Kidney and Urinary Tract Flashcards
1
Q
Nephrotic syndrome
A
- Group of conditions characterized by increased basement membrane permeability, permitting the urinary loss of plasma proteins
- Clinical; massive proteinuria, hypoalbuminemia, generalized edema, hyperlipidemia and hypercholesterolemia
2
Q
Minimal change disease (lipoid nephrosis)
A
- The prototype of the nephrotic syndrome
- Lipid-laden renal cortices
- EM: fusing of epithelial foot processes
- Responds well to adrenal steroid therapy
3
Q
Focal segmental glomerulosclerosis
A
- Major cause of nephrotic syndrome, clinically similar to MCD
- Characterized by sclerosis within capillary tufts of deep juxtamedullary glomeruli
- Focal distribution: some of the glomeruli
- Segmental distribution: part of the glomerulus
- Do not respond to steroids
- More common in African Americans
- Associated with HIV
4
Q
Membranous glomerulonephritis
A
- Major primary cause of the nephrotic syndrome - accompanied by azotemia
- Immune complex disease
- Thickened capillary walls
- Immune complexes in intramembranous and epimembranous locations within the BM
- “Spike and dome” appearance - stain
- Granular deposits of IgG or C3 - granular immunofluorescence
- Sometimes associated with; HBV, syphilis, malaria infection, drugs or malignancy
5
Q
Diabetic nephrophathy
A
- Clinically manifested by nephrotic syndrome
- Increase in mesangial matrix
1) Diffuse glomerulosclerosis
2) Nodular glomerulosclerosis
6
Q
Renal amyloidosis
A
- Cause of nephrotic syndrome
- Subendothelial and mesangial amyloid deposits
1) Primary (AL) amyloidosis - in patients with plasma cell dyscrasias - monoclonal lambda light chains - most common type
2) Secondary (AA) amyloidosis - in patins with long-standing neoplasia or inflammation - serum amyloid A protein
7
Q
Nephritic syndrome
A
- Characterized by inflammatory rupture of the glomerular capillaries - bleeding into the urinary space
- Clinical; Oliguria, azotemia, hypertension, hematuria (leakage of red cells directly from glomerular capillaries into Bowman space - red cell casts)
8
Q
Poststreptococcal glomerulonephritis
A
- Prototype of the nephritic syndrome
- Immune-complex disease - antigen of streptococcal origin - most often group A beta-hemolytic streptococci
- Lab; urinary red cells and red cell casts, azotemia, decreased serum C3, and increased titers of anti-streptococcal antibodies
- Electron-dense “humps” on epithelial side
- “Lumpy-bumpy” immunofluorescence
9
Q
Rapidly pregressive glomerulonephritis (RPGN)
A
- Nephritic syndrome that progresses rapidly to renal failure within weeks or months
- Formation of crescents between the Bowman capsule and the glomerular tuft - result from deposition of fibrin in the Bowman capsule and from proliferation of parietal epithelial cells of the Bowman capsule.
- Etiology: 50% - poststreptococcal - immune complex deposition
Type I: ANCA-negative - antiglomerular BM antibody
Type II: ANCA-negative - immune-complex type
Type III: ANCA-positive - antineutrophil cytoplasmic antibodies
10
Q
Goodpasture syndrome
A
- Cause: antibodies directed against antigens in glomerular and pulmonary alveolar BM
- IgG - linear immunofluorescence
- Clinical: nephritic syndrome, pneumonitis with hemoptysis, hematuria, RPGN crescentic morphology
11
Q
Focal glomerulonephritis
A
- Focal and segmental disease, but differs from focal segmental glomerulosclerosis in that the changes are inflammatory and proliferative rather than sclerotic
- Most often - immune complex disease
12
Q
Alport syndrome
A
- Hereditary nephritis
- Associated with nerve deafness and ocular disorders - lens dislocation and cataracts
- Clinical characteristics: nephritic syndrome progressing to end-stage renal disease by 30y
- Cause: mutation in the gene for the α5 chain of type IV collagen
13
Q
IgA nephropathy (Berger disease)
A
- Deposition of IgA in the mesangium
- Most frequently, the disease is characterized by benign recurrent hematuria in children, usually following an infection - 1 to 2 days
- Can be component of Henoch-Schönlein disease
14
Q
Membranoproliferative glomerulonephritis
A
- Clinical characteristic - slow progression to chronic renal disease
- BM thickening and cellular proliferation
- Reduplication of glomerular basement membrane into two layers - “tram-track” appearance
Type I: immune-complex nephritis - unknown antigen
Type II: dense deposit - IgG antibody with specificity for the C3 convertase
15
Q
Most common pathogen of urinary tract and kidney infection?
A
- Most frequently, the infection involves the normal flora of the colon, most often Escherichia coli
- Most common pathogen in young, sexually active woman is Staphylococcus saprophyticus
16
Q
Acute Pyelonephritis
A
- Infection of the renal parenchyma
- Fever, leukocytosis, flank tenderness, urinary white cells, and white cells casts in the urine
17
Q
Cystitis
A
- Infection of lower urinary tract - bladder
- Pyuria and often hematuria, but unitary white cell casts are not found.
18
Q
Acute tubular necrosis
A
- Most common cause of acute renal failure
- Revesible condition
- Can lead to cardiac standstill from hyperkalemia
- Precipitated by renal ischemia, caused by prolonged hypotension or shock - induced by gram-negative sepsis, trauma, or hemorrhage
19
Q
Fanconi syndrome
A
- Dysfunction of the proximal renal tubules
- May be hereditary or acquired
- Impaired reabsorption of glucose, amino acids, phosphate, and bicarbonate
- Clinical: glycosuria, hyperphosphaturia and hypophosphatemia, aminoaciduria, and systemic acidosis
20
Q
Cystinuria
A
- Impaired tubular reabsorption of cysteine
- Cystine stones
21
Q
Hartnup disease
A
- Impaired tubular reabsorption of tryptophan
- Leads to pellagra-like manifestations
22
Q
Chronic pyelonephritis
A
- Coarse, asymmetric corticomedullary scarring
- Interstitial inflammatory infiltrate –> interstitial fobrosis and tubular atrophy
- Causes: chronic urinary tract obstruction and repeted buts of acute inflammation
23
Q
Urolithiasis
A
- Characterized by calculi (stones) in the urinary tract
A) Calcium stones - most common (80-85%) - calcium oxalate or/both calcium phosphate- Increased intestinal absorption of calcium
- Increased primary excretion of calcium
- Hypercalcemia –> caused by hyperparathyroidism, malignancy, sarcoidosis, vit. D intoxication, or milk-alkali syndrome
B) Ammonium magnesium phosphate stones
C) Uric acid stones
D) Cystine stones
24
Q
Adult polycystic kidney disease
A
- Autosomal dominant inheritance
- Most comma inherited disorder of the kidney
- Partial replacement of renal parenchyma by cysts - bilaterally, enlarged kidneys
- Often associated with berry aneurysm of the circle of Willis
- Clinical: hypertension, hematuria, palpable renal masses, progression to renal failure
25
Q
Benign tumors of the kidney
A
- Adenoma - derived from renal tubules, may be precursor lesion to renal carcinoma
- Angiomyolipoma - hamartoma consisting of fat, smooth muscle, and blood vessels
- Oncocytoma - comprised of cells with abundant granular acidophilic cytoplasm replete with mitochondria
26
Q
Renal cell carcinoma
A
- Most common renal malignancy
- More common in men, higher incidence in cigarette smokers
- Gene deletions in chromosome 3
- Originates in renal tubules, most often arises in upper pole
- Frequently invade renal veins or the vena cava - early hematogenous dissemination
1) Clear cell RCC - most common type
2) Papillary RCC
3) Chromophobe RCC - Triad of flank pain, palpable mass, and hematuria (hematuria as the most frequent presenting sign)
- Fever, secondary polycythemia, ectopic production of carious hormones or hormone-line substances
27
Q
Wilms tumor (nephroblastoma)
A
- Most common renal malignancy of early childhood (peak 2-4 years of age)
- Originates from primitive metanephric tissue
- Palpable flank mass
- Deletions of short arm of chromosome 11. WT-1 and WT-2 genes - cancer suppressor genes.
- WAGR complex - Wilms tumor, Aniridia, Genitourinary malformations, and mental-motor Retardation –> WT-1 gene
- Hemihypertrophy (gross asymmetry of the body)
- Associated with Denys-Drash syndrome
28
Q
Transitional cell carcinoma
A
- Most common tumor of the urinary collecting system - renal calyces, pelvis, ureter, or bladder
- Often multifocal in origion - field effect
- Early sign is hematuria
- Marked tendency to recur after resection
- Tend to spread contiguously
