Nephrology

Question 1

What is BERGERs disease

Answer 1

IgA nephropathy
Respiratory tract infection + GROSS hematuria
IgA- immune complexes which deposit in the kidneys leading to scarring

Question 2

What is the treatment for BERGERs disease

Answer 2

ACE-I- renals progression of CKD
Prednisolone

Question 3

What is dx of igA nephropathy

Answer 3

Renal biopsy- light microscopy will show focal or diffuse mesangial proliferation and extracellular matrix expansion

Question 4

What other dx are typically associated with IgA nephropathy

Answer 4

Liver cirrhosis due decreased clearange of IgA causing liver damage
AIDS

Question 5

What is good pastures disease

Answer 5

Known as Anti-GBM
Progressive glomerulonephritis and pulmonary hemorrhage
Dx: urine analysis shows RBC and proteins. renal biopsy -FSGN and rupture of bowmans capsule. Immunofluorescence- linear capillary wall staining for IgG ad C3 deposition

Question 6

What is alport Syndrome

Answer 6

X-linked hereditary disorder (FEMALE Carriers, AFFECTED males)
Defect in basement membrane formation in cochlear, eye and kidney glomerulus
caused

Question 7

What is the triad found in Alport syndrome

Answer 7

Progressive nephropathy, Sensorineural deafness and ocular abnormalities

Question 8

What is the mutation leading to Alport syndrome

Answer 8

80% are thought X-linked mutation in the gene that mades type 4 collagen

Question 9

What is the difference shown on light microscopy between Alport and igA nephropathy renal biopsy

Answer 9

Alport- normal
IgA nephropathy: focal or diffuse mesangial proliferation and ECM expansion

Question 10

What is type 1 RTA

Answer 10

Affects the distal tubules
reduces the ability to excrete acid and absorb potassium
leads to:acidosis, hypOkalemia and low bicarbonate and high urine PH
causes: inablity to acidify the urine
commonly causes renal stones and neprocalconosis,

Question 11

What is type 4 RTA

Answer 11

common RTA
hyporeninemic hypoaldosteronism
features: hyperkalemia, aciosis, mild chronic renal insuffincyce
caused y DM or tubulointersitial disease

Question 12

What is type 2 RTA

Answer 12

impaired retention if bicarbonate in proximal tubules which leads to systemic acidosis
causes: myeloma, amyloidosis, cytinosis, wilsons dx
features: hypOkalemia, bicarb is low, urine ph is normal