title: Clinical-Epidemiological Pattern of Primary Immunodeficiencies in Malaysia 1987-2006: A 20 year experience in Four Malaysian Hospitals.
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  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.
authors: Universiti Kebangsaan, Department Pediatrics, Faculty of Medicine, Universiti Kebangsaan (National University of Malaysia) Jalan Yaacob Latif, Bandar Tun Razak, Cheras 56000, Kuala Lumpur. lokman.m.noh@gmail.com.
authors: L M
authors: LM
authors: Noh
authors: B A
authors: BA
authors: Nasuruddin
authors: A H
authors: AH
authors: Abdul Latiff
authors: R M
authors: RM
authors: Noah
authors: M R
authors: MR
authors: Kamarul Azahar
authors: M Z
authors: MZ
authors: Norzila
authors: I
authors: I
authors: Zulkifli
authors: H K
authors: HK
authors: Gill
authors: N I
authors: NI
authors: Nik Zainal
authors: A W M
authors: AW
authors: Suhaimi
authors: N Y
authors: NY
authors: Nik Khairulddin
authors: H
authors: H
authors: Maraina
authors: H I
authors: HI
authors: Intan
authors: I
authors: I
authors: Mat
authors: M
authors: M
authors: Shahnaz
authors: Azizi
authors: A
authors: B H O
mesh_terms: D057210:Delayed Diagnosis; D006801:Humans; D007153:Immunologic Deficiency Syndromes; D008296:Malaysia; D012042:Registries; D011795:Surveys and Questionnaires