■探讨中枢神经系统(CNS)累及儿童嗜酸性肉芽肿合并多血管炎(EGPA)的临床特点及治疗效果。
■一个因中枢神经系统受累而出现EGPA的儿童于2023年6月入院。回顾性分析其临床特点,并对相关文献进行了综述,对这一状况进行了全面概述。
■一个十岁的女孩,有反复咳嗽和哮喘病史,伴有外周血嗜酸性粒细胞增多八个月,住进了我们的医院.一入场,在她的手和脚上可见斑点丘疹,可听到双侧肺部啰音。实验室检查显示嗜酸性粒细胞(EOS)的比例超过白细胞的10%,抗中性粒细胞胞浆抗体(MPO-ANCA)阳性,免疫球蛋白G水平为15.80g/L,免疫球蛋白E水平大于2500.00IU/mL。影像学检查显示双肺以及鼻窦炎有多个斑片状和结节状高密度阴影。肺功能检查提示中度通气和弥散功能障碍。骨髓细胞学证实嗜酸性粒细胞比例显著增加。皮肤病理证实为白细胞碎裂性血管炎。住院期间,这孩子抽搐了。大脑的磁共振成像(MRI)扫描显示双侧大脑皮层有多个异常信号阴影,脑电图(EEG)显示癫痫波。甲基强的松龙冲击治疗联合环磷酰胺治疗后,她的咳嗽和哮喘缓解了,皮疹消失,没有任何进一步的抽搐。我们发现,以前仅报道了一名年轻的中枢神经系统受累的EGPA患者。先前报告的病例始于长期发烧,减肥,紫癜性皮疹.两名患者对糖皮质激素和环磷酰胺治疗反应良好,他们的临床症状显着改善,外周血嗜酸性粒细胞正常化。
■在儿童中诊断EGPA可能具有挑战性。当孩子受到EGPA的影响时,必须对中枢神经系统受累的迹象保持警惕.糖皮质激素和环磷酰胺治疗可有效控制儿童EGPA。
UNASSIGNED: To explore the clinical characteristics and treatment outcomes of children with central nervous system (CNS) involvement in eosinophilic granulomatosis with polyangiitis (EGPA).
UNASSIGNED: A child who presented with EGPA complicated by CNS involvement was admitted to our hospital in June 2023. The clinical features were analyzed retrospectively, and relevant literatures were reviewed to provide a comprehensive overview of this condition.
UNASSIGNED: A ten-year-old girl, who had a history of recurrent cough and asthma accompanied by peripheral blood eosinophilia for eight months, was admitted to our hospital. On admission, spotted papules were visible on her hands and feet, bilateral pulmonary rales were audible. The laboratory examination revealed that the proportion of eosinophils (EOS) exceeded 10% of white blood cells, the anti-neutrophil cytoplasmic antibody (MPO-ANCA) was positive, the immunoglobulin G level was 15.80g/L, and the immunoglobulin E level was greater than 2500.00IU/mL. The imaging examination showed multiple patchy and nodular high-density shadows in both lungs as well as sinusitis. Pulmonary function tests indicated moderate ventilation and diffusion dysfunction. Bone marrow cytology demonstrated a significant increase in the proportion of eosinophils. Skin pathology confirmed leukocytoclastic vasculitis. During the hospitalization, the child had a convulsion. The magnetic resonance imaging (MRI) scan of the brain showed multiple abnormal signal shadows in the bilateral cerebral cortex and the electroencephalogram (EEG) showed epileptic waves. Following the administration of methylprednisolone pulse therapy in combination with cyclophosphamide treatment, her cough and asthma resolved, the skin rash disappeared without any further convulsions. We found that only a young EGPA patient with CNS involvement had been previously reported. The previously reported case began with long-term fever, weight loss, and purpuric rash. Both patients responded well to treatment with glucocorticoids and cyclophosphamide, experiencing significant improvement in their clinical symptoms and normalization of their peripheral blood eosinophils.
UNASSIGNED: The diagnosis of EGPA in children can be challenging. When a child is affected by EGPA, it is essential to remain vigilant for signs of CNS involvement. The treatment with glucocorticoids and cyclophosphamide is effective in managing EGPA in children.