A Review of Data from the Inborn Errors of Immunity Database in Malaysia: Clinical, Demographic, and Epidemiological Patterns Over Three Decades (1987-2016).

@article{noh2026review,
  title = {{A Review of Data from the Inborn Errors of Immunity Database in Malaysia: Clinical, Demographic, and Epidemiological Patterns Over Three Decades (1987-2016).}},
  author = {Lokman Mohd Noh and Amir Hamzah Abdul Latiff and Intan Hakimah Ismail and Adli Ali and Intan Juliana Abd Hamid and Mohd Azri Zainal Abidin and Jalilah Jamaluddin and Kent Chee Keen Woo and Adiratna Mat Ripen and Rahim Md Noah and Asrul Abdul Wahab and Kamarul Azhar Mohd Razali and Norzila Mohamed Zainudin and Siti Mardiana Mohamad and Zarina Thasneem Zainudeen and Ilie Fadzilah Hashim and Khairoon Nisa Mohamed Nashrudin and Nik Khairulddin Nik Yusoff and Marina Md Sham and Sangeetha Sinniah and Mohd Shahrir Mohamed Said and Sabeera Begum Kader Ibrahim and Nik Zainal Abidin Nik Ismail and Mohd Suhaimi Ab Wahab and Rus Anida Awang and Thiyagar Nadarajaw and Choo Chong Ming and Florence Bakon and Alia Zainudin and Zulkifli Ismail},
  journal = {Journal of clinical immunology},
  year = {2026},
  month = aug,
  doi = {10.1007/s10875-026-02037-7},
  url = {https://europepmc.org/article/MED/42972176},
  note = {CC BY-NC-ND},
}

TY  - JOUR
TI  - A Review of Data from the Inborn Errors of Immunity Database in Malaysia: Clinical, Demographic, and Epidemiological Patterns Over Three Decades (1987-2016).
AU  - Lokman Mohd Noh
AU  - Amir Hamzah Abdul Latiff
AU  - Intan Hakimah Ismail
AU  - Adli Ali
AU  - Intan Juliana Abd Hamid
AU  - Mohd Azri Zainal Abidin
AU  - Jalilah Jamaluddin
AU  - Kent Chee Keen Woo
AU  - Adiratna Mat Ripen
AU  - Rahim Md Noah
AU  - Asrul Abdul Wahab
AU  - Kamarul Azhar Mohd Razali
AU  - Norzila Mohamed Zainudin
AU  - Siti Mardiana Mohamad
AU  - Zarina Thasneem Zainudeen
AU  - Ilie Fadzilah Hashim
AU  - Khairoon Nisa Mohamed Nashrudin
AU  - Nik Khairulddin Nik Yusoff
AU  - Marina Md Sham
AU  - Sangeetha Sinniah
AU  - Mohd Shahrir Mohamed Said
AU  - Sabeera Begum Kader Ibrahim
AU  - Nik Zainal Abidin Nik Ismail
AU  - Mohd Suhaimi Ab Wahab
AU  - Rus Anida Awang
AU  - Thiyagar Nadarajaw
AU  - Choo Chong Ming
AU  - Florence Bakon
AU  - Alia Zainudin
AU  - Zulkifli Ismail
PY  - 2026
DA  - 2026/08/05
JO  - Journal of clinical immunology
DO  - 10.1007/s10875-026-02037-7
UR  - https://europepmc.org/article/MED/42972176
AB  - Introduction  The need to establish inborn errors of immunity (IEI) database arose from the recognition that IEI existed in Malaysia, beginning in 1977, followed by more reports a decade later. As awareness grew, it became necessary to develop a database to systematically capture IEI data to support planning, monitoring, and improvement of patient care in this emerging area of clinical medicine in Malaysia.Materials and methods  Patients were managed at participating hospitals under the care of clinical immunologists. Each patient underwent a comprehensive clinical assessment, including evaluation of relevant clinical features and immunological laboratory investigations at selected centres. In 2007, a database, the Malaysian Primary Immunodeficiency Network (MyPIN) Registry, was established to collate and store this information.Results  Of 423 cases referred to immunologist-led clinics, 300 were identified as IEI, giving an estimated prevalence of 0.95 per 100,000 population. Defects in cellular immunity, including combined immunodeficiencies and well-defined combined immunodeficiency syndromes, were the most frequent (44.7%), followed by predominantly antibody immunodeficiencies (PAD) (30.7%) and phagocytic defects (12.3%). Males outnumbered females with a ratio of 1.75:1, and 24.0% of patients had a positive family history. Indigenous populations accounted for 4.0% of IEI cases. Increased clinical immunology expertise had improved diagnosis and treatment, with a marked rise in case detection as more clinical immunologists joined hospital services, especially during the later part of the third decade.Conclusion  In developing countries where awareness of IEI remains limited, the establishment of a database managed by clinical immunology experts can play an important role in improving strategies for the diagnosis and care of patients with IEI.
SN  - 0271-9142
SN  - 1573-2592
ER  -