Incidence of hip problems in developmental central hypotonia: A scoping review

IF 4.3 2区 医学 Q1 CLINICAL NEUROLOGY Developmental Medicine and Child Neurology Pub Date : 2024-11-06 DOI:10.1111/dmcn.16166
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Abstract

Children with rare genetic conditions often have hypotonia or low muscle tone and delayed motor skills like standing and walking. We know that children with cerebral palsy (CP) with limited standing and walking abilities are at increased risk for hip subluxation or dislocation (where the hips are partly or fully out of position), whether they have high or low muscle tone. Hip surveillance (a program of regular orthopaedic assessments and x-rays) helps to recognize the early signs of hip problems and makes sure children get the best interventions at the right time. However, children with hypotonia who do not have a CP diagnosis often do not receive hip surveillance.

We searched for journal articles or book chapters describing children or adults with rare genetic conditions (like Prader-Willi, Kabuki, Joubert, 49, XXXXY, PURA, as well as other rare syndromes) who were reported to have both hypotonia and hip problems.

We found reports of 544 children and adults aged 1 month to 63 years with hypotonia and hip problems due to rare genetic or undiagnosed conditions. Some had differences in hip shape when they were born, and hip problems also got worse over time. We found that children with hypotonia have a much higher rate of hip problems than children with normal muscle tone, and these problems are often missed by typical infant screening programs. Some children dislocate their hips after they start walking, and older children and adults can also develop painful dislocated hips.

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发育性中枢性肌张力低下患者髋关节问题的发生率:范围综述。
患有罕见遗传疾病的儿童通常会出现张力低下或肌肉张力低,站立和行走等运动技能延迟。我们知道,站立和行走能力有限的脑瘫(CP)患儿,无论肌肉张力高低,髋半脱位或脱位(髋关节部分或完全脱位)的风险都在增加。髋关节监测(一个定期的骨科评估和x光检查项目)有助于识别髋关节问题的早期迹象,并确保儿童在正确的时间得到最好的干预。然而,没有CP诊断的低张力儿童通常不接受髋关节监测。我们搜索了描述患有罕见遗传疾病(如Prader-Willi, Kabuki, Joubert, 49, XXXXY, PURA以及其他罕见综合征)的儿童或成人的期刊文章或书籍章节,这些儿童或成人同时患有张力低下和髋关节问题。我们发现544例儿童和成人,年龄1个月至63岁,由于罕见的遗传或未诊断的疾病而出现张力低下和髋关节问题。有些人出生时臀部形状不同,随着时间的推移,臀部问题也越来越严重。我们发现,与肌肉张力正常的儿童相比,肌张力过低的儿童患髋关节问题的几率要高得多,而这些问题往往被典型的婴儿筛查项目所遗漏。一些儿童在开始走路后髋关节脱臼,年龄较大的儿童和成人也会出现疼痛的髋关节脱臼。
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来源期刊
CiteScore
7.80
自引率
13.20%
发文量
338
审稿时长
3-6 weeks
期刊介绍: Wiley-Blackwell is pleased to publish Developmental Medicine & Child Neurology (DMCN), a Mac Keith Press publication and official journal of the American Academy for Cerebral Palsy and Developmental Medicine (AACPDM) and the British Paediatric Neurology Association (BPNA). For over 50 years, DMCN has defined the field of paediatric neurology and neurodisability and is one of the world’s leading journals in the whole field of paediatrics. DMCN disseminates a range of information worldwide to improve the lives of disabled children and their families. The high quality of published articles is maintained by expert review, including independent statistical assessment, before acceptance.
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