Rubin's 16: Kidney Flashcards
Give the dx: Undifferentiated tubular structures surrounded by primitive mesenchyme; Often seen with cysts; Palpable flank mass seen shortly after birth; Usually unilateral
Renal dysplasia (multicystic is one form)
Give the dx: Multiple small cysts in the renal papillae
Medullary sponge kidney
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
Autosomal dominant polycystic kidney disease **1/3 of patients also have hepatic cysts
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;
Minimal change disease (aka lipoid nephrosis); Treat with corticosteroids; Will not progress to renal failure
What is nephrotic syndrome?
Heavy proteinuria; Hypoalbuminemia; Hyperlipidemia; Edema;
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”
Nephritic syndrome from poststreptococcal glomerulonephritis
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
Amyloid nephropathy Renal amyloidosis leads to nephrotic syndrome
Give the dx: Eosinophils in the urine
Allergic nephritis
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
Pyelonephritis
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);
Membranous glomerulopathy
Give the dx: Chronic immune complex disease; Granular deposition of immunoglobulin and complement in glomerular capillary loops and mesangium
Membranoproliferative glomerulonephritis type 1 (MPGN-1)
Give the dx: Deposition of antiglomerular basement membrane antibody
Goodpasture syndrome
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
Membranous nephropathy of SLE
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
Granulomatosis with Polyangiitis (Wegener’s) –can result in rapidly progressive glomerulonephritis
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
Focal segmental glomerulosclerosis (FSGS) **Most common renal complication of IV drug use
Renin is release by the:
Juxtaglomerular apparatus
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
Berger disease (a kind of focal proliferative glomerulonephritis)
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
Hereditary nephritis (Alport syndrome)
Give the dx: Pathologic changes in some glomeruli but not others; Intermittent hematuria;
Focal proliferative glomerulonephritis - includes: –lupus nephritis –Henoch-Schonlein purpura –Berger disease (IgA nephropathy)
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;
Proliferative glomerulonephritis (severe form of lupus nephritis)
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
Anti-GBM antibody glomerulonephritis
