Successful bilateral lung transplantation in pulmonary alveolar microlithiasis: A case report and review of literature

IF 1.9 4区 医学 Q3 RESPIRATORY SYSTEM Clinical Respiratory Journal Pub Date : 2024-05-09 DOI:10.1111/crj.13773
Parviz Mardani, Reyhaneh Naseri, Reza Shahriarirad, Hadiseh Mahram, Masoud Shafi, Tahmoores Niknam, Mohammad Bagher Khosravi, Mohammad Javad Fallahi, Armin Amirian
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Abstract

Background

Pulmonary alveolar microlithiasis (PAM) is a rare autosomal recessive genetic disorder with approximately 1000 known cases worldwide, in which calcium phosphate microliths deposit in the alveolar air spaces. As of writing this report, no definitive conventional therapy exists, and many PAM cases may progress to severe respiratory failure and potential death. Bilateral lung transplantation (BLx) seems to be the most optimal solution; however, this procedure is challenging along with limited reports regarding the outcome in PAM. We report a case of PAM successfully treated with BLx for the first time in Iran.

Method

We present the case of a 42-year-old female with a longstanding history of cough, not responding to conventional antitussive medication, who was diagnosed as a case of PAM following a hospitalization due to coughing, dyspnea on exertion, and hemoptysis. Despite treatment with corticosteroid and medical treatment, no improvement was achieved and she subsequently developed respiratory and right ventricular failure, with oxygen ventilation dependence. Eventually, she was scheduled for BLx. The operation was successful and during her 2-year follow-up, no recurrence or significant postoperative complications has been reported.

Conclusion

This case presentation and literature review confirm the effectiveness of BLx as a promising treatment for PAM-diagnosed patients, improving both life expectancy and quality of life.

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肺泡微石症患者成功进行了双侧肺移植:病例报告和文献综述。
背景:肺泡微结石症(PAM)是一种罕见的常染色体隐性遗传疾病,全世界已知约有 1000 例,患者的肺泡气隙中会沉积磷酸钙微结石。截至撰写本报告时,尚无明确的常规疗法,许多 PAM 病例可能会发展为严重的呼吸衰竭,甚至死亡。双侧肺移植(BLx)似乎是最理想的解决方案;然而,这种手术具有挑战性,而且有关 PAM 结果的报道也很有限。我们首次在伊朗报告了一例通过 BLx 成功治疗 PAM 的病例:我们报告了一例 42 岁女性的病例,她有长期咳嗽史,对常规止咳药无反应,因咳嗽、用力时呼吸困难和咯血住院后被诊断为 PAM。尽管她接受了皮质类固醇治疗和药物治疗,但病情仍不见好转,随后出现呼吸衰竭和右心室衰竭,并依赖氧气通气。最终,她被安排接受 BLx 手术。手术很成功,在两年的随访中,没有发现复发或明显的术后并发症:本病例和文献综述证实,BLx 是治疗 PAM 诊断患者的有效方法,可改善患者的预期寿命和生活质量。
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来源期刊
Clinical Respiratory Journal
Clinical Respiratory Journal 医学-呼吸系统
CiteScore
3.70
自引率
0.00%
发文量
104
审稿时长
>12 weeks
期刊介绍: Overview Effective with the 2016 volume, this journal will be published in an online-only format. Aims and Scope The Clinical Respiratory Journal (CRJ) provides a forum for clinical research in all areas of respiratory medicine from clinical lung disease to basic research relevant to the clinic. We publish original research, review articles, case studies, editorials and book reviews in all areas of clinical lung disease including: Asthma Allergy COPD Non-invasive ventilation Sleep related breathing disorders Interstitial lung diseases Lung cancer Clinical genetics Rhinitis Airway and lung infection Epidemiology Pediatrics CRJ provides a fast-track service for selected Phase II and Phase III trial studies. Keywords Clinical Respiratory Journal, respiratory, pulmonary, medicine, clinical, lung disease, Abstracting and Indexing Information Academic Search (EBSCO Publishing) Academic Search Alumni Edition (EBSCO Publishing) Embase (Elsevier) Health & Medical Collection (ProQuest) Health Research Premium Collection (ProQuest) HEED: Health Economic Evaluations Database (Wiley-Blackwell) Hospital Premium Collection (ProQuest) Journal Citation Reports/Science Edition (Clarivate Analytics) MEDLINE/PubMed (NLM) ProQuest Central (ProQuest) Science Citation Index Expanded (Clarivate Analytics) SCOPUS (Elsevier)
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