French congenital neutropenia registry

{
"level_sex_clusion_I": [
{
"value": "Male",
"concept": {
"vocab": "MeSH",
"vocabURI": "http:\/\/id.nlm.nih.gov\/mesh\/D008297"
}
},
{
"value": "Female",
"concept": {
"vocab": "MeSH",
"vocabURI": "http:\/\/id.nlm.nih.gov\/mesh\/D005260"
}
}
],
"level_age_clusion_I": [
{
"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"
}
},
{
"value": "Young Adult (19 to 24 years)",
"concept": {
"vocab": "MeSH",
"vocabURI": "http:\/\/id.nlm.nih.gov\/mesh\/D055815"
}
},
{
"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"
}
},
{
"value": "Aged (65 to 79 years)",
"concept": {
"vocab": "MeSH",
"vocabURI": "http:\/\/id.nlm.nih.gov\/mesh\/D000368"
}
},
{
"value": "Aged, 80 and over (80 years and more)",
"concept": {
"vocab": "MeSH",
"vocabURI": "http:\/\/id.nlm.nih.gov\/mesh\/D000369"
}
}
],
"level_type_clusion_I": [
"Patients population"
],
"level_type_clusion_other": "",
"clusion_I": "All of the criteria must be present: 1. Patient suffering from severe chronic neutropenia: - permanent neutropenia: absolute polynuclear rate < 0.5 109\/l, measured at at least three intervals over the three months prior to the study or absolute polynuclear rate < 1 109\/l, measured at at least three intervals over the three months prior to the study and presence of either a severe infection (septicemia- cellulitis- bacterial or mycotic pneumonia) or chronic gingivo-stomatitis. - intermittent neutropenia: After a surveillance period of at least six weeks, the neutrophilia rate must be less than 0.5 109 \/l in at least three blood counts. 2. Myelogram performed and cytological aspect compatible with the diagnosis (in the opinion of the registry's contact cytologist) 3. Subject aged over three months 4. Patients suffering from glycogen storage disease Ib, Shwachman Diamond syndrome or WHIM syndrome are all included 5. Consent by the patient and\/or his\/her parents ",
"clusion_E": "EXCLUSION CRITERIA (except glycogen storage disease Ib, Shwachman Diamond syndrome, WHIM syndrome or large granular lymphocytosis LGL): - all types of neutropenia caused by drugs - all medical histories with chemotherapy - medullary aplasia, irrespective of its etiology (idiopathic, Fanconi syndrome, etc.)- anemia < 8gr\/dl or thrombopenia (except inflammatory or iron-deficiency anemia, glycogen storage disease Ib and Shwachman Diamond syndrome). - progressive malignant pathology or medical history of malignant pathology - neutropenia linked to HIV infection - macrophage activation syndrome - initial myelodysplasia"
}