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Acquired Cystic Kidney Disease

Acquired Cystic Kidney Disease - Acquired Cystic Kidney...

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Acquired Cystic Kidney Disease September 21, 2010 Jun-Ki Park
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1977 Mike Dunnill from Oxford first identified the presence of renal tumors in a new form of cystic kidney disease occuring in pts on long term intermittent hemodialysis. Systemic autopsy study of 30 HD patients. 14 had extensive renal cysts 6 of them had renal carcinomal 1 with metastatic disease.
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Diagnostic Criteria Acquired cystic kidney disease Minimum of 3 cysts Renal failure No cysts before onset of renal failure No family history or clinical features of other renal cystic disease Kidney usually small or normal size Extensive chronic renal damage
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Incidence Matson 1990 : In 130 pts with advanced CKD or ESRD 7% ACKD in CKD and 22% in dialysis pts. Duration of dialysis: 28 months in those with 1 3cysts, 49 months in those with ACKD. 50 80% of pts may be affected after >10yrs of dialysis. Kojima 2006 : In2624 dialysis patients 81.8% had ACKD on a median dialysis time of 11yrs. 44pts (1.68%) showed evidence of renal carcinoma.
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Multiple and bilateral cysts Usually less than 0.5cm but can be as large as 2 3cm Cysts are located only in the kidney Can begin prior to dialysis (occurrence in pts with CrCl as high as 70ml/min, however majority in pts w/ CrCl 50ml/min) Men and blacks are at much higher risk Features of ACKD
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Pathogenesis Grantham 1991
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In rats with normal kidneys diphenylamine, diphenylthiazole , or nordihydroguaiacetic acid causes damage of renal epithelium and leads to focal tubule dilatation and expansion. (Welling 1985) Cyst formation can be reversed by stopping chemical exposure.
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