, Mi Seung Shin, Duk Hee Kang, Gue Bock Choi, Kyun Il Youn 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.
, Jung Yeon Lee, Su Jin Heo, Yoen Kyung Kee, Seung Hyeok Han Among the possible venous thromboembolic events in nephrotic syndrome, renal vein thrombosis and pulmonary embolism are common, while portal vein thrombosis (PVT) is rare. This report describes a 26-year-old man with histologically proven minimal change disease (MCD) complicated by PVT. The patient presented with epigastric pain and edema. He had been diagnosed with MCD five months earlier and achieved complete remission with corticosteroids, which were discontinued one month before the visit. Full-blown relapsing nephrotic syndrome was evident on laboratory and clinical findings, and an abdominal computed tomography revealed PVT. He immediately received immunosuppressants and anticoagulation therapy. An eight-week treatment resulted in complete remission, and a follow-up abdominal ultrasonography showed disappearance of PVT. In conclusion, PVT is rare and may not be easily diagnosed in patients with nephrotic syndrome suffering from abdominal pain. Early recognition of this rare complication and prompt immunosuppression and anticoagulation therapy are encouraged to avoid a fatal outcome.
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