[Immunodeficiency in The Netherlands: clinical and immunological survey, 1970-1983. Interfacultaire werkgroep Immunodeficiëntie].
OBJECTIVE
Inventory of patients with immunodeficiency in the Netherlands.


METHODS
Retrospective over the period 1970-1983.


METHODS
Dutch Working Group on Immunodeficiency, University Children's Hospital, 'Het Wilhelmina Kinderziekenhuis', Utrecht.


METHODS
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.


RESULTS
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%).


CONCLUSIONS
Results were similar to those of other European countries.
Zegers
B J
BJ
Interfacultaire werkgroep Immunodeficiëntie, Wilhelmina Kinderziekenhuis, afd. Immunologie, Utrecht.
Weemaes
C M
CM
Weening
R S
RS
van der Meer
J W
JW
Vossen
J M
JM
D000917:Antibody Formation; D001327:Autoimmune Diseases / Q000276:immunology; D003165:Complement System Proteins / Q000172:deficiency; D005260:Female; D006801:Humans; D007111:Immunity, Cellular; D007153:Immunologic Deficiency Syndromes / Q000453:epidemiology / Q000276:immunology*; D008297:Male; D009426:Netherlands / Q000453:epidemiology; D010587:Phagocytosis; D012189:Retrospective Studies
D003165:Complement System Proteins
Immuundeficiëntie in Nederland: klinische en immunologische inventarisatie, 1970-1983.