Clinical-Epidemiological Pattern of Primary Immunodeficiencies in Malaysia 1987-2006: A 20 year experience in Four Malaysian Hospitals.

AIM OF STUDY: To determine the clinical and epidemiological characteristics of patients seen with primary immunodeficiencies referred at four Malaysian Hospitals between 1987 to 2007. METHODS: Patient data were retrospectively obtained from patient records and supplemented by information from a standardized questionnaires taken at the time of diagnosis from 4 participating hospitals. The completed data were transferred to document records kept by the first author. The diagnoses made were based on criteria set by WHO Scientific Committee 1986. RESULTS: Fifty one (51) patients with completed records satisfied the criteria of primary immunodeficiencies based on WHO Scientific Committee 1986. Predominant Antibody deficiency (40.4%) is the commonest of the class of primary immunodeficiency (based on modified IUIS classification) followed by phagocytic defect (17.3%), combined immunodeficiencies (15.4%) and other cellular immunodeficiencies (11.5%). The commonest clinical presentation is pneumonia (54%) . A positive Family history with a close family relative afflicted was a strong pointer to diagnosis for PID (52.6%) Primary immnodeficiencies are seen in all the major ethnic groups of Malaysia, predominantly among Malays. As observed in other patient registries, diagnostic delay remains the major cause of morbidity and mortality. CONCLUSION: Primary immunodeficiencies is relative rare but is an emerging disease in Malaysia. Creating awareness of the disease, may reveal more cases within the community. It is sufficient to be a health issue in Malaysia as in other developing countries in the future.

Epidemiology of Haemophilus influenzae invasive disease in hospitalised Kelantanese children, 1985-1994.

AIM: Data is lacking with regard to the epidemiology of invasive haemophilus influenzae (HI) disease in Malaysia. This study was carried out to document the epidemiology of invasive HI disease in hospitalised Kelantanese children. METHODS: We conducted a retrospective study of 65 children who had invasive HI disease from June 1985 to December 1994. Data regarding age, sex, duration of illness, weight, diagnosis, complications, duration of hospitalisation, outcome, full blood count and sensitivity pattern of HI to various antibiotics were reviewed. RESULTS: The age distribution varied from one day to 72 months with a mean of 13 months. Peak incidence occurred in the 7-12 months age group. Majority (89.1%) was below two years of age. The relative frequencies of the 75 clinical entities documented were as follows: meningitis 64%, pneumonia 29.3%, septicaemia 5.4%, and abscess 1.3%. In addition, 13.5% of cases had meningitis associated with pneumonia. Serotype b accounted for all strains in cases where serotyping was done. Anaemia (Hb < 10 g%) was seen in 71.4% of cases. Long term complications were noted in 41.5% of cases of meningitis. Case fatality rate was 12.3%. The percentage of HI strains sensitive to penicillin, ampicillin, chloramphenicol and co-trimoxazole were 83.7%, 87.7%, 98.2% and 89.7%, respectively. CONCLUSION: The data suggest that invasive HI disease causes considerable morbidity and mortality in Kelantanese children.