Autosomal dominant polycystic kidney disease
From IDWiki
Classification
- Autosomal dominant PKD (ADPKD): presents in adulthood; family history
- Autosomal recessive PKD (ARPKD): presents in childhood; severe
Clinical Presenation
- Flank pain from renal hemorrhage, calculi, or UTI
- Hypertension and hematuria
- CKD, usually starting in the 4th decade
- Risk factors for progressive CKD include hypertension, early onset of symptoms, male gender, and increased renal size
Extrarenal complications
- Cerebral aneurysms (5-20%), hepatic and pancreatic cysts, cardiac valve disease, colonic diverticula, abdominal wall and inguinal hernias and seminal vesicle cysts
Management
CKD
- Manage risk factors, especially hypertension
- ACEi/ARB for hypertension and to slow CKD (especially if proteinuric)
- Eventually needs hemodialysis or renal transplant
Extrarenal Complications
- Needs screening for cerebral aneurysms
