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优化治疗效果:一名患有卡塔格内综合征的28岁女性支气管扩张和肺大疱的管理

Optimizing outcomes: Management of bronchiectasis and air bullae in a 28-year-old female with kartagener syndrome.

作者信息

Alrahil Ali, Aljanadi Mazen, Zaal Kinana Abo, Shaaban Mohammad Ebrahim, Altabaa Kasem, Hasan Tammam

机构信息

Department of Thoracic Surgery, Damascus Hospital, Damascus, Syria.

Department of Thoracic Surgery, Damascus Hospital, Damascus, Syria.

出版信息

Int J Surg Case Rep. 2025 Apr;129:111097. doi: 10.1016/j.ijscr.2025.111097. Epub 2025 Mar 5.

Abstract

INTRODUCTION AND SIGNIFICANCE

Kartagener syndrome is a primary ciliary dyskinesia disorder characterized by the classic triad of chronic sinusitis, bronchiectasis, and situs inversus. The clinical presentation and age of diagnosis of this syndrome are variable, and patients require meticulous care, including antibiotics and respiratory therapy, to prevent deterioration of pulmonary function.

CASE PRESENTATION

We present the case of a female patient with a history of recurrent respiratory infections since birth, treated with antibiotics and complicated by middle ear disease. Investigations revealed a rare presentation of air bullae associated with bronchiectasis. The patient was referred to our care and diagnosed with Kartagener syndrome, subsequently undergoing urgent surgical intervention, which contributed significantly to her overall improvement and the resolution of her thoracic condition.

CLINICAL DISCUSSION

In clinical practice, it is crucial to emphasize daily chest physiotherapy, appropriate antibiotic therapy, supportive pulmonary care, and prompt and careful therapeutic intervention to achieve optimal health outcomes.

CONCLUSION

While complications of Kartagener syndrome are relatively uncommon, particularly emphysema with bronchiectasis leading to significant pulmonary lobe damage, they can occur.

摘要

引言与意义

卡塔格内综合征是一种原发性纤毛运动障碍性疾病,其特征为慢性鼻窦炎、支气管扩张和内脏转位这一经典三联征。该综合征的临床表现和诊断年龄各不相同,患者需要精心护理,包括使用抗生素和进行呼吸治疗,以防止肺功能恶化。

病例介绍

我们报告一例女性患者,自出生以来有反复呼吸道感染病史,接受过抗生素治疗,并伴有中耳疾病。检查发现一种与支气管扩张相关的罕见肺大疱表现。该患者被转诊至我们处并被诊断为卡塔格内综合征,随后接受了紧急手术干预,这对她的整体康复以及胸部疾病的缓解有显著帮助。

临床讨论

在临床实践中,强调每日胸部物理治疗、适当的抗生素治疗、支持性肺部护理以及及时且谨慎的治疗干预以实现最佳健康结果至关重要。

结论

虽然卡塔格内综合征的并发症相对不常见,尤其是肺气肿合并支气管扩张导致显著的肺叶损伤,但仍可能发生。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/283b/11930696/6c948f44c0a8/gr1.jpg

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