure of foramen oval, increased O2 shuts off prostaglandin --> close ductus arterioles
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downs heart disease
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ASD(fixed split S2, need abx prophylaxis), endocardial cushion defect (loud P2, L-->R shunt, pul HTN)
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stroke in child?
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sickle cell
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papillary necrosis
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analgesic overuse, DM, infection, UT obstruction, hemoglobinopathies, cirrhosis, CHF, shock, hemophilia
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VSD tx
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ACEI, diuretics
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infant with reps farilure, fever, lethargy, poor feeding, hypotonia, seizures, bulging fontanel
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meningitis, in newborn, most likely GBS
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HSV in newborn
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temporal lobes, focal neurologic deficits
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listeria meningitis in newborn
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also abscesses and pneumonia
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before intubating pt with croup...
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try epi
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recent viral infection or trauma, limp and/or uni hip or knee pain, flexed, slightly abd and externally rotated, limitation of motion, pain with palpation or passive motion
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transient synovitis tx: NSAIDs and rest, can see slight increased WBC, ESR
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septic arthritis from transient synovitis
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WBC >12, temp>39/102, ESR>40, refusal to bear weight
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is breath holding spells assoc with anything (age 6-18m)
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iron def anemia, no long term sequele,
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PKU
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def phenylalanine hydroxylase, increased serum phenylalanine and urinary excretion of phenyl acetic acid, irreversible MR, seizure d/o, psychotic episodes, blond hair, fair skin, blue eyes, musty or mousy odor, seborrheic lesions, eczematous rash, vomiting, failure to thrive, dev delay, met acidosis, phenyalanine >20, nl to low nl tyrosine, increased urinary levels of phenylalanine metabolites (phenylpyruvic and o hydroxyphenylacetic acids), nl tetrahydrobiopterin level
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transient hyperphenylalaninemia
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transient tyrosinemia of new bone- def oxidize tyrosine
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tyrosinemia
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def fumarylacetoacetate hydrolase, sym 2 wks, fever, irriability, vomiting ,hemorrhage, hepatomegaly, jaundice, elevated serum transaminases, epi of acute peripheral neuropathy, fanconi like synd, hypoglycemia, smell like boiled cabbage
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alkaptonuria
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def homogentisic acid oxidase, dark/ black urine, ochronosis, arthritis, homogentisic acid in urine
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chediak higashi
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decreased granulation, chemotaxis and granulopoiesis, mild coagulopathy, peripehral and cranial neuropathy, HSM, pancytopenia, partial oculocutaneous albinism, frequent bacterial infections, progressie lymphoprolif synd,giant lysosomes tx: TMP SMX and ascorbic acid
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leukocyte adhesion defect
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defective tethering, adhesion, targeting of myeloid leukocytes to sites of microbial invasion, neutrophilia w/o polymorphs, delayed separation of umbilical cord, recurrent bacterial infections, necrotic skin lesions, severe gingivitis, periodontitis, alveolar bone loss --> early loss of deciduous and permanent teeth
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atlantoaxial instability
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10-15% down synd, excessive laxity in posterior transverse ligament, increased mobility btw C1 and C2, compression of spinal cord, behavioral changes, torticollis, urinary incontinence, vertebrobasilar sympt, dizziness, vertigo, diplopia, leg spasticity, hyperreflexia, positive babinski, clonus
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metatarsus adductus
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type I: overcorrects passively and actively- no tx; type II: feet that correct to neural tx orthosis or corrective shoes; type III rigid feel do not correct- serial casts
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febrile seizures that need to be worked up
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focal lasts >15min, or multiple with total >30min
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neonatal tetanus
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muscle spasms, hypertonicity, poor suckling, fatigue, opisthotonus, death from apnea and septicemia
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ADHD
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6m, persistent since age 7, in more than one setting, poor conc, distractibility, elevated motor activity, impulsivity
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signs of hydrocephalus
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poor feeding, irritability, committing ,decreased activity, scalp veins may appear prominent with shiny, tight skin overlying the vessels, cranial sutures may be widely spaced, CT brain, tx: shunt
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tx of DDH
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pavlik harness <6m, reduction and spica cast 6m-2yrs, open reduction after 2yo, no correction after 8yo
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when to prophylactic pin contralateral side in slipped apical femoral epiphysis
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hypothyroidism
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x linked gammaglobulinemia/ bruton's agammaglobulinemia
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tx: IVIG; recurrent bacterial infections early in life
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cause of exudate
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increased capillary permeability
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what causes effusion in liver failure
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transudate- decreased plasma oncotic pressure
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what causes transudative effusion in CHF
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elevated hydrostatic pressure
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cretinism
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poor feeding, lethargy, large fontanelles, thick tongue, constipation, umbilical hernia, poor growth, hypotonicity, dry skin, hypothermia, jaundice, decreased T4, increased TSH, prolonged jaundice
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difference between pseudo precocious puberty and true?
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true causes testicular or ovarian enlargement
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diamond blackfan syndome/ congential hypopalstic anemia
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defect ofintrinsic defect of erythroid progenitor cells, macrocytosis, pallor, web neck, cleft lip, shielded chest, triphalangeal thumbs, palse mucous membranes tx:steroids
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fanconi's anemia
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AR, pancytopenia and macrocytosis, cae au lait, microcephaly, microphthalia, short, horseshoe kidney, absent thumbs
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neuroblastoma vs wilms
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neuroblastoma crosses midline and have catecholamine metabolites in urine, wilms DOES NOT cross midline and has hematuria
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congential syphillis
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lesions on plasm and soles, HSM, jaundice, anemia, rhinorhea, metaphyseal dystrophy, periostitis
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ricketts
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open fontanels, pingpong ball over occiput, enlargement of costochondral junctions, thickening of wrists and ankles, cupping and fraying of distal ends of radius and ulna, horizontal depression along lower border of chest, LOW calcifediol level, increased Alk phos,
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chondrodystrophy
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irregular concave outlines of distal ends of bone
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stridor, diff breathing, sitting up, leaning forward, drooling,
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epiglottitis, need to intubate- H flu and s. pyogenes
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depression and dexamethasone suppression test
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abnl in 50%
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newborn hand paralysis and horners
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klumpkes
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laryngomalacia/ congenital flaccid larynx
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hold baby up for 30 mins after feeding, don't feed supine, rolling epiglottis side to side, noisy inspiration, subsides by 18m
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AFP
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from yolk sac and fetal liver, increased in neural tube defect, multi gestations, wrong gestational age, abd wall defect
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stranger anxiety
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normal till 3yo
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causes of neonatal shock
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gastroenteritis, GBS, CAH, CF, gi obstruction, galactomsemia
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allergic interstitial nephritis
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from many drugs, along with arthralgias and rash, WBC casts and eos in urine, caused by sulfa, penicillin, cephalosporin rifampicin
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vaccinations for pre term babies given according to...
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chronological age, except no hep b till >2kg
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wet the bed at least 2 x/wk for 3 months
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behavior mod --> desmopressin --> TCA (imipramine)
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overproduction uric acid, behavioral problems, neurologic disability, def of HPRT
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nesch nyhan
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what tests of get on BPH pt?
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U/A and Cr
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hip is externally rotated, won't walk on it, extreme pain
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septic arthritis -- tx drainage immediately
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HUS
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e.coli 0157H7, shigella, salmonella, yersinia, campylobacter, preceded by URI, GI bleeding, purpura, htn, microangiopathic hemolytic anemia, acute renal failure, fever, oliguria, thrombocytopenia, increased LDH, indirect bili, retic count
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androgen insensitivity
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primary amenorrhea, b/l inguinal masses, breast development w/o pubic or axillary hair
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hyperca, single lytic lesion in head of humerus in 6yo
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langerhans cell histiocytosis, benign and treated conservatively
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newborn, cyanotic, left axis deviation
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tricuspid atresia
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lennox gastaut synd
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seizures and MR
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most common brain tumor in children
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astrocytoma, usually infratentorial
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what is in excess with 21 hydroxlase def?
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17 alpha hydroxyprogesterone--> hirsutism or virilization
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molluscum
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in child prob not sexual abuse
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tx of rosacea
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metronidazole or tetracycline
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increased gastric residual first few days of life, abd distention
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necrotizing entercolitis
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restrictive lung disease
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all lung volumes go DOWN except FEV1/FVC
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test amniotic fluid for lung maturity
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L/S>2 or phosphatidylglycerol present
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tx of CF
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deoxyribonuclease, pancreatic enzymes
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what children get RDS?
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premature and diabetic mother
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CF
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rectal prolapse, esophageal varices, pancreatic insufficency, infertility,
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erythema toxicum
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red halo surrounding lesions, presence of eosinophils in pustules
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tx of get spell
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put knee to chest, fluids, morphine, propranolol
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staph scalded skin syndrome
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perioral, flexures, nikolsky sign is positive, target desmoglein 1, resolve 1-2 wks, cultures of skin are sterile (caused by toxin)
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medial longitudinal fasciculus lesion (MS)
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left eye nystagmus when looking left and right eye nystagmus when looking right
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ALL
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children, >25% lymphoblasts in bone marrow, HSM, petechiae, lymphadenopathy
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what is meconium aspiration common in?
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post mature and SGA infants, flattenign of diaphragm, hyperinflation, coarse streaking and patch opacities over both lung fields
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complications of meconium aspiration
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pneumothorax, infection, pul HTN, residual lung problems, neuro problems from asphyxia
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rubella
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posterior cervical lymphadenopathy, patchy erthyema throat and palate, 3 day measles
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atypical measles
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inactivated vaccine, atypical rash, arthritis, hepatitis, lung involvement, edema of hands and feet
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scarlet fever
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punctate or finely papular texture, gray white tonsilar exudates, chills, abd pain, etc, tx: pen V or erthyromycin, clinda
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diabetic mother... increased risk for...
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caudal regression synd, transposition of great vessels, duodenal atresia and small elft colon, anencephaly and neural tube defects
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polycythemia in newborn (HCT>65)
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from delayed clamping of umbilical cord, find plethora, lethargy, irritability, jitterienss, seizures, resp distress, tachypnea, cyanosis
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lead levels and tx
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>10 recheck serum lead, if <20 can recheck in 1 mon, if >45 chelation
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cystathionine synthase def
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elevations of homocysteine and methionine tx: high dose Vit B6 --> restrict methionine with supplementation of cysteine
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cholesteatoma
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secondary to chronic middle ear infection, continuous drainage despite abx, granulation and skin debris; complications hearing loss, CN palsies, vertigo, brain abscess, meningitis
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prophylaxis for malaria
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mefloquine
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suspect congenital diaphragmic hernia
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Orogastric tube placement
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best predictor of extent of TCA overdose
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QRS interval
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infant that drinks a lot of cows milk? dx? tx?
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iron def anemia, tx oral iron w/o testing
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complications of aortic dissection
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stroke, ARF, aortic regurg, cardiac tamponade
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what dev of secondary sex characteristic is worrisome for CNS disorder
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pubic hair before 8yo (pubarche), okay to have adrenarchy (axillary hair-premature adrenal activation) or thelarche
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type 1 gaucher
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acid beta glucosidase, fatigue, easy bruise, thrombocytopenia, bone pain, path fractures, erlenmeye fast of femur, wrinkled paper on bone marrow study
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patients with RA are increased risk of what other bone d/o
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osteopenia, osteoporosis
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osteitis fibrosis cystic
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brown tumors, increased osteoclast, hyperparathyroidsism causes it
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osteomalacia
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d/o of bone mineralization from decreased fit D, ca, from parathyroidectomy and rental tubular acidosis
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acute renal trxplant rejection
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heavy lymphocyte infiltration, vascular involvement with swelling of intima
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tx of tet spells
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increase systemic vascular resistance--> improved pul blood flow
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hyperIgM
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recurrent sinopul infections, pneumocystis jiroveci,
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bulous myingitis
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bullae on tympanic membrane in patients with otitis media, more severe pain, assoc mycoplasma pneumoniae
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childhood disintegrative disorder
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in men, nl 2 yrs, loss of skills in receptive/ expressive language, social skills, bowel or bladder control, play and motor skills, severely disabled for life
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rett syndrome
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usually female, hand movements, nl for 6 m, decal head growth, poor coordination, seizure, ataxia, MR, diminished social interactions
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c. botulism
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2wk-9m with constipation --> hypotonia, weakness, loss of DTR, CN abnl, respiratory difficulty, symptoms from organism
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parinaud's synd
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paralysis of vertical gaze, assoc with pupillary disturbances, eyelid retraction, can disturb hypothal --> increased beta hog --> increase leydig cell stimlation
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milk protein intolerance
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can be intolerant to cows milk or breast, stool has RBCs and dos,
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acute unilateral lymphadenopathy
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usually bacterial, Staph A,
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tularemia
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francisella tularenis, acute uni cervical lymphadenopathy, headache, fever, malaise, infected animal
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peptostreptococcus
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periodontal disease, acute uni lymphadenopathy,
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child with cerebellar signs
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fredreich's ataxia, arnold chiari, dandy walker, tumor, ataxia telangiectasia
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prostate cancer
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abnl nodules, sympts of BPH, osteoblastic lesions, acid phosphatase increased if broke through capsule
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bunch of grapes out of vagina
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sarcoma botryoides
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unicameral bone cyst
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lytic benign, prox humerus, pathologic fx possible
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MCC of precocious puberty in boys
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adrenal hyperplasia
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hyaline membrane disease of newborn
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60-80% <28 wks, tachypnea, grunt, retractions, grunt, dusky, fine reticularity of lung parenchyma on xray
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painless melena
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meckels
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fredreich's ataxia
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necrosis and degeneration of cardiac muscle fibers leading to myocarditis, cardiac arrhythmias, CHF, wide gait, cervical spinal cord and cerebellar atrophy, high plantar arches, from tocopherol abnl
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NF1
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cafe au lait, macrocephaly, short stature, feeding difficulties, learning disabilities
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risks for RDS in infant
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male, maternal diabetes, premature, c/s w/o labor, perinatal asphyxia
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obesity hypoventilation syndrome
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weight loss, ventilator, O2, progestins
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reye's synd
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vomiting, aggitation irritation, --> restless, lethargy, stupor; see convulsions, hyperammonium, increased bili, alk phos, PTT, AST,ALT, LD,hypoglycemia, microvesicular steatosis
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risks SGA
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hypoglycemia, polycythemia, hypoxia, meconium, hypocalcemia, hypothermia
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