Renal

Question 1

AD PKD

Answer 1

Cause: PKD1 (chromosome 16), and PKD2 (chromosome 4)
Pathogenesis: cysts arise from everywhere in kidney
ROS: older pt, liver/spleen/pancreas cysts, berry aneurysms, diverticulosis, mitral valve prolapse
Biopsy: sclerosis

Question 2

AR PKD

Answer 2

Cause: PKHD1 (chromosome 6)
Pathogenesis: cysts arise from collecting ducts
ROS: pt is baby + oligohydramnios in mother, small kidney, liver fibrosis/cirrhosis (abnml. LFTs)

Question 3

Renal Cell Carcinoma (RCC)

Answer 3

Cause: VHL (hereditary), smoking, cadmium exposure (batteries), horseshoe kidney, MCD
Pathogenesis: arises from proximal tubule cell
Biopsy: clear cells w/ lipid and glycogen in cytoplasm
Paraneoplasia: EPO, Renin, PTH-like peptide, ACTH, AA amyloid, leukemoid reaction

Question 4

Horseshoe Kidney

Answer 4

Assoc. w/ Turner’s and trisomies
Pathogenesis: IMA prevents kidney from ascending
Complication: RCC, ischemic bowel dz from compression of IMA

Question 5

Minimal Change Dz

Answer 5

LM: normal
EM: effacement of foot processes
Tx: good response to steroids

Question 6

FSGS

Answer 6

“Fat black prostitute”

- assoc w/ obesity, Sickle cell, HBV

Question 7

Membranous Nephropathy

Answer 7

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Question 8

MPGN Type I

Answer 8

Assoc: HCV (cryoglobulins)
LM: tram-track appearance d/t GBM splitting
EM: subendothelial deposits
IF: granular (IgG and C3)

Question 9

MPGN Type II

Answer 9

Aka Dense Deposit Dz
Assoc: C3 nephritic factor (stabilizes C3 convertase which decreases serum C3 levels)
LM/EM: dense uniform thickening of GBM

Question 10

RPGN Type I

Answer 10

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Question 11

RPGN Type II

Answer 11

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Question 12

RPGN Type III

Answer 12

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Question 13

IgA Nephropathy (MAPGN)

Answer 13

S/p GI or respiratory infection
LM: mesangial proliferation
EM: mesangial IC deposits
IF: mesangial IgA deposits

Question 14

Alport’s

Answer 14

ROS: can’t see, can’t pee, can’t hear a bee
Mechanism: mutation of type IV collagen
EM: splitting of BGM w/ “basket-weave” appearance

Question 15

Henoch-Schonlein Purpura (HSP)

Answer 15

Assoc w/ IgA nephropathy, palpable purpura d/t IgA deposits in skin etc.

Question 16

TIN

Answer 16

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Question 17

Acute Tubular Necrosis

Answer 17

Labs: increased FENa, “muddy brown” casts
1. Exciting event
2. Maintenance phase - oliguria
3. Recovery phase - polyuria (BUN and creatinine fall)
Causes: ischemia d/t decreased renal blood flow with PCT and ThAL are most susceptible, OR nephrotoxicity d/t injury from toxic substances (drugs, myoblobin, hemoglobin) PCT is most susceptible

Question 18

Papillary Necrosis

Answer 18

Cause: Sickle cell, acute pyelonephritis, NSAIDs, DM
Gross: sloughing of necrotic renal papillae causing gross hematuria and proteinuria