| Popis: |
Inventory of patients with immunodeficiency in the Netherlands.Retrospective over the period 1970-1983.Dutch Working Group on Immunodeficiency, University Children's Hospital, 'Het Wilhelmina Kinderziekenhuis', Utrecht.A questionnaire with specific information on immunodeficient patients was completed by clinical members of the Working Group i.e. paediatricians and internists. Diagnosis of the immunodeficiency was according to WHO recommendations.The data of 336 patients could be included and analysed. Defects in humoral immunity were the most common (62.9%), followed by combined defects (18.75%), T cell defects (7.4%), phagocytic disorders (6.8%) and complement component deficiencies (2.7%). Sixty-two patients (15.5%) died, notably those with combined T and B cell defects, selective T cell defects and those with immunodeficiency based on DNA repair defects. Autoimmunity was relatively frequent in immunodeficiency (7.7%) as was cancer (2.7%).Results were similar to those of other European countries. |
