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女性卡塔格内综合征的偶然影像学发现:一例报告。

Incidental imaging detection of Kartagener syndrome in a female: A case report.

作者信息

Dhakal Natasha, Dahal Prajwal

机构信息

Manmohan Cardiothoracic Vascular and Transplant Center, Kathmandu, Nepal.

Department of Radiology and Imaging, Grande International Hospital, Kathmandu, Nepal.

出版信息

Radiol Case Rep. 2024 Dec 23;20(3):1517-1521. doi: 10.1016/j.radcr.2024.11.087. eCollection 2025 Mar.

Abstract

Kartagener syndrome is a rare ciliopathic genetic disorder characterized by a triad of chronic sinusitis, situs inversus, and bronchiectasis. The underlying pathophysiology involves reduced ciliary motility due to defects in ciliary structure and function within the respiratory tract and fallopian tubes. Diagnosis is typically confirmed through imaging studies such as X-rays, CT scans, and echocardiograms, which reveal the abnormal orientation of the heart and other organs. Treatment depends on the presence of associated conditions, such as congenital heart defects, respiratory infections and appropriate interventions with regular follow-up can help prevent complications. In this case, we present a 32-year-old female with a history of secondary infertility who presented with recurrent cough and fever. A thorough clinical examination was conducted, followed by radiological investigations that revealed chronic sinusitis, bronchiectasis, dextrocardia, and situs inversus totalis. She was subsequently treated with oral antibiotics, mucolytics, and initiated on chest physiotherapy. This case highlights the importance of considering Kartagener syndrome in patients presenting with recurrent respiratory infections and fertility issues.

摘要

卡塔格内综合征是一种罕见的纤毛病性遗传病,其特征为慢性鼻窦炎、内脏反位和支气管扩张三联征。潜在的病理生理学机制涉及由于呼吸道和输卵管内纤毛结构和功能缺陷导致的纤毛运动能力降低。诊断通常通过X线、CT扫描和超声心动图等影像学检查来确诊,这些检查可显示心脏和其他器官的异常方位。治疗取决于是否存在相关病症,如先天性心脏缺陷、呼吸道感染,定期随访并采取适当干预措施有助于预防并发症。在此病例中,我们报告一名32岁有继发性不孕病史的女性,她出现反复咳嗽和发热。进行了全面的临床检查,随后的影像学检查显示有慢性鼻窦炎、支气管扩张、右位心和完全性内脏反位。她随后接受了口服抗生素、黏液溶解剂治疗,并开始进行胸部物理治疗。该病例强调了在出现反复呼吸道感染和生育问题的患者中考虑卡塔格内综合征的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/efb8/11727315/67651b838b4c/gr1.jpg

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