Korean Journal of Nephrology 2001;20(2):212-220.

원저 : 말기 신부전으로 진행하는 한국인 상염색체우성 다낭신 환자의 임상적 특성 (Clinical Characteristics of End-stage Renal Disease in Korean Autosomal Dominant Polycystic Kidney Disease)

황영환(Young Hwan Hwang),안규리(Cu Rie Ahn),황대연(Dae Yeon Hwang),이은주(Eun Joo Lee),어현선(Hyun Seon Eo),채희진(Hee Jean Chae),이정은(Jung Eun Lee),김연수(Yon Su Kim),한진석(Jin Suk Han),김성권(Suhng Gwon Kim)

Abstract

End stage renal disease(ESRD) is a well-known major complication of autosomal polycystic kidney disease(ADPKD). Several risk factors of renal progression in ADPKD were identified, such as PKD1 gene, male gender and earlier age of onset. In Korea, ADPKD is a cause of ESRD in 2Yo of hemodi- alysis patients. Until now, only a few detailed studies have been performed in regarding to evaluate the risk factor for ESRD especially in the Asian population. 148 ADPKD patients were registered to PKD clinic in our hospital(Mar. 1996-Dec. 1999). Among them, 34 patients(male : female 14: 20) who had started renal replacement therapy were studied to elucidate clinical characteristics including the nature of progression of renal failure. These data were compared with 14 patients(male : female 3: 11) who did not develop renal failure(serum creatinine ≤1.4 mg/dL) at the age of 50 years. Median age at the diagnosis of ADPKD was 43 years(range : 22-65 years), median age at initiation of renal replacement therapy(RRT) was 52.5 years(28-73) and median duration from the diagnosis to RRT were 6 years(0-30). The prevalence of gross hematuria, proteinuria (>1g/24h), urolithiasis, upper urinary tract infection, hypertension and liver cysts were 69, 54, 16, 29, 85 % and 85%, respectively. 84% of these patients had family members with ADPKD and 10% of them had ESRD family members. PKD1 vs. PKD2 was 7: 1 in 8 patients with ESRD and 1: 1 in 2 patients of control group. Gross hematuria and proteinuria were more prevalent in ESRD patients than the control group(p=0.001 and p=0.0008, respectively). In 18 patients with ESRD, rates of renal progression were traced using a reciprocal of serum creatinine(1/Cr) curve. Once azotemia(serum creatinine value ≥1.5 mg/dL) developed, the median rate of decline of 1/Cr was -0.073dL/mg/year(range : -0.046--0.114dL/mg/ year), which was constant irrespective of either the age of onset or sex. In summary, in 34 patients, the renal function seemed to be maintained to a certain age. But, once azotemia developed, the renal function was rapidly declining with similar rate, ended up ESRD in 8.2 years. Presence of gross hematuria and proteinuria were associated with poor prognosis.