To define the diagnostic meaning of antineutrophil cytoplasmic antibodies (ANCA) positivity in children, we analysed 1485 consecutive sera sent for routine immunological investigation to our department from January to August 1996.
Using this large screening, we identified the most typical clinical disorders associated with ANCA in childhood.
Out of 1485 indirect immunofluorescence (IIF) tests for ANCA. 143 were ANCA positive. 70 had a cytoplasmic IIF pattern (c-ANCA). and 73 a perinuclear IIF pattern (p-ANCA).
The ANCA associated diseases in childhood were cystic fibrosis (CF) (31 c-ANCA. 7 p-ANCA positive out of 71 CF children investigated), juvenile chronic arthritis (JCA) (21 p-ANCA positive out of 78), auto-immune hepatitis (AIH) (4 c-ANCA and 12 p-ANCA positive out of 19). and ulcerative colitis (UC) (2 c-ANCA, 5 p-ANCA positive out of 15).
In cases of c-ANCA positivity we determined the antigenic specificity of ANCA for proteinase 3 and/or bactericidal permeability increasing protein.
Borderline anti-proteinase 3 levels were found in CF, and in high levels in one boy with Wegener granulomatosis.
Bactericidal permeability increasing protein was characteristic target antigen in children with CF. p-ANCA positive children were further tested for the specificity for myeloperoxidase. which was detected mostly in children with JCA. (...)
Mots-clés Pascal : Anticorps anticytoplasme polynucléaire neutrophile, Association, Diagnostic, Mucoviscidose, Biologie clinique, Immunofluorescence indirecte, Arthrite chronique juvénile, Etude statistique, Epidémiologie, République tchèque, Europe, Nourrisson, Homme, Enfant, Adolescent, Chronique, Appareil respiratoire pathologie, Appareil digestif pathologie, Pancréas pathologie, Maladie héréditaire, Métabolisme pathologie, Immunohistochimie, Système ostéoarticulaire pathologie, Rhumatisme inflammatoire, Immunopathologie
Mots-clés Pascal anglais : Antineutrophil cytoplasmic antibody, Association, Diagnosis, Cystic fibrosis, Clinical biology, Indirect immunofluorescence, Juvenile rheumatoid arthritis, Statistical study, Epidemiology, Czech Republic, Europe, Infant, Human, Child, Adolescent, Chronic, Respiratory disease, Digestive diseases, Pancreatic disease, Genetic disease, Metabolic diseases, Immunohistochemistry, Diseases of the osteoarticular system, Inflammatory joint disease, Immunopathology
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 99-0049611
Code Inist : 002B06E03. Création : 31/05/1999.