peds46

Question 1

decreased complement (C3 and C4)

Answer 1

seen in active disease in SLE

Question 2

diagnostic criteria for SLE (4 of 11 needed)

Answer 2

SOAP BRAIN MD; serositis, oral ulcers, arthritis, photosens, blood cytopenias, renal disease, ANA pos, immunoserology abnormalities, neuro, malar rash, discoid lupus

Question 3

serositis

Answer 3

pleuritis or pericardial inflamm

Question 4

immunoserology abnormalities in SLE

Answer 4

antibodies to DS DNA, Smith antigen, false pos RPR or VDRL assays

Question 5

treatment of SLE

Answer 5

NSAIDs, immunosuppressants (glucocticoids are the mainstay of therapy; cyclophosphamide for severe lupus nephritis)

Question 6

prognosis of SLE

Answer 6

survival over 90%; major causes of mortality are infection, renal failure, and CNS complications

Question 7

dermatomyositis

Answer 7

infalmm of msucle results in progressive muscle weakness w characteristic skin findings

Question 8

clincial features of dermatomyositis

Answer 8

constitutional sx; cutaneous findings in sun exposed areas, proximal muscle weakness

Question 9

skin findings in dermatomyositis

Answer 9

periorbital vilaceous heliotrope rash; gottron’s papules (skin over fingers may become erythematous and hypertrophic

Question 10

proximal muscle weakness of dermatomyositis is in which joints?

Answer 10

hip girdle and legs; occurs weeks to months after eruption of skin findings

Question 11

gower’s sign in dermatomyositis

Answer 11

pos

Question 12

diagnosis of dermatomyositis

Answer 12

classic clnical pres; abnormal EMG; abnormal muscle biopsy; incr muscle enzymes (CPK, etc.)

Question 13

treatment of dermatomyositis

Answer 13

steroids, other immunosupp, vit D and calcium supp to decr fractures

Question 14

complications of dermatomyositis

Answer 14

aspiration pneumonia due to diminishd gag reflex;intestinal perforation from GI vasculitis; osteopenia 2/2 steroids and muscle weakness

Question 15

other manifestations of dermatomyositis

Answer 15

neck flexor muscle weakness, calcinosis, nail bed telangiectasias, constipation, dysphagia, cardiac involvement

Question 16

prognosis for dermatomyositis

Answer 16

better in kids than adults;no association with malignancy in kids (though there is in adults); motality rate 3%

Question 17

rheumatic fever

Answer 17

delayed autoimmune complication of URI with strep; characterized by inflamm of connective tissue

Question 18

strep of strep throat

Answer 18

strep pyogenes; group A beta hemolytic

Question 19

rheumatic fever most common in what age group?

Answer 19

5-15 yo

Question 20

clinical features of rheumatic fever

Answer 20

cardiac involement, polyarthritis, sydenham’s chorea, skin involvement

Question 21

cardiac involvement in rheumatic fever

Answer 21

all layers of the heart; endocarditis is most common and causes insuff of the left sided valves; myocarditis manifested as tachycardia out of proportion to fever; pericarditis is less common

Question 22

polarthritis in rheumatic fever

Answer 22

migratory, asymmetric, and exquisitely painful; does not result in chronic joint disease

Question 23

sydenham’s chorea in rheumatic fever

Answer 23

occurs months later; reflects involvement of the basal gang and caudate nuc involvement

Question 24

skin involvement in rheumatic fever

Answer 24

erythema marginatum (nonpruritic rash); subcutaneous nodules on bony prominences of extensor surfaces of extremities