促肾上腺皮质激素治疗婴儿痉挛的随访研究

目的对促肾上腺皮质激素(ACTH)治疗的婴儿痉挛(IS)患儿进行追踪随访，了解其预后并评价影响预后的因素。 方法(1)对北京大学第一医院儿科1993年1月至2003年12月住院的53例ACTH治疗的IS患儿的临床随访资料进行回顾性研究。(2)ACTH治疗方法：采用ACTH静滴，25~40IU/d,共4周。 结果(1)对53例ACTH治疗患儿随访6个月至10年，ACTH治疗发作完全缓解的31例，随访2年以上，共有16例复发，复发率为51.6%，其中于停用ACTH后2~3个月复发者占50.0%(8/16),复发形式以肌阵挛发作、痉挛发作、部分运动性发作为主；(2)临床发作预后与开始用ACTH治疗时的病程、ACTH的近期疗效有关(病程≤2个月者、ACTH近期疗效好的临床发作预后好)，与发病年龄、病因无关；(3)智力预后与ACTH开始治疗时的病程、病因、发作预后有关(病程≤2个月者、隐原性者、发作完全缓解者智力预后好)，与发病年龄、ACTH近期疗效无关。 结论(1)IS患儿临床发作预后与开始用ACTH治疗时的病程、ACTH的近期疗效有关。(2)智力预后与ACTH开始治疗时的病程、病因、发作预后有关。

Outcome of adrenocorticorticotropia hormone therapy for infantile spasms.

Follow up the IS patients treated with ACTH in order to analyze the long-term outcome and its influenced factors. Methods1.The medical records of 53 patients with IS were analyzed with retrospective studies.2.53 patients with IS,who were treated with ACTH,the dosage ranging from 25IU to 40IU daily for 4 weeks,were analyzed. ResultsFactors influencing seizure outcome were treatment lag and the initial effects of ACTH on seizure.The patients with shorter treatment lag (≤2 months) were better than those with longer treatment lag (>2 months);the patients with the better initial effects of ACTH on seizure were better than those with the worse initial effects of ACTH on seizure.Seizure outcome had not correlates with the age of onset and etiologies.Patients with shorter treatment lag (≤2 months) showed a better mental outcome than those with longer treatment lag (>2 months).Cryptogenic group showed a better mental outcome than symptomatic group.Mental outcome in seizure-free patients were better than that in those having seizures.Mental outcome had not correlated with the age of onset and the initial effects of ACTH on seizures. Conclusion1.The patients with shorter treatment lag and with the better initial effects of ACTH on seizures showed a better seizure outcome.2.Mental outcome correlated with treatment lag,etiology and seizure outcome(patients with shorter treatment lag,cryptogenic and seizure-free showed a better mental outcome).