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Pulmonary hypertension associated with neurofibromatosis type 1

Etienne-Marie Jutant, Barbara Girerd, Xavier Jaïs, Laurent Savale, Caroline O'Connell, Frederic Perros, Olivier Sitbon, Marc Humbert, David Montani
European Respiratory Review 2018 27: 180053; DOI: 10.1183/16000617.0053-2018
Etienne-Marie Jutant
1Université Paris–Sud, Faculté de Médecine, Paris, France
2AP-HP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Dépt Hospitalo-Universitaire (DHU) Thorax Innovation (TORINO), Service de Pneumologie, Hôpital de Bicêtre, Paris, France
3UMR_S 999, Univ. Paris–Sud, INSERM, Laboratoire d'Excellence (LabEx) en Recherche sur le Médicament et l'Innovation Thérapeutique (LERMIT), Hôpital Marie-Lannelongue, Paris, France
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Barbara Girerd
1Université Paris–Sud, Faculté de Médecine, Paris, France
2AP-HP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Dépt Hospitalo-Universitaire (DHU) Thorax Innovation (TORINO), Service de Pneumologie, Hôpital de Bicêtre, Paris, France
3UMR_S 999, Univ. Paris–Sud, INSERM, Laboratoire d'Excellence (LabEx) en Recherche sur le Médicament et l'Innovation Thérapeutique (LERMIT), Hôpital Marie-Lannelongue, Paris, France
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Xavier Jaïs
1Université Paris–Sud, Faculté de Médecine, Paris, France
2AP-HP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Dépt Hospitalo-Universitaire (DHU) Thorax Innovation (TORINO), Service de Pneumologie, Hôpital de Bicêtre, Paris, France
3UMR_S 999, Univ. Paris–Sud, INSERM, Laboratoire d'Excellence (LabEx) en Recherche sur le Médicament et l'Innovation Thérapeutique (LERMIT), Hôpital Marie-Lannelongue, Paris, France
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Laurent Savale
1Université Paris–Sud, Faculté de Médecine, Paris, France
2AP-HP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Dépt Hospitalo-Universitaire (DHU) Thorax Innovation (TORINO), Service de Pneumologie, Hôpital de Bicêtre, Paris, France
3UMR_S 999, Univ. Paris–Sud, INSERM, Laboratoire d'Excellence (LabEx) en Recherche sur le Médicament et l'Innovation Thérapeutique (LERMIT), Hôpital Marie-Lannelongue, Paris, France
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Caroline O'Connell
4Service de Chirurgie Thoracique, Vasculaire et Transplantation Pulmonaire, Hôpital Marie-Lannelongue, Paris, France
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Frederic Perros
1Université Paris–Sud, Faculté de Médecine, Paris, France
3UMR_S 999, Univ. Paris–Sud, INSERM, Laboratoire d'Excellence (LabEx) en Recherche sur le Médicament et l'Innovation Thérapeutique (LERMIT), Hôpital Marie-Lannelongue, Paris, France
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Olivier Sitbon
1Université Paris–Sud, Faculté de Médecine, Paris, France
2AP-HP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Dépt Hospitalo-Universitaire (DHU) Thorax Innovation (TORINO), Service de Pneumologie, Hôpital de Bicêtre, Paris, France
3UMR_S 999, Univ. Paris–Sud, INSERM, Laboratoire d'Excellence (LabEx) en Recherche sur le Médicament et l'Innovation Thérapeutique (LERMIT), Hôpital Marie-Lannelongue, Paris, France
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Marc Humbert
1Université Paris–Sud, Faculté de Médecine, Paris, France
2AP-HP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Dépt Hospitalo-Universitaire (DHU) Thorax Innovation (TORINO), Service de Pneumologie, Hôpital de Bicêtre, Paris, France
3UMR_S 999, Univ. Paris–Sud, INSERM, Laboratoire d'Excellence (LabEx) en Recherche sur le Médicament et l'Innovation Thérapeutique (LERMIT), Hôpital Marie-Lannelongue, Paris, France
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David Montani
1Université Paris–Sud, Faculté de Médecine, Paris, France
2AP-HP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Dépt Hospitalo-Universitaire (DHU) Thorax Innovation (TORINO), Service de Pneumologie, Hôpital de Bicêtre, Paris, France
3UMR_S 999, Univ. Paris–Sud, INSERM, Laboratoire d'Excellence (LabEx) en Recherche sur le Médicament et l'Innovation Thérapeutique (LERMIT), Hôpital Marie-Lannelongue, Paris, France
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Article Information

vol. 27 no. 149
DOI 
https://doi.org/10.1183/16000617.0053-2018
PubMed 
30158278

Published By 
European Respiratory Society
Print ISSN 
0905-9180
Online ISSN 
1600-0617
History 
  • Received June 2, 2018
  • Accepted July 28, 2018
  • Published online August 29, 2018.

Copyright & Usage 
Copyright ©ERS 2018. ERR articles are open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.

Author Information

  1. Etienne-Marie Jutant1,2,3,
  2. Barbara Girerd1,2,3,
  3. Xavier Jaïs1,2,3,
  4. Laurent Savale1,2,3,
  5. Caroline O'Connell4,
  6. Frederic Perros1,3,
  7. Olivier Sitbon1,2,3,
  8. Marc Humbert1,2,3 and
  9. David Montani1,2,3
  1. 1Université Paris–Sud, Faculté de Médecine, Paris, France
  2. 2AP-HP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Dépt Hospitalo-Universitaire (DHU) Thorax Innovation (TORINO), Service de Pneumologie, Hôpital de Bicêtre, Paris, France
  3. 3UMR_S 999, Univ. Paris–Sud, INSERM, Laboratoire d'Excellence (LabEx) en Recherche sur le Médicament et l'Innovation Thérapeutique (LERMIT), Hôpital Marie-Lannelongue, Paris, France
  4. 4Service de Chirurgie Thoracique, Vasculaire et Transplantation Pulmonaire, Hôpital Marie-Lannelongue, Paris, France
  1. David Montani, Service de pneumologie, Centre de Référence de l'hypertension Pulmonaire, CHU de Bicêtre, 78 rue du Général Leclerc, 94275 Le Kremlin Bicetre, France. E-mail: david.montani{at}aphp.fr
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Vol 27 Issue 149 Table of Contents
European Respiratory Review: 27 (149)
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Pulmonary hypertension associated with neurofibromatosis type 1
Etienne-Marie Jutant, Barbara Girerd, Xavier Jaïs, Laurent Savale, Caroline O'Connell, Frederic Perros, Olivier Sitbon, Marc Humbert, David Montani
European Respiratory Review Sep 2018, 27 (149) 180053; DOI: 10.1183/16000617.0053-2018

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Pulmonary hypertension associated with neurofibromatosis type 1
Etienne-Marie Jutant, Barbara Girerd, Xavier Jaïs, Laurent Savale, Caroline O'Connell, Frederic Perros, Olivier Sitbon, Marc Humbert, David Montani
European Respiratory Review Sep 2018, 27 (149) 180053; DOI: 10.1183/16000617.0053-2018
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  • Article
    • Abstract
    • Abstract
    • Introduction
    • Clinical presentation of NF1
    • Genetics, biology and pathophysiology in NF1
    • Pulmonary hypertension
    • Pathophysiology of PH in NF1
    • Treatment of PH associated with NF1
    • Interstitial lung disease and thoracic complications
    • Conclusion
    • Footnotes
    • References
  • Figures & Data
  • Info & Metrics
  • PDF

Subjects

  • Pulmonary vascular disease
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Series

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Group 5 Pulmonary Hypertension

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