Descamps H, Bataille J, Estournet B, Barois A
Service de Réanimation Infantile, Rééducation Neuro-Respiratoire, Hôpital Raymond-Poincaré, Garches.
Ann Med Interne (Paris). 1987;138(8):615-9.
Twenty three children with myasthenia were followed up in our Department for 10 years. There were no familial forms of the disease; there were no patients with the pure ophthalmic form but 61 p. 100 of patients had serious respiratory disease. Steroid therapy was possible in 10 cases: in 4 patients under 15 years of age, short courses of steroids were ineffective; in 6 patients over the age of 15, long term steroid therapy resulted in some improvement but cortico-dependence developed in 4 cases. One child responded to treatment with veinoglobulin (R). Sixteen children underwent thymectomy; the symptoms in the 7 non-thymectomized patients were equivalent. The myasthenic syndrome was stable or worsened in 75 p. 100 of cases after thymectomy; only 56 p. 100 of children improved 2 years after surgery. The incidence of complete remission was the same in the children undergoing thymectomy and in those not treated surgically at 7 years after thymectomy. Our attitude is therefore not to refer myasthenic children for routine thymectomy and to avoid steroid therapy whenever possible.
我们科室对23例重症肌无力患儿进行了10年的随访。该疾病无家族性发病形式;无单纯眼肌型患者,但61%的患者患有严重的呼吸系统疾病。10例患者可行类固醇治疗:4例15岁以下患者,短期类固醇治疗无效;6例15岁以上患者,长期类固醇治疗有一定改善,但4例出现皮质类固醇依赖。1例儿童对静脉注射免疫球蛋白(R)治疗有反应。16例儿童接受了胸腺切除术;7例未接受胸腺切除术患者的症状与之相当。胸腺切除术后75%的病例中重症肌无力综合征稳定或恶化;术后2年只有56%的儿童病情改善。胸腺切除术后7年,接受胸腺切除术的儿童与未接受手术治疗的儿童完全缓解率相同。因此,我们的态度是不建议重症肌无力患儿常规进行胸腺切除术,并尽可能避免类固醇治疗。