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58 Cards in this Set
- Front
- Back
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kid swallow's battery?
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- do xray
- if in esoph, take out endoscopically - if in stomach, 90% will pass ok |
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meconium ileus vs plug?
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- fhx of CF = ileus => increased risk of perf
- no fhx = plug => no increased risk |
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intussuception?
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- abd pain, red current jelly
- seen in CF around age 2 |
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double bubble sign?
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- duodenal atresia => bilious vomiting
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anemia of prematurity
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in premature or low birth weight infants
- see normal smear, low retic, normal bili - 2/2 transition of erythropoiesis sites, shorter RBC life - giving Fe doesn't help |
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signs of alpha or beta thal?
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- see hypochromic, microscytic anemia, +fhx
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dactylitits?
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- work up for sickle cell disease
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mcCune albright?
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- precocious puberty, cafe au lait spots, bone defects
- sporadic, G protein cAMP kinase defect |
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ddx cafe au lait?
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- large irregular borders = McCune
- associated with axillary or genital freckles = Von Recklinghausen syndrome |
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Peutz-Jeghers
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- GI polyposis, mucocutaneous pigmentation
- some with estrogen secreting tumor => precocious puberty |
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Sturge Weber
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- port-wine stain over trigeminal region => MR, seizures, visual impairment
- intracranial calcifications |
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MCC of heterosexual precocious puberty?
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- e.g. male genitalia in female = adrenal tumor
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tuberous sclerosis?
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- adenoma subaceum cutaneous abnormality, seizures
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galatosemia
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- 2/2 galactose-1-phosphate uridyl transferase deficiciency
- bilateral cataracts, jaundice, hypoglycemia, increased urine galactose - increased risk of E coli neonatla sepsis - liver cirrhosis - tx: eliminate dietary galactose |
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baby with bilateral cataracts, jaundice?
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- galactosemia
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child falls on outstretched hand, see anterior fat pad displacement?
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- ischemia of forearm tissue
- supracondylar fx of humerus => Volkmann's ischemic contracture = type of compartment syndrome => fasciotomy - even though distal pulse is palpable, there may be brachial artery injury |
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GI malformation in Down's? Cardiac?
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- duodenal atresia, hirschprug, esoph atresia, pyloric stenosis, malrotation
- heart: endocardial cusion defect, VSD, PDA |
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2 y.o. with painless vs painful rectal bleeding?
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- painless = meckel
- painful = intussusception |
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tetralogy of fallot?
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- cyanosis that doesn't improve with O2
- squat => decrease peripheral resistance => decrease right to left shunt => improves cyansosi - tet spells: hypoxic episodes with paroxysms of deep, rapid breathing 2/2 increased pulm vascular resistance precipitated by crying, infx, exercise => give O2, knee-chest position, fluids, morphine, propranolo |
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VSD
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- left to right shunt, spontaneously heals, else eisenmenger => cyanosis
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ASD
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- left to right shunt, v. beneign, rarely eisenmenger
- doesn't lead to endocarditis |
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systolic murmur radiating to back
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- coarctation => noteced ribs
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eisenmenger tx
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lung transplant
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cyanosis that doesn't resolve with O2?
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- tetraology of fallot
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tuberous sclerosis
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- hypopigmented macules = ash leaf spots, infantile spasm = brief symmetrical contractions of body, cortical tubers on head CT
- ACTH for infantile spasms |
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tx seizures?
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- cabamazepine = tonic clonic, partial
- ethosuximide = absence seizures - phenobarbital = generalized tonic clonic - phenytoin = primary, secondary generalized tonic-clonic seizures, partial, status epilecticus |
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hypopigmented macules?
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- ash leaf in tuberous sclerosis
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enuresis in 7 y.o.
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- always do urinalysis even if bedwetting entire life
- IV pyelogram only if recurrent UTI or hematuris - if all benign, behavior modification, if doesn't work then imipramine or desmpressin |
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ddx scalp swelling in newborn?
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- overlies on scalp bone, benign lokoing = cephalohematoma, no tx
- pulsations, increased pressre with crying, bony defect = cranial meningocele |
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biggest worry with baby getting into vitamin pills?
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- iron poisoning => corrosive to GI mucosa, mit injury => cellular damage, systemic tox => increased lactic acid, liver cirrhosis
- long term = gastric scarring, pyloric stenosis - xray = radioopaque iron tabs - tx: ipecac or deferoxamien (Chelator), no lavage |
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edward's syndrome?
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small jaw, small head, rocker bottom feet, overlapping fingers, no creases
- MC heart defect = VSD |
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heart defect in congenital dz?
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- edwards (18) = VSD
- downs = ASH, endocardial cushion defect - williams' = supravalvular aortic stenosis - DiGeorge = Truncus arteriosis, tet of allot, aortic arch - neonatal lupus = congenital heart block - kawasaki = coronary artery aneurysm - PDA = congenital rubella |
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**homocysteinuria
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- cystathione synthase def = methionine met abnormality
- marfan like + thombeembolic events 2/2 vessel wall changes and increased adhesiveness of plt - lens dislocation in downwards (upwards in Marfans) - increased homocysteine, methionine - tx: high dose B6 or low methionine high cysteine diet |
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weird metabolic d/o
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- Krabbe's dz: beta galactosidase, no myelin, MR, blind, deaf, seizures
- tay sachs: beta-hexosaminidase A def, MR, blindness, cherry red macula - fabrys: sphingolipidosis, 2/2 alpha galactosidase def; angiokeratomas, periph neuropathy, corneal dystrophy, kidney, heart failure |
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Freidrich's ataxia
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- gait disturbance, pes cavus, ataxia, no ankle jerk
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infantile spinal muscular atrophy
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- muscular atrophy, normal social, language
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pertussis infx?
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- macrolide, e.g. erythromicin, give to close contacts too even if they are immunized
- immunize those not immune |
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gaucher's dz
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- Aschkenazi Jews, def of acid beta glucosidsae
- bone pain and easy brusing - dx: erlenmyerer flask = deformity of distal femure + gaucher cells = wrinkled paper appearance |
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erlenmyer flask bone?
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- Gaucher's dz
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Neimann Pick
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- fatal d/o of infancy
- sphingomyelinase |
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GMI gangliosidosis
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- beta galactosidsaes, present at birth, hepatosplenomegaly, skeleta abnormalities
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Farber's dz
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- lysosomal enzyme ceramidase
- accumulatse in joints |
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CNS tumors
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- infratentorial, supratentorial = astrocytoma
- posterior fossa = medulloblastoma - pinealoma = paralysis of vertical gaze, collier sign = eyelid retraction - sella turnica = visual field defects, endocrine = craniopharyngeoma |
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meningitis with rash
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= always N. meningitides, all others = no rash
- note: L. mono is transmitted from vagina to newborn as is GBS |
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s/p viral or trauma now with hip pain?
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- transiet synovitis => pain => decreased ROM, limp, ext rotated
- ddx septic arthritis: WBC > 12, T >102, ESR > 40, no weight bearing - do xray to r/o Legg- Calves Perthes - tx: nsaids, rest |
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marfans
ehlers danlos congenital contractural arachnodactyly |
- fibrillin 1
- collagen - multiple contractures |
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still birth with blue sclera, fx, limb deformities?
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- osteogenesis imperfecta = type I collagen defect = perinatal death
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9 y.o. girl with unsteady gait, EKG changes?
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- freidrich's ataxia: increased trinucleotide repeast, poor prognosis, necrosis and degeneration of cardiac muscles => myocarditis, fibrosis, cardiomyopathy
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milestone
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cross and circle = age 3
- square and rectangle = age 4 - triangle = age 5 - diamond = age 6 |
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migraines?
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- MCC HA in pediatric population
- only image if behavioral changes, decline school performance, decreased growth, morning HA, seizures |
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blue gray macule on sacral area? salmon patch? cafe au lait?
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- mongolian spot 2/2 entrapped melanocytes
- flat salmon = vascular lesion, goes away - cafe au lait = NF if > 0.5 mm and >5 lesions |
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cutis marmarata?
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- lace like pattern on skin in response to cold/dress
- can persist in Downs or Edwards |
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premature infant with sudden decline in consciousness, tense fontanelles, CT shows dilation of ventricles?
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- communicating hydrocephalus secondary to SAH 2/2 intraventricular hemorrhage, common in premature
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von gierke's dz?
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- type 1 glycogen storage dz = glucose 6 phosphatase def
- typical scene: 3-4 mo, hypoglycemia, lactic acidosis, hyperlipidemia - DOLL LIKE FACE = fat face, thin body |
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pompes
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- acid maltase deficiency
- type II - floppy baby, feeding difficulties |
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endocardial cushion defect?
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- left to right shunt => pulm HTN => loud P2
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continuous murmur along chest wall
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PDA
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MC leukemia in kids?
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- ALL, see increased lymphoblasts
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