Register of thalassaemia patients in France (Qualified Register)

{
"level_sex_clusion_I": [
{
"value": "Male",
"concept": {
"vocab": "MeSH",
"vocabURI": "http:\/\/id.nlm.nih.gov\/mesh\/D008297"
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},
{
"value": "Female",
"concept": {
"vocab": "MeSH",
"vocabURI": "http:\/\/id.nlm.nih.gov\/mesh\/D005260"
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],
"level_age_clusion_I": [
{
"value": "Infant, Newborn (birth to 28 days)",
"concept": {
"vocab": "MeSH",
"vocabURI": "http:\/\/id.nlm.nih.gov\/mesh\/D007231"
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},
{
"value": "Infant (28 days to 2 years)",
"concept": {
"vocab": "MeSH",
"vocabURI": "http:\/\/id.nlm.nih.gov\/mesh\/D007223"
}
},
{
"value": "Child, Preschool (2 to 5 years)",
"concept": {
"vocab": "MeSH",
"vocabURI": "http:\/\/id.nlm.nih.gov\/mesh\/D002675"
}
},
{
"value": "Child (6 to 12 years)",
"concept": {
"vocab": "MeSH",
"vocabURI": "http:\/\/id.nlm.nih.gov\/mesh\/D002648"
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},
{
"value": "Young Adult (19 to 24 years)",
"concept": {
"vocab": "MeSH",
"vocabURI": "http:\/\/id.nlm.nih.gov\/mesh\/D055815"
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},
{
"value": "Adult (25 to 44 years)",
"concept": {
"vocab": "MeSH",
"vocabURI": "http:\/\/id.nlm.nih.gov\/mesh\/D000328"
}
},
{
"value": "Middle Aged (45 to 64 years)",
"concept": {
"vocab": "MeSH",
"vocabURI": "http:\/\/id.nlm.nih.gov\/mesh\/D008875"
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},
{
"value": "Aged (65 to 79 years)",
"concept": {
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],
"level_type_clusion_I": [
"Patients population"
],
"level_type_clusion_other": "",
"clusion_I": "Beta-thalassemias (TM) major and intermediate (TI), including HbE\/thalassemia composite hereterozygosity and thalassemia patients who have had a HSC transplant. Diagnosis of a severe form of beta-thalassemia is formally established on the basis of data from a biochemical study of hemoglobin with no real differential diagnosis. Distinguishing between thalassemia major and intermediate requires the combination of 3 consensual criteria: • for TM: Severe anemia indicating the setup of a systematic transfusion routine (at least 8 transfusions\/year) before the age of 4. • for TI: Moderate or medium intensity anemia that have no or only occasional need for transfusion. The date and circumstances of the diagnosis are asked for upon inclusion and the characteristics of the transfusion treatment upon inclusion and when each follow-up sheet is completed. Identification of the beta-thalassemia mutations or type of beta 0 or beta + mutation are available in most cases, allowing for a complementary approach - even if the TM or TI diagnosis retains a clinical definition. HbE\/thalassemia composite hereterozygosity is of biological definition (biochemical or molecular) and can lead to an intermediate or major form of thalassemia: as a result they are subject to the same classification according to the same clinical criteria (age at diagnosis and transfusion needs).",
"clusion_E": ""
}