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对通过中小动脉的坏死性血管炎或者血管造影所得的数据诊断为结节性多动脉炎(PAN)的患儿进行分析。要求儿科医生将患儿分为4个提意的儿童PAN 组。全世界21个儿科中心的110例患儿参与了试验。受试女孩和男孩的比率为56:54。其平均年龄为9.05±3.57岁。病例分类如下:33例皮肤PAN,占30%;5例典型的PAN伴有乙肝表面抗原阳性,占4.6%;9例抗中性粒细胞胞浆抗体(ANCA)相关的成人显微镜下多动脉炎,占8.1%;63例系统性PAN,占57.2%。皮肤PAN 是局限在皮肤骨骼肌肉系统中的疾病。所有乙肝表面抗原相关PAN都经过肾血管造影诊断,且大部分病例都进行了抗病毒治疗。显微镜下多动脉炎患儿存在肺和肾病,或两部位中至少一种疾病。87%的患儿存在ANCA, 2例患儿最终发展为终末期肾衰。典型系统性PAN患儿存在多系统损伤,大部分都有全身的症状和急性期反应物含量增加。皮质类固醇和环磷酰胺是免疫治疗的首选药物。总死亡率为1.1%。PAN患儿之间存在着明显的差异,与成人相比,儿童的PAN有不同的临床表现、较高的生存率和较低的复发率。
Analysis of children diagnosed with polyarteritis nodosa (PAN) by data from necrotizing vasculitis or angiography of small and medium arteries. Pediatricians are asked to divide their children into four groups of children who are referred to PAN. 110 children from 21 pediatric centers around the world participated in the trial. The ratio of girls to boys tested was 56:54. The average age is 9.05 ± 3.57 years old. The cases were classified as follows: 33 cases of skin PAN, 30%; 5 cases of typical PAN with hepatitis B surface antigen positive, accounting for 4.6%; 9 cases of anti-neutrophil cytoplasmic antibody (ANCA) related to adult microscopic polyarteritis , Accounting for 8.1%; 63 cases of systemic PAN, accounting for 57.2%. Skin PAN is a disease confined to the skin and skeletal muscle system. All HBsAg-associated PANs are diagnosed by renal angiography and most have been antivirally treated. Polyarteritis under the microscope in children with lung and kidney disease, or at least one of two diseases. Eighty-seven percent of children had ANCA, and two eventually developed end-stage renal failure. There are multiple systemic lesions in a typical systemic PAN and most have systemic symptoms and increased levels of acute phase reactants. Corticosteroids and cyclophosphamide are the drug of choice for immunotherapy. The overall mortality rate was 1.1%. PAN children there is a clear difference between children compared with adults, children with different clinical manifestations of PAN, a higher survival rate and lower recurrence rate.