ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED GLOMERULONEPHRITIS IN CHILDREN

被引：12

作者：

NASH, MC ^{[1
]}

JONES, CL ^{[1
]}

WALKER, RG ^{[1
]}

POWELL, HR ^{[1
]}

机构：

[1] ROYAL CHILDRENS HOSP,VICTORIAN PAEDIAT RENAL SERV,PARKVILLE,VIC 3052,AUSTRALIA

来源：

PEDIATRIC NEPHROLOGY | 1993年 / 7卷 / 01期

关键词：

CRESCENTIC GLOMERULONEPHRITIS; ANTINEUTROPHIL CYTOPLASMIC ANTIBODY; MICROSCOPIC POLYARTERITIS;

D O I：

10.1007/BF00861551

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Two cases of anti-neutrophil cytoplasmic antibody (ANCA)-associated necrotizing and crescentic glomerulonephritis are reported. A 12-year-old girl and a 10-year-old boy presented with polyarthritis, anaemia, haematuria, proteinuria, impaired renal function, anorexia, nausea, marked loss of weight and lethargy. The boy also had a vasculitic rash and anterior uveitis. Both children had diffuse cytoplasmic ANCA identified by indirect immunofluorescence and confirmed by specific enzyme-linked immunosorbent assay. Renal biopsies showed severe focal and segmental necrotizing glomerulonephritis with 100% crescents. They were treated with plasma exchange, prednisolone, cyclophosphamide and heparin. Within 1 month of commencing treatment, both had normal serum creatinine concentrations and ANCA was not detectable. Renal biopsies 6 weeks following commencement of treatment revealed quiescent disease, although up to 40% of glomeruli were sclerosed or had fibrous crescents. Following cessation of cyclophosphamide and heparin after 7 months and reduction in steroid dose, a biopsy at 10 months in the boy revealed quiescent disease, but the girl had recurrent disease associated with reappearance of a low titre of ANCA and small cellular crescents in 20% of the glomeruli. These cases reflect the potential usefulness of ANCA determination for categorizing paediatric patients, helping in the selection of therapy and as a possible marker of disease activity, similar to the experience in adults.

引用

页码：11 / 14

页数：4

共 14 条

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