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Trasplante pulmonar en la linfangioleiomiomatosis esporádica. Estudio de 7 casos.

Fecha de publicación:

Autores de IIS La Fe

  • Emilio Ansótegui Barrera

    Autor

  • Juan Carlos Peñalver Cuesta

    Autor

  • Francisco José Vera Sempere

    Autor

  • Jose Diego Padilla Alarcon

    Autor

Abstract

Sporadic lymphangioleiomyomatosis (S-LAM) is a rare disease that affects only women. It is characterized by an abnormal proliferation of immature smooth muscle cells (LAM cells) that grow in an aberrant manner in the airway, parenchymal lung lymph and blood vessels, determining the onset of pulmonary cystic lesions. The disease has no treatment, progressing to respiratory failure, and lung transplantation (LT) may be a treatment option at this stage. Our goal was to study 7 patients undergoing LT for S-LAM.

Datos de la publicación

ISSN/ISSNe:
0025-7753, 1578-8989

MEDICINA CLINICA  ELSEVIER ESPANA SLU

Tipo:
Abstract of Published Item
Páginas:
349-352
PubMed:
23937818
Factor de Impacto:
0,254 SCImago ℠
Cuartil:
Q3 SCImago ℠

Citas Recibidas en Web of Science: 3

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Keywords

  • Enfermedades raras, Linfangioleiomiomatosis, Lung transplant, Lymphangioleiomyomatosis, Rare diseases, Trasplante pulmonar

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