Primary immunodeficiency diseases at Red Cross War Memorial Children's Hospital.
OBJECTIVE
To describe the spectrum of primary immunodeficiency diseases (PIDs) diagnosed at Red Cross War Memorial Children's Hospital.


METHODS
Retrospective, descriptive study.


METHODS
Tertiary, referral hospital.


METHODS
All patients investigated by the immunology service because of suspected PIDs, between January 1983 and December 1996.


METHODS
Review of immunology service database and hospital case records.


RESULTS
During the 14-year review period, 515 patients were investigated, a mean of 36.8 new patients per annum. Ninety-three patients with PIDs were diagnosed, a mean of 6.6 new patients per annum. The spectrum of PIDs was similar to that reported in developed countries. As in other series, antibody deficiencies predominated, accounting for 56% (52/93) of diagnoses. The male/female ratio was 1.5:1; 73% (62/85) came from the Western Cape, the remaining 27% (23/85) resided in five other provinces. Eighty per cent (70/87) presented with recurrent or atypical infection, with or without failure to thrive. Sinopulmonary infections (80%), diarrhoeal disease (19%) and candidiasis (18%) were the most common preceding infections. By the age of 5 years, only 60% had been diagnosed, compared with about 80% in developed countries. During the study period, 20% (19/93) were known to have died.


CONCLUSIONS
The results show a pattern of PIDs incidence similar to that in developed countries. Diagnosis was delayed in many patients, which probably contributed to morbidity. To facilitate earlier diagnosis and to improve outcome, children should be considered for an immunological assessment if they exhibit increased susceptibility to infection.
Eley
B S
BS
Department of Paediatrics and Child Health, University of Cape Town.
Hughes
J
J
Cooper
M
M
Pienaar
S
S
Beatty
D W
DW
D000293:Adolescent; D000361:Agammaglobulinemia / Q000175:diagnosis / Q000453:epidemiology; D017677:Age Distribution; D001260:Ataxia Telangiectasia / Q000175:diagnosis / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D004406:Dysgammaglobulinemia / Q000175:diagnosis / Q000453:epidemiology; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000175:diagnosis / Q000453:epidemiology*; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D016511:Severe Combined Immunodeficiency / Q000175:diagnosis / Q000453:epidemiology; D017678:Sex Distribution; D013019:South Africa / Q000453:epidemiology