title: A rare indication for lung transplantation - pulmonary alveolar microlithiasis: institutional experience of five consecutive cases.
abstract: BACKGROUND
  Pulmonary alveolar microlithiasis (PAM) is a rare lung disease caused by calcifications within the alveolar space. The only known effective treatment for an end-stage PAM is lung transplantation (LuTX).
  
  
  METHODS
  We performed a retrospective chart review of all individuals that underwent lung transplantation at our center between 1989 and 2013. Five consecutive patients with PAM were identified.
  
  
  RESULTS
  Four females and one male with a mean age of 46.3 yr were identified. Extracorporeal membrane oxygenation (ECMO) support was required intraoperatively in four cases and post-operatively in one case. Mean post-operative intubation time was 3.3 (range, 2-5) d and mean intensive care unit (ICU) stay was 8.3 (range, 4-12) d. No intraoperative complications were observed. One early patient (operated in 1995) underwent acute re-transplantation on the second post-operative day (POD) and died from sepsis on the 11 POD. In one patient reperfusion edema was observed requiring a prolonged weaning process. No other severe perioperative complications were observed. Four of five patients are currently still alive with normal follow-up parameters. No recurrence of PAM was observed.
  
  
  CONCLUSIONS
  Lung transplantation is a feasible therapy option in patients with end-stage PAM showing good post-operative results comparable to other indications for LuTX.
authors: Division of Thoracic Surgery, Department of Surgery, Medical University of Vienna, Vienna, Austria.
authors: Thomas
authors: T
authors: Klikovits
authors: Division of Thoracic Surgery, Department of Surgery, Medical University of Vienna, Vienna, Austria.
authors: Alexis
authors: A
authors: Slama
authors: Division of Thoracic Surgery, Department of Surgery, Medical University of Vienna, Vienna, Austria.
authors: Konrad
authors: K
authors: Hoetzenecker
authors: Division of Thoracic Surgery, Department of Surgery, Medical University of Vienna, Vienna, Austria.
authors: Ryuichi
authors: R
authors: Waseda
authors: Division of Thoracic Surgery, Department of Surgery, Medical University of Vienna, Vienna, Austria.
authors: Christopher
authors: C
authors: Lambers
authors: Division of Thoracic Surgery, Department of Surgery, Medical University of Vienna, Vienna, Austria.
authors: Gabriella
authors: G
authors: Murakoezy
authors: Division of Thoracic Surgery, Department of Surgery, Medical University of Vienna, Vienna, Austria.
authors: Peter
authors: P
authors: Jaksch
authors: Division of Thoracic Surgery, Department of Surgery, Medical University of Vienna, Vienna, Austria.
authors: Clemens
authors: C
authors: Aigner
authors: Division of Thoracic Surgery, Department of Surgery, Medical University of Vienna, Vienna, Austria.
authors: Shahrokh
authors: S
authors: Taghavi
authors: Division of Thoracic Surgery, Department of Surgery, Medical University of Vienna, Vienna, Austria.
authors: Walter
authors: W
authors: Klepetko
authors: Division of Thoracic Surgery, Department of Surgery, Medical University of Vienna, Vienna, Austria.|Department of Thoracic Surgery, Semmelweis University Budapest, Budapest, Hungary.
authors: Gyoergy
authors: G
authors: Lang
authors: Division of Thoracic Surgery, Department of Surgery, Medical University of Vienna, Vienna, Austria.
authors: Mir Alireza
authors: MA
authors: Hoda
mesh_terms: D000293:Adolescent; D000328:Adult; D002114:Calcinosis / Q000601:surgery*; D015199:Extracorporeal Membrane Oxygenation; D005240:Feasibility Studies; D005260:Female; D005500:Follow-Up Studies; D030342:Genetic Diseases, Inborn / Q000601:surgery*; D006085:Graft Survival; D006801:Humans; D008171:Lung Diseases / Q000601:surgery*; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D055815:Young Adult
keywords: extracorporeal membrane oxygenation; lung transplantation; outcome; pulmonary alveolar microlithiasis