Loeys-Dietz Syndrome and Asthma: Pathophysiological Insights and Clinical Dilemmas.

IF 0.8 Q4 RESPIRATORY SYSTEM
Respirology Case Reports Pub Date : 2025-08-13 eCollection Date: 2025-08-01 DOI:10.1002/rcr2.70312
Ella Brockwell-Mole, John D Blakey, Vidya V Navaratnam
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引用次数: 0

Abstract

A 23-year-old man presented with a life-threatening asthma exacerbation following a respiratory infection. Although he had a childhood history of asthma, he had remained asymptomatic in adulthood and reported regular use of both vapes and marijuana. His medical history included Type 2 Loeys-Dietz syndrome (LDS), requiring multiple vascular surgeries, rendering standard asthma therapies relatively contraindicated. Due to the severity of his condition, nebulised beta-agonists were cautiously administered, and his beta-blocker therapy was substituted. Imaging and forced oscillation technique (FOT) demonstrated severe obstructive airways disease. Marked and persistent eosinophilia was noted. While asthma is a recognised comorbidity in LDS, earlier recognition and intervention, such as inhaled corticosteroids, smoking cessation support, and judicious use of beta-blockers, may have reduced the severity. Increased awareness of the prevalence and severity of asthma in LDS could support earlier diagnosis, preventive strategies and timely escalation of therapy, thereby reducing the risk of catastrophic respiratory events.

Loeys-Dietz综合征和哮喘:病理生理学见解和临床困境。
一名23岁男子在呼吸道感染后出现危及生命的哮喘恶化。尽管他童年时有哮喘病史,但成年后仍无哮喘症状,并经常使用电子烟和大麻。他的病史包括2型Loeys-Dietz综合征(LDS),需要多处血管手术,使得标准哮喘治疗相对禁忌。由于病情严重,雾化β受体激动剂被谨慎使用,β受体阻滞剂被替代。影像和强迫振荡技术(FOT)显示严重的阻塞性气道疾病。可见明显且持续的嗜酸性粒细胞增多。虽然哮喘是公认的LDS的合并症,但早期的识别和干预,如吸入皮质类固醇、戒烟支持和明智地使用β受体阻滞剂,可能降低了严重程度。提高对低度发展中国家哮喘患病率和严重程度的认识,可以支持早期诊断、预防策略和及时升级治疗,从而降低灾难性呼吸事件的风险。
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来源期刊
Respirology Case Reports
Respirology Case Reports RESPIRATORY SYSTEM-
CiteScore
1.40
自引率
0.00%
发文量
178
审稿时长
8 weeks
期刊介绍: Respirology Case Reports is an open-access online journal dedicated to the publication of original clinical case reports, case series, clinical images and clinical videos in all fields of respiratory medicine. The Journal encourages the international exchange between clinicians and researchers of experiences in diagnosing and treating uncommon diseases or diseases with unusual presentations. All manuscripts are peer-reviewed through a streamlined process that aims at providing a rapid turnaround time from submission to publication.
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