View source for Autosomal dominant polycystic kidney disease

== 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

[[Category:Nephrology]]