A Case of HELLP Syndrome Combined with Acute Renal Failure / 대한신장학회잡지

The HELLP syndrome(hemolysis, elevated liver enzymes, and low platelets) is a complication of severe preeclampsia or eclampsia and associated with increased maternal and perinatal mortality. Exact pathogenic mechanism is still unclear but the microangiopathic hemolytic anemia combined with endothelial cell damage is suspected to be closely associated with disease process. Variable degree of disseminated intravascular coagulation(DIC) may be accompanied with the HELLP syndrome. We report a case of postpartum HELLP syndrome. She is 24 year-old woman. She showed definite evidence of microangiopathic hemolytic anemia, DIC, elevated liver enzymes and acute renal failure. Liver biopsy revealed fatty change with focal fibrin like material deposition in the portal tract area. She recovered completely with conservative treatment in 2 weeks.

Clinical Characteristics of Minimal Change Nephrotic Syndrome in Adults / 대한신장학회잡지

We evaluated retrospectively the clinical characteristics of 26 adult nephrotic syndrome patients with minimal-change disease who were followed up for more than six months. Median follow up period was 23months. They consisted of 16 men and 10 women, and the mean age was 34.3 years (range 18-68 years). We administered prednisolone 1.0mg/Kg body weight to the patients for eight weeks and tapered it gradually. Complete remission was obtained in 22 (85%) patients treated with prednisolone and 54% of them reached complete remission in four weeks after beginning prednisolone treatment. Nine patients who initially responded to prednisolone therapy relapsed. Two of these nine patients showed single relapse and seven patients had frequent relapses during the study period. Cyclophosphamide or cyclosporin A was added when the patients did not respond to prednisolone and showed frequent relapse. Of the six patients treated with cyclophosphamide, four patients who were frequent relapsors and one patient who was a non-reponder to prednisolone went into complete remission. The other prednisolone non-responder did not remit. Microscopic hematuria was found in three patients and these patients showed elevated serum creatinine level at the time of diagnosis. Seven patients showed renal impairment at the time of renal biopsy. All of them recovered from renal failure after treatment. Six patients showed complete remission of nephrotic syndrome after prednisolone and/or cyclophosphamide treatment. There was no patient who showed a deterioration of renal function during the study period. In conclusion, complete remission was obtained in 85% of adult minimal change nephrotic syndrome patients and 41% of patients relapsed within one year after attaining complete remission. The serum level of creatinine and microscopic hematuria at the time of diagnosis were not associated with poor prognosis during the study period.

The Clinicopathological Characteristics of Thin Glomerular Basement Membrane Nephropathy / 대한신장학회잡지

Thin glomerular basement membrane nephropathy, also called benign recurrent hematuria, is characterized by diffuse thinning of the glomerular basement membrane and by hematuria. The present study was based on a retrospective review of 366 native kidney biopsies performed at Chungnam National University Hospital from January 1993 to November 1996. Fifteen of these cases satisfied the criteria for definition of thin glomerular basement membrane nephropathy, placing the incidence of the disease at 4.0%. The criteria for definition of the disease are 1) normal findings by light microscopy, 2) no deposits by immunofluorescent microscopy, and 3) diffuse thinning of the glomerular basement membrane by electronmicroscopy. The studied patients had a mean age of 35.8 years (range from 14 to 59) and included 12 females and 3 males. A positive family history was present in one case. All pateints were normotensive and had hematuria (four cases showed gross hematuria). Seven patients had mild proteinuria, but two patients showed proteinuria of nephrotic range. The serum creatinine level was within normal limits except one. Abnormalities were not observed in immunoglobulins, complement component, serologic tests (RA, ASO) and HBsAg. Renal biopsy findings by light and immunofluoroscent microscopy did not reveal any abnormalities but diffuse thinning of the glomerular basement membrane (range from 154 to 279nm) was observed by electronmicroscopy. With the above results, patients with idiopathic renal hematuria with normal renal function and normal blood pressure, thin glomerular basement membrane nephropathy should be considered.