黏多糖贮积症IV型患者的听力损失：一项范围综述的方案
Hearing Loss in Patients With Morquio Syndrome: Protocol for a Scoping Review.
作者信息
Diaz-Ordoñez Lorena, Candelo Estephania, Silva-Cuero Katherine, Saldarriaga Wilmar, Murgašová Lenka, Magner Martin, Pachajoa Harry
机构信息
Department of Basic Medical Sciences, Center for Research on Congenital Anomalies and Rare Diseases (CIACER), Universidad Icesi, Cali, Colombia.
School of Basic Sciences, Universidad del Valle, Cali, Colombia.
出版信息
JMIR Res Protoc. 2022 Jun 7;11(6):e32986. doi: 10.2196/32986.
BACKGROUND
Mild to moderate hearing loss is common in patients with mucopolysaccharidosis (MPS) IVA. The hearing loss can be conductive, sensorineural, or mixed. However, in these patients, the mixed form is frequent, attributed to the combination of conductive and neurosensory elements, with slowly progressive evolution. Conductive hearing loss may be secondary to recurrent upper respiratory tract infections, serous otitis media, and deformities of the ear ossicles due to the accumulation of glycosaminoglycans (GAGs). Meanwhile, the sensorineural form is mainly attributed to the accumulation of GAGs in the auditory system.
OBJECTIVE
The aim of this scoping review is to understand the extent and type of evidence in relation to the physiopathology, classification, epidemiology, and clinical management of hearing loss and the effect of therapy for hearing loss in patients with MPS IVA.
METHODS
This scoping review includes participants across all genders and of no particular age group who are diagnosed with MPS IVA and develop hearing loss as a comorbidity. No exclusion criteria (country, language, or document type) will be applicable. The information sources will include experimental and quasi-experimental, analytical observational, observational, and qualitative studies. Unpublished literature will not be covered. Grey literature will be covered. A total of 2 independent reviewers will participate in the process of screening the literature, paper selection, and data extraction, and this process will be performed blindly. When all manuscripts have been selected, disagreements that arise between the 2 reviewers at each stage of the selection process will be resolved through discussion or with an additional reviewer. Results will be reported with descriptive statistics and information will be displayed in a diagrammatic or tabular manner, as explained in the JBI guidelines.
RESULTS
The literature search was performed in November 2021 in MEDLINE, LILACS (Literatura Latino-Americana e do Caribe em Ciências da Saúde), the Cochrane Library, ScienceDirect, Google Scholar, and OpenGrey; a total of 780 results were retrieved. Completion of the review is expected in mid-2022.
CONCLUSIONS
This scoping review will be the first to describe the extent of the information regarding the development of hearing loss in the MPS IVA population. The data gathered by this review may lead to an understanding of the grade of hearing loss in this population and allow for the assessment of possible interventions according to the disease pattern.
INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): PRR1-10.2196/32986.
背景
轻度至中度听力损失在黏多糖贮积症IVA型（MPS IVA）患者中很常见。听力损失可为传导性、感音神经性或混合性。然而，在这些患者中，混合性形式较为常见，这归因于传导性和神经感觉成分的结合，且呈缓慢进展。传导性听力损失可能继发于反复上呼吸道感染、浆液性中耳炎以及由于糖胺聚糖（GAGs）蓄积导致的听小骨畸形。同时，感音神经性形式主要归因于GAGs在听觉系统中的蓄积。
目的
本范围综述的目的是了解与MPS IVA患者听力损失的病理生理学、分类、流行病学和临床管理以及听力损失治疗效果相关的证据范围和类型。
方法
本范围综述纳入所有性别、无特定年龄组且被诊断为MPS IVA并伴有听力损失合并症的参与者。不适用任何排除标准（国家、语言或文献类型）。信息来源将包括实验性和准实验性、分析性观察性、观察性和定性研究。不涵盖未发表的文献。将涵盖灰色文献。共有2名独立评审员参与文献筛选、论文选择和数据提取过程，且此过程将在盲态下进行。当所有手稿都被选定后，在选择过程的每个阶段，2名评审员之间出现的分歧将通过讨论或由另一名评审员解决。结果将用描述性统计报告，信息将以图表或表格形式呈现，如JBI指南中所解释。
结果
2021年11月在MEDLINE、拉丁美洲和加勒比卫生科学文献数据库（LILACS）、考克兰图书馆、ScienceDirect、谷歌学术和OpenGrey中进行了文献检索；共检索到780条结果。预计2022年年中完成综述。
结论
本范围综述将首次描述关于MPS IVA人群听力损失发生情况的信息范围。本综述收集的数据可能有助于了解该人群的听力损失程度，并根据疾病模式评估可能的干预措施。
国际注册报告识别号（IRRID）：PRR1-10.2196/32986
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