A patient-specific measurement technique to model shoulder joint kinematics.

BACKGROUND: Measuring dynamic in vivo shoulder kinematics is crucial to better understanding numerous pathologies. Motion capture systems using skin-mounted markers offer good solutions for non-invasive assessment of shoulder kinematics during dynamic movement. However, none of the current motion capture techniques have been used to study translation values at the joint, which is crucial to assess shoulder instability. The aim of the present study was to develop a dedicated patient-specific measurement technique based on motion capture and magnetic resonance imaging (MRI) to determine shoulder kinematics accurately. HYPOTHESIS: Estimation of both rotations and translations at the shoulder joint using motion capture is feasible thanks to a patient-specific kinematic chain of the shoulder complex reconstructed from MRI data. MATERIALS AND METHODS: We implemented a patient-specific kinematic chain model of the shoulder complex with loose constraints on joint translation. To assess the effectiveness of the technique, six subjects underwent data acquisition simultaneously with fluoroscopy and motion capture during flexion and empty-can abduction. The reference 3D shoulder kinematics was reconstructed from fluoroscopy and compared to that obtained from the new technique using skin markers. RESULTS: Root mean square errors (RMSE) for shoulder orientation were within 4° (mean range: 2.0°-3.4°) for each anatomical axis and each motion. For glenohumeral translations, maximum RMSE for flexion was 3.7mm and 3.5mm for empty-can abduction (mean range: 1.9-3.3mm). Although the translation errors were significant, the computed patterns of humeral translation showed good agreement with published data. DISCUSSION: To our knowledge, this study is the first attempt to calculate both rotations and translations at the shoulder joint based on skin-mounted markers. Results were encouraging and can serve as reference for future developments. The proposed technique could provide valuable kinematic data for the study of shoulder pathologies. LEVEL OF EVIDENCE: Basic Science Study.

Enzyme replacement therapy for mucopolysaccharidosis VI (Maroteaux-Lamy syndrome): experience in Hong Kong.

Mucopolysaccharidosis VI (Maroteaux-Lamy syndrome) is a very rare inherited lysosomal storage disease. We evaluated the efficacy and safety of weekly infusions of recombinant human arylsulfatase B as enzyme replacement therapy for two patients in whom this condition was advanced. The primary outcome variables were the distance walked in a 6-minute walk test, forced vital capacity, and ejection fraction. The secondary outcome variables were the number of stairs climbed in a 3-minute stair climbing test, joint mobility, urinary glycosaminoglycan excretion, auto-continuous positive airway pressure study and liver size. After 24 weeks of treatment, patient A walked 40 m (36%) and patient B walked 66 m (58%) more in the walk test than at baseline. After 48 weeks, in patient A the corresponding improvements were 142 m (129%) in the walk test and 33 stairs (60%) in the 3-minute stair climbing test, and in patient B the respective improvements were 198 m (174%) and 77 stairs (140%). There was a significant decline in urinary glycosaminoglycan excretion and improvement in range of motion of joints in both patients. The auto-continuous positive airway pressure study revealed improvements in patient A, while other efficacy variables remained static. There were no drug-related adverse events or allergic reactions reported during and after the infusions of recombinant human arylsulfatase B. Recombinant human arylsulfatase B significantly improves endurance and reduces urinary glycosaminoglycan excretion. The drug is generally safe and well tolerated.