Korean J Nephrol.  1999 Sep;18(5):707-713.

Clinical Progression and Complications of Autosomal Dominant Polycystic Kedney Disease in Korea

• Lee KB
• Kim H
• Lee YR
• Cho SK
• Cho WY
• Kim HK
• Shin JH
• Kwon YJ
• Pyo HJ
• Cha DR
• Kim YS
• Park SE
• Seo SY

Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is one of most common hereditary disorders that is potentially fatal. To elucidate clinical and genetic characteristics of ADPKD in Korea, 166 patients were analyzed retrospectively. The results were as follows ; 1) The male-to-female ratio was 83: 83, and age of patients was 4813(meanSD) years. 6496 of patients have a family history of the ADPKD. 2) Most common symptom was flank and ab- dominal pain. In urinalysis, proteinuria(42%), hematuria(18%) were found. Other organ involvement included hepatic cysts(58%), pancreatic cysts(8%) and splenic cyst(296). 3) The age at diagnosis was 44% 12 years. Hypertension was observed in 65%, the age of detection was 45% 11 years. Azotemia was observed in 36%, the age of detection was 52 +/- 11 years. 4) Echocardiography showed left ventricular hypertrophy 5896(19/33), valve regurgitation 1596(5/33) and mitral valve prolapse 3%(1/33). Cerebrovascular events occurred in 14 patients(8.4%), and 6 patients confirmed cerebral aneurysm rupture. 5) 29 patients(17%) reached end stage renal fail- ure(ESRD), the age was 53 +/- 10 years. The provability of being alive and not having ESRD was 88% by age 50, 76% by age 58, and 48% by age 65. Sex and hepatic cyst were not associated significantly with the course of renal function(p>0.05). 6) 15 patients(9%) died, the age of death was 55 +/- 12 years. 6 patients died after reaching ESRD. The cause of death were was cancer(4), cerebrovascular event(3), cessation of dialysis(2), sudden death (2), liver cirrhosis(1), sepsis(1), suicide(1) and unknown(1).
Results
of our study revealed the complications of ADPKD in Korea, hypertension 6596, ESRD 17% and cerebrovascular event 8%. Functional survival was 88M by age 50, 76% by age 58, and 48M by age 65. The extrarenal manifestations are more important contributors to mortality than renal manifestations of ADPKD.

MeSH Terms

Azotemia
Cause of Death
Death, Sudden
Diagnosis
Echocardiography
Humans
Hypertension
Hypertrophy, Left Ventricular
Intracranial Aneurysm
Kidney Failure, Chronic
Korea*
Liver
Mitral Valve Prolapse
Mortality
Polycystic Kidney, Autosomal Dominant
Retrospective Studies
Rupture
Urinalysis