Yang Zhi-xiao, Xiong Hui, Zhang Yue-hua, Bao Xin-Hua, Jiang Yu-wu, Wu Ye, Wang Shuang, Chang Xing-zhi, Qin Jiong, Lin Qing, Wu Xi-ru
Department of Peadiatrics, Peking University First Hospital, Beijing 100034, China.
Beijing Da Xue Xue Bao Yi Xue Ban. 2011 Jun 18;43(3):455-9.
To investigate and analyze the clinical manifestations, classification, therapeutic approaches and follow-up of myasthenia gravis (MG) in children in order to improve its management and prognosis.
Clinical information of 135 children with MG, who were diagnosed between January 1993 to January 2008, were collected and retrospectively analyzed. And prospective following-up of these patients were conducted.
Among the 135 cases, 59 were males and 76 females, giving the ratio of M/F around 1:1.3. Totally, 115 cases (85.2%) were type I MG (ocular type), of which only 4.2% developed to generalized type during the subsequent clinical course. Type II MG (generalized type)was found in 18 cases (13.4%) and type III MG in two cases(1.5%). The onset age ranged from 5 month to 15 years, with 50.3% before three years and 80.7% before seven years. Upper respiratory tract infection was presented in 26.7% (36/135) of the sick children before the onset of MG. Among the 106 children being followed up, recurrence of the disease identified in 50.9% and the number of relapse ranged from 1 to 9. Altogether, 40.19% (43/106) of the cases were positive for anti-acetylcholine receptor antibodies (AchR-Ab) on the initial examination, and the AchR-Ab postitive rate showed no difference among different clinical subtypes and states. However, during the follow-up, 53% (9/17) of the recurrent cases, who were negative at the first onset, turned to be positive, and 37.97% (30/79) were positive for repetitive nerve stimulation in electromyogram test. There were 71 % (45/63) of all the cases showed reduced levels of CD4+ and/or CD3+ and/or CD8+. Thymus proliferation was found in 5.93% (8/135) through CT scan and thymoma in 1.48% (2/135). Steroids and anti-cholinesterase administration were effective in most cases with good prognosis.
Childhood MG, mainly type I, is relatively common in China, with specific characteristics which are different from western patients or adult MG in morbidity, sex distribution, progress, laboratory examination and treatment. The prevalence of myasthenia gravis crisis and mortality rate in MG children is low, and few are accompanied with thymoma. Most MG cases have a satisfied prognosis and few have neuropsychic sequela.
探讨和分析儿童重症肌无力（MG）的临床表现、分类、治疗方法及随访情况，以改善其管理和预后。
收集1993年1月至2008年1月期间确诊的135例儿童MG患者的临床资料，并进行回顾性分析。同时对这些患者进行前瞻性随访。
135例患者中，男性59例，女性76例，男女比例约为1:1.3。其中，115例（85.2%）为I型MG（眼肌型），后续临床过程中仅4.2%发展为全身型。II型MG（全身型）18例（13.4%），III型MG 2例（1.5%）。发病年龄为5个月至15岁，3岁前发病者占50.3%，7岁前发病者占80.7%。26.7%（36/135）的患儿在MG发病前有上呼吸道感染史。在106例接受随访的患儿中，疾病复发率为50.9%，复发次数为1至9次。初诊时，40.19%（43/106）的病例抗乙酰胆碱受体抗体（AchR-Ab）呈阳性，不同临床亚型和状态的AchR-Ab阳性率无差异。然而，随访期间，首次发病时阴性的复发病例中有53%（9/17）转为阳性，肌电图重复神经刺激检查阳性率为37.97%（30/79）。所有病例中71%（45/6）的CD4+和/或CD3+和/或CD8+水平降低。CT扫描发现胸腺增生5.（8/135），胸腺瘤1.48%（2/135）。大多数病例使用类固醇和抗胆碱酯酶药物治疗有效，预后良好。
儿童MG在中国以I型为主，较为常见，在发病率、性别分布、病情进展、实验室检查和治疗等方面具有不同于西方患者或成人MG的特点。儿童MG危象的发生率和死亡率较低，合并胸腺瘤者较少。大多数MG病例预后良好，很少有神经精神后遗症。
