{"study_desc":{"title_statement":{"idno":"FRESH-PEF236-en","IDno":{"metadata_no":[{"agency":"PEF","code":"236"},{"agency":"FReSH","code":"FRESH-PEF236"}]},"title":"French Langerhans cell histiocytosis registry","alternate_title":""},"study_authorization":{"agency":[{"name":"CNIL"},{"name":"CESREES"}]},"authoring_entity":[{"name":"Jean;DONADIEU","firstname":"Jean","lastname":"DONADIEU","type":"investigator","extlink":[{"title":"ROR","uri":"https:\/\/ror.org\/00pg5jh14","role":"organisation id"},{"title":"SIREN","uri":"267500452","role":"organisation id"},{"uri":"0000-0002-4485-146X","role":"pi id","title":"ORCID"},{"uri":"073435910","role":"pi id","title":"IdRef"}],"email":"jean.donadieu@aphp.fr","affiliationName":"ASSISTANCE PUBLIQUE HOPITAUX DE PARIS (AP-HP)","PILabo":"Hopital Trousseau \/ Service d\u2019H\u00e9mato Oncologie P\u00e9diatrique","isContact":true}],"oth_id":[{"name":""}],"production_statement":{"prod_place":"Epidemiology France Portal (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":""}]},"distribution_statement":{"contact":[{"name":"Jean;DONADIEU","lastname":"DONADIEU","firstname":"Jean","type":"contact","email":"jean.donadieu@aphp.fr","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":"Morbidity"},{"keyword":"Incidence"},{"keyword":"Prevalence"},{"keyword":"Mortality"}],"topics":[{"topic":"Hematology","vocab":"health theme","extLink":[{"title":"ESV","uri":"http:\/\/data.europa.eu\/8mn\/euroscivoc\/9239f328-78c9-40a5-a0b9-8e66fd9b6245"},{"title":"MeSH","uri":"D006402"}]},{"topic":"Paediatrics","vocab":"health theme","extLink":[{"title":"ESV","uri":"http:\/\/data.europa.eu\/8mn\/euroscivoc\/bf8aaaac-ddee-41cd-bb8a-c5a63de3fcbd"},{"title":"MeSH","uri":"D010372"}]},{"topic":"Pulmonary medicine","vocab":"health theme","extLink":[{"title":"ESV","uri":"http:\/\/data.europa.eu\/8mn\/euroscivoc\/f6e18c52-6087-4662-904c-5c4b8174e40e"},{"title":"MeSH","uri":"D015272"}]},{"topic":"Langerhans cell histiocytosis","vocab":"cim-11","extLink":[{"uri":"http:\/\/id.who.int\/icd\/entity\/216625985","title":"CIM-11"}]},{"topic":"Biological determinants: Genetic predisposition","vocab":"health determinant"},{"topic":"Biological determinants","vocab":"health determinant"}],"purpose":"The public health objectives of this registry are to: - evaluate the incidence and prevalence of the disease - determine the risk factors of the disease manifesting and the possible prevention means - evaluate death rates in the population - The incidence and prevalence of long-term sequelae of this disease (pituitary affects \u2013 sclerosing cholangitis \u2013 respiratory failure \u2013 neurological and psychiatric problems) and to evaluate prevention methods - Evaluate the impact of therapies on the long-term progression of the disease - particularly mortality - and long-term sequelae - Enable the implementation of basic biological research on broad samples of patients whose progressive profiles have been determined. Such studies have two objectives: The determination of factors for the disease manifesting and the improvement of determining factors of the disease's progression - particularly the sequelae.","abstract":"","coll_dates":[{"start":"1994-01-01"}],"nation":[{"name":"France","abbreviation":"fr","extLink":{"vocab":"ISO","vocabURI":"fr"}}],"geog_coverage":"","analysis_unit":"Individuals","universe":"{\"level_sex_clusion_I\":[{\"value\":\"Male\",\"concept\":{\"vocab\":\"MeSH\",\"vocabURI\":\"D008297\"}},{\"value\":\"Female\",\"concept\":{\"vocab\":\"MeSH\",\"vocabURI\":\"D005260\"}}],\"level_age_clusion_I\":[{\"value\":\"Infant, Newborn (birth to 28 days)\",\"concept\":{\"vocab\":\"MeSH\",\"vocabURI\":\"D007231\"}},{\"value\":\"Infant (28 days to 2 years)\",\"concept\":{\"vocab\":\"MeSH\",\"vocabURI\":\"D007223\"}},{\"value\":\"Child, Preschool (2 to 5 years)\",\"concept\":{\"vocab\":\"MeSH\",\"vocabURI\":\"D002675\"}},{\"value\":\"Child (6 to 12 years)\",\"concept\":{\"vocab\":\"MeSH\",\"vocabURI\":\"D002648\"}},{\"value\":\"Adolescent (13 to 18 years)\",\"concept\":{\"vocab\":\"MeSH\",\"vocabURI\":\"D000293\"}},{\"value\":\"Young Adult (19 to 24 years)\",\"concept\":{\"vocab\":\"MeSH\",\"vocabURI\":\"D055815\"}},{\"value\":\"Adult (25 to 44 years)\",\"concept\":{\"vocab\":\"MeSH\",\"vocabURI\":\"D000328\"}},{\"value\":\"Middle Aged (45 to 64 years)\",\"concept\":{\"vocab\":\"MeSH\",\"vocabURI\":\"D008875\"}},{\"value\":\"Aged (65 to 79 years)\",\"concept\":{\"vocab\":\"MeSH\",\"vocabURI\":\"D000368\"}},{\"value\":\"Aged, 80 and over (80 years and more)\",\"concept\":{\"vocab\":\"MeSH\",\"vocabURI\":\"D000369\"}}],\"level_type_clusion_I\":\"Patients population\",\"level_type_clusion_other\":\"\",\"clusion_I\":\"all cases of Langerhans cell histiocytosis in children under 15 years of age  - proven by histology  - defined by radio-clinical criteria if the diagnosis is validated by at least two physicians from the reference center on the basis of the following criteria:            \u2022 Typical radiological lesions of the bone associated with diabetes insipidus \",\"clusion_E\":\"a malignant tumor, angiomatosis of the bones or an infection\u2022 Typical radiological lesions of the lung, demonstrating an association of cystic and nodular lesions\"}","data_kind":"['Clinical data','Paraclinical data (non-biological)','Paraclinical data (non-biological) : Imaging','Paraclinical data (non-biological) : Function testing','Biological data','Socio-demographic data']","quality_statement":{"standards":[{"name":"['D76.0 C960 C 961 D76.3']","committee":"","governance":""}],"other_quality_statement":""}},"study_development":{"development_activity":[{"activity_type":"primary evaluation","activity_description":"Health event\/morbidity\r\nHealth event\/mortality\r\nHealth care consumption and services"}]},"method":{"data_collection":{"time_method":"","frequency":"","sampling_procedure":"['{\"concept\":{\"vocabURI\":\"Other\",\"vocab\":\"CESSDA\"},\"value\":\"Other\"}']","sample_frame":{"frame_unit":{"unit_type":"['']"}},"coll_mode":["{\"concept\":{\"vocabURI\":\"Interview\",\"vocab\":\"CESSDA\"},\"value\":\"Interview with the participant (including clinical)\"}"],"research_instrument":"Access on specific project only","sources":[],"target_sample_size":"[1000-10000[ individuals","response_rate":"1454 (02\/2011)"},"method_notes":"Observational Study","study_class":"Unknown","notes":[{"subject":"research type","values":["Observational Study"]},{"subject":"observational study method","values":["Morbidity registry"]}]},"data_access":{"dataset_availability":{"access_place":"","complete":"","status":"{\"value\":\"Restricted access\",\"extLink\":[{\"title\":\"COAR\",\"uri\":\"http:\/\/purl.org\/coar\/access_right\/c_16ec\"}]}","notes":""},"dataset_use":{"restrictions":"","conditions":"","conf_dec":[{"txt":""}],"spec_perm":[{"txt":"","required":""}],"contact":[{"name":""}],"deposit_req":"","cit_req":""}}},"additional":{"versionLang":"en","originLang":"en","autoTranslation":false,"status":"Imported","creationDate":"04-01-2011","lastUpdatedManual":"23-01-2015","isContributorPI":"","contributorName":"","contributorAffiliation":"","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)","Private non-profit"],"otherFundingAgentType":["","",""]},"sponsor":{"sponsorType":["Public (France)"],"otherSponsorType":[""]},"governance":{"committee":""},"collaborations":{"networkConsortium":""},"theme":{"complementaryInformation":"","RareDiseases":true},"activeFollowUp":{"isActiveFollowUp":true,"followUpModeOther":""},"interventionalStudy":{"researchPurpose":[],"trialPhase":[],"interventionalStudyModel":"","isClinicalTrial":"","otherResearchPurpose":""},"isInclusionGroups":"","allocation":{"allocationMode":"","allocationUnit":""},"masking":{"maskingType":"","blindedMaskingDetails":[]},"arms":[],"intervention":[],"inclusionGroups":[],"collectionProcess":{"collectionModeDetails":"The data is recorded from patients' clinical records obtained from pediatric hematology or general and specialist pediatric departments. These are consulted by post, telephone or on-site monitoring.","collectionModeOther":""},"dataCollection":{"inclusionStrategyOther":"","samplingModeOther":"","recruitmentSourceOther":"","otherDocumentation":""},"dataCollectionIntegration":{"isDataIntegration":false},"geographicalCoverage":{"geoDetail":"All of french territory"},"dataTypes":{"clinicalDataDetails":"","biologicalDataDetails":"Hematology, biochemistry, genetic polymorphisms, immunology","isDataInBiobank":true,"biobankContent":[],"biobankContentOther":"","dataTypeOther":""},"variableDictionnary":{"variableDictionnaryAvailable":"","variableDictionnaryLink":""},"dataQuality":{"otherDocumentation":""},"mockSample":{"mockSampleAvailable":"","mockSampleLocation":""},"thirdPartySource":{"otherSourceType":[]},"isHealthTheme":true,"fileDscr":{"fileTxt":{"fileCitation":{"titlStmt":{"IDno":[]}}}}},"schematype":"survey"}