Rapidly progressive glomerulonephritis in a child with Henoch-Schönlein Vasculitis and familial Mediterranean fever
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Date
2009
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Abstract
Henoch-Schonlein Vasculitis (HSV) is systemic small vessel vasculitis involving the skin, kidney, joints, and gastrointestinal tract. The proportion of patients reported to have renal involvement varies between 20% and 80%. Rapidly progressive glomerulonephritis (RPGN)is rare syndrome in children, characterized by clinical features of glomerulonephritis (GN) and rapid loss of renal function. We present a severe kidney involvement in a 14 year old boy with HSV in who is carring MEFV mutation. A 14 year old boy had developed sudden onset of palpable purpuric rash on his extensor surfaces of lower extremities. He had elevated an erythrocyte sedimentation rate (ESR) (45 mm/h), C-reactive protein (3.74 mg/dl), serum urea 66 mg/dl, serum creatinine 1.8 mg/dl. Also, he had hypocomplementemia. Antinuclear antibody, anti ds DNA, antineutrophil cytoplasmic antibody, anticardiolipine antibodies were negative. Urinalysis revealed macroscopic hematuria and proteinuria with a 24-h urinary protein excretion of 55 mg/m2/h. The renal biopsy specimen showed crescentic and necrotizing glomerulonephritis. He had also M694V/E148Q compound heterozygote mutation. Clinical symptoms and renal failure resolved with intermittant hemodialysis and medical therapy. © 2009 Sozeri et al; licensee BioMed Central Ltd.
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alanine aminotransferase , albumin , antinuclear antibody , antistreptolysin , aspartate aminotransferase , C reactive protein , calcium , cardiolipin antibody , cholesterol , colchicine , complement component C3 , complement component C4 , creatinine , cyclophosphamide , double stranded DNA antibody , glutamic acid , glutamine , hemoglobin , immunoglobulin , methionine , methylprednisolone , neutrophil cytoplasmic antibody , prednisolone , triacylglycerol , urea , valine , adolescent , article , backache , case report , DNA determination , echocardiography , echography , electrolyte blood level , erythrocyte sedimentation rate , familial Mediterranean fever , familial Mediterranean fever gene , foot edema , gene , gene mutation , hematuria , hemodialysis , Henoch Schonlein vasculitis , heterozygote , histopathology , human , human tissue , hypertension , hypocomplementemia , kidney biopsy , leg pain , magnetic resonance angiography , male , mitral valve regurgitation , occult blood test , pericardial effusion , priority journal , protein blood level , proteinuria , purpuric rash , rapidly progressive glomerulonephritis , urinary excretion , vasculitis