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Lymphangioleiomyomatosis: an explosive presentation of a rare disease

Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease, commonly affecting women in the reproductive age group. Exacerbation of pre-existing disease is common during pregnancy likely due to the up-regulation of estrogen and progesterone receptors present within the proliferating smooth muscle...

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Detalles Bibliográficos
Autores principales: Pais, Faye, Fayed, Mohamed, Evans, Timothy
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5461433/
https://www.ncbi.nlm.nih.gov/pubmed/28616248
http://dx.doi.org/10.1093/omcr/omx023
Descripción
Sumario:Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease, commonly affecting women in the reproductive age group. Exacerbation of pre-existing disease is common during pregnancy likely due to the up-regulation of estrogen and progesterone receptors present within the proliferating smooth muscle cells. This case highlights a dramatic presentation of LAM for the first time in pregnancy, its rapid progression during gestation, and a partial resolution with delivery. The unusual radiographic imaging in this patient, lacked the characteristic cystic lesions commonly associated with LAM, but instead demonstrated a dense interstitial pattern with micronodular expansion of the interlobular septa suggesting severe lymphatic obstruction.