Dataset Information

Subjective and Objective Assessment of Hand Function in Mucopolysaccharidosis IVa Patients.

ABSTRACT: INTRODUCTION:Morquio A is classically described as a skeletal dysplasia, but the impact of the abnormalities on the upper limb has not been described. AIM:The aim of our study is to assess the level of subjective and objective functions of the hand and upper limb in Morquio A patients in order to advance understanding as this may change future management for this complex group of patients. METHOD:We have evaluated a series of ten patients with Morquio syndrome who are currently part of the enzyme replacement therapy trials in our unit. To establish functional levels, we developed a questionnaire based on the principles of the World Health Organization International Classification of Functioning, Disability and Health (WHO ICF). Furthermore, we measured patients' grip function and range of motion. RESULTS:All patients showed hypermobility and limited control of the wrist. Their grip strength was reduced and they showed higher difficulty levels for activities involving strength. It is feasible that the wrist abnormalities may be the major cause of functional loss for Morquio patients. CONCLUSION:Our findings suggest the wrist itself represents an area that we should focus on to be able to improve the patients' functional abilities and hence their level of independence on day-to-day basis.

SUBMITTER: Aslam R

PROVIDER: S-EPMC3565635 | biostudies-literature | 2013

REPOSITORIES: biostudies-literature

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Subjective and Objective Assessment of Hand Function in Mucopolysaccharidosis IVa Patients.

Aslam Riffat R van Bommel Annelotte C M ACM Hendriksz Christian J CJ Jester Andrea A

JIMD reports 20121013

<h4>Introduction</h4>Morquio A is classically described as a skeletal dysplasia, but the impact of the abnormalities on the upper limb has not been described.<h4>Aim</h4>The aim of our study is to assess the level of subjective and objective functions of the hand and upper limb in Morquio A patients in order to advance understanding as this may change future management for this complex group of patients.<h4>Method</h4>We have evaluated a series of ten patients with Morquio syndrome who are curre ...[more]

PMID: 23430548

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Project description:ObjectivesTo investigate the correlation of objective audiometry with user satisfaction as measured with the questionnaire scores.MethodsTwenty patients with hearing loss, who agreed to wear a hearing aid and were referred for hearing aid fitting, were included in this prospective clinical study. All patients used the in-the-canal type of Wide7 hearing aid provided by BSL Co., Ltd. We performed the Korean version of the Hearing Handicap Inventory for the Elderly (K-HHIE) and the International Outcome Inventory for Hearing Aids (K-IOI-HA) before and 1, 3, and 6 months after wearing the hearing aid. We also performed pure tone audiometry (PTA), speech audiometry (SA), functional gain (FG), hearing in noise test (HINT), and central auditory processing disorder tests, such as frequency pattern test (CA-f), duration pattern test (CA-d), and dichotic test (CA-Di). Patients were divided into two groups (group A-HHIE, improved; group B-HHIE, same or worse) by comparing the score of K-HHIE before and 6 months after wearing the hearing aid. In the 6-month K-IOI-HA questionnaire, 21 points were considered as the average score. Based on this, we further divided patients into two groups (group A-IOI, >21 points; group B-IOI, ≤21 points).ResultsGroup A-HHIE included six patients and group B-HHIE included 14 patients. In PTA, SA, HINT, CA-d, and CA-Di, group A-HHIE showed higher improvements than group B-HHIE, which were not statistically significant. Group A-IOI included 12 patients and group B-IOI included eight patients. No statistically significant difference was noted in the improvement of audiometric results over a period of 6 months after wearing the hearing aid between groups A-IOI and B-IOI.ConclusionThere were no significant and consistent audiometric results to reflect patient's satisfaction with the hearing aid. Therefore, when analyzing the hearing aid-fitting outcome, both the objective audiometric tests and subjective questionnaire should be performed together for validating hearing aid performance.

Dataset Information

Subjective and Objective Assessment of Hand Function in Mucopolysaccharidosis IVa Patients.

Publications

Subjective and Objective Assessment of Hand Function in Mucopolysaccharidosis IVa Patients.

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