DEPARTMENT OF PNEUMONOLOGY, MEDICAL UNIVERSITY OF SILESIA IN KATOWICE, KATOWICE, POLAND.
DEPARTMENT OF CARDIAC, VASCULAR AND ENDOVASCULAR SURGERY AND TRANSPLANTOLOGY, SILESIAN CENTER FOR HEART DISEASES IN ZABRZE, MEDICAL UNIVERSITY OF SILESIA, KATOWICE, POLAND.
Wiad Lek. 2021;74(9 cz 1):2235-2240.
Pulmonary alveolar microlithiasis is a rare genetic disorder, inherited autosomally recessively, which is characterized by intra-alveolar deposition of microliths built mostly of calcium salts and phosphorus. This case study describing management of patient with pulmonary alveolar microlithiasis. A 49-year-old woman, diagnosed with pulmonary microlithiasis in 1979 was admitted to Pneumology Department due to increased dyspnea. On admission there were no clinical signs of active infection. The chest computer tomography scan confirmed the presence of advanced microlithiasis. Pulmonary function test revealed mild restriction with moderate diffusion impairment, due to severe hypoxemia present on 6-minute walking test patient was sent for specific assessment to local lung transplant team in Zabrze for consideration for lung transplantation. According to International Society for Heart & Lung Transplantation guidelines the patient was observed in 6 months intervals to reveal whether further disease progression will be observed. Clinical condition of our patient does not correlate with radiological scans, severe respiratory symptoms and cardiological complications. Computer tomography scan should not be the only indication for lung transplant.
肺泡微结石症是一种罕见的遗传性疾病，呈常染色体隐性遗传，其特征是肺泡内沉积主要由钙盐和磷组成的微结石。本病例研究描述了一位肺泡微结石症患者的治疗过程。一位 49 岁的女性，1979 年被诊断为肺微结石症，因呼吸困难加重而被收入肺病科。入院时无活动性感染的临床体征。胸部计算机断层扫描确认存在进展性微结石症。肺功能测试显示轻度限制和中度弥散功能障碍，由于 6 分钟步行试验中存在严重低氧血症，患者被送往当地扎布热肺移植团队进行特定评估，以考虑进行肺移植。根据国际心肺移植学会的指南，对患者进行 6 个月间隔的观察，以确定是否会观察到进一步的疾病进展。我们患者的临床状况与影像学扫描、严重的呼吸症状和心脏并发症不相关。计算机断层扫描不应是肺移植的唯一指征。
