English
| dc.contributor.author | Sozeri, B | |
| dc.contributor.author | Mir, S | |
| dc.contributor.author | Ertan, P | |
| dc.contributor.author | Kara, OD | |
| dc.contributor.author | Sen, S | |
| dc.date.accessioned | 2024-07-18T11:57:41Z | |
| dc.date.available | 2024-07-18T11:57:41Z | |
| dc.description.abstract | BMC | |
| dc.identifier.issn | 1546-0096 | |
| dc.identifier.uri | http://akademikarsiv.cbu.edu.tr:4000/handle/123456789/7094 | |
| dc.language.iso | Article | |
| dc.subject | 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. | |
| dc.title | English | |
| dc.type | PURPURA | |
| dc.type | NEPHRITIS | |
| dc.type | INVOLVEMENT | |
| dc.type | MUTATIONS | |
| dc.type | PATIENT | |
| dc.type | RISK |