childhood polycystic kidney disease
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Pathology
- renal medullary ductal ectasia
- may be associated with hepatic fibrosis
- portal hypertension
Genetics
Clinical manifestations
- presents in childhood
- progressive, severe renal failure at an early age
Complications
- adenomas in 5-10%
- adenocarcinomas in 1.5%
Management
- treatment of hypertension
- dietary salt restriction, DASH diet
- ACE inhibitor or ARB[1]
More general terms
References
- ↑ Jump up to: 1.0 1.1 Medical Knowledge Self Assessment Program (MKSAP) 11, 15, American College of Physicians, Philadelphia 1998, 2009
- ↑ Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 1324