Joint Contracture and Radiologic Features in Mucopolysaccharidosis.
10.4055/jkoa.2004.39.7.825
- Author:
Jong Sup SHIM
1
;
Dong Kyu JIN
;
Seung Jun PARK
;
Sung Min KIM
;
Byung Dam RYU
Author Information
1. Department of Orthopedic Surgery, Samsung Medical Center Sungkyunkwan University School of Medicine, Seoul, Korea. sjsra@smc.samsung.co.kr
- Publication Type:Original Article
- Keywords:
Mucopolysaccharidosis;
Joint contracture;
Radiographic feature
- MeSH:
Child;
Congenital Abnormalities;
Contracture*;
Elbow;
Enzyme Assays;
Fibroblasts;
Hip Joint;
Humans;
Joints*;
Lower Extremity;
Mucopolysaccharidoses*;
Orthopedics;
Shoulder;
Upper Extremity;
Wrist
- From:The Journal of the Korean Orthopaedic Association
2004;39(7):825-829
- CountryRepublic of Korea
- Language:Korean
-
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.
