Journal of the Korean Orthopaedic Association (대한정형외과학회지)

Korean Orthopaedic Association (대한정형외과학회)
권호 표지

Joint Contracture and Radiologic Features in Mucopolysaccharidosis

점액다당증의 관절 구축 및 방사선학적 소견

Shim, Jong-Sup;Jin, Dong-Kyu;Park, Seung-Jun;Kim, Sung-Min;Ryu, Byung-Dam
심종섭;진동규;박승준;김성민;류병담

  • Published : 20040000
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Abstract

Purpose: To evaluate the clinical manifestations of contracted joints and the radiological features in mucopolysaccharidosis (MPS). Materials and Methods: From January 1996 to March 2002, 15 children from a pool of children diagnosed with MPS through a fibroblast culture and an enzyme assay, who could tolerate a large umber of examinations, underwent orthopedic physical and radiological examinations.Results: There were limitations in the abduction and internal rotation of the shoulder, extension limitation of the wrist, and internal rotation of the hip joint and flexion contractures of the elbow and DIP joints. Scoliotic deformities were all within the physiological curve. There were only three cases of a dorsolumbar kyphotic change and there were no neurological deficits due to the spinal deformities. The mean bone age was delayed compared with the mean chronological age. As the chronological age increased, the gap between the chronological age and the bone age increased. Conclusion: Joint contracture, which is the most severe symptom in orthopedic aspect, was more severe in the upper extremities than in the lower extremities. The spinal deformity was relatively mild and there were no neurological problems. The bone age tended to be delayed as the chronological age increased.

목 적: 점액다당증 환자에서 구축된 관절의 임상 양상과 방사선학적 특징을 분석하여 보고하고자 한다. 대상 및 방법: 1996년 1월부터 2002년 3월까지 점액다당증으로 확진을 받은 환아 중, 골격계에 대한 방사선학적 검사와 충분한 이학적 관절 운동 범위 계측이 가능하였던 환아 15예를 대상으로 이학적 검진과 방사선 촬영, 기립상 전 척추 촬영, 그리고 골 연령 측정을 위한 수부촬영을 시행하였다. 결 과: 견관절의 외전과 내회전 제한, 주관절의 굴곡 구축, 수근 관절의 수배굴곡 제한, 원위 지간 관절의 굴곡 구축, 고관절의 내회전 제한 등이 임상적으로 문제가 되었다. 척추 측만증은 모두 생리적 만곡 범위 내에 분포하였고, 상요추부의 후만 변형은 3예에서 경미하게 관찰되었다. 척추 변형으로 인한 신경학적 이상을 보이는 사례는 없었다. 골 연령은 지연되어 있었으며, 역 연령이 증가할수록 골 연령의 지연은 증가하였다(Spearman's correlation coefficient=0.936, p<0.001). 결 론: 관절 구축 및 관절 운동 장애는 상지에서 하지보다 심하였다. 척추의 변형은 비교적 경미하였고, 신경학적 증세는 발견되지 않았다. 골 연령의 지연은 나이가 들수록 증가되는 추세를 나타내었다.

Keywords

References

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