Thoracic and Vascular Surgery Research Center, Shiraz University of Medical Science, Shiraz, Iran.
Shiraz Transplant Center, Abu-Ali Sina Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.
Clin Respir J. 2024 May;18(5):e13773. doi: 10.1111/crj.13773.
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.
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.
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.
肺肺泡微结石症（PAM）是一种罕见的常染色体隐性遗传疾病，全世界约有 1000 例已知病例，其中磷酸钙微结石沉积在肺泡气腔中。截至本报告撰写之时，尚无明确的常规治疗方法，许多 PAM 病例可能进展为严重呼吸衰竭和潜在死亡。双侧肺移植（BLx）似乎是最理想的解决方案；然而，由于 PAM 术后结果的相关报告有限，该手术具有挑战性。我们报告了伊朗首例成功接受 BLx 治疗的 PAM 病例。
我们介绍了一名 42 岁女性的病例，她长期咳嗽，对常规镇咳药无反应，因咳嗽、用力呼吸困难和咯血住院后被诊断为 PAM 病例。尽管接受了皮质类固醇和药物治疗，但病情并未改善，随后她出现呼吸和右心室衰竭，需要吸氧通气。最终，她接受了 BLx。手术成功，在 2 年的随访期间，未报告复发或明显的术后并发症。
本病例报告和文献复习证实了 BLx 作为 PAM 确诊患者的一种有前途的治疗方法的有效性，提高了患者的预期寿命和生活质量。
