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Updated: Mar 26, 2026
Development of Obliterative Bronchiolitis in a Murine Model of Orthotopic Lung Transplantation
Published on: July 10, 2012
A rare indication for lung transplantation - pulmonary alveolar microlithiasis: institutional experience of five
Thomas Klikovits1, Alexis Slama1, Konrad Hoetzenecker1
1Division of Thoracic Surgery, Department of Surgery, Medical University of Vienna, Vienna, Austria.
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.
Insights
Lung transplantation (LuTX) is a viable treatment for end-stage Pulmonary Alveolar Microlithiasis (PAM). This study shows good post-operative outcomes, suggesting LuTX is a feasible option for PAM patients.
Area of Science:
- Pulmonology
- Thoracic Surgery
- Transplantation Medicine
Background:
- Pulmonary alveolar microlithiasis (PAM) is a rare condition characterized by calcifications in the lung's air sacs.
- Lung transplantation (LuTX) is currently the only effective treatment for end-stage PAM.
Purpose of the Study:
- To evaluate the feasibility and outcomes of lung transplantation in patients with end-stage Pulmonary Alveolar Microlithiasis.
- To assess the perioperative and long-term results of LuTX for PAM.
Main Methods:
- A retrospective chart review was conducted for patients undergoing LuTX between 1989 and 2013.
- Five consecutive patients diagnosed with PAM were identified and their data analyzed.
Main Results:
- Four females and one male, with a mean age of 46.3 years, underwent LuTX.
- Extracorporeal membrane oxygenation (ECMO) was required in four cases intraoperatively and one post-operatively.
- Four out of five patients survived with normal follow-up parameters; no recurrence of PAM was observed. One patient required re-transplantation and died from sepsis, while another experienced reperfusion edema.
Conclusions:
- Lung transplantation is a feasible therapeutic option for patients suffering from end-stage Pulmonary Alveolar Microlithiasis.
- Post-operative results for LuTX in PAM patients are comparable to those for other lung disease indications.
