Bartter Syndrome ; complications ; diagnosis ; pathology ; Child, Preschool ; Humans ; Male ; Nephrosis, Lipoid ; complications ; diagnosis ; pathology ; Treatment Outcome

We studied 60 cases of minimal change nephrotic syndrome (MCNS) with mesangial IgA deposits occurring over a 6 year period. There were 43 adults and 17 children. Hematuria occurred in 69.0% of the adults and 88.2% of the children. Two adults and six children had gross hematuria during the course of the disease. Mesangial IgA deposits were noted in 100% of the cases, and concomitant IgG or IgM deposits were found in 78.6% of adults and 73.7% of children. The fluorescent intensity of mesangial IgA deposits was trace (+/-) to 1+ in 86.1% and 70.6% of the adults and children respectively. Most of the patients showed electron microscopic findings consistent with minimal change nephrotic syndrome. We speculate that most of our cases are variants of minimal change nephrotic syndrome but are neither IgA nephropathy nor an overlapping syndrome, and that environmental or genetic factors may be related to the deposition of IgA in these MCNS patients.
Adult ; Child ; Comparative Study ; Female ; Glomerular Mesangium/immunology/*pathology ; Hematuria/etiology ; Human ; Immunoglobulin A/*analysis ; Male ; Nephrosis, Lipoid/complications/immunology/*pathology

The renal glomeruli of 12 male Osborne-Mendel (OM) rats 3 to 24 weeks old were examined by electron microscopy. Effacement of podocyte foot processes (FPs) developed at 3 weeks of age and became progressively worse over time. Loss or dislocation of the slit membrane was also found. Vacuoles and osmiophilic lysosomes appeared in the podocytes starting at 6 weeks of age. Podocyte detachment from the glomerular basement membrane (GBM) was apparent at 18 weeks of age. Laminated GBM was occasionally observed in all animals. These features might lead to the development of spontaneous proteinuria and glomerulosclerosis in OM rats.
Animals ; Animals, Outbred Strains ; Glomerular Basement Membrane/*pathology/ultrastructure ; Kidney Diseases/complications/etiology/*pathology ; Male ; Microscopy, Electron, Transmission ; Nephrosclerosis/etiology/pathology ; Nephrosis/complications/pathology ; Podocytes/*pathology/ultrastructure ; Proteinuria/etiology/pathology ; Rats

C1q nephropathy is a proliferative glomerulopathy with extensive mesangial deposition of C1q. A three-year old boy presented with a nephrotic-range proteinuria during an acute phase of Epstein-Barr virus (EBV) infection, and he had a family history of Dent's disease. The renal biopsy findings were compatible with C1q nephropathy. However, EBV in situ hybridization was negative. The CLCN5 gene analysis revealed an R637X hemizygous mutation, which was the same as that detected in his maternal cousin, the proband of the family. The causal relationship between EBV infection and C1q nephropathy remains to be determined. Moreover, the effects of underlying Dent's disease in the process of C1q nephropathy has to be considered.
Biopsy ; Child, Preschool ; Epstein-Barr Virus Infections/metabolism ; Glomerulonephritis/pathology ; Humans ; In Situ Hybridization ; Infectious Mononucleosis/*complications/*diagnosis ; Kidney Diseases/*complications/*diagnosis ; Kidney Tubules/*pathology ; Male ; Membrane Glycoproteins/*chemistry ; Mutation ; Nephrosis ; Proteinuria/*complications/*diagnosis ; Receptors, Complement/*chemistry ; Treatment Outcome

The World Health Organization classifies lupus nephritis as class I to V or VI. However, a few cases of minimal change glomerulopathy have been reported in association with systemic lupus erythematosus (SLE). Mycophenolate mofetil has been shown to be effective for treatment of minimal change disease and lupus nephritis. A 24-year-old woman diagnosed with SLE five years prior to presentation complained of a mild generalized edema. The urinalysis showed microscopic hematuria and proteinuria. The assessed amount of total proteinuria was 1,618 mg/24 hours. A renal biopsy demonstrated diffuse fusion of the foot processes of podocytes on electron microscopy. Mycophenolate mofetil was started in addition to the maintenance medications of prednisolone 10 mg/day and hydroxychloroquine 400 mg/day. After six months of treatment, the microscopic hematuria and proteinuria resolved, and the total urine protein decreased to 100 mg/24 hours.
Antirheumatic Agents/therapeutic use ; Female ; Glucocorticoids/therapeutic use ; Humans ; Hydroxychloroquine/therapeutic use ; Immunosuppressive Agents/*therapeutic use ; Lupus Erythematosus, Systemic/complications/*pathology ; Mycophenolic Acid/*analogs & derivatives/therapeutic use ; Nephrosis, Lipoid/*drug therapy/etiology/pathology ; Prednisone/therapeutic use ; Young Adult