Rubin's 16: Kidney

Question 1

Give the dx: Undifferentiated tubular structures surrounded by primitive mesenchyme; Often seen with cysts; Palpable flank mass seen shortly after birth; Usually unilateral

Answer 1

Renal dysplasia (multicystic is one form)

Question 2

Give the dx: Multiple small cysts in the renal papillae

Answer 2

Medullary sponge kidney

Question 3

Give the dx: Enlarged, multicystic (in the parenchyma) kidneys; Often mutation in the gene that codes for polycystin; Half of all patients develop end-stage renal failure; Bilateral flank and abdominal masses; Passage of blood clots in the urine; Azotemia progressing to uremia is also common

Answer 3

Autosomal dominant polycystic kidney disease **1/3 of patients also have hepatic cysts

Question 4

Give the dx: Proteinuria from effacement of the visceral epithelial cell foot processes; NO morphologic abnormalities seen by light microscopy; most common cause of nephrotic syndrome in children; Fusion of foot processes can be seen on EM;

Answer 4

Minimal change disease (aka lipoid nephrosis); Treat with corticosteroids; Will not progress to renal failure

Question 5

What is nephrotic syndrome?

Answer 5

Heavy proteinuria; Hypoalbuminemia; Hyperlipidemia; Edema;

Question 6

Give the dx: Facial edema; Headaches, malaise, dizziness; Oliguria; Hematuria; HTN; Usually seen in children who have recently been ill; Subepithelial and subendothelial depositsof complement that look like “humps”

Answer 6

Nephritic syndrome from poststreptococcal glomerulonephritis

Question 7

Give the dx: High proteinuria; Deposition of kappa or lambda light chains in the glomerular basement membranes and mesangial matrix; Amorphous material blocks glomerular capillaries

Answer 7

Amyloid nephropathy Renal amyloidosis leads to nephrotic syndrome

Question 8

Give the dx: Eosinophils in the urine

Answer 8

Allergic nephritis

Question 9

Give the dx: WBC casts in the urine; Most commonly caused by E. Coli (gram-negative); Bacteriuria; Diabetic patients are at increased risk; Patients present with fever, chills, malaise and flank pain

Answer 9

Pyelonephritis

Question 10

Give the dx: Frequent cause of nephrotic syndrome in adults; Accumulation of immune complexes in the subepithelial zone of glomerular capillaries (IgG deposits on immunofluorescence);

Answer 10

Membranous glomerulopathy

Question 11

Give the dx: Chronic immune complex disease; Granular deposition of immunoglobulin and complement in glomerular capillary loops and mesangium

Answer 11

Membranoproliferative glomerulonephritis type 1 (MPGN-1)

Question 12

Give the dx: Deposition of antiglomerular basement membrane antibody

Answer 12

Goodpasture syndrome

Question 13

Give the dx: Immune complexes against DNA, RNA and autologous proteins get deposited along the BM of the glomeruli ad form a similar pattern to idiopathic membranous nephropathy; Mesangial and subendothelial deposits of immunoglobulins also present

Answer 13

Membranous nephropathy of SLE

Question 14

Give the dx: Systemic, necrotizing vasculitis; Granulomatous lesions in the nose, sinuses, lungs; Renal glomerular disease is often present (hematuria with RBC casts, focal glomerular necrosis with crescents and vasculitis affecting the aterioles and venules); C-ANCA often present

Answer 14

Granulomatosis with Polyangiitis (Wegener’s) –can result in rapidly progressive glomerulonephritis

Question 15

Give the dx: Glomerular scarring; Initially involves only part of a glomerular tuft; Segmental obliteration of capillary loops by collagen and increased lipids/proteins; Cause od nephrotic syndrome in 30% of adults (and 10% of children); Proteinuria

Answer 15

Focal segmental glomerulosclerosis (FSGS) **Most common renal complication of IV drug use

Question 16

Renin is release by the:

Answer 16

Juxtaglomerular apparatus

Question 17

Give the dx: Most common type of glomerulonephritis in adults; Deposition of IgA-dominant immune complexes causes nephropathy; Exacerbations occur after RTIs or GI bugs; Renal biopsy shows intense mesangial IgA, almost always with C3; Mesangial cell proliferation; Patients present with hematuria, proteinuria, some RBC casts

Answer 17

Berger disease (a kind of focal proliferative glomerulonephritis)

Question 18

Give the dx: Abnormal type IV collagen in the glomerular BM; Hematuria presents early in life; Proteinuria, progressive renal failure and HTN develop later in life

Answer 18

Hereditary nephritis (Alport syndrome)

Question 19

Give the dx: Pathologic changes in some glomeruli but not others; Intermittent hematuria;

Answer 19

Focal proliferative glomerulonephritis - includes: –lupus nephritis –Henoch-Schonlein purpura –Berger disease (IgA nephropathy)

Question 20

Give the dx: Widespread involvement of the glomeruli; Diffuse proliferation of mesangial and endothelial cells (biopsy shows hypercellularity); Immune complex deposition present on both sides of the BM, in the mesangium and capillary loops; Glomeruli with thickened basement membranes called “wire loop” lesions;

Answer 20

Proliferative glomerulonephritis (severe form of lupus nephritis)

Question 21

Give the dx: Uncommon but aggressive form of glomerulonephritis; When combined with pulmonary hemorrhage, the dx is Goodpasture syndrome; Autoimmune attack on GBM component of collagen type IV; Diffuse linear staining of GBMs for IgG, which indicates autoantibodies bound to the BM; Over 90% have glomerular crescents (crescentic glomerulonephritis); Presents as rapidly progressive renal failure with nephritis

Answer 21

Anti-GBM antibody glomerulonephritis