Renal Diseases

  1. define azotemia
    elevation of the blood urea nitrogen and creatine levels, due to decreased filtration of blood through the glomeruli
  2. define uremia
    association of azotemia with clinical signs and symptoms (gastroenteritis, pericarditis, dermatitis, hyperkalemia, acidosis)
  3. list/define 3 major clinical renal syndromes
    • 1. acute nephritic syndrome: acute onset of hematuria, mild to moderate proteinuria and hypertension
    • 2. nephrotic syndrome: heavy proteinuria (>3.5g/day), hypoalbuminemia, edema, hyperlipidemia and lipiduria.
    • 3. acute renal failure: acute onset of azotemia with oliguria or anuria
  4. list 2 congenital cystic renal diseases
    • 1. autosomal dominant polycystic kidney disease (adult)
    • 2. autosomal recessive polycystic kidney disease (childhood)
  5. 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
  6. 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
  7. list 2 mechanisms of glomerular injury
    • 1. immune complex deposits in glomerular BM or mesangium
    • 2. epi/endothelial cell injury
  8. list 3 types of pathologic evaluations of kidney biopsies
    • 1. light microscopy
    • 2. immunofluorescence
    • 3. electron microscopy
  9. 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
  10. 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
  11. 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
  12. 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.
  13. describe membranoproliferative glomerulonephiritis in terms of nephrotic syndrome
    immune complex deposition and proliferation of mesangial and endothelial cells
  14. 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
  15. 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
  16. list 2 aspects of hematuria
    • 1. IgA nephropathy (Berger disease)
    • 2. hereditary nephritis
  17. 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.
  18. what is Henoch-Schonlein syndrome?
    when the glomerular disease is associated with systemic manifestations like a skin rash and arthritis
  19. describe hereditary nephritis
    mutation in a gene that codes for the alpha chain of type IV collagen resulting in an abnormal BM
  20. define crescentic or rapidly progressive glomerulonephritis
    antibodies directed against a glomerular basement antigen, deposition of immune complexes or lack of immune complex deposition
  21. 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.
  22. 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.
  23. 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
  24. 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
  25. 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
  26. 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
  27. describe malignant nephrosclerosis
    rapid onset of renal failure. arterioles show hyperplastic arterioloslcerosis
  28. 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.
  29. 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"
    • types of stones: calcium, magnesium ammonium phosphate (persistent alkaline urine), uric acid (persistent acidic urine).
  30. describe the clinical/etiology and pathology of renal cell carcinomas
    • clinical: hematuria, polycythemia, paraneoplastic syndromes
    • 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
  31. 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.
  32. 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
Card Set
Renal Diseases
renal diseases