Idiopathic Retroperitoneal Fibrosis Associated with a Horseshoe Kidney / 대한신장학회지

A case of a 52-year-old man with retroperitoneal fibrosis and a horseshoe kidney is presented. Horseshoe kidney is one of the most common renal anomalies and complicated with urinary tract infection, hydronephrosis, calculi, tumor of the renal pelvis, and other multiple congenital abnormalities. Idiopathic retroperitoneal fibrosis is a rare disease characterized by the presence of a retroperitoneal tissue, consisting of chronic inflammation and marked fibrosis, which often entraps ureters or other abdominal organs. The correlation between horseshoe kidney and retroperitoneal fibrosis has not been described. We report a rare case of idiopathic retroperitoneal fibrosis with horseshoe kidney disease demonstrating good response to steroid therapy.

Perirenal Lymphangioma Combined With Multiple Splenic and Hepatic Cysts / 대한신장학회지

Lymphangioma usually occurs in children and usually involves neck and axillary region. Renal or perirenal cystic lymphangioma, hepatic lymphangiomatosis and splenic lymphangiomatosis are very rare disorders. Perirenal cystic lymphangioma combined with multiple hepatic cysts or multiple splenic cysts suspected to be lymphangiomatosis has not been reported in adults in this country until now. The patient was a 43-year-old woman who had been diagnosed with multiple splenic cysts about ten years ago. She presented with a perirenal cystic lesion discovered incidentally and we detected small multiple hepatic cysts additionally with abdominal CT. We removed perirenal cyst surgically and a perirenal lymphangioma was confirmed.

Renoprotective effect of deflazacort in IgA nephropathy with proteinuria / 대한내과학회지

BACKGROUND/AIMS: Steroid therapy is reported to improve the clinical outcome of IgA nephropathy. In addition, recent studies have revealed that deflazacort has fewer side effects than prednisolone. This study examined the effect of steroids and compared the clinical efficacy of deflazacort and prednisolone in patients with IgA nephropathy. METHODS: We retrospectively reviewed 136 patients with biopsy-proven IgA nephropathy who received deflazacort (n=50), prednisolone (n=29), or neither (n=59), and in whom blood pressure was controlled with angiotensin converting enzyme inhibitors or angiotensin receptor blockers. The mean duration of steroid administration was 9.5+/-9.1 months. The initial clinical status and change in the amount of protein in the 24-hour urine were compared among the three groups. RESULTS: The baseline characteristics (age, blood pressure, serum creatinine level, initial protein in the 24-hour urine, and creatinine clearance) did not differ significantly among the groups. The decrement of protein in the 24-hour urine was higher in the deflazacort and prednisolone groups, as compared with the control group (4.4+/-5.4, 4.2+/-1.5, and 2.1+/-3.1 g/day, respectively, p=0.013). The increment in the creatinine clearance was higher in the deflazacort and prednisolone groups, as compared with the control group (11.5+/-16.4, 12.3+/-26.2, and 4.8+/-14.91.3+/-0.9, respectively, p=0.009). There were no significant differences in the above parameters between the deflazacort and prednisolone groups. CONCLUSIONS: Steroid therapy reduces urinary protein excretion in IgA nephropathy, and the clinical efficacy of deflazacort and prednisolone was found to be similar.