Korean J Nephrol.  2001 Mar;20(2):212-220.

Clinical Characteristics of End-stage Renal Disease in Korean Autosomal Dominant Polycystic Kidney Disease

• Hwang YH
• Ahn C
• Hwang DY
• Lee EJ
• Eo HS
• Chae HJ
• Lee JE
• Kim YS
• Han JS
• Kim S
• Lee JS
• Lee JG
• Cho JT

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 2% of hemodialysis 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 < OR =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 > OR =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.

Keyword

Polycystic kidney disease; End stage renal disease; Renal progression

MeSH Terms

Age of Onset
Asian Continental Ancestry Group
Azotemia
Creatinine
Diagnosis
Female
Hematuria
Humans
Hypertension
Kidney Failure, Chronic*
Korea
Liver
Male
Polycystic Kidney Diseases
Polycystic Kidney, Autosomal Dominant*
Prevalence
Prognosis
Proteinuria
Renal Dialysis
Renal Insufficiency
Renal Replacement Therapy
Risk Factors
Urinary Tract Infections
Urolithiasis
Creatinine