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.
Acute Kidney Injury* ; Anemia, Hemolytic ; Biopsy ; Dacarbazine ; Eclampsia ; Endothelial Cells ; Female ; Fibrin ; HELLP Syndrome* ; Humans ; Liver ; Perinatal Mortality ; Postpartum Period ; Pre-Eclampsia ; Pregnancy ; Young Adult

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.
Adult* ; Biopsy ; Body Weight ; Creatinine ; Cyclophosphamide ; Cyclosporine ; Diagnosis ; Female ; Follow-Up Studies ; Hematuria ; Humans ; Male ; Nephrosis, Lipoid* ; Nephrotic Syndrome ; Prednisolone ; Prognosis ; Recurrence ; Renal Insufficiency ; Retrospective Studies

Although the exact pathogenetic mechanisms of the renal ischemia-reperfusion injury(I-R injury) have not been clearly elucidated yet, the reactive oxygen spe-cies(ROS) and mononuclear cell infiltration have been suggested to contribute to this renal injury process. The cytokines and growth factors produced by infiltrated leukocytes and renal parenchymal cells could accelerate the process of tissue damage by inducing cellular proliferation, fibrosis, and further recruitment of other inflammatory cells. The renin-angiotensin sys- tem(RAS) also has been suggested to be one of the most important mediators in the renal injury process of many animal models and human renal diseases. In order to evaluate the change of the levels of TNF-alpha, endothelin-l, and TGF-beta gene expression in unilateral I-R injured renal cortical tissue, competitive RT-PCR was performed for the above mRNAs in Sprague-Dawley rats(60 minutes of ischemic time by non-traumatic vessel clamp and 24 hours of reperfusion). Also the plasma renin activity(PRA) and an-giotensin II(AII) level were measured at the time of sacrifice by radioimmunoassay. On the light miscroscopic examination, I-R injured renal cortical tissue showed typical findings of acute tubular necrosis, such as mononuclear ceU infiltration, necrosis, swelling and denudation of proximal tubular cells. Compared to control sham operated group, I-R injured group tissues showed significantiy increased level of TGF-beta(p<0.05), endothelin-l(p<0.05) and TNF-alpha(p<0.05) gene expression in 24 hours after I-R renal injury. The levels of PRA and AIl were also significantly elevated compared to sham group(p< 0.05, p<0.05, respectively). In conclusion, we speculate that the early activation of RAS and elevated gene expression of TNF-alpha, endothelin and TGF-beta of renal cortieal tissue may contribute to the early pathogenetic mechanism of I-R renal injury process.
Animals ; Cell Proliferation ; Cytokines ; Endothelin-1* ; Endothelins ; Fibrosis ; Gene Expression ; Humans ; Intercellular Signaling Peptides and Proteins ; Kidney ; Leukocytes ; Models, Animal ; Necrosis ; Oxygen ; Plasma ; Radioimmunoassay ; Rats* ; Rats, Sprague-Dawley ; Renin ; Renin-Angiotensin System ; Reperfusion Injury ; RNA, Messenger ; Transforming Growth Factor beta* ; Tumor Necrosis Factor-alpha*

Acute cortical necrosis is a rare cause of the acute renal failure. It is frequently associated with complications of pregnancy. The renal biopsy is the key of diagnosis of these disease. However, the contrast enhanced CT scan can be a tool for the diagnosis of acute renal cortical necrosis, because of its noninvasiveness and constant findings(enhancement of subcapsular rim, nonenhancement of the renal cortex, enhancement of medulla, and lack of excretion of contrast media to the collecting system). This is a case of acute renal cortical necrosis diagnosed by CT scan. She had an anuria for 16 days and feature of hemolytic uremic syndrome and polycystic kidney.
Acute Kidney Injury ; Anuria ; Biopsy ; Contrast Media ; Diagnosis ; Hemolytic-Uremic Syndrome ; Humans ; Kidney Cortex Necrosis ; Necrosis* ; Polycystic Kidney Diseases* ; Pregnancy ; Tomography, X-Ray Computed

Thrombosis is one of the important complications of nephrotic syndrome. Still, the mechanism of thrombosis of nephrotic syndrome is under debate, but the presence of hypercoagulable state in nephrotic syndrome is regarded as the principal contributing factor to that. Venous thrombosis in nephrotic syndrome has been increasingly reported, but arterial thrombosis is relatively rare and has been reported mainly in nephrotic children. Authors experienced a 35-year old male patient with frequently relapsing nephrotic syndrome of focal segmental glomerulosclerosis, who complained sudden onset of claudication, cold sensation, numbness and cyanosis of right lower extremity. A diagnosis of thrombosis in right common iliac artery and thromboses in septal and distal branch of left anterior descending coronary artery was made by arterial angiography. The patient fully recovered after emergent thrombectomy and anticoagulation therapy.
Adult ; Angiography ; Child ; Coronary Vessels* ; Cyanosis ; Diagnosis ; Glomerulosclerosis, Focal Segmental ; Heart* ; Humans ; Hypesthesia ; Iliac Artery* ; Lower Extremity ; Male ; Nephrotic Syndrome* ; Sensation ; Thrombectomy ; Thrombosis* ; Venous Thrombosis

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.
Biopsy ; Blood Pressure ; Chungcheongnam-do ; Complement System Proteins ; Creatinine ; Female ; Glomerular Basement Membrane* ; Hematuria ; Hepatitis B Surface Antigens ; Humans ; Immunoglobulins ; Incidence ; Kidney ; Male ; Membranes ; Microscopy ; Proteinuria ; Retrospective Studies ; Serologic Tests