Rapidly progressive glomerulonephritis in a child with Henoch-Schönlein Vasculitis and familial Mediterranean fever

dc.contributor.authorSozeri B.
dc.contributor.authorMir S.
dc.contributor.authorErtan P.
dc.contributor.authorKara O.D.
dc.contributor.authorSen S.
dc.date.accessioned2024-07-22T08:21:39Z
dc.date.available2024-07-22T08:21:39Z
dc.date.issued2009
dc.description.abstractHenoch-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.
dc.identifier.DOI-ID10.1186/1546-0096-7-8
dc.identifier.issn15460096
dc.identifier.urihttp://akademikarsiv.cbu.edu.tr:4000/handle/123456789/18719
dc.language.isoEnglish
dc.rightsAll Open Access; Gold Open Access; Green Open Access
dc.subjectalanine aminotransferase
dc.subjectalbumin
dc.subjectantinuclear antibody
dc.subjectantistreptolysin
dc.subjectaspartate aminotransferase
dc.subjectC reactive protein
dc.subjectcalcium
dc.subjectcardiolipin antibody
dc.subjectcholesterol
dc.subjectcolchicine
dc.subjectcomplement component C3
dc.subjectcomplement component C4
dc.subjectcreatinine
dc.subjectcyclophosphamide
dc.subjectdouble stranded DNA antibody
dc.subjectglutamic acid
dc.subjectglutamine
dc.subjecthemoglobin
dc.subjectimmunoglobulin
dc.subjectmethionine
dc.subjectmethylprednisolone
dc.subjectneutrophil cytoplasmic antibody
dc.subjectprednisolone
dc.subjecttriacylglycerol
dc.subjecturea
dc.subjectvaline
dc.subjectadolescent
dc.subjectarticle
dc.subjectbackache
dc.subjectcase report
dc.subjectDNA determination
dc.subjectechocardiography
dc.subjectechography
dc.subjectelectrolyte blood level
dc.subjecterythrocyte sedimentation rate
dc.subjectfamilial Mediterranean fever
dc.subjectfamilial Mediterranean fever gene
dc.subjectfoot edema
dc.subjectgene
dc.subjectgene mutation
dc.subjecthematuria
dc.subjecthemodialysis
dc.subjectHenoch Schonlein vasculitis
dc.subjectheterozygote
dc.subjecthistopathology
dc.subjecthuman
dc.subjecthuman tissue
dc.subjecthypertension
dc.subjecthypocomplementemia
dc.subjectkidney biopsy
dc.subjectleg pain
dc.subjectmagnetic resonance angiography
dc.subjectmale
dc.subjectmitral valve regurgitation
dc.subjectoccult blood test
dc.subjectpericardial effusion
dc.subjectpriority journal
dc.subjectprotein blood level
dc.subjectproteinuria
dc.subjectpurpuric rash
dc.subjectrapidly progressive glomerulonephritis
dc.subjecturinary excretion
dc.subjectvasculitis
dc.titleRapidly progressive glomerulonephritis in a child with Henoch-Schönlein Vasculitis and familial Mediterranean fever
dc.typeArticle

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