2015 Exam Flashcards
Boy with lupus. Most likely test consistent with lupus:
- ANCA (+)
- HIGH plt
- HLA B27 (+)
- (+) RPR
(+) RPR
SLE: Thrombocytopenia typically (high much less common), ANA, anti-dsDNA antibodies, anti-Sm, False (+) RPR (syphilis test)
Absent reflexes. Fasciculation in 6 wk old with poor feed and hypotonia. What is next most diagnostic test?
- MRI
- thyroid function
- metabolic screen
- genetic testing
Genetics!
SMA= absent reflexes +/- tongue fasciculation
= SMN1 gene testing
3 y.o. with CP with pneumonia. Consolidation with effusion. best tx:
- cipro
- clinda + gent
- cefuroxime + azithro
- amp+ gent
Clinda + gent
CLIN= anaerobes, staph, strep Gent= gram (-) bacilli and pseudomonas
Post tonsillectomy. Na 121 with serum osmolality 260. most consistent with:
- urine osmolality > serum
- serum osmolality> urine
- Urine Na < 0
- high U/O
Urine osmolality > serum.
Two factors:
- excessive hypotonic fluid in Sx
- SIADH due to non osmotic causes post-op (pain, low fluid etc.)
SIAD= low UO= urine osm > serum
2 y.o. with 2 wk proximal muscle weakness:
- congenital myopathy
- neuromuscular
- peripheral neuropathy
- SC injury
Congenital Myopathy
Central= HIE= DTR up, muscle bulk and power normal/low
Anterior Horn Cell= PROXIMAL; DTR low or absent
i.e. SMA= hypotonia, proximal, fasciculation, areflexia. SMN1 gene testing!
Peripheral= DISTAL.
i.e. Charcot Marie Tooth= teens, slow DISTAL weakness, tripping, foot drop, inverted champagne of lower leg, high arch, hammer tone, flat feet
W/U: nerve conduction study, EMG +/- genetic testing
Neuromuscular: muscle bulk and power normal but fluctuate.
i.e. Myasthenia Gravis= Ptosis, EOM weak, normal pupil, feeding issue, symmetric progressive distal limb with rapid fatigue. EMG!
Muscular Dystrophy=
i.e. DMD= distal= calf hypertrophy, muscle bx.
Myotonic Dystrophy = tent mouth, low DTR
When is the live flu vaccine contraindicated?
< 2
severe asthma (active wheeze, PO or high dose ICS, medically attended wheezing in last 7d)
immunocompromised
pregnancy
on ASA
<48h after antiviral agent against flu given
AOM with myringotomy tubes. Purulent drainage. Afebrile.
- Cx and wait
- Topical Abx and corticosteroid drop
- High dose amox
- Standard dose amox
Topical Abx and Corticosteroid Drop
Lots of pRBC. ECG complication?
Peaked T waves= high K
Acute rheumatic fever ECG finding?
Prolonged PR interval.
HTN, high temp. tachycardia. Due to?
- cocaine
- LSD
- marijauna
Cocaine= Sympathomimetic
Describe APGAR:
0-1-2
Appear= Blue, Acro, Pink Grimace= None, grimace w/ stimulated, vs. 2 is sneezing, coughing or pulling away with stimulation Pulse= None, < 100, > 100 Activity/Tone= 0, some flexion, full Resp= None, Irregular, Full
Best test for nutritional vitamin D deficiency?
- Ca
- 1, 25 (OH)D
- 25-Vitamin D
- PTH
25 Vitamin D
BB screen comes back (+) for Carnitine palmitoyltransferase 2 deficiency. Best course until confirmed:
- start PO carnitine
- feed frequent (q3h)
- a.a. formula
- hyper caloric formula
= F.a. Oxidation Defect
= Avoid fasting!
What tumour is beck with Wiedemann at higher risk for?
Wilm’s Tumour
Hepatoblastoma
Neuroblastoma
Adrenocortical carcinoma
Reminder: macrosomia, macrogloassia, omphalocele, HSM, ear pit, hypoglycaemia due to hyperinsulinemia, hemihyperplasia
Why do we irradiate blood given to prem?
- less CMV
- less GVHD
- sterilize RBC
- less hemolytic rxn
Less GVHD
Goal= prevent transfusion associated graft-versus host disease
Indication: infant, immunosuppressed or dficienct, bone marrow/stem cell transplant, intrauterine transfusion