The adult polycystic kidney disease(APCK) is inheritant disease in which cysts aredistributed throught the cortex and medulla of both kidneys. The disease process is usually notclinically apparent until the third or fourh decade of life. Mild persistent proteinuria(> 200mg/d) can be developed in 20% to 40% of cases, but nephrotic-range proteinuria is rare and whenfound, should prompt a search for the other concomitant renal disorder, such as IgA nephropathy, rapidly progressive glomerulonephropathy, focal glomerulosclerosis.

We report a case of APCK presented with nephrotic syndrome. A 76-year-old man was admitted due to generalized edema with weakness. The blood pressure was120/80mmHg. The BUN/creatinine were 15/1.2mg/dl, serum albumin 2.9g/dl, cholesterol / triglyceride 432/273mg/dl, total urinary protein excretion 8.41g/day.

The serum level of IgG, IgAS, IgM, C₃, C₄ were within normal range and anti-nuclear antibody and anti-double strand DNA antibody were also negative. The abdominal ultrasonography and computerized tomography revealed multiple large cysts in both kidneys.The patient was treated with Enalapril®, an angiotensin converting enzyme inhibitors, 5mg forcontrol of proteinuria.

The APCK presented with nephrotic syndrome is rare and to our knowledge,this is a first report in Korea.

Immunoglobulin A nephropathy(IgA nephropathy) Is one of the most prevalent glomerulonephritis in Korea, and nearly one third of them progress to end stage renal disease(ESR:D) over 20 to 30 years. The exact pathogenesis and therapeutic modality to inhibit theprogress of IgA nephropathy into BSRD are still uncertain in spite of lots of reports on beneficial effects of several therapeutic strategy. The present study was undertaken to know the incidence of IgA nephropathy, the mode of presentation, the characteristic pathologic findingsand the course of disease with the possible prognostic factor.

I reviewed the medical records including the pathologic reports of 37 cases of IgA nephropathy who performed renal biopsy between Jan. 1988 and Oct. 1995. The initialpresenting sypmtoms and laboratory finding, pathologic characteristic and follow-up data werealso investigated with the relationship between the initial laboratory or pathologic findings andthe deterioration of renal function.

The incidence of IgA nephropathy was 16.5%. IgA nephropathy was more prevalent in male in their 3rd decade. Gross hematuria (27%) and microscopic hematuria with significant proteinuria (24%) were the most common clinical symptoms/signs. The incidence ofneprotic syndrome among IgA nephropathy was 22%. The amount of proteinuria in total 37 subjects was 3.5±4.9g/day. Mesangial expansion (41%) and hypercellularity (41%) were themost common light microscopic finding. We couldn't find any statistically significant differencein initial blood pressure, serum creatinine and proteinuria according to the extent of mesangial IgA deposition. With the follow-up of mean duration of 22.4±0.8 months, serum creatinine increased significantly with the development of ESRD in 3 cases of subjects. These cases of ESRD all presented nephrotic syndrome initially, and did not respond to steroid therapy. The pathologic findings in 2 of them were global glomerular sclerosis and crescent formation.

IgA nephropathy is no longer the unusual and benign disease. Further prospective, controlled study is necessary to know which is the best therapeutic modality to inhibitor slow-down the progression of IgA nephropathy.