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Primary immunodeficiency diseases at Red Cross War Memorial Children's Hospital
Insights
Primary immunodeficiency diseases (PIDs) in children were studied, revealing a spectrum similar to developed nations. Delayed diagnosis contributed to morbidity, highlighting the need for earlier immunological assessment in susceptible children.
Area of Science:
- Clinical Immunology
- Pediatric Infectious Diseases
- Primary Immunodeficiency Diseases (PIDs)
Background:
- Primary immunodeficiency diseases (PIDs) are a heterogeneous group of disorders affecting the immune system.
- Understanding the spectrum and incidence of PIDs is crucial for diagnosis and management, particularly in pediatric populations.
- Previous studies have characterized PIDs in various global regions, but data from specific tertiary referral centers are valuable.
Purpose of the Study:
- To delineate the spectrum of primary immunodeficiency diseases (PIDs) diagnosed at a major South African pediatric hospital.
- To analyze the incidence, clinical presentation, and diagnostic patterns of PIDs over a 14-year period.
- To compare PID characteristics with those reported in developed countries and identify areas for improved diagnostic timelines.
Main Methods:
- A retrospective, descriptive study design was employed.
- Data were collected from patients investigated for suspected PIDs at the Red Cross War Memorial Children's Hospital between 1983 and 1996.
- Review of the immunology service database and hospital case records formed the basis of the analysis.
Main Results:
- A total of 515 patients were investigated, with 93 diagnosed with PIDs.
- Antibody deficiencies were the most common type, accounting for 56% of diagnoses.
- Delayed diagnosis was observed, with only 60% diagnosed by age 5, and a significant mortality rate of 20%.
Conclusions:
- The PID spectrum in this cohort mirrors that of developed countries, suggesting universal patterns.
- Delayed diagnosis is a significant issue, likely contributing to increased morbidity and mortality.
- Early immunological assessment for children with recurrent or atypical infections is recommended to improve outcomes.
Objective:
To describe the spectrum of primary immunodeficiency diseases (PIDs) diagnosed at Red Cross War Memorial Children's Hospital.
Design:
Retrospective, descriptive study.
Setting:
Tertiary, referral hospital.
Patients:
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.