小儿反复性发热性非化脓性结节性脂膜炎(附6例报告)  

Recurrent Nodular Nonsuppurative Panniculitis of Children——Report of 6 Cases

作　　者：张巧芳[1] 路雪英[1] 李秀英[1] 

机构地区：[1]天津市儿童医院,300074

出　　处：《天津医药》1995年第10期590-592,共3页Tianjin Medical Journal

摘　　要：结节性脂膜炎为脂肪组织自身免疫性疾病,罕见但预后严重。1979～1991年我院收治6例,随诊8～14年,死亡、存活各3例。存活3例中治愈1例,反复发作持续用药1例,伴发糖尿病1例。临床特征为持续高热,分批出现皮下结节,高脂蛋白血症。监测3例免疫功能表现不一,例4 IgG正常、例5 IgG、IgM增高(IgG145g/L、IgM1360 mg/L)及CD_3、CD_4(％)减低(CD_336％、CD_413.3％),例6 IgG、IgM增高(IgG111g/L,IgM1060mg/L)。因发病初期表现复杂需与相应疾病鉴别。本文6例均做皮下结节活检确诊。治疗首选类固醇激素,无效时选用环磷酰胺、氯喹、保泰松、环胞霉素A等。Nodular pannicusis is an adipose autoimmune disease ,which is rare and whose prognosis is poor- From 1979 to 1991,six cases were studied with 8 to 14 years of follow-up. Three cases were died and three cases survived;one was cured.one with recurrent attacks of subcutaneous nodules on any part of the body and hy-perlipidemia- Immunoglobulins and T cell changes:case 4 had normal .serum leveis of IgG,case 5 had high serum leveis of IgG and IgMGgG 145g/L,IgM l 360mg/L)and low CD3 and CD4(CD3 36. 0% ,CD4 13. 3%), case 6 had high serum levels of IgG and I'gM(IgG 111g/L,IgM 1060mg/L), During mitial phase its climcal manifestations were complicated. In order to differentiate the disease from other similar diseases.the diagnosis of six cases were deterimned by biopsies of subcutaneous nodules. In therapy .corticosteruids are recommend-ed as the first choice drug.other therapeutic agents used for patient who failed 10 respond to steroids indude cyclophosphamide.ch!oroquineTphenylbutazone and cyclosporin A.

关 键 词：儿童 结节性脂膜炎 脂膜炎 自身免疫性 发热性 

分 类 号：R725.939[医药卫生—儿科]