Rapidly progressive glomerulonephritis in a child with Henoch-Schönlein Vasculitis and familial Mediterranean fever
| dc.contributor.author | Sozeri B. | |
| dc.contributor.author | Mir S. | |
| dc.contributor.author | Ertan P. | |
| dc.contributor.author | Kara O.D. | |
| dc.contributor.author | Sen S. | |
| dc.date.accessioned | 2025-04-10T11:16:06Z | |
| dc.date.available | 2025-04-10T11:16:06Z | |
| dc.date.issued | 2009 | |
| dc.description.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. | |
| dc.identifier.DOI-ID | 10.1186/1546-0096-7-8 | |
| dc.identifier.uri | http://hdl.handle.net/20.500.14701/51544 | |
| dc.title | Rapidly progressive glomerulonephritis in a child with Henoch-Schönlein Vasculitis and familial Mediterranean fever | |
| dc.type | Article |