Skin injuries and joint contractures of the upper extremities in Rett syndrome
Skin injuries and joint contractures of the upper extremities in Rett syndrome
复制标题
雷特综合征上肢皮肤损伤和关节挛缩
DOI：
10.1111/jir.12452
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发表时间：
2018
影响因子：
3.6
通讯作者：
Taniguchi T
中科院分区：
文献类型：
--
作者：
Hirano D;Taniguchi T
目的 本研究的目的是调查 Rett 综合征患者上肢皮肤损伤和关节挛缩的发生率和类型。 方法 2016 年，向 1016 名特殊需要教育学校主任和 204 名康复科主任发送了一份关于皮肤损伤和关节挛缩的调查问卷[包括 130 个严重运动和智力障碍者 (SMID) 设施、73 个 SMID 患者病房和日本国立医院组织和国立中心医院、国立神经病学和精神病学中心]。使用描述性统计数据来表明每个问题的出现频率。结果信息来自 216 例雷特综合征患者（3-53 岁）。雷特综合征患者中分别有 41% 和 49% 的人出现皮肤损伤和上肢关节挛缩。大多数皮肤损伤发生在手（19%）和手指（29%）。皮肤损伤的发生率不受年龄或疾病严重程度的影响。在肩关节（33%）和肘关节（29%）中观察到许多关节挛缩。关节挛缩往往发生在 10 岁以上或有严重运动障碍的个体中。结论 本研究中评估的雷特综合征受试者中几乎一半有皮肤损伤和关节挛缩。特别是，关节挛缩的发生率受年龄和疾病严重程度的影响。因此，医务人员努力预防该患者群体发生皮肤损伤和关节挛缩非常重要。
ObjectivesThe purpose of this study was to investigate the incidence and type of skin injuries and joint contractures of the upper extremities in individuals with Rett syndrome.MethodsIn 2016, a questionnaire regarding skin injuries and joint contractures was sent to 1016 directors of schools for special needs education and 204 directors of departments of rehabilitation [consisting of 130 facilities for persons with severe motor and intellectual disabilities (SMID), 73 wards for patients with SMID, and the National Hospital Organisation and National Centre Hospital, National Centre of Neurology and Psychiatry] in Japan. Descriptive statistics were used to indicate frequency in each question.ResultsInformation was acquired from 216 cases (3–53 years old) with Rett syndrome. Skin injuries and joint contractures of the upper extremities were observed in 41% and 49% of individuals with Rett syndrome, respectively. Most of the skin injuries were observed on the hands (19%) and fingers (29%). The incidence of skin injuries was not affected by age or disease severity. Many joint contractures were observed in the shoulder (33%) and elbow (29%) joints. Joint contractures tended to occur in individuals aged over 10 years or with severe locomotor impairment.ConclusionAlmost half of the Rett syndrome subjects assessed in the present study had skin injuries and joint contractures. Especially, the incidence of joint contractures was affected by age and disease severity. Thus, it is important that medical staff attempt to prevent the occurrence of skin injuries and joint contractures in this patient population.