Abstract
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.
Highlights
Pulmonary alveolar microlithiasis (PAM) is an autosomal recessive disease characterized by the deposition of phosphate and calcium in the alveoli
Pulmonary alveolar microlithiasis (PAM) was first described by Harbitz in 19181. This rare disease which progresses with calcium and phosphate deposition in the alveolar space is an autosomal recessive disorder caused by the SLC34A2 gene mutation[2,3]
Moon et al reported the case of a patient who survived for 15 postoperative years with no recurrence after the lung transplantation was performed after PAM diagnosis[8]
Summary
Pulmonary alveolar microlithiasis (PAM) was first described by Harbitz in 19181. This rare disease which progresses with calcium and phosphate deposition in the alveolar space is an autosomal recessive disorder caused by the SLC34A2 gene mutation[2,3]. We present the case report of the first Turkish patient followed-up in the intensive care unit (ICU) with the diagnosis of PAM, who needed ventilator support at the time of discharge from the ICU and received lung transplantation in Austria. The chest X-ray and thoracic computed tomography (CT) taken at the ICU admission revealed bilateral, diffuse involvement (sandstorm) and decreased aeration areas of both lungs (Figure 1, Figure 2). The patient underwent bilateral lung transplantation in Vienna 14 months after initial admission (she was under oxygen and NIMV support during these 14 months). She was followed-up during the first seven postoperative days in the ICU and discharged on the 21st day from the hospital.
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