Clinical features, prothrombotic risk factors, and long-term follow-up of eight pediatric Moyamoya patients.
Clinical features, prothrombotic risk factors, and long-term follow-up of eight pediatric Moyamoya patients.
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DOI：
10.3988/jcn.2012.8.2.100
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发表时间：
2012-06
期刊：
影响因子：
--
通讯作者：
Kırış T
中科院分区：
文献类型：
--
作者：
Tatlı B;Ekici B;Sencer A;Sencer S;Aydın K;Aydınlı N;Calışkan M;Ozmen M;Kırış T
本研究的目的是阐明儿童烟雾病患者的临床特征、血栓形成危险因素和结果。 2000 年 1 月至 2006 年 12 月期间在三级中心诊断为烟雾病的患者参加了这项研究。回顾性分析患者的临床表现、基础疾病、血栓形成危险因素、血栓家族史、影像学表现、治疗和结局。经血管造影证实，八名患者患有烟雾病，其中一名患有 I 型神经纤维瘤病，一名患有唐氏综合症。诊断时的年龄在 19 个月至 11 岁之间变化（73.4±41.8 个月，平均值±SD）。诊断后随访时间为52.5±14.8个月。六名患者的最初临床表现是偏瘫。没有患者有任何可识别的促血栓因素。尽管进行了药物和手术治疗，仍有三名患者复发，一名患者死亡。只有两名患者康复且没有留下后遗症。血栓前危险因素评估对于烟雾病患者的价值似乎有限；儿科患者的结果仍然令人沮丧。
The aim of this study was to elucidate the clinical features, prothrombotic risk factors, and outcome of pediatric Moyamoya patients. Patients diagnosed with Moyamoya disease at a tertiary center between January 2000 and December 2006 were enrolled in this study. The clinical presentations, underlying diseases, prothrombotic risk factors, family history of thrombosis, radiological findings, treatment, and outcome of the patients were reviewed retrospectively. Eight patients with angiographically proven Moyamoya disease were identified, one of whom had neurofibromatosis type I and one had Down syndrome. The age at diagnosis varied between 19 months and 11 years (73.4±41.8 months, mean±SD). The follow-up period after diagnosis was 52.5±14.8 months. In six patients, the initial clinical presentation was hemiparesis. None of the patients had any identifiable prothrombotic factors. Despite medical and surgical treatment, three patients had recurrences and one died. Only two patients recovered without sequelae. The value of prothrombotic risk factor evaluation appears to be limited in Moyamoya patients; the outcome for pediatric patients remains dismal.
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