Combined pulmonary fibrosis and emphysema syndrome in connective tissue disease
✍ Scribed by Vincent Cottin; Hilario Nunes; Luc Mouthon; Delphine Gamondes; Romain Lazor; Eric Hachulla; Didier Revel; Dominique Valeyre; Jean-François Cordier; Groupe d'Etudes et de Recherche sur les Maladies “Orphelines” Pulmonaires
- Publisher
- John Wiley and Sons
- Year
- 2010
- Tongue
- English
- Weight
- 213 KB
- Volume
- 63
- Category
- Article
- ISSN
- 0004-3591
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Pulmonary disease is a common presenting feature and complication of T-cell immunodeficiency. We retrospectively reviewed 15 children with severe combined immune deficiency (SCID) and 19 children with DiGeorge syndrome at the time of their first presentation to the Royal Children's Hospital in the 1
## Abstract ## Objective Most of the data about the prevalence of pulmonary arterial hypertension (PAH) are from tertiary centers that are biased toward seeing more severe cases; therefore, the true prevalence of PAH among patients with connective tissue disease is unknown. We sought to determine