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Pulmonary Manifestations of Systemic Disease: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease II |

Life-Threatening Multi-Level Airway Stenosis Due to Myhre Syndrome: A Case Report

Daniel Alape, MD; Erik Folch, MD; Sebastian Fernandez-Bussy, MD; Alejandro Folch, MD; Adnan Majid, MD
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Division of Thoracic Surgery and Interventional Pulmonology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2016;150(4_S):1099A. doi:10.1016/j.chest.2016.08.1206
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SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease II

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Myhre syndrome (MS) is characterized by cognitive disability, constrictive pericarditis, laryngotracheal stenosis, prognathism, tapered fingers, joint tenderness and short stature.1 MS has been related with SMAD4 gene mutations. This gene encodes for the SMAD4 protein that is the central node in the TFGβ-/BMP pathway. In fibroblasts, this alteration results in matrix metalloproteinase overexpression and impaired matrix deposition leading to abnormal scarring.2

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