12

Question 1

autosomal recessive disorder, congenital marrow failure, poor growth, morphologic abnormalities, macrocytic anemia

Answer 1

Fanconi anemia

Question 2

chelators for iron and lead

Answer 2

iron: deferoxamine
lead: oral succimer (mild-mod), calcium EDTA (mod-sev)

Question 3

limitation of upward gaze, bilateral eyelid retraction and light-near dissociation (Parinaud syndrome)

Answer 3

pineal gland tumors

also headaches and vomiting

Question 4

most common pneumo bug in infants/young kids with CF?

in adults with CF?

Answer 4

staph aureua

pseudomonals

Question 5

migraine tx

Answer 5

NSAIDs
triptans: rizatriptan (for ages 6-17 years) and almotriptan (for adolescents), anti emetics, abortive: topiramate, valproic acid, β-blockers, TCAs and cyproheptadine

Question 6

migraine criteria: 5+ attacks that meet the following criteria

Answer 6

1. Last 1-72 hours (untreated or unsuccessfully treated)
2. 2+:
   Unilateral or bilateral frontal or temporal pain
   Pulsing or throbbing in nature
   Moderate to severe pain
   Aggravated or causes avoidance of routine physical activity
3. Have at least one of the following:
   Nausea or vomiting
   Photophobia or phonophobia
4. Is not due to another disorder

Question 7

? is imaging of choice to evaluate for secondary headache, unless ?

Answer 7

MRI

hemorrhage or fracture is suspected based on history and physical in which case CT is recommended

Question 8

To establish the diagnosis, a patient must demonstrate 4 of the 11 features of SLE
(SOAP BRAIN MD)

Answer 8

Serositis
Oral ulcers
Arthritis
Photosensitivity
Blood Disorders
Renal involvement
Antinuclear antibodies
Immunological disorders (dsDNA, anti-Smith, anti-phospholipid antibodies)
Neurological disorder
Malar Rash
Discord Rash

Question 9

SLE

Answer 9

A multisystem disease in which widespread inflammatory involvement of the connective tissues occurs along with an immune-complex vasculitis

Question 10

sensitive and specific SLE markers

Answer 10

ANA positivity is present in 95% to 99% of children with SLE
Anti–double-stranded (ds) DNA levels are more specific, and in some individuals, levels do correlate with disease severity

Question 11

Renal disease in SLE is often asymptomatic so if hypertension, elevated creatinine, or findings of nephritis on urinalysis are noted, do what?

Answer 11

biopsy is required for staging the level of disease

Question 12

Involvement of other organs may present as ?

Answer 12

cerebritis, pleuritis, pericarditis, hepatitis, and hypersplenism.Hematologic abnormalities include cytopenias and antiphospholipid antibodies.

Question 13

ddx SLE

Answer 13

JIA (-renal involvement)
Dermatomyositis (+proximal muscle weakness, Gottron papules, erythema over the elbows and knees, and the finding of malar rash crossing the nasolabial folds)
Reactive and postinfectious arthritis

Question 14

SLE treatment

Answer 14

avoid sun exposure
Hydroxychloroquine (mild), NSAIDs for joint pain, Glucocorticoids (acute/moderate), immunosuppressive agents (cyclophosphamide, rituximab, methotrexate, and mycophenolate mofetil) if severe including evidence of renal or neurologic involvement

Question 15

lab studies in PSGN

Answer 15

C3 (low in 90% of cases), C4 (usually normal); antistreptolysin-O (ASO) enzyme antibodies, and antideoxyribonuclease B (anti-DNase B) antibodies provide evidence of recent streptococcal infection.
hematuria, proteinuria, red cell casts

Question 16

? is characterized by recurrent painless hematuria, usually preceded by an upper respiratory tract infection.

Answer 16

Immunoglobulin A (Berger) nephropathy

Question 17

ddx PSGN

Answer 17

Rhabdomyalysis, Immunoglobulin A (Berger) nephropathy, HSP, SLE

Question 18

Generally the interval between GABHS pharyngitis and APSGN is ?; the interval between GABHS impetigo and APSGN is?

Answer 18

1 to 2 weeks

3 to 6 weeks

Question 19

PSGN presentation

Answer 19

almost all patients have microscopic hematuria, only 30% to 50% develop gross hematuria. In addition, 85% present with edema and 60% to 80% develop hypertension.

Question 20

PSGN treatment

Answer 20

supportive; fluid balance is crucial; diuretics, fluid restriction, or both may be necessary. Sodium and potassium intake may require restriction. HTN controlled with CCBs

Question 21

resolution timeframe of PSGN

Answer 21

edema resolves in 5 to 10 days, and patients usually are normotensive within 3 weeks. C3 levels usually normalize in 2 to 3 months; a persistently low C3 level is uncommon and suggests an alternate diagnosis. Microscopic hematuria may persist for 1 to 2 years.

Question 22

appendicitis workup

Answer 22

uCG, surgery consult if suspected
abdominal US then CT
CBC that may show leukocytosis, a CMP (to identify diabetes, hypercalcemia, abnormal creatinine, transaminitis), pancreatic enzymes, and a urinalysis to eliminate other causes of the pain

Question 23

why surgical consult needs to be done ASAP for appendicitis

Answer 23

perforation rates exceed 65% if diagnosis is delayed beyond 36 to 48 hours from symptom onset

• most common complications of appendicitis are wound infection and intra-abdominal abscess or phlegmon formation, all of which occur more frequently with appendices perforation
  others: sepsis, shock, ileus, peritonitis, and adhesions causing SBO

Question 24

CBC in appendicitis

Answer 24

leukocytosis with a predominance of PMNs (a “left shift”) on a CBC supports an inflammatory process. However, the CBC may be normal in the first 48 hours of the illness. Thereafter, it would be expected to be greater than 10,000/mm3 and in cases of perforation, it may be greater than 20,000/mm3.