PATCH研究:衰老过程中听力损失的发生率和 osteogenesis imperfecta 中的治疗选择:丹麦全国性的基于注册的队列研究
Sara Kretzschmar Haumann1, Jesper Roed Sørensen2,3, Jesper Hvass Schmidt2,3
1Department of Endocrinology, Bone and Mineral Unit, Odense University Hospital, Kløvervænget 6, 5 Floor, 5000, Odense C, Denmark.
Calcified tissue international
|July 16, 2024
概括
患有骨质发育不完善 (OI) 的人面临着明显更高的听力损失 (HL) 和相关并发症的风险,经常在较年轻时出现症状. 这项研究强调了OI患者中HL,耳硬化,助听器使用和中耳手术的患病率增加.
科学领域:
- 遗传学和遗传性疾病
- 耳鼻喉科 耳鼻喉科 耳鼻喉科
- 流行病学 流行病学
背景情况:
- 骨质发生不完善 (Osteogenesis Imperfecta,简称OI) 是一组罕见的遗传性原体疾病.
- 听力损失 (HL) 是OI的一种已知的并发症,通常与中耳骨的结构变化有关.
研究的目的:
- 为了确定患有OI的个体的流行,发病率,发病年龄和听力损失 (HL) 风险.
- 在OI人群中调查耳硬化,中耳手术和助听器使用的风险和发病年龄.
主要方法:
- 一项全国性的丹麦队列研究,利用1977年1月1日至2018年12月31日的基于登记册的数据.
- 包括864名OI患者,根据出生年份和性别,与4276名普通人口 (Ref.Pop) 的1:5匹配.
- 细危险比率 (SHR) 的分析,以评估HL,耳硬化,手术和助听器使用的风险和患病率.
主要成果:
- 任何HL的患病率在OI队列中为17.0%,在Ref.Pop中为4.0%,风险明显更高 (SHR4.56).
- 与Ref.Pop.相比,OI患者的耳硬化和/或中耳手术风险明显增加 (SHR 22.51),较早发病.
- 听力辅助器的使用在OI队列中更频繁 (SHR 4.16),也在早期开始使用.
结论:
- 患有 osteogenesis imperfecta 的人显著增加听力损失和相关干预的风险和患病率.
- 与一般人群相比,OI的听力损失和相关并发症在较年轻时表现出来.
- 这些发现强调了听力学监测和早期干预对被诊断患有OI的个体的重要性.
相关概念视频
01:29Bone Disorders
3.5K
Aging and its effect on bone remodeling is the most common cause of bone disorders. In young and healthy people, bone deposition and resorption happen at an equal rate to maintain optimal bone health.
Bone deposition is also affected by the levels of sex hormones like estrogen and testosterone that promote osteoblast activity and bone matrix synthesis. When the level of these hormones decreases due to aging, it causes a reduction in bone deposition. As a result, bone resorption by osteoclasts...
Bone deposition is also affected by the levels of sex hormones like estrogen and testosterone that promote osteoblast activity and bone matrix synthesis. When the level of these hormones decreases due to aging, it causes a reduction in bone deposition. As a result, bone resorption by osteoclasts...
3.5K
01:11The Auditory Ossicles
1.6K
The auditory ossicles of the middle ear transmit sounds from the air as vibrations to the fluid-filled cochlea. The auditory ossicles consist of two malleus (hammer) bones, two incus (anvil) bones, and two stapes (stirrups), one on each side. These bones develop during the fetal stage and are the ones to ossify first. They are fully mature at birth and do not grow afterward.
The aptly named stapes look very much like a stirrup. The three ossicles are unique to mammals, and each plays a role in...
The aptly named stapes look very much like a stirrup. The three ossicles are unique to mammals, and each plays a role in...
1.6K