Humans ; Infant, Newborn ; Male ; Nephrotic Syndrome ; congenital ; Syphilis, Congenital ; complications ; diagnosis ; drug therapy

Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Mycoses ; blood ; diagnosis ; drug therapy ; Nephrotic Syndrome ; blood ; complications ; beta-Glucans ; blood

Adult ; Humans ; Intracranial Thrombosis ; diagnosis ; drug therapy ; etiology ; Male ; Methylprednisolone ; therapeutic use ; Nephrotic Syndrome ; complications ; drug therapy ; Venous Thrombosis ; diagnosis ; drug therapy ; etiology

REVERSIBLE posterior leukoencephalopathy syndrome (RPLS) is a rare neurological syndrome characterized by headache, altered mental status, seizures, and visual disturbance, associated with reversible white matter changes.1 It has been commonly reported in patients with severe hypertension and pre-eclampsia. Here we report a case with nephrotic syndrome complicated by RPLS.
Brain ; diagnostic imaging ; drug effects ; pathology ; Child ; Female ; Humans ; Magnetic Resonance Imaging ; Nephrotic Syndrome ; complications ; diagnosis ; drug therapy ; Posterior Leukoencephalopathy Syndrome ; complications ; diagnosis ; drug therapy ; Tomography, X-Ray Computed ; Treatment Outcome

Nephrotic syndrome is an unusual manifestation of IgA Nephropathy (IgAN). Some cases respond to steroid treatment. Here we describe a case-series of IgAN patients with steroid-responsive nephrotic syndrome. Twelve patients with IgAN with steroidresponsive nephrotic syndrome were evaluated and followed up. All patients presented with generalized edema. Renal insufficiency was found in two patients. The renal biopsy of eight patients revealed wide foot process effacement in addition to the typical features of IgAN. They showed complete remission after steroid therapy. Seven relapses were reported in five patients; six of the relapsed cases responded to steroid therapy. Compared with steroid-non-responsive patients, the patients with steroid-responsive nephrotic syndrome had shorter symptom duration, more weight gain, more proteinuria, and lower histologic grade than did those that had steroid-non-responsive nephrotic syndrome at presentation. None of the responders progressed to end stage renal disease, whereas five (38%) non-responders required dialysis or renal transplantation. Patients with IgAN who have steroid-responsive nephrotic syndrome likely have both minimal change disease and IgAN. The clinical features of sudden onset of generalized edema, initial heavy proteinuria and initial severe hypoalbuminemia might help identify the subset of patients, especially in low grade IgAN.
Adult ; Aged ; Female ; Glomerulonephritis, IGA/complications/*diagnosis ; Humans ; Kidney Transplantation ; Korea ; Male ; Microscopy, Fluorescence/methods ; Middle Aged ; Nephrotic Syndrome/complications/*diagnosis/therapy ; Remission Induction ; Retrospective Studies ; Steroids/*therapeutic use ; Treatment Outcome

Nephrotic syndrome associated with Tsutsugamushi disease has not been previously reported. We are describing a case of Tsutsugamuchi disease presenting with nephrotic syndrome. A 72-year-old woman presented with fever and generalized edema. Laboratory studies revealed a leukocytosis, hypoalbuminemia, and hypercholesterolemia. Her urine protein excretion was 5.4 g/day. The anti-Tsutsugamushi antibody test was strongly positive (1:2,560). A renal biopsy was performed, and pathologic findings revealed membranous glomerulonephritis. The patient's clinical symptoms improved markedly after treatment with doxycycline.
Aged ; Anti-Bacterial Agents/therapeutic use ; Antibodies, Bacterial/blood ; Biopsy ; Doxycycline/therapeutic use ; Female ; Glomerulonephritis, Membranous/diagnosis/*etiology ; Humans ; Nephrotic Syndrome/diagnosis/*etiology ; Orientia tsutsugamushi/immunology ; Scrub Typhus/*complications/diagnosis/drug therapy/microbiology ; Treatment Outcome

Nephrotic syndrome is a rare manifestation of malignancy associated with paraneoplastic syndrome. Paraneoplastic nephrotic syndrome has been reported in various malignancies: malignant lymphoma, colon cancer, lung cancer and prostate cancer. However, an ovarian carcinoma associated with nephrotic syndrome has rarely been reported. Only six cases of ovarian carcinoma associated paraneoplastic nephrotic syndrome has been reported worldwide, but no cases have been reported in Korea. Here, we report a case of paraneoplastic nephrotic syndrome in a patient with an ovarian carcinoma. The patient presented with ascites, proteinuria and hypoalbuminemia. An initial computed tomography (CT) scan and ultrasonography evaluations showed no specific findings suggestive of an ovarian tumor. Despite treatment for nephrotic syndrome, the symptoms became more aggravated. There after, follow up evaluation at Yonsei University Medical Center, including serum CA 125, pelvis MRI and peritoneal fluid examination were performed. On the pelvis MRI, a left ovarian mass was detected with an ascitic fluid collection. The serum CA 125 level was elevated to 2211 U/ml. The peritoneal fluid cytological examination showed malignant cells suggestive of an ovarian carcinoma. Combination chemotherapies including paclitaxel plus carboplatin, topotecan plus gemcitabine and oxaliplatin plus capecitabine were administered to the patient, and complete remission was achieved on image and tumor marker studies. There was complete recovery from the nephrotic syndrome with no evidence of ascites and proteinuria. These findings suggest that nephrotic syndrome caused by paraneoplastic syndrome can be resolved only after the complete control of the underlying malignancy.
Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Carcinoma/*complications/diagnosis/drug therapy ; Female ; Human ; Magnetic Resonance Imaging ; Middle Aged ; Nephrotic Syndrome/*complications/drug therapy ; Ovarian Neoplasms/*complications/diagnosis/drug therapy ; Paraneoplastic Syndromes/*complications/drug therapy ; Remission Induction ; Support, Non-U.S. Gov't ; Tomography, Emission-Computed ; Tomography, X-Ray Computed

Biomarkers ; analysis ; Delayed Diagnosis ; Diagnosis, Differential ; Female ; Humans ; Infant, Newborn ; Liver Diseases ; diagnosis ; drug therapy ; etiology ; Liver Function Tests ; Nephrotic Syndrome ; diagnosis ; drug therapy ; etiology ; Penicillin G ; administration & dosage ; therapeutic use ; Skin Diseases ; diagnosis ; drug therapy ; etiology ; Syphilis, Congenital ; complications ; diagnosis ; drug therapy

OBJECTIVEIdiopathic collapsing glomerulopathy (ICG) is a clinically and pathologically distinct variant of focal segmental glomerulosclerosis, which is characterized by proteinuria (often nephrotic range) and rapid progression to end-stage renal failure. The typical pathological changes are global/segmental glomerular collapse, hypertrophy and hyperplasia of podocytes and severe tubulointerstitial lesions. Most ICG patients who have been reported in previous published papers are adults. ICG in children is rare. The study aimed to analyze and investigate clinical manifestations, renal histopathological findings, treatment and outcomes of ICG in children.

METHODSData of two cases of ICG, a 7-year-old boy and a 12-year-old girl, were analyzed. Both of them were Chinese and Han. Clinical characteristics, results of laboratory tests, renal histopathological findings, treatment, outcomes and prognosis of the two children with ICG were retrospectively analyzed. Results were compared with published data.

RESULTSThese two children presented typical clinical features of nephrotic syndrome. The quantity of 24 hr urine protein was 7.6 g/d (0.47 g/kg x d for boy) and 10.67 g/d (0.35 g/kg x d for girl). Both of them had hypertension (blood pressure ranged from 130/90 to 150/110 mmHg) and hypercholesterolemia (15.4 mmol/L for the boy and 11.3 mmol/L for the girl). The serum albumin was 12 g/L for girl and 23 g/L for boy. The creatinine clearance rate gradually decreased from normal range to 30 ml/min for the girl. The histopathological changes in renal biopsy of them were focal segmental or global glomerular collapse, hypertrophy and hyperplasia of podocytes and severe tubulointerstitial lesions. These two cases were steroid-resistant and were treated with pulse intravenous methylprednisolone and pulse intravenous cyclphosphamade in one case, who rapidly progressed to end-stage renal failure and died half a year later. Another one was treated with cyclosporine. He showed continuous hypertention and heavy proteinuria for eight months.

CONCLUSIONICG in the 2 children was a severe disease which presented steroid-resistant nephrotic syndrome and rapidly progressive renal failure. The pathological characteristics was global/segmental glomerular collapse, hypertrophy and hyperplasia of podocytes and severe tubulointerstitial lesions. In children with ICG treatment was difficult and the prognosis was poor.

Child ; Disease Progression ; Female ; Glomerulosclerosis, Focal Segmental ; complications ; diagnosis ; pathology ; therapy ; Glucosinolates ; Humans ; Kidney ; pathology ; Kidney Failure, Chronic ; etiology ; Kidney Glomerulus ; pathology ; Male ; Nephrotic Syndrome ; etiology ; Proteinuria ; etiology ; Treatment Outcome