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先天性多发性关节挛缩症儿童研究平台:初步登记结果。

Research platform for children with arthrogryposis multiplex congenita: Findings from the pilot registry.

机构信息

Shriners Hospital for Children, Montreal, Quebec, Canada.

School of Physical and Occupational Therapy, McGill University, Montreal, Quebec, Canada.

出版信息

Am J Med Genet C Semin Med Genet. 2019 Sep;181(3):427-435. doi: 10.1002/ajmg.c.31724. Epub 2019 Jul 29.

DOI:10.1002/ajmg.c.31724
PMID:31359631
Abstract

A pediatric registry for arthrogryposis multiplex congenita (AMC) proposes to advance research by providing the platform to inform the distribution, etiology, and natural history of AMC. The registry was piloted on 40 families of children (mean = 8.25 years, 48% males) presenting with AMC across two hospitals in North America. Data on the child's demographic and newborn variables, mothers' and fathers' demographic variables, lifestyle habits, and medical history were collected using a telephone interview with the primary caregiver and review of medical charts. Mean gestational age was 38 weeks, 97% of children presented with lower extremity deformities, and 74% of neonatal interventions targeted the lower extremity. Newborns spent an average of 14 days in the hospital (range 2-56 days) mostly for diagnostic workup and feeding difficulties. Half (49%) of the sample had internal organ involvement. Genetic testing was done on 48% of the children, including chromosome studies, single gene, whole-exome/genome sequencing, and/or microarray studies. Genetic findings were inconclusive in most. Two-thirds of mothers (67%) reported inconsistently feeling fetal movements. This pilot study contributed to the refinement of participant selection, identification of data source, expansion of data sets, and areas for future exploration prior to the implementation of a multisite AMC pediatric registry.

摘要

一个小儿多发性关节挛缩症(AMC)的注册研究旨在通过提供信息平台来推进研究,以了解 AMC 的分布、病因和自然史。该注册研究在北美两家医院的 40 个 AMC 患儿家庭中进行了试点(平均年龄=8.25 岁,48%为男性)。使用主要照顾者的电话访谈和病历回顾收集了儿童的人口统计学和新生儿变量、母亲和父亲的人口统计学变量、生活方式习惯和病史数据。平均胎龄为 38 周,97%的儿童出现下肢畸形,74%的新生儿干预针对下肢。新生儿平均住院 14 天(范围 2-56 天),主要是为了进行诊断和喂养困难的治疗。该样本的一半(49%)有内脏器官受累。对 48%的儿童进行了基因检测,包括染色体研究、单基因、全外显子/基因组测序和/或微阵列研究。大多数基因检测结果不确定。三分之二的母亲(67%)报告说感觉胎儿运动不规律。这项试点研究为参与者选择、确定数据来源、扩展数据集以及在实施多中心 AMC 儿科注册研究之前的未来探索领域做出了贡献。

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引用本文的文献

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Eur J Hum Genet. 2025 Apr 7. doi: 10.1038/s41431-025-01848-3.
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Human Phenotype Ontology Annotations for Rare Congenital Conditions: Application to Arthrogryposis Multiplex Congenita.罕见先天性疾病的人类表型本体注释:在多发性先天性关节挛缩症中的应用。
Am J Med Genet A. 2025 Aug;197(8):e64067. doi: 10.1002/ajmg.a.64067. Epub 2025 Apr 3.
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Health-related quality of life in 205 children with arthrogryposis multiplex congenita.
205例先天性多发性关节挛缩症患儿的健康相关生活质量
Qual Life Res. 2025 Jan;34(1):247-260. doi: 10.1007/s11136-024-03808-8. Epub 2024 Oct 22.
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Epidemiology, aetiology, interventions and genomics in children with arthrogryposis multiplex congenita: protocol for a multisite registry.先天性多发性关节挛缩症患儿的流行病学、病因学、干预措施和基因组学:多中心注册研究方案。
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Test-retest reliability for performance-based outcome measures among individuals with arthrogryposis multiplex congenita.关节挛缩症多发性先天畸形患者基于表现的结果测量的重测信度。
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