Matches in SemOpenAlex for { <https://semopenalex.org/work/W2070292641> ?p ?o ?g. }
Showing items 1 to 79 of
79
with 100 items per page.
- W2070292641 endingPage "131" @default.
- W2070292641 startingPage "127" @default.
- W2070292641 abstract "La fibrose pulmonaire idiopathique (FPI) familiale est une maladie très rare, progressivement fatale, dont la pathogénie, incomplètement élucidée, fait intervenir des lésions des cellules alvéolaires épithéliales dont l’origine pourrait être immunologique, microbiologique ou chimique, aboutissant à une cicatrisation fibrosante. Un terrain génétique prédisposant a été démontré. Nous rapportons l’observation d’une patiente ayant un frère décédé à l’âge de 29 ans suite à une FPI. Elle est impubère, ayant une épidermodysplasie verruciforme depuis l’enfance, développe à l’âge de 31 ans une pneumopathie interstitielle diffuse dont la biopsie chirurgicale fait le diagnostic de FPI. Les explorations endocriniennes trouvent un hypogonadisme hypogonadotrope, une hypothyroïdie périphérique et une selle turcique vide à l’imagerie par résonance magnétique. Cette association entre FPI familiale, polyendocrinopathie auto-immune et dermatose d’origine génétique due à un déficit de l’immunité cellulaire, renforce l’hypothèse d’un dysfonctionnement immunitaire dans la pathogénie de la FPI. Familial idiopathic pulmonary fibrosis (IPF) is a very rare and progressively fatal disease. Its pathogenesis is not fully understood and involves damage to alveolar epithelial cells of possibly immunological, microbiological or chemical origin, leading to fibrosing healing. A genetic predisposition has been demonstrated. The authors report the case of a female patient whose brother died at the age of 29 from IPF. She had epidermodysplasia verruciformis since childhood, with the absence of pubertal development. At the age of 31, she presented diffuse interstitial pneumonia. A lung biopsy confirmed the diagnosis of IPF. Endocrine explorations detected hypogonadotropic hypogonadism, primary hypothyroidism and magnetic resonance imaging revealed an empty sella turcica. The association of familial IPF, autoimmune polyendocrinopathy and genetic dermatosis caused by a cellular immune deficiency supports the hypothesis of an immune dysfunction in the pathogenesis of IPF." @default.
- W2070292641 created "2016-06-24" @default.
- W2070292641 creator A5002054165 @default.
- W2070292641 creator A5005780211 @default.
- W2070292641 creator A5010620515 @default.
- W2070292641 creator A5041918760 @default.
- W2070292641 creator A5045409122 @default.
- W2070292641 creator A5051069724 @default.
- W2070292641 creator A5064241321 @default.
- W2070292641 creator A5070938303 @default.
- W2070292641 creator A5088795165 @default.
- W2070292641 date "2010-04-01" @default.
- W2070292641 modified "2023-09-27" @default.
- W2070292641 title "Fibrose pulmonaire idiopathique familiale associée à une polyendocrinopathie auto-immune et à une épidermodysplasie verruciforme" @default.
- W2070292641 cites W1490750046 @default.
- W2070292641 cites W1970078758 @default.
- W2070292641 cites W1974638682 @default.
- W2070292641 cites W1991407692 @default.
- W2070292641 cites W2028707212 @default.
- W2070292641 cites W2032414925 @default.
- W2070292641 cites W2050634855 @default.
- W2070292641 cites W2059935397 @default.
- W2070292641 cites W2061497263 @default.
- W2070292641 cites W2079266996 @default.
- W2070292641 cites W2108543831 @default.
- W2070292641 cites W2108683967 @default.
- W2070292641 cites W2115176808 @default.
- W2070292641 cites W2120997345 @default.
- W2070292641 cites W2132298700 @default.
- W2070292641 cites W2144017344 @default.
- W2070292641 cites W2150448842 @default.
- W2070292641 cites W2152815838 @default.
- W2070292641 cites W2157753406 @default.
- W2070292641 cites W2315096416 @default.
- W2070292641 cites W2412450684 @default.
- W2070292641 doi "https://doi.org/10.1016/j.pneumo.2009.10.001" @default.
- W2070292641 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/20413048" @default.
- W2070292641 hasPublicationYear "2010" @default.
- W2070292641 type Work @default.
- W2070292641 sameAs 2070292641 @default.
- W2070292641 citedByCount "2" @default.
- W2070292641 countsByYear W20702926412014 @default.
- W2070292641 countsByYear W20702926412015 @default.
- W2070292641 crossrefType "journal-article" @default.
- W2070292641 hasAuthorship W2070292641A5002054165 @default.
- W2070292641 hasAuthorship W2070292641A5005780211 @default.
- W2070292641 hasAuthorship W2070292641A5010620515 @default.
- W2070292641 hasAuthorship W2070292641A5041918760 @default.
- W2070292641 hasAuthorship W2070292641A5045409122 @default.
- W2070292641 hasAuthorship W2070292641A5051069724 @default.
- W2070292641 hasAuthorship W2070292641A5064241321 @default.
- W2070292641 hasAuthorship W2070292641A5070938303 @default.
- W2070292641 hasAuthorship W2070292641A5088795165 @default.
- W2070292641 hasConcept C142724271 @default.
- W2070292641 hasConcept C71924100 @default.
- W2070292641 hasConceptScore W2070292641C142724271 @default.
- W2070292641 hasConceptScore W2070292641C71924100 @default.
- W2070292641 hasIssue "2" @default.
- W2070292641 hasLocation W20702926411 @default.
- W2070292641 hasLocation W20702926412 @default.
- W2070292641 hasOpenAccess W2070292641 @default.
- W2070292641 hasPrimaryLocation W20702926411 @default.
- W2070292641 hasRelatedWork W1506200166 @default.
- W2070292641 hasRelatedWork W1995515455 @default.
- W2070292641 hasRelatedWork W2048182022 @default.
- W2070292641 hasRelatedWork W2080531066 @default.
- W2070292641 hasRelatedWork W2604872355 @default.
- W2070292641 hasRelatedWork W2748952813 @default.
- W2070292641 hasRelatedWork W2899084033 @default.
- W2070292641 hasRelatedWork W3031052312 @default.
- W2070292641 hasRelatedWork W3032375762 @default.
- W2070292641 hasRelatedWork W3108674512 @default.
- W2070292641 hasVolume "66" @default.
- W2070292641 isParatext "false" @default.
- W2070292641 isRetracted "false" @default.
- W2070292641 magId "2070292641" @default.
- W2070292641 workType "article" @default.