Clinical Progression and Complications of Autosomal Dominant Polycystic Kedney Disease in Korea.
- Author:
Kyu Beck LEE
1
;
Hyang KIM
;
Young Rea LEE
;
Sang Kyung CHO
;
Won Yong CHO
;
Hyung Kyu KIM
;
Jin Ho SHIN
;
Young Joo KWON
;
Hee Jung PYO
;
Dae Ryong CHA
;
Yong Seup KIM
;
Sang Eun PARK
;
Sang Youl SEO
Author Information
1. Hemorrhagic fever with renal syndrome, Gastrointestinal tract, Hypoalbuminemia
- Publication Type:Original Article
- MeSH:
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
- From:Korean Journal of Nephrology
1999;18(5):707-713
- CountryRepublic of Korea
- Language:Korean
-
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.
