describe the clinical presentation, etiology, and pathology of autosomal dominant polycystic kidney disease
clinical: gradual onset of renal failure, urinary tract hemorrhage
etiology: defective PKD1 gene on Chomosome 16. encodes polycystin-1
pathology: cysts in liver, aneurysms in the circle of Willis, large kidneys with numerous cysts
describe the clinical aspects and pathology of autosomal recessive polycystic kidney disease
clinical: renal failure develops from infancy to several years of age
pathology: cycsts and progressive liver fibrosis, cysts in collecting tubules in the cortex and medulla of the kidney
list 2 mechanisms of glomerular injury
1. immune complex deposits in glomerular BM or mesangium
2. epi/endothelial cell injury
list 3 types of pathologic evaluations of kidney biopsies
1. light microscopy
3. electron microscopy
list 5 aspects of nephrotic syndrome
1. minimal change disease
2. membranous glomerulonephritis
3. focal segmental glomerulosclerosis
4. mebranoproliferative glomerulonephritis
5. glomerular disease in diabetes mellitus
describe minimal change disease in terms of nephrotic syndrome
- most common cause of nephrotic syndrome in children.
- electron microscopy demostrates effacement of epithelial cell foot processes
describe membranous glomerulonephritis in terms of nephrotic syndrome
- most common ages 30-50
- can be primary or secondary
- immune complexes in the epithelial side of the GBM are shown via immunofluorescence and electron microcscopy
describe focal segmental glomerulosclerosis in terms of nephrotic syndrome
- one of the most common causes of nephrotic syndrome in adults
- primary (idiopathic) or secondary
- partial sclerosis of some golmeruli characerized by increased mesangial matrix collagen with obliteration of capillary loops. idiopathic form has no immune complexes.
describe membranoproliferative glomerulonephiritis in terms of nephrotic syndrome
immune complex deposition and proliferation of mesangial and endothelial cells
describe glomerular disease in diabetes mellitus in terms of nephrotic syndrome
- minimal proteinuria progresses, over 10-15 years, to severe proteinuria
- thick GBM, increase in mesangial matrix and formation of mesangial nodules
describe nephritic syndrome
- most commonly occurs in children following a streptococcal infection
- proliferation of endothelial and mesangial cells; immune complexes in GBM
- progression to chronic renal disease in adults most likely
list 2 aspects of hematuria
1. IgA nephropathy (Berger disease)
2. hereditary nephritis
describe IgA nephropathy (Berger Disease)
- usually in children/young adults
- worse after upper respiratory tract viral infection
- pathogenesis: increased IgA production
- pathology: mesangial deposition of immune complexes containing IgA and variable proliferation of mesangial and endothelial cells.
what is Henoch-Schonlein syndrome?
when the glomerular disease is associated with systemic manifestations like a skin rash and arthritis
describe hereditary nephritis
mutation in a gene that codes for the alpha chain of type IV collagen resulting in an abnormal BM
define crescentic or rapidly progressive glomerulonephritis
antibodies directed against a glomerular basement antigen, deposition of immune complexes or lack of immune complex deposition
describe end stage renal disease
loss of glomeruli and tubules leads to fibrosis. damaged glomeruli become completely sclerotic (global sclerosis). leads to progressive renal failure.
describe acute pyelonephritis
renal disease affecting tubules, interstitium and pelvis and is most often secondary to bacterial infection. the infection may spread from the urinary bladder up the ureters and into the renal pelvis and kidneys.
describe the clincal, pathology and etiology of acute pyelonephritis
clinical: sudden onset with pain at the costovertebral angle and systemic evidence of infection. dysuria, frequency and urgency
pathology: patchy interstitial and tubular neutrophilic inflammation
etiology: UT obstruction, vesicoureteral reflux, diabetes mellitus and immunosupression
describe drug-induced interstitial nephritis
etiology: antibiotics, NSAIDs
pathogenesis: hypersensitivity reaction to drugs
pathology: interstitial infiltration of mononuclear inflammatory cells, neutrophils and eosinophils. granulomas may be present. glomeruli are not involved in the inflammation. large doeses of analgesics may lead to interstitial nephritis with papillary necrosis
describe the clinical, etiology and pathology of acute tubular necrosis
clinical: renail failure, reduced urine output, electrolyte imbalance. reversible over a period of weeks.
etiology: ischemia or toxin
pathology: dilation of tubules, interstitial edema, necrosis of epithelium
describe clinical/pathology/hisopathology of benign nephrosclerosis
clinical: hypertension and diabetes. gradual onset of chronic renal failure
pathology: reduction in size, cortex is thin
histopath: narrowing of the lumens of arterioles and arteries by hyaline type arteriolosclerosis and fibroelastic hyperplasia of muscular arteries. also tubular atrophy, interstitial fibrosis and global sclerosis of glomeruli
describe malignant nephrosclerosis
rapid onset of renal failure. arterioles show hyperplastic arterioloslcerosis
list/define two types of thrombotic microangiopathies
1. thrombotic thrombocytopenia purpura (TTP): acquired defect in a protease enzyme that degrades vWF. mostly widespread involvement.
2. Hemolytic-uremic syndrome (HUS): endothelial cell injury due to shiga-toxin from e. coli or shigella. leads to platelet activation. predominantly kidney involvement.
- both disorders have microthrombus formation in capillaries.
describe the clinical/pathology and types of renal stones (urolithiasis)
clinical: UT obstruction, ulceration of urogenital lining and bleeding. small stones go to ureters while large stones remain in the pelvis and cause hematuria and infection
pathology: 80% are unilateral. most frequent site of formation is within calyces and pelvis. also in the bladder. large stones called "staghorn calculi"
etiology: risk factors are smoking, hypertension, obesity, cadmium exposure and von Hippel-Lindau syndrome
pathology: arise from tubular epithelium and reach a large size prior to diagnosis, often invades the renal vein
describe the clincal/pathology of Wilm's Tumor
clinical: abdominal mass, children aged 2-5
pathology: triphasic pattern with epithelial structures resembling primitive tubules or glomeruli, stroma and blastema. illustrates abortive formation of renal structures in various stages of renal development.
describe the clinical/pathology of tumors of the bladder and collecting system
clinical: most common tumor is transitional cell carcinoma. dominant manifestation is painless hematuria. predisposing factors are smoking, chronic cystitis, infection with Schistosomiasis, and exposure to beta-napthylene.
pathology: papillary growth pattern and resembles transitional epithelium