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
Hemoglobin electrophoresis patterns:
HbAS (sickle trait)= ++ A + S Hb SS (sickle dx)= ++ S; no A Beta thal minor= A + variable F Beta thal major= +++ F. No A Alpha major= ++ A; HbH HbBart= ++ HbH; no A.
8 y.o. with T1DM with abdo pain. Lost wt. Pruritic bulls lesions on extensor surface of arms + trunk. Likely dx?
- celiac dx
- IBD
- eosinophilic gsatroenteropathy
- eosinophilic gastroenteropathy
- parasitic infection
Celiac Dx
Dermatitis herpetiformis
What glomerular dx has low C3?
Post infectious
Membranoproliferazive
AI and Vasculitis (SLE)
Subacute bacterial endocarditis
Versus Normal
= IgA, anti-GBM, HSP, alport, polyarteritis nodosa
Leukocoria. Name 3 DX?
Retinoblastoma Cataracts Advanced ROP Coats Dx Toxocariasis
What do you do for SIADH
Fluid restrict
Most imp and two other organism for splenic pt?
Streptococcus pneumoniae
Other: Salmonella, Neisseria meningitidis, Haemophilus influenzae
Contractility vs. Peripheral vascular resistance. Describe the pattern for:
- Dopamine
- high dose epi
- low dose epi
- dobutamine
Dopamine: Up both
High dose: Up both
Low dose Epi: Contractibility up but no change to PVR
Dobutamine: Same as low dose epi
What are two investigations or dermatomyositis?
CK, AST, LD
EMG
+/- Muscle Bx
Reminder clinical dx:
= Classic Rash (heliotrope of eye lid + grotton) + min. 3 of
> weakness (symmetrical proximal)
> muscle enzyme (CK, AST, LDH)
> EMG change (Short small motor unit, fibrillation, (+) sharp wave)
> Muscle bx (necrosis, inflammation)
Short gut syndrome sequelae. Other than malnutrition and malabsorption:
➢ Dehydration
➢ Vitamin deficiency
➢ Malnutrition (i.e. Vitmain B12, folate, fat soluble vitamins, zinc, copper)
➢ ** Bacterial Overgrowth
➢ High rate of sepsis and cholestasis since may depend on TPN for long time
o ** Catheter infections
o **** Cholestasis
➢ Central line infection, liver failure w/ long TPN use
o ** *Sepsis
Describe good control asthma:
< 4 daytime /week;
no nightly;
activity normal
no missed school
Moderate= daily daytime but no weekly night Severe= weekly night use
Describe 4 organs other than MSK involved with DMD and their effect:
Cardiac: Cardiomyopathy
Resp: Hypoventilation
GI: constipation
OTHER: GI: obesity
OTHER: Steriod affect= osteopenia
CNS: intellectual impairment
MSK: intoning, calf hypertrophy, scoliosis, mobility down
Methanol long term complication
Blindness
Methanol treatment
ABC
Fomepizole
Give ethanol to stop methanol metabolism
+/- Na bicarb, folate, hemodialysis
4 features of atypical febrile seizure
More than 1 in 24 hour
Seizure > 15 minute
Focal symptom
+/- age < 6 or > 6 y.o.?
5 absolute contraindications for OCP int teen?
Migraine with aura Severe cirrhosis Severe uncontrolled HTN History of Stroke History of VTE or DVT Current breast cancer
Name 3 RF for refeeding syndrome?
Weight < 80% of ideal weight for age, height and sex.
Not eating > 10 days.
Other: fast re-feeding
4 High risk groups for sever influenzae?
Pregnancy
< 5 year old (Neonates and Infants) (6-59 months)
Aboriginal, Inuit and First Nation.
Children with chronic disease. (i.e. CVS, liver, renal dx, neuro, anemia, CA etc.)
On Chronic ASA therapy
Elderly
FEV1 drop in CF. 5 reasons?
Infection: Pseudomonas Infection: Stenotrophomonas Infection: Burkholderia cepacia Viral LRTI ABPA Smoking Natural progression= Worsening bronchiectasis (progression of dx) Non adherence to treatment (although they said she is)
Neonatal lupus. Two most common antibodies?
Anti-Ro
Anti-Lau
Three signs of neonatal lupus aside from congenital heart block:
Discoid rash
Hepatitis
Thrombocytopenia/ cytopenia
