Güçyetmez Bülent, Ogan Aylin, Cimet Ayyıldız Aylin, Yalçın Güder Berrin, Klepetko Walter
Intensive Care Unit, International Hospital, Istanbul, 34149, Turkey.
Division of Thoracic Surgery, Medical University of Vienna, Vienna, A-1090, Austria.
F1000Res. 2014 May 28;3:118. doi: 10.12688/f1000research.4035.1. eCollection 2014.
Pulmonary alveolar microlithiasis (PAM) is an autosomal recessive disease characterized by the deposition of phosphate and calcium in the alveoli. The disease progresses asymptomatically until later stages. When it becomes symptomatic, lung transplantations performed before the onset of right heart failure may improve life expectancy and quality. Here we present a case report concerning the very first Turkish PAM patient to have undergone lung transplantation surgery. Patient information: A 52 year-old female, Caucasian patient, already diagnosed with PAM in infancy, was admitted to the intensive care unit, diagnosed with pneumonia and hospitalized for 20 days. We decided to refer the patient to a specialized center for lung transplantation. Bilateral lung transplantation was performed in Vienna 14 months later and no recurrence was observed during the first postoperative year.
Bilateral lung transplantation may improve both the life expectancy and the quality of life of PAM diagnosed patients with severe respiratory failure who do not suffer from right heart failure. The risk of recurrence should not be considered as a justifying reason to avoid transplantation as a treatment method.
肺泡微结石症(PAM)是一种常染色体隐性疾病,其特征是肺泡内磷酸钙沉积。该疾病在后期之前通常无症状进展。出现症状时,在右心衰竭发作前进行肺移植可能会改善预期寿命和生活质量。在此,我们报告首例接受肺移植手术的土耳其PAM患者的病例。患者信息:一名52岁的白种女性患者,婴儿期即被诊断为PAM,入住重症监护病房,诊断为肺炎并住院20天。我们决定将该患者转诊至专门的肺移植中心。14个月后在维也纳进行了双侧肺移植,术后第一年未观察到复发。
双侧肺移植可改善诊断为PAM且患有严重呼吸衰竭但未出现右心衰竭患者的预期寿命和生活质量。复发风险不应被视为避免将移植作为一种治疗方法的正当理由。