{"doc_desc":{"title":"Pneumopathies Interstitielles Diffuses Idiopathiques (PID) de l\u2019enfant et de l\u2019adulte","idno":"FRESH-PEF73380-fr","producers":[{"name":"Sonia GUEGUEN","affiliation":"SORBONNE UNIVERSITE"}]},"study_desc":{"title_statement":{"idno":"FRESH-PEF73380-fr","IDno":{"metadata_no":[{"agency":"PEF","code":"73380"},{"agency":"FReSH","code":"FRESH-PEF73380"}]},"title":"Pneumopathies Interstitielles Diffuses Idiopathiques (PID) de l\u2019enfant et de l\u2019adulte","alternate_title":"RaDiCo-PID"},"study_authorization":{"agency":[{"name":"CNIL"},{"name":"CESREES"}]},"authoring_entity":[{"type":"investigator","name":"Vincent;COTTIN","PILabo":"French National Institute for Health and Medical Research (Inserm) \/ Inserm UMR754","affiliationName":"INSTITUT NATIONAL DE LA SANTE ET DE LA RECHERCHE MEDICALE (INSERM)","extlink":[{"title":"ORCID","uri":"0000-0002-5591-0955","role":"pi id"},{"title":"IdRef","uri":"166774731","role":"pi id"},{"title":"SIREN","uri":"180036048","role":"organisation id"},{"title":"RNSR","uri":"199517675N","role":"labo id"}],"email":"vincent.cottin@chu-lyon.fr","isContact":"Oui"}],"oth_id":[{"name":"R\u00e9seau Europ\u00e9en de R\u00e9f\u00e9rence ERN-LUNG \/ Fili\u00e8re de Sant\u00e9 Maladies Rares Pulmonaires RespiFil","type":"collaboration"}],"production_statement":{"prod_place":"Portail Epid\u00e9miologie France (PEF)","producers":[{"name":"INSTITUT NATIONAL DE LA SANTE ET DE LA RECHERCHE MEDICALE (INSERM)","extlink":[{"title":"ROR","uri":"https:\/\/ror.org\/02vjkv261","role":"sponsor id"},{"title":"SIREN","uri":"180036048","role":"sponsor id"}],"role":"sponsor"}],"funding_agencies":[{"name":"AGENCE NATIONALE DE LA RECHERCHE (ANR)","extlink":[{"title":"ROR","uri":"https:\/\/ror.org\/00rbzpz17"},{"title":"SIREN","uri":"130002504"}]},{"name":"","extlink":[]}]},"distribution_statement":{"contact":[{"name":"Vincent;COTTIN","email":"vincent.cottin@chu-lyon.fr","type":"contact","affiliationName":"INSTITUT NATIONAL DE LA SANTE ET DE LA RECHERCHE MEDICALE (INSERM)","contactPointLabo":"","extlink":[{"uri":"https:\/\/ror.org\/02vjkv261","role":"organisation id","title":"ROR"},{"uri":"180036048","role":"organisation id","title":"SIREN"}]}]},"study_info":{"keywords":[],"topics":[{"topic":"P\u00e9diatrie","vocab":"health theme","extLink":[{"title":"ESV","uri":"http:\/\/data.europa.eu\/8mn\/euroscivoc\/bf8aaaac-ddee-41cd-bb8a-c5a63de3fcbd"},{"title":"MeSH","uri":"http:\/\/id.nlm.nih.gov\/mesh\/D010372"}]},{"topic":"Pneumologie","vocab":"health theme","extLink":[{"title":"ESV","uri":"http:\/\/data.europa.eu\/8mn\/euroscivoc\/f6e18c52-6087-4662-904c-5c4b8174e40e"},{"title":"MeSH","uri":"http:\/\/id.nlm.nih.gov\/mesh\/D015272"}]},{"topic":"Maladies respiratoires affectant principalement l'interstitium pulmonaire","vocab":"cim-11","extLink":[{"uri":"http:\/\/id.who.int\/icd\/entity\/111658096","title":"CIM-11"}]},{"topic":"D\u00e9terminants environnementaux : Climat","vocab":"health determinant"},{"topic":"D\u00e9terminants socio-d\u00e9mographiques et \u00e9conomiques","vocab":"health determinant"},{"topic":"D\u00e9terminants biologiques : Pr\u00e9dispositions g\u00e9n\u00e9tiques","vocab":"health determinant"},{"topic":"D\u00e9terminants comportementaux","vocab":"health determinant"},{"topic":"D\u00e9terminants environnementaux","vocab":"health determinant"},{"topic":"D\u00e9terminants li\u00e9s au syst\u00e8me de sant\u00e9 : Consommation des soins","vocab":"health determinant"},{"topic":"Syst\u00e8mes de soins et acc\u00e8s aux soins","vocab":"health determinant"},{"topic":"D\u00e9terminants socio-d\u00e9mographiques et \u00e9conomiques : Emploi","vocab":"health determinant"},{"topic":"D\u00e9terminants biologiques","vocab":"health determinant"},{"topic":"D\u00e9terminants li\u00e9s au syst\u00e8me de sant\u00e9","vocab":"health determinant"}],"purpose":"Primary Objective The main objective is to describe the phenotypic features of the paediatric and adult patients with IIP\/PID, at diagnosis and during the follow-up. These data will be critical for the description of the natural history of the various forms of IIP\/PID. Secondary Objectives The secondary objectives are to: \u2022 Identify gene factors involved in disease initiation and progression, \u2022 Investigate the extent to which environmental and co-morbidity factors may influence disease severity and outcome \u2022 Identify and validate biomarkers for disease diagnosis and progression Exploratory objectives \u2022 Production of improved strategies for patient recruitment and enrolment into clinical trials \u2022 Development of novel strategy for patient follow-up \u2022 Development of novel diagnostic approaches \u2022 Evaluation of effect on natural history of disease, and tolerance of therapy, in a large population in real life \u2022 Development of novel therapeutic approaches Information Technology Objectives \u2022 Develop and diffuse an electronic tool of data collection from various sources linked to a database integrating a system of management and follow-up of data-management allowing collection of data for IIP\/PID paediatric and adult patients. \u2022 Include data generated by patients and, where relevant, their parents and\/or carers.","abstract":"","coll_dates":[{"start":"2017-01-01","end":"2021-01-01"}],"nation":[{"name":"France","abbreviation":"fr","extLink":{"vocab":"ISO","vocabURI":"fr"}}],"geog_coverage":"","analysis_unit":"Individus","universe":"{\n    \"level_sex_clusion_I\": [\n        {\n            \"value\": \"Masculin\",\n            \"concept\": {\n                \"vocab\": \"MeSH\",\n                \"vocabURI\": \"http:\\\/\\\/id.nlm.nih.gov\\\/mesh\\\/D008297\"\n            }\n        },\n        {\n            \"value\": \"F\u00e9minin\",\n            \"concept\": {\n                \"vocab\": \"MeSH\",\n                \"vocabURI\": \"http:\\\/\\\/id.nlm.nih.gov\\\/mesh\\\/D005260\"\n            }\n        }\n    ],\n    \"level_age_clusion_I\": [\n        {\n            \"value\": \"Nouveau-n\u00e9 (naissance \u00e0 28j)\",\n            \"concept\": {\n                \"vocab\": \"MeSH\",\n                \"vocabURI\": \"http:\\\/\\\/id.nlm.nih.gov\\\/mesh\\\/D007231\"\n            }\n        },\n        {\n            \"value\": \"Nourrisson (28j \u00e0 2 ans)\",\n            \"concept\": {\n                \"vocab\": \"MeSH\",\n                \"vocabURI\": \"http:\\\/\\\/id.nlm.nih.gov\\\/mesh\\\/D007223\"\n            }\n        },\n        {\n            \"value\": \"Petite enfance (2 \u00e0 5 ans)\",\n            \"concept\": {\n                \"vocab\": \"MeSH\",\n                \"vocabURI\": \"http:\\\/\\\/id.nlm.nih.gov\\\/mesh\\\/D002675\"\n            }\n        },\n        {\n            \"value\": \"Enfance (6 \u00e0 12 ans)\",\n            \"concept\": {\n                \"vocab\": \"MeSH\",\n                \"vocabURI\": \"http:\\\/\\\/id.nlm.nih.gov\\\/mesh\\\/D002648\"\n            }\n        },\n        {\n            \"value\": \"Adolescence (13 \u00e0 18 ans)\",\n            \"concept\": {\n                \"vocab\": \"MeSH\",\n                \"vocabURI\": \"http:\\\/\\\/id.nlm.nih.gov\\\/mesh\\\/D000293\"\n            }\n        },\n        {\n            \"value\": \"Adulte (19 \u00e0 24 ans)\",\n            \"concept\": {\n                \"vocab\": \"MeSH\",\n                \"vocabURI\": \"http:\\\/\\\/id.nlm.nih.gov\\\/mesh\\\/D055815\"\n            }\n        },\n        {\n            \"value\": \"Adulte (25 \u00e0 44 ans)\",\n            \"concept\": {\n                \"vocab\": \"MeSH\",\n                \"vocabURI\": \"http:\\\/\\\/id.nlm.nih.gov\\\/mesh\\\/D000328\"\n            }\n        },\n        {\n            \"value\": \"Adulte (45 \u00e0 64 ans)\",\n            \"concept\": {\n                \"vocab\": \"MeSH\",\n                \"vocabURI\": \"http:\\\/\\\/id.nlm.nih.gov\\\/mesh\\\/D008875\"\n            }\n        },\n        {\n            \"value\": \"Personne \u00e2g\u00e9e (65 \u00e0 79 ans)\",\n            \"concept\": {\n                \"vocab\": \"MeSH\",\n                \"vocabURI\": \"http:\\\/\\\/id.nlm.nih.gov\\\/mesh\\\/D000368\"\n            }\n        },\n        {\n            \"value\": \"Grand \u00e2ge (80 ans et plus)\",\n            \"concept\": {\n                \"vocab\": \"MeSH\",\n                \"vocabURI\": \"http:\\\/\\\/id.nlm.nih.gov\\\/mesh\\\/D000369\"\n            }\n        }\n    ],\n    \"level_type_clusion_I\": \"Patients\",\n    \"level_type_clusion_other\": \"\",\n    \"clusion_I\": \"Crit\u00e8res d'inclusion :      Patients ayant re\u00e7u un diagnostic d'IIP\\\/PID. Le diagnostic d'IIP\\\/PID est \u00e9tabli sur la base de l'anamn\u00e8se, des constatations cliniques, radiologiques et fonctionnelles, et si disponibles, des r\u00e9sultats pathologiques. Les crit\u00e8res d'inclusion comprennent :         Crit\u00e8res cliniques : manifestations de l'insuffisance respiratoire chronique, notamment dyspn\u00e9e\\\/tachypn\u00e9e, toux et cyanose pendant l'exercice ou au repos.         Crit\u00e8res radiologiques : anomalies caract\u00e9ristiques de la tomodensitom\u00e9trie thoracique haute r\u00e9solution (HRCT), notamment une att\u00e9nuation \u00e9tendue du verre d\u00e9poli ou alv\u00e9olaire, une r\u00e9ticulation souvent associ\u00e9e \u00e0 une bronchiectasie de traction et un aspect en rayon de miel.         Crit\u00e8res fonctionnels : anomalies des tests de fonction pulmonaire refl\u00e9tant un sch\u00e9ma restrictif, notamment perte de volume pulmonaire, capacit\u00e9 vitale (VC), capacit\u00e9 pulmonaire totale (TLC) ; r\u00e9duction de la capacit\u00e9 de diffusion pulmonaire pour le monoxyde de carbone (DLCO), anomalies de l'\u00e9change gazeux et alt\u00e9ration de la r\u00e9ponse ventilatoire \u00e0 l'exercice.      Patients (parents\\\/tuteurs pour les patients p\u00e9diatriques) ayant donn\u00e9 leur consentement \u00e9clair\u00e9 pour participer au protocole.      Patients affili\u00e9s au \\\"R\u00e9gime National d'Assurance Maladie\\\".  \",\n    \"clusion_E\": \"Patients atteints de maladies pulmonaires parenchymateuses diffuses caus\u00e9es par la toxicit\u00e9 m\u00e9dicamenteuse, une immunod\u00e9ficience, des troubles prolif\u00e9ratifs incluant l'histiocytose et des troubles m\u00e9taboliques     Patients (parents\\\/tuteurs pour les patients p\u00e9diatriques) incapables d'approuver\\\/comprendre le protocole\"\n}","data_kind":"['Donn\u00e9es cliniques','Donn\u00e9es rapport\u00e9es par le participant de l'\u00e9tude','Donn\u00e9es paracliniques','Donn\u00e9es biologiques']","quality_statement":{"standards":[{"name":"['HPO, ICD10, Snomed CT, Codes Orpha et ORDO, Dictionnaire des m\u00e9dicaments (DCIs).']","committee":"","governance":""}],"other_quality_statement":"['Gestion continue des donn\u00e9es ; Plan de gestion des donn\u00e9es et Plan de validation des donn\u00e9es. Contr\u00f4les natifs et syst\u00e8me de queries']"}},"study_development":{"development_activity":[{"activity_type":"primary evaluation","activity_description":"Ev\u00e9nements de sant\u00e9\/morbidit\u00e9\r\nEv\u00e9nements de sant\u00e9\/mortalit\u00e9\r\nConsommation de soins\/services de sant\u00e9\r\nQualit\u00e9 de vie\/sant\u00e9 per\u00e7ue\r\nAutres"}]},"method":{"data_collection":{"time_method":"","frequency":"","sampling_procedure":"['{\"concept\":{\"vocabURI\":\"Other\",\"vocab\":\"CESSDA\"},\"value\":\"Autre\"}']","sample_frame":{"frame_unit":{"unit_type":"['Via des structures (services ou \u00e9tablissements de sant\u00e9, \u00e9coles, entreprises\u2026)']"}},"coll_mode":["{\"concept\":{\"vocabURI\":\"Transcription\",\"vocab\":\"CESSDA\"},\"value\":\"Transcription et saisie d\u2019informations dans un enregistrement structur\u00e9\"}"],"research_instrument":"Acc\u00e8s restreint sur projet sp\u00e9cifique","sources":[{"sourceCitation":{"titlStmt":{"titl":""},"holdings":"","notes":{"subject":"source purpose","value":""}},"srcOrig":["Base m\u00e9dico-administrative"]}],"target_sample_size":"[1000-10000[ individus","response_rate":"2550"},"method_notes":"Etude observationnelle","study_class":"Inconnu","notes":[{"subject":"research type","values":["Etude observationnelle"]},{"subject":"observational study method","values":["Registre de morbidit\u00e9"]}]},"data_access":{"dataset_availability":{"access_place":"","complete":"","status":"{\"value\":\"Acc\u00e8s r\u00e9serv\u00e9\",\"extLink\":[{\"title\":\"COAR\",\"uri\":\"http:\/\/purl.org\/coar\/access_right\/c_16ec\"}]}"},"dataset_use":{"restrictions":"","conditions":"","conf_dec":[{"txt":""}],"spec_perm":[{"txt":"","required":""}],"contact":[{"name":""}],"deposit_req":"","cit_req":""},"notes":""}},"additional":{"IsImport":true,"versionLang":"fr","originLang":"fr","autoTranslation":false,"status":"imported","creationDate":"03-01-2017","lastUpdatedAuto":null,"lastUpdatedManual":"03-12-2024","isContributorPI":"Non","contributorName":"Sonia GUEGUEN","contributorAffiliation":"SORBONNE UNIVERSITE","addTeamMember":"","obtainedAuthorization":{"otherAuthorizingAgency":["",""]},"relatedDocument":[],"regulatoryRequirements":{"conformityDeclaration":""},"fundingAgent":{"fundingAgentType":["Public (France)","Industrie"],"otherFundingAgentType":["",""]},"sponsor":{"sponsorType":["Public (France)"],"otherSponsorType":[""]},"governance":{"committee":"Oui"},"collaborations":{"networkConsortium":"Oui"},"theme":{"complementaryInformation":"","RareDiseases":"Oui"},"activeFollowUp":{"isActiveFollowUp":"Oui","followUpModeOther":""},"interventionalStudy":{"researchPurpose":[],"trialPhase":[],"interventionalStudyModel":"","isClinicalTrial":"","otherResearchPurpose":""},"isInclusionGroups":"","allocation":{"allocationMode":"","allocationUnit":""},"masking":{"maskingType":"","blindedMaskingDetails":[]},"arms":[],"intervention":[],"inclusionGroups":[],"collectionProcess":{"collectionModeDetails":"eCRF utilisant REDCap ; Plateforme bas\u00e9e sur le cloud, s\u00e9curis\u00e9e par conception, accessible via le web. Ressource certifi\u00e9e d'h\u00e9bergement de donn\u00e9es de sant\u00e9","collectionModeOther":""},"dataCollection":{"inclusionStrategyOther":"","samplingModeOther":"","recruitmentSourceOther":"","otherDocumentation":""},"dataCollectionIntegration":{"isDataIntegration":"Oui"},"geographicalCoverage":{"geoDetail":"Couverture nationale compl\u00e8te \u00e0 travers le r\u00e9seau des centres de r\u00e9f\u00e9rence et de comp\u00e9tence pour les maladies pulmonaires rares"},"dataTypes":{"clinicalDataDetails":"les principales variables collect\u00e9es comprennent des aspects d\u00e9mographiques, des ant\u00e9c\u00e9dents familiaux, des r\u00e9sultats d'examens cliniques, des donn\u00e9es environnementales, des d\u00e9tails socio-professionnels, des ant\u00e9c\u00e9dents m\u00e9dicaux des patients, des caract\u00e9ristiques anatomopathologiques, des examens biologiques, microbiologiques et d'imagerie. Les deux \u00e9tudes impliquent \u00e9galement des endoscopies bronchiques, des mesures de la fonction respiratoire, le suivi de l'\u00e9volution de la maladie, la collecte de donn\u00e9es de fertilit\u00e9, des aspects g\u00e9n\u00e9tiques, le recueil des traitements, des questionnaires sur la qualit\u00e9 de vie, et l'inclusion d'informations sur l'\u00e9pisode de Covid-19.","biologicalDataDetails":"Saisie des donn\u00e9es des r\u00e9sultats biologiques (h\u00e9matologie, biochimie, immunit\u00e9, s\u00e9rologie) ; Saisie des donn\u00e9es pour : bact\u00e9riologie, virologie, parasitologie, mycologie, bronchoscopie, examen par lavage broncho-alv\u00e9olaire ; Saisie des donn\u00e9es des tests de la fonction pulmonaire, des gaz du sang art\u00e9riel et de la spirom\u00e9trie (si disponibles, \u00e9changes gazeux nocturnes et polysomnographie) ; Si disponible\/r\u00e9alis\u00e9, saisie des donn\u00e9es des examens tissulaires pulmonaires, des biopsies pulmonaires (chirurgicales, transbronchiques), des explants pulmonaires ; Si disponible\/r\u00e9alis\u00e9, saisie des donn\u00e9es de l'\u00e9valuation de la fonction d'autres organes (y compris les examens digestifs et cardiaques).","isDataInBiobank":"Oui","biobankContent":[],"biobankContentOther":"","dataTypeOther":""},"variableDictionnary":{"variableDictionnaryAvailable":"","variableDictionnaryLink":""},"dataQuality":{"otherDocumentation":""},"mockSample":{"mockSampleAvailable":"","mockSampleLocation":""},"thirdPartySource":{"otherSourceType":[""]},"isHealthTheme":"Oui","fileDscr":{"fileTxt":{"fileCitation":{"titlStmt":{"IDno":[]}}}}},"schematype":"survey"}