{"doc_desc":{"title":"Registre de l'Histiocytose Langerhansienne (registre qualifi\u00e9)","idno":"FRESH-PEF236-fr","producers":[{"name":"Jean DONADIEU","affiliation":"ASSISTANCE PUBLIQUE HOPITAUX DE PARIS (AP-HP)"}]},"study_desc":{"title_statement":{"idno":"FRESH-PEF236-fr","IDno":{"metadata_no":[{"agency":"PEF","code":"236"},{"agency":"FReSH","code":"FRESH-PEF236"}]},"title":"Registre de l'Histiocytose Langerhansienne (registre qualifi\u00e9)","alternate_title":""},"study_authorization":{"agency":[{"name":"CNIL"},{"name":"CESREES"}]},"authoring_entity":[{"type":"investigator","name":"Jean;DONADIEU","PILabo":"Hopital Trousseau \/ Service d\u2019H\u00e9mato Oncologie P\u00e9diatrique","affiliationName":"ASSISTANCE PUBLIQUE HOPITAUX DE PARIS (AP-HP)","extlink":[{"title":"ORCID","uri":"0000-0002-4485-146X","role":"pi id"},{"title":"IdRef","uri":"073435910","role":"pi id"},{"title":"SIREN","uri":"267500452","role":"organisation id"},{"title":"","uri":"","role":"labo id"}],"email":"jean.donadieu@aphp.fr","isContact":"Oui"}],"oth_id":[{"name":""}],"production_statement":{"prod_place":"Portail Epid\u00e9miologie France (PEF)","producers":[{"name":"ASSISTANCE PUBLIQUE HOPITAUX DE PARIS (AP-HP)","extlink":[{"title":"ROR","uri":"https:\/\/ror.org\/00pg5jh14","role":"sponsor id"},{"title":"SIREN","uri":"267500452","role":"sponsor id"}],"role":"sponsor"}],"funding_agencies":[{"name":"INSTITUT NATIONAL DE LA SANTE ET DE LA RECHERCHE MEDICALE (INSERM)","extlink":[{"title":"ROR","uri":"https:\/\/ror.org\/02vjkv261"},{"title":"SIREN","uri":"180036048"}]},{"name":"SANTE PUBLIQUE FRANCE","extlink":[{"title":"ROR","uri":"https:\/\/ror.org\/00dfw9p58"},{"title":"SIREN","uri":"130022338"}]},{"name":"","extlink":[]}]},"distribution_statement":{"contact":[{"name":"Jean;DONADIEU","email":"jean.donadieu@aphp.fr","type":"contact","affiliationName":"ASSISTANCE PUBLIQUE HOPITAUX DE PARIS (AP-HP)","contactPointLabo":"","extlink":[{"uri":"https:\/\/ror.org\/00pg5jh14","role":"organisation id","title":"ROR"},{"uri":"267500452","role":"organisation id","title":"SIREN"}]}]},"study_info":{"keywords":[{"keyword":"Mortalit\u00e9"},{"keyword":"Morbidit\u00e9"},{"keyword":"Incidence"},{"keyword":"Pr\u00e9valence"}],"topics":[{"topic":"H\u00e9matologie","vocab":"health theme","extLink":[{"title":"ESV","uri":"http:\/\/data.europa.eu\/8mn\/euroscivoc\/9239f328-78c9-40a5-a0b9-8e66fd9b6245"},{"title":"MeSH","uri":"http:\/\/id.nlm.nih.gov\/mesh\/D006402"}]},{"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":"Histiocytose \u00e0 cellules de Langerhans","vocab":"cim-11","extLink":[{"uri":"http:\/\/id.who.int\/icd\/entity\/216625985","title":"CIM-11"}]},{"topic":"D\u00e9terminants biologiques : Pr\u00e9dispositions g\u00e9n\u00e9tiques","vocab":"health determinant"},{"topic":"D\u00e9terminants biologiques","vocab":"health determinant"}],"purpose":"Les objectifs de sant\u00e9 publique de ce registre sont de mesurer : -L\u2019incidence et la pr\u00e9valence de la maladie -De d\u00e9terminer les facteurs de risque d\u2019apparition de la maladie et les moyens de pr\u00e9vention \u00e9ventuels -La mortalit\u00e9 en population -L\u2019incidence et la pr\u00e9valence des s\u00e9quelles \u00e0 long terme dans cette maladie (atteinte hypophysaire \u2013 cholangite scl\u00e9rosante \u2013 insuffisance respiratoire \u2013 trouble neurologique et psychiatrique) et d\u2019\u00e9valuer les m\u00e9thodes de pr\u00e9vention -Evaluer l\u2019impact des prises en charge th\u00e9rapeutiques sur l\u2019\u00e9volution \u00e0 long terme de la maladie en particulier la mortalit\u00e9, et les s\u00e9quelles \u00e0 long terme -Permettre la r\u00e9alisation d\u2019\u00e9tudes biologiques fondamentales sur des \u00e9chantillons larges de patients dont le profil \u00e9volutif est d\u00e9termin\u00e9. Ces \u00e9tudes visent 2 objectifs : la d\u00e9termination des facteurs d\u2019apparition de la maladie et l\u2019am\u00e9lioration des facteurs d\u00e9terminants l\u2019\u00e9volution de la maladie et en particulier les s\u00e9quelles.","abstract":"","coll_dates":[{"start":"1994-01-01"}],"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\": \"tous les cas d'Histiocytose langerhansienne chez l\u2019enfant de moins de 15 ans  -prouv\u00e9s par une histologie  -d\u00e9finis par des crit\u00e8res radio cliniques si le diagnostic est valid\u00e9 par au moins 2 m\u00e9decins du centre de r\u00e9f\u00e9rence en fonction des crit\u00e8res suivant:            \u2022 L\u00e9sions radiologiques osseuses typiques associ\u00e9s \u00e0 un diab\u00e8te insipide \",\n    \"clusion_E\": \"une tumeur maligne, une angiomatose osseuse, une infection           \u2022 L\u00e9sions radiologiques typiques du Poumon, montrant une association de l\u00e9sions kystiques et nodulaires\"\n}","data_kind":"['Donn\u00e9es cliniques','Donn\u00e9es paracliniques','Donn\u00e9es biologiques','Donn\u00e9es socio-d\u00e9mographiques','Donn\u00e9es paracliniques (hors biologiques) : Imagerie','Donn\u00e9es paracliniques (hors biologiques) : Exploration fonctionnelle']","quality_statement":{"standards":[{"name":"['D76.0C960C 961 D76.3']","committee":"","governance":""}],"other_quality_statement":""}},"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"}]},"method":{"data_collection":{"time_method":"","frequency":"","sampling_procedure":"['{\"concept\":{\"vocabURI\":\"Other\",\"vocab\":\"CESSDA\"},\"value\":\"Autre\"}']","sample_frame":{"frame_unit":{"unit_type":""}},"coll_mode":["{\"concept\":{\"vocabURI\":\"Interview\",\"vocab\":\"CESSDA\"},\"value\":\"Entretien avec le participant (dont clinique)\"}"],"research_instrument":"Acc\u00e8s restreint sur projet sp\u00e9cifique","sources":[],"target_sample_size":"[1000-10000[ individus","response_rate":"1454 (02\/2011)"},"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":"04-01-2011","lastUpdatedAuto":null,"lastUpdatedManual":"23-01-2015","isContributorPI":"Non","contributorName":"Jean DONADIEU","contributorAffiliation":"ASSISTANCE PUBLIQUE HOPITAUX DE PARIS (AP-HP)","addTeamMember":"","obtainedAuthorization":{"otherAuthorizingAgency":["",""]},"relatedDocument":[{"type":"","title":"","link":"http:\/\/www.orpha.net\/consor\/cgi-bin\/OC_Exp.php?Expert"}],"regulatoryRequirements":{"conformityDeclaration":""},"fundingAgent":{"fundingAgentType":["Public (France)","Public (France)","Priv\u00e9 \u00e0 but non lucratif"],"otherFundingAgentType":["","",""]},"sponsor":{"sponsorType":["Public (France)"],"otherSponsorType":[""]},"governance":{"committee":""},"collaborations":{"networkConsortium":""},"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":"Les donn\u00e9es sont enregistr\u00e9es \u00e0 partir des dossiers cliniques des patients issus des services d'h\u00e9mato-p\u00e9diatrique ou de p\u00e9diatrie g\u00e9n\u00e9rale et sp\u00e9cialis\u00e9e. Ceux-ci sont consult\u00e9 par courrier, t\u00e9l\u00e9phone, ou monitoring sur site.","collectionModeOther":""},"dataCollection":{"inclusionStrategyOther":"","samplingModeOther":"","recruitmentSourceOther":"","otherDocumentation":""},"dataCollectionIntegration":{"isDataIntegration":"Non"},"geographicalCoverage":{"geoDetail":"Ensemble du territoire fran\u00e7ais"},"dataTypes":{"clinicalDataDetails":"","biologicalDataDetails":"H\u00e9matologieBiochimiePolymorphismes g\u00e9n\u00e9tiquesImmunologie","isDataInBiobank":"Oui","biobankContent":[],"biobankContentOther":"","dataTypeOther":""},"variableDictionnary":{"variableDictionnaryAvailable":"","variableDictionnaryLink":""},"dataQuality":{"otherDocumentation":""},"mockSample":{"mockSampleAvailable":"","mockSampleLocation":""},"thirdPartySource":{"otherSourceType":[]},"isHealthTheme":"Oui","fileDscr":{"fileTxt":{"fileCitation":{"titlStmt":{"IDno":[]}}}}},"schematype":"survey"}