Rheum, collagen, multi-organ

Question 1

Marfan - what respiratory condition is fairly specific for it and is minor criteria for dx

Answer 1

spontaneous pneumothorax

Question 2

Triad of primary symptoms for Marfan

Answer 2

Skeletal, cardio, ocular

Question 3

What skeletal features are associated with Marfan

Answer 3

Pectus carinatum, pectus excavatum, reduced upper to lower segment ratio, scoliosis >20 degress or spondylolisthesis, reduced elbow etension, joint hypermobility

Question 4

What cardiac conditions are associated with Marfan

Answer 4

dilation or dissection of ascending aorta, mitral valve prolapse, dilation of pulmonary artery and calcification of mitral annulus

Question 5

What ocular features are associated with Marfan

Answer 5

Ectopia, lentis, flat cornea, hypoplastic iris, and increased axial length of the globe, myopia

Question 6

What physical activities are Marfan pts asked to avoid

Answer 6

not to participate in contact or competitive sports or isometric exercise, avoid activities that place them at increased risk for joint injury or pain

Question 7

What is the triad for Ehlers Danlos

Answer 7

hyperextensible skin, hyperextensible joins, and tissue fragility

Question 8

What is the genetics pattern for EDS

Answer 8

AD mostly, but can see AR, and X-linked

Question 9

What is the highest risk for Ehlers Danlos?

Answer 9

Spontaneous rupture of medium-sized arterisa

Question 10

What precautions should Ehlers Danlos pt take?

Answer 10

Avoid collision sports, weight training and heavy lifting

Question 11

What is the immune basis for sarcoidosis?

Answer 11

Interaction between macrophages, Th1 phenotype CD4+ lymphocytes, resultant cytokines and chemokines that cuase an inflammatory cascade that produces granuloma, persistent antigenic stimulation maintains the process (mycobacterium, propionibacterium acnes?)

Question 12

What is ophtho exam for patient with sarcoidosis?

Answer 12

redness, eye pain, blurry vision, photophobia, eye examination, uveitis with firmly edged keratitic precipitates, conjunctival granulomas, peripheral multifocal choroiditis

Question 13

What are typical skin manifestations in sarcoidosis?

Answer 13

red, flat-topped papules involving the face, erythema nodosum, ulcers, subcutaneous tumors, nonspecific mac, pap, plaw, nods

Question 14

Typical neuro symptoms for adults with sarcoidosis

Answer 14

facial nerve palsy, HA, sensory deficits, cerebellar signs

Question 15

Sarcoidosis in children less than 5 years of age have what triad?

Answer 15

arthritis, rash, and uveitis, seizures - prognosis is worse

Question 16

Preferred method for confirming sarcoidosis

Answer 16

biopsy of periphearl lymph nodes, skin lesions, enlarged salivary glands, lacrimal glands or conjunctival nodules

Question 17

What is pulm function test results for sarcoidosis

Answer 17

restrictive pattern

Question 18

What lab tests typically abnormal in sarcoidosis?

Answer 18

ESR, anemia, leukpenia, eosinophilia, hypergammaglob, hypercalcemia, hypercalciuria, ACE 9n 50% with late-onset disease

Question 19

Pattern of rheumatologic disorder, 13 to 15 year old AA from southern state?

Answer 19

Sarcoidosis

Question 20

Child with prolonged fever and uveitis. What three rheum diseases are highest on list?

Answer 20

JIA, SLE, sarcoidosis

Question 21

Diagnostic criteria for metabolic syndrome

Answer 21

Central obesity and two of the following:
High triglyc
Low HDL
HTN
Elevated fasting plasma glucose

Question 22

What are symptoms of craniopharyngiomas?

Answer 22

Hypopituitarism - decline in growth rate, decreased muscle mass, infantile body proportiona, DI, headache, visual field abnormalities, increased fat mass (GH deficiency and hypothyroidism), if extends to hypothalamus can have weight gain

Question 23

What is diencepahlic syndrome?

Answer 23

Tumors affected appetite center, decrase food intake, leading to weight loss