Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
40 Cards in this Set
- Front
- Back
What maternal infection is PDA associated with?
|
– rubella
|
|
Coarct Exam
|
– late systolic murmur in left axilla
– apical impulse is forceful – treat recurrence w/ balloon |
|
Which part of DTAP causes problems
|
– pertussis
– you can leave that out for next vaccine |
|
HBV vaccine
|
– birth, 2 m, 6 m
|
|
Dtap vaccine
|
– 2, 4, 6 months
– 18 months – 5 years |
|
Hib vaccine
|
– 2,4,6 months
– 15 months – this is the same for PPV |
|
IPV vaccine
|
– 2,4,6 months
– 5 years |
|
MMR and Varicella vaccine
|
– 15 months
– repeat MMR at 5 years |
|
Fabry’s disease
|
– lysosomal storage
– deficiency of a-galactosidase A – accumulation of ceramide trixidose – the only one that’s x-linked besides Hunters – get renal failure |
|
Krabbe’s disease
|
– lysosomal storage aka GM1
– deficiency of galactosylceramide B-galactoside – accumulation of galactocerebroside – get optic atrophy, vertebral beaking, spacity, and die early |
|
Gaucher’s disease
|
– lysosomal storage
– deficiency of B-glucocerebroside – glucocerebroside accumulated in brain, liver, spleen and marrow to make Gauchers cells – normal life span – erlynmeyer flask bones |
|
Tay Sachs disease
|
– lysosomal storage
– deficiency of Hexosaminidase A – accumulation of GM2 ganglioside (as does Sandhoff’s) – death by 3 – big in jews |
|
Metachromatic leukodystrophy
|
– lysosomal storage
– deficiency of Arylsulfatase A – accumulation of sulfatide – gets brain, kidney, liver, and peripheral nerves |
|
Hunter’s vs. Hurlers
|
– lysosomal storage
– deficiency of A-L-iduronidase vs. iduronate sulfatase |
|
Common variable immunodeficiency
|
– associated w/ lymphoma and autoimmune disease
– Ig drops to 20s and 30s and all but A are low – tend to present later than Brutons |
|
Ataxia telangectasia
|
– Combined immunodeficiency from DNA repair defect
– associated w/ non hodgkins and gastric carcinoma – No tx |
|
Wiskott-Aldrich
|
– Combined immunodeficiency
– increased E and A and decreased M – associated w/ atopy and leukemia |
|
Chronic granulomatous disease
|
– get anemia, LA, and hypergammagolublinemia
– Nitroblue tetrazolium Dx – Tx w/ daily TMP-SMX and IFN-a |
|
Chediak higashi
|
– associated w/ occulocutaneous albinism, neuropathy, neutropenia
– tx w/ BMT |
|
C1esterase deficiency dx and tx
|
– CH50
– danzol |
|
Terminal compliment deficiency
|
– meningococcal and gonococcal
– lupus and glomerulonephritis |
|
Subacute phase of Kawasake
|
– increase ESR and thrombocytosis
|
|
When are corticosteroids contraindicated
|
– Kawasakis and SJS
|
|
Roseloa Rash
|
– from HHV-6
– starts on trunk and quickly spreads to face and extremities – associated w/ febrile seizures |
|
Kernicterius Hx
|
– high pitched cry, hypertonicity, seizures
|
|
How do you treat CP spaticity?
|
– diazepam, dantrolene, or baclofen pump
|
|
Neuroblastoma associations
|
– Hirschprungs, neurofibromatosis, N-myc
– kids less than 2 |
|
Neuroblastoma history/exam
|
– Horners syndrome
– Opsoclonus/myoclonus – raccoon eyes – subcutaneous tumor nodules – it likes to hemorrhage and calcify |
|
Which Ab’s are specific for Lupus
|
– anti smith and anti ds-DNA
|
|
Cru-du-chat
|
– 5p deletion
– microcephaly, hypotonia, short stature |
|
What should you give an atopic asthmatic?
|
– mast cell stabilizer
– aka sodium cromolyn |
|
Chlamydia blindness exam
|
– lymphoid follicles on conjunctiva
– scarring and limbal follicles |
|
Von Gierke’s
|
– G6P deficiency (type1) presents at 3-4 months
– hypoglycemia, lactic acidosis – increased uric acid and lipids – doll face w/ protuberant abdomen and thin arms |
|
Pompe’s
|
– Acid maltace deficiency (type 2) present is 1st weeks
– floppy baby w/ big tounge – feeding difficulties and hypertrophy CM |
|
Type 3 GSD
|
– deficiency of glycogen debranching enzyme
– clinically like type 1 but normal lactate and uric acid – increased LFTs – fasting ketosis |
|
Type 4 GSD
|
– deficiency of branching enzyme
– 18 months w/ FFT and HSM |
|
TCA overdose treatment
|
– sodium barcarb will treat the acidosis and narrow the QRS
|
|
Most common pediatric brain tumor
|
– benign astrocytoma (supra and infra)
– then medulo |
|
Gastroschisis 1st step
|
– wrap up exposed bowel
|
|
Faber’s disease
|
– ceramidase deficiency -> accumulation in joints
|