ABIM NEP Inh dz Word Scramble
|
Embed Code - If you would like this activity on your web page, copy the script below and paste it into your web page.
Normal Size Small Size show me how
Normal Size Small Size show me how
Question | Answer |
EM basket weave pattern w thick and thin areas of GBM? | Alport Syndrome. Very early in disease, uniform thinning noted. |
Diagnosis of Alport Syn is confirmed by ____ | EM pattern of basketweaving; IF shows abnormal collagen but variable depending on inheritance |
Normal adult bowman’s capsule stains for what collagen chains? | alpha-3, -4, and -5 |
Alport bowman’s capsule stains for what collagen chains? | alpha-5, -5, and -6 |
Linear staining of GBM and bowman capsules by alpha-5 collagen? | Can be normal! Also seen w thin basement membrane disease. May also stain distal tubule in linear fashion. Same staining patient w alpha-3. |
Alport heterozygous carries have GBM on IF stain for collagen ___. | alpha-5 and alpha-3 |
Alport patients have bowman’s capsule on IF stain for collagen ___. | no staining for either alpha-5 and alpha-3 |
Thin basement membrane clinical picture. | hematuria w/o proteinuria. DDX Alport. LM nl;IF stains reveal all alpha-3 (no -4 or -5) |
Gene associated with Thin basement membrane? | COL4A3 or COL4A4 |
Describe PKD dx based on # cysts required. | 15-39 need >3 cysts total; 40-59 need 2 or more cysts each kidney; over 40 need 4 or more cysts per kidney |
T/F mutated proteins in APKD are located on kidney cilia. | TRUE. polycistin-1, -2 and -3 |
CRISP study described height adjusted kidney volume of ____ predicts risk of developing CKDIII and beyond. | 600 cm3/m. considered most sensitive marker of progression. GFR fall not seen until volume > 1500. |
Goal therapies in APKD? | BP <120/80, 3.5 liters/day fluid, 2 gram Na/day, follow MRI q2yrs, statin |
Management of bleeding cysts from APKD: | epsilon amino caproic acid (EACA), aprotinin, DDAVP |
Most useful test to detect infected APKD cysts? | PET-CT |
Recommendations for Intracranial aneurysm (ICA) screening in APKD | perform if have family or personal hx or prior to surgery/txp (?), high risk occupation,. Screen every 3-5 yrs until mid 40s. |
What drugs should be avoided in APKD? | SSRIs and TCA release or potentiate effect of vasopressin! ? Caffeine. |
Created by:
ka1usg
Popular Medical sets