A rare indication for lung transplantation - pulmonary alveolar microlithiasis: institutional experience of five consecutive cases.
Clin Transplant
; 30(4): 429-34, 2016 Apr.
Article
em En
| MEDLINE
| ID: mdl-26841075
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.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Calcinose
/
Transplante de Pulmão
/
Doenças Genéticas Inatas
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Pneumopatias
Tipo de estudo:
Observational_studies
/
Prognostic_studies
Limite:
Adolescent
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Adult
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Female
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Humans
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Male
/
Middle aged
Idioma:
En
Revista:
Clin Transplant
Assunto da revista:
TRANSPLANTE
Ano de publicação:
2016
Tipo de documento:
Article
País de afiliação:
Áustria