Sozeri B.Mir S.Ertan P.Kara O.D.Sen S.2024-07-222024-07-22200915460096http://akademikarsiv.cbu.edu.tr:4000/handle/123456789/18719Henoch-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.EnglishAll Open Access; Gold Open Access; Green Open Accessalanine aminotransferasealbuminantinuclear antibodyantistreptolysinaspartate aminotransferaseC reactive proteincalciumcardiolipin antibodycholesterolcolchicinecomplement component C3complement component C4creatininecyclophosphamidedouble stranded DNA antibodyglutamic acidglutaminehemoglobinimmunoglobulinmethioninemethylprednisoloneneutrophil cytoplasmic antibodyprednisolonetriacylglycerolureavalineadolescentarticlebackachecase reportDNA determinationechocardiographyechographyelectrolyte blood levelerythrocyte sedimentation ratefamilial Mediterranean feverfamilial Mediterranean fever genefoot edemagenegene mutationhematuriahemodialysisHenoch Schonlein vasculitisheterozygotehistopathologyhumanhuman tissuehypertensionhypocomplementemiakidney biopsyleg painmagnetic resonance angiographymalemitral valve regurgitationoccult blood testpericardial effusionpriority journalprotein blood levelproteinuriapurpuric rashrapidly progressive glomerulonephritisurinary excretionvasculitisArticle10.1186/1546-0096-7-8