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Pulmonary hemosiderosis in children with Down syndrome: a national experience

dc.rights.licenseopenen_US
dc.contributor.authorALIMI, A.
dc.contributor.authorTAYTARD, J.
dc.contributor.authorABOU TAAM, R.
dc.contributor.authorHOUDOUIN, V.
dc.contributor.authorFORGERON, A.
dc.contributor.authorLUBRANO LAVADERA, M.
dc.contributor.authorCROS, P.
dc.contributor.authorGIBERTINI, I.
dc.contributor.authorDERELLE, J.
dc.contributor.authorDESCHILDRE, A.
dc.contributor.authorTHUMERELLE, C.
dc.contributor.authorEPAUD, R.
dc.contributor.authorREIX, P.
hal.structure.identifierBordeaux population health [BPH]
dc.contributor.authorFAYON, Michael
dc.contributor.authorROULLAUD, S.
dc.contributor.authorTROUSSIER, F.
dc.contributor.authorRENOUX, M. C.
dc.contributor.authorDE BLIC, J.
dc.contributor.authorLEYRONNAS, S.
dc.contributor.authorTHOUVENIN, G.
dc.contributor.authorPERISSON, C.
dc.contributor.authorRAVEL, A.
dc.contributor.authorCLEMENT, A.
dc.contributor.authorCORVOL, H.
dc.contributor.authorNATHAN, N.
dc.date.accessioned2020-10-19T07:32:12Z
dc.date.available2020-10-19T07:32:12Z
dc.date.issued2018-04-20
dc.identifier.issn1750-1172 (Electronic) 1750-1172 (Linking)en_US
dc.identifier.urihttps://oskar-bordeaux.fr/handle/20.500.12278/11393
dc.description.abstractEnBACKGROUND: Pulmonary hemosiderosis is a rare and complex disease in children. A previous study from the French RespiRare(R) network led to two important findings: 20% of the children presented with both pulmonary hemosiderosis and Down syndrome (DS), and at least one tested autoantibody was found positive in 50%. This study investigates the relationships between pulmonary hemosiderosis and DS. METHODS: Patients younger than 20 years old and followed for pulmonary hemosiderosis were retrieved from the RespiRare(R) database. Clinical, biological, functional, and radiological findings were collected, and DS and non-DS patients' data were compared. RESULTS: A total of 34 patients (22 girls and 12 boys) were included, among whom nine (26%) presented with DS. The mean age at diagnosis was 4.1 +/- 3.27 years old for non-DS and 2.9 +/- 3.45 years old for DS patients. DS patients tended to present a more severe form of the disease with an earlier onset, more dyspnoea at diagnosis, more frequent secondary pulmonary hypertension, and an increased risk of fatal evolution. CONCLUSIONS: DS patients have a higher risk of developing pulmonary hemosiderosis, and the disease seems to be more severe in this population. This could be due to the combination of an abnormal lung capillary bed with fragile vessels, a higher susceptibility to autoimmune lesions, and a higher risk of evolution toward pulmonary hypertension. A better screening for pulmonary hemosiderosis and a better prevention of hypoxia in DS paediatric patients may prevent a severe evolution of the disease.
dc.language.isoENen_US
dc.rightsAttribution 3.0 United States*
dc.rights.urihttp://creativecommons.org/licenses/by/3.0/us/*
dc.title.enPulmonary hemosiderosis in children with Down syndrome: a national experience
dc.title.alternativeOrphanet J Rare Disen_US
dc.typeArticle de revueen_US
dc.identifier.doi10.1186/s13023-018-0806-6en_US
dc.subject.halSciences du Vivant [q-bio]/Santé publique et épidémiologieen_US
dc.identifier.pubmed29678139en_US
bordeaux.journalOrphanet journal of rare diseasesen_US
bordeaux.page60en_US
bordeaux.volume13en_US
bordeaux.hal.laboratoriesBordeaux Population Health Research Center (BPH) - U1219en_US
bordeaux.issue1en_US
bordeaux.institutionUniversité de Bordeauxen_US
bordeaux.peerReviewedouien_US
bordeaux.inpressnonen_US
hal.exportfalse
bordeaux.COinSctx_ver=Z39.88-2004&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.jtitle=Orphanet%20journal%20of%20rare%20diseases&rft.date=2018-04-20&rft.volume=13&rft.issue=1&rft.spage=60&rft.epage=60&rft.eissn=1750-1172%20(Electronic)%201750-1172%20(Linking)&rft.issn=1750-1172%20(Electronic)%201750-1172%20(Linking)&rft.au=ALIMI,%20A.&TAYTARD,%20J.&ABOU%20TAAM,%20R.&HOUDOUIN,%20V.&FORGERON,%20A.&rft.genre=article


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