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Pediatric Just The Facts - part 8

Thay đổi bệnh tối thiểu (lipoid nephrosis) tiêu cự đoạn glomerulosclerosis Mesangial IgM bệnh thận Diffuse mesangial hội chứng bẩm sinh phổ biến hạt nhân thận hư Membranoproliferative viêm cầu thận màng bệnh thận HIV bệnh thận | 442 SECTION 15 DISEASES OF THE KIDNEY URETERS AND BLADDER TABLE 119-1 Glomerular Disorders That Usually Present with Nephrotic Syndrome Minimal change disease lipoid nephrosis Focal segmental glomerulosclerosis Mesangial IgM nephropathy Diffuse mesangial proliferation Congenital nephrotic syndrome Membranoproliferative glomerulonephritis Membranous nephropathy HIV nephropathy ages 1 and 8. Kidney function blood pressure and C3 complement levels are normal. Microscopic hematuria may be noted. Response to corticosteroids occurs within 3 weeks in the majority of children who then may continue a relapsing remitting course for 5-10 years usually followed by a permanent remission. Potentially serious complications include infections peritonitis pneumonia bacteremia and meningitis as well as vascular thromboses. For children with frequent relapses 20-25 of cases or the minority who do not respond to steroids other medications that may be useful include cyclophosphamide cyclosporine tacrolimus and mycophenolate mofetil. The cause of minimal change nephrotic syndrome is unknown. Focal segmental glomerular sclerosis FSGS is the second most common glomerular disorder with nephrotic syndrome. Patients with FSGS rarely respond to steroids but may improve with cyclosporine tacrolimus cyclophosphamide or mycophenolate mofetil. Untreated most children progress from having normal kidney function to end-stage kidney disease and require transplantation. Unfortunately the condition may recur after transplantation as well but unlike the disease in native kidneys it may remit after a course of intensive plasmapheresis. Membranoproliferative glomerulonephritis MPGN and membranous glomerulonephropathy MGN often present with nephrotic-range proteinuria often with overt nephrotic syndrome. MPGN may present with or without hematuria and is usually accompanied by profound hypocomplementemia. There are three subtypes of MPGN each distinguished by kidney biopsy. Treatment with alternate day .