Sells J M, Jaffe K M, Hall J G
Department of Pediatrics, University of Washington, Seattle, USA.
Pediatrics. 1996 Feb;97(2):225-31.
Amyoplasia is the most commonly seen diagnostic subgroup of children with arthrogryposis. The natural history of these children has not been well described previously.
Review of the medical records of 38 children with amyoplasia enabled us to describe their birth characteristics, therapeutic interventions, and functional outcomes.
Eighty-four percent of the children had symmetrical, four-limb involvement, which was similar to the original descriptions of amyoplasia, at birth. There was an average of 5.7 orthopedic procedures per child, and the children had multiple castings and splintings of their limbs and participated in physical and occupational therapy on a regular basis. By the age of 5 years, 85% were ambulatory, most were relatively or completely independent in their activities of daily living, and most were in regular classrooms at the appropriate grade level.
Although children with amyoplasia have pronounced musculoskeletal involvement at birth, which requires orthopedic and rehabilitative interventions during their childhood, their functional outcome in both physical and educational areas is excellent.
先天性多发性关节挛缩症是关节挛缩症患儿中最常见的诊断亚组。此前,这些患儿的自然病史尚未得到充分描述。
回顾38例先天性多发性关节挛缩症患儿的病历,使我们能够描述他们的出生特征、治疗干预措施和功能结局。
84%的患儿出生时四肢均有对称性受累,这与先天性多发性关节挛缩症的最初描述相似。每个患儿平均接受5.7次骨科手术,患儿四肢多次接受石膏固定和夹板固定,并定期接受物理治疗和职业治疗。到5岁时,85%的患儿能够行走,大多数患儿在日常生活活动中相对独立或完全独立,并且大多数在合适的年级进入普通教室上课。
尽管先天性多发性关节挛缩症患儿出生时存在明显的肌肉骨骼受累,在儿童期需要进行骨科和康复干预,但他们在身体和教育方面的功能结局都非常好。