Histological patterns of idiopathic steroid resistant nephrotic syndrome in Egyptian children: A single centre study.

BACKGROUND: Idiopathic steroid resistant nephrotic syndrome (ISRNS) represents about 10-20% of children with nephrotic syndrome with variable outcome. OBJECTIVES: To determine the histological patterns of ISRNS in Egyptian children and the histological details of the commonest types which might be the reason for the steroid resistance. PATIENTS AND METHODS: The study included 53 cases with ISRNS. Their renal biopsies were retrieved from the archive of Electron microscopy unit and pathology department, Ain Shams University Specialized Hospital (ASUSH) in the duration from 2005-2011. The biopsies were examined histologically, with immunohistochemistry, and by electron microscopy. RESULTS: They were 36 males (67.9%) and 17 females (32.1%), the age at diagnosis ranged from 1.5- 16 years with a mean of 6.71 years. Lower limb oedema was the commonest presentation (100%), haematuria was revealed in (17%) of cases. Histological examination showed three major patterns; Focal segmental glomerulosclerosis (FSGS) in 30.2%, minimal change glomerulopathy (MCG) in 24.5% and IgA nephropathy in 13.2 %. Mesangial hypercellularity was very common among MCG patients (85.3% ±6.7). Tubulointerstitial inflammation and fibrosis were common among cases with IgA nephropathy (40.4% ±11, 53.7% ±8 respectively). CONCLUSIONS: ISRNS in Egyptian children could be attributed mainly to three major diseases (FSGS, MCG and IgA nephropathy). Mesangial hypercellularity and severe tubulointerstitial disease might be the major causes for steroid resistance in MCG and IgA nephropathy respectively. Renal biopsy with electron microscopy examination should be done for all children with nephrotic syndrome at first time of presentation for proper assignment of treatment protocol.

Concurrent Thin Basement Membrane Disease and Minimal Change Disease: A Case Report / 대한내과학회지

Thin basement membrane disease is defined as diffuse thinning of the glomerular basement membrane, and is clinically characterized by persistent hematuria, minimal proteinuria, normal renal function, and a benign course. It can occur together with other glomerular diseases. We experienced a case of thin basement membrane disease concurrent with minimal-change disease. Treatment with corticosteroid resulted in improved proteinuria and peripheral edema during the follow-up period.

Neurotoxicity Induced by Cefepime in a Patient with Minimal Change Disease / 대한신장학회지

A 71-year-old woman with minimal change disease visited our clinic complaining of pleuritic chest pain. Cefepime was given under the impression that she had pneumonia. Three days after cefepime administration, she became unconscious. A brain MRI scan was non-revealing and an EEG showed triphasic waves. As there was no evidence of septic, uremic or hepatic encephalopathy, we suspected cefepime-induced neurotoxicity. Cefepime was stopped and she underwent hemodialysis to decrease the blood levels of the drug. Following hemodialysis, she regained consciousness.

C1q Nephropathy: A Distinct Pathologic Entity

BACKGROUND: C1q nephropathy (C1qN) is a controversial diagnostic entity defined by Jennette and Hipp in 1985. The prevalence is very low and a few large scale studies have been reported. Application of the criteria for clinical diagnostics of C1qN may cause confusion with other glomerulonephropathies, such as minimal change disease (MCD) or focal segmental glomerulosclerosis (FSGS). In order to clarify the confusion with glomerulonephropathies, we did this study to identify the clinicopathological characteristics and the exact disease entity of C1qN. METHODS: A total of 5,258 kidney biopsies at Kangnam St Mary's Hospital were reviewed. Twenty three cases (0.44%) met the criteria of C1qN. Twenty eight cases showing dominant C1q deposits without electron dense depostis (EDD) grouped as C1q+EDD-, and previously diagnosed typical cases of MCD and FSGS were selected for this study. Four groups were compared to each other with regard to the clinical and pathological aspects of the disease. RESULTS: C1qN patients had an average age of 30.4 years. Eighteen were males and 5 were females. Eighty seven percent had proteinuria and 18% had hematuria. By electron microscopy analysis, 100% had mesangial EDD and 47.8% showed foot process effacement. C1qN had some significant differences compared with C1q+EDD-, MCD and FSGS. CONCLUSIONS: C1qN is clinically and morphologically different from MCD and FSGS. However, additional long term studies are needed to fully define C1qN from other glomerulonephritis with C1q deposits.

Concurrent IgA Nephropathy and Minimal Change Disease in a Patient with Polycythemia Vera: A Case Report / 대한신장학회지

Polycythemia vera is a hematopoietic stem cell disease, characterized by sustained and excessive proliferation of erythrocytic, granurocytic and megakaryocytic cells in the bone marrow resulting in pancytosis in peripheral blood. There have been a few reports of glomerulonephritis with polycythemia vera, most of which were IgA nephropathy. We report a case of a polycythemia vera associated with proteinuria. We confirmed the polycythemia vera according to World Health Organization criteria. Renal pathology showed IgA nephropathy and minimal change disease. Periodic phlebotomy was done and hydroxyurea was administered without specific managements for renal disease. After 3-month treatment, hemoglobin level decreased and proteinurea disappeared.

Nephrotic Syndrome Associated with Thymic Neuroendocrine Tumor; Case Report and Review of Literature / 대한신장학회지

We report a case of nephrotic syndrome with thymic neuroendocrine tumor. A 50-year-old woman was admitted with generalized edema. 24-hour urine collection revealed proteinuria of 20 gram daily and creatinine clearance of 28 mL/min. During examination, a thymic mass was found, which is unresectable due to metastasis to the lung. Biopsy of the thymus and the kidney revealed large cell neuroendocrine carcinoma of thymus origin and minimal change disease, respectively. Despite high dose steroid therapy, massive proteinuria continued for about 30 days. However, when the patient was given a chemotherapy (cyclophosphamide, adriamycin, vincristine), proteinuria began to decrease dramatically. The carcinoma showed the partial response after six cycles of chemotherapy. This is the first report on remission of malignancy-related nephrotic syndrome after chemotherapy, especially for thymic neuroendocrine tumor.