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Laryngological presentations and patient-reported outcome measures in Ehlers-Danlos syndrome.喉科学表现和埃勒斯-当洛斯综合征患者报告的结局测量。
J Laryngol Otol. 2022 Oct;136(10):947-951. doi: 10.1017/S0022215121004072. Epub 2021 Dec 10.
2
Throat and voice problems in Ehlers-Danlos syndromes and hypermobility spectrum disorders.埃勒斯-当洛综合征和高活动型谱系障碍中的咽喉及嗓音问题。
Am J Med Genet C Semin Med Genet. 2021 Dec;187(4):527-532. doi: 10.1002/ajmg.c.31956. Epub 2021 Nov 20.
3
A review of respiratory manifestations and their management in Ehlers-Danlos syndromes and hypermobility spectrum disorders.Ehlers-Danlos 综合征和高迁移率族蛋白谱障碍的呼吸表现及其处理的综述。
Chron Respir Dis. 2021 Jan-Dec;18:14799731211025313. doi: 10.1177/14799731211025313.
4
Problems in the orofacial region associated with Ehlers-Danlos and Marfan syndromes: a case series.与埃勒斯-当洛综合征和马凡综合征相关的口面部区域问题:病例系列
Br J Oral Maxillofac Surg. 2020 Feb;58(2):208-213. doi: 10.1016/j.bjoms.2019.11.018. Epub 2019 Dec 16.
5
Prevalence of Obstructive Sleep Apnea in Joint Hypermobility Syndrome: A Systematic Review and Meta-Analysis.关节过度活动综合征中阻塞性睡眠呼吸暂停的患病率:系统评价和荟萃分析。
J Clin Sleep Med. 2019 Feb 15;15(2):293-299. doi: 10.5664/jcsm.7636.
6
Arytenoid Prolapse in 3 Patients With Ehler-Danlos Syndrome Leading to Respiratory Compromise.3例埃勒斯-当洛综合征患者出现杓状软骨脱垂导致呼吸功能受损。
Mayo Clin Proc. 2017 May;92(5):851-853. doi: 10.1016/j.mayocp.2017.01.016.
7
Prevalence and Occupation of Patients Presenting With Dysphonia in the United States.美国出现声音障碍患者的患病率及职业情况
J Voice. 2017 Sep;31(5):594-600. doi: 10.1016/j.jvoice.2017.01.011. Epub 2017 Apr 13.
8
The 2017 international classification of the Ehlers-Danlos syndromes.2017年埃勒斯-当洛综合征国际分类法。
Am J Med Genet C Semin Med Genet. 2017 Mar;175(1):8-26. doi: 10.1002/ajmg.c.31552.
9
Two patients with Ehlers-Danlos syndrome type VIII with unexpected hoarseness.两名患有 VIII 型埃勒斯-当洛综合征且伴有意外声音嘶哑的患者。
Clin Exp Dermatol. 2016 Oct;41(7):771-4. doi: 10.1111/ced.12911.
10
Laryngological presentations of Ehlers-Danlos syndrome: case series of nine patients from two London tertiary referral centres.埃勒斯-丹洛斯综合征的喉科学表现:来自伦敦两家三级转诊中心的9例患者病例系列
Clin Otolaryngol. 2017 Aug;42(4):860-863. doi: 10.1111/coa.12708. Epub 2016 Aug 3.

喉科主诉在高活动度埃勒斯-当洛斯或高活动度谱系障碍中的发生率。

Laryngological Complaint Prevalence in Hypermobile Ehlers-Danlos or Hypermobility Spectrum Disorders.

机构信息

Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Jacksonville, Florida, U.S.A.

Department of Cardiovascular Medicine, Mayo Clinic, Jacksonville, Florida, U.S.A.

出版信息

Laryngoscope. 2024 Feb;134(2):773-778. doi: 10.1002/lary.30964. Epub 2023 Aug 19.

DOI:10.1002/lary.30964
PMID:37597175
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10841389/
Abstract

OBJECTIVE

The aim was to study laryngological complaints in patients with hypermobile Ehlers-Danlos syndrome (hEDS) or hypermobility spectrum disorders (HSD).

METHODS

A total of 363 patients met inclusion for the study by completing questions related to voice, upper airway, and swallowing between July 7, 2020 and July 13, 2022. Demographic data, voice-related questions, and hypermobility diagnosis were analyzed retrospectively. From those, 289 patients were diagnosed with hEDS or HSD with 74 that did not meet the diagnostic criteria for either diagnosis serving as controls.

RESULTS

There were no statistically significant differences between patients with hEDS and HSD regarding Voice Handicap Index (VHI-10) scores, voice, upper airway, or swallow complaints. However, more hEDS/HSD patients answered positively to the laryngeal dysfunction question versus controls (p = 0.031). 22.5% of hEDS/HSD patients (n = 65) reported hoarseness, of which 52.3% reported hoarseness >2 days/month. 33.9% (n = 98) with hEDS/HSD reported symptoms of dysphagia, and 27.0% (n = 78) reported laryngeal dysfunction symptoms. Controls demonstrated 20.3% prevalence of hoarseness, of which 46.7% reported hoarseness >2 days/month. 24.3% of controls had dysphagia and 14.9% laryngeal dysfunction symptoms. Of the 363 patients, VHI-10 scores >11 were more likely in patients reporting >2 days of hoarseness/month (p = 0.001) versus those with <2 days of hoarseness/month. There was an increased prevalence of voice, upper airway, and dysphagia symptoms in hEDS/HSD patients compared with previously reported prevalence data in the general population.

CONCLUSION

A significant proportion of patients diagnosed with hypermobility due to hEDS or HSD were found to have voice, upper airway, and dysphagia symptoms. These rates are higher than those previously reported in the general population.

LEVEL OF EVIDENCE

3 Laryngoscope, 134:773-778, 2024.

摘要

目的

研究患有高弹性埃勒斯-当洛斯综合征(hEDS)或高弹性谱障碍(HSD)的患者的喉科投诉。

方法

共有 363 名患者在 2020 年 7 月 7 日至 2022 年 7 月 13 日期间通过完成与声音、上呼吸道和吞咽相关的问题,符合研究条件。分析了人口统计学数据、与声音相关的问题和高弹性诊断情况。其中,289 名患者被诊断为 hEDS 或 HSD,74 名患者未符合这两种诊断中的任何一种诊断标准,作为对照组。

结果

在 hEDS 和 HSD 患者的嗓音障碍指数(VHI-10)评分、声音、上呼吸道或吞咽投诉方面,没有统计学上的显著差异。然而,hEDS/HSD 患者中回答有喉功能障碍问题的比例高于对照组(p=0.031)。22.5%的 hEDS/HSD 患者(n=65)报告声音嘶哑,其中 52.3%的患者报告声音嘶哑>2 天/月。33.9%(n=98)的 hEDS/HSD 患者报告有吞咽困难症状,27.0%(n=78)报告有喉功能障碍症状。对照组显示声音嘶哑的患病率为 20.3%,其中 46.7%的患者报告声音嘶哑>2 天/月。对照组中有 24.3%的患者有吞咽困难,14.9%的患者有喉功能障碍症状。在 363 名患者中,VHI-10 评分>11 更可能发生在报告声音嘶哑>2 天/月的患者中(p=0.001),而报告声音嘶哑<2 天/月的患者则较少见。与之前报道的普通人群中发病率数据相比,hEDS/HSD 患者的声音、上呼吸道和吞咽困难症状的发病率更高。

结论

由于 hEDS 或 HSD 而被诊断为高弹性的患者中,有相当比例的患者存在声音、上呼吸道和吞咽困难症状。这些比率高于之前在普通人群中报道的比率。

证据水平

3 级喉镜检查,134:773-778,2024。