{
  "abstract": "Introduction\n\nThe 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\n\nPatients 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\n\nOf 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\n\nIn 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.",
  "abstract_shown": true,
  "affiliations": [
    "Medical Advisory Board, Malaysian Patients Organisation for Primary Immunodeficiencies (MyPOPI), Formerly Professor of Paediatric Immunology, Universiti Sains Malaysia, Pulau Pinang, Malaysia.",
    "Allergy and Immunology Centre, Pantai Hospital Kuala Lumpur, Kuala Lumpur, Malaysia.",
    "Sunway Centre for Planetary Health, Sunway University, Petaling Jaya, Malaysia.",
    "Clinical Immunology Unit, Department of Paediatrics, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia, 43400, Serdang, Selangor, Malaysia. intanhakimah@upm.edu.my.",
    "Advanced Medical Research in Allergy and Clinical Immunology (AMRAC), Hospital Sultan Abdul Aziz Shah, Universiti Putra Malaysia, Selangor, Malaysia. intanhakimah@upm.edu.my.",
    "Department of Paediatrics, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia.",
    "Research Center, Hospital Tunku Ampuan Besar Tuanku Aishah Rohani, Universiti Kebangsaan Malaysia (UKM) Specialist Children's Hospital, Kuala Lumpur, Malaysia.",
    "Department of Clinical Medicine, Pusat Kanser Tun Abdullah Ahmad Badawi, Universiti Sains Malaysia, Kepala Batas, Pulau Pinang, Malaysia.",
    "Clinical Immunology Unit, Department of Paediatrics, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia, 43400, Serdang, Selangor, Malaysia.",
    "Advanced Medical Research in Allergy and Clinical Immunology (AMRAC), Hospital Sultan Abdul Aziz Shah, Universiti Putra Malaysia, Selangor, Malaysia.",
    "Allergy and Immunology, Gleneagles Hospital, Kuala Lumpur, Malaysia.",
    "Cancer Research Centre, Institute for Medical Research (IMR), National Institutes of Health, Shah Alam, Malaysia.",
    "Institute of Medical Science Technology, Universiti Kuala Lumpur, Selangor, Malaysia.",
    "Department of Medical Microbiology and Immunology, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia.",
    "Institute of Paediatrics, Hospital Kuala Lumpur, Kuala Lumpur, Malaysia.",
    "Sunway Medical Centre, Subang Jaya, Selangor, Malaysia.",
    "Department of Community Health, Advanced Medical and Dental Institute, Universiti Sains Malaysia, Pulau Pinang, Malaysia.",
    "Department of Paediatrics, Hospital Raja Perempuan Zainab II, Ministry of Health Malaysia, Kelantan, Malaysia.",
    "Department of Paediatrics, Hospital Tunku Azizah, Ministry of Health Malaysia, Kuala Lumpur, Malaysia.",
    "Sunway Medical Centre Velocity, Kuala Lumpur, Malaysia.",
    "Unit of Rheumatology, Department of Medicine, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia.",
    "Department of Paediatrics, School of Medical Sciences, Universiti Sains Malaysia, Kelantan, Malaysia.",
    "Hospital Pulau Pinang, Ministry of Health Malaysia, George Town, Pulau Pinang, Malaysia.",
    "Hospital Sultanah Bahiyah, Ministry of Health Malaysia, Alor Setar, Kedah, Malaysia.",
    "Hospital Sultan Abdul Halim, Ministry of Health Malaysia, Sungai Petani, Kedah, Malaysia.",
    "KPJ Kuching Specialist Hospital, Kuching, Sarawak, Malaysia.",
    "KPJ Selangor Specialist Hospital, Shah Alam, Selangor, Malaysia."
  ],
  "article_type": "review article",
  "arxiv": null,
  "author_total": 30,
  "authors": [
    {
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        0
      ],
      "name": "Lokman Mohd Noh"
    },
    {
      "affs": [
        1,
        2
      ],
      "name": "Amir Hamzah Abdul Latiff"
    },
    {
      "affs": [
        3,
        4
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      "name": "Intan Hakimah Ismail"
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        5,
        6
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      "name": "Adli Ali"
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        7
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      "name": "Intan Juliana Abd Hamid"
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        8,
        9
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      "name": "Mohd Azri Zainal Abidin"
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    {
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        8,
        9
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      "name": "Jalilah Jamaluddin"
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        10
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      "name": "Kent Chee Keen Woo"
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        11
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      "name": "Adiratna Mat Ripen"
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      "name": "Rahim Md Noah"
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      "name": "Asrul Abdul Wahab"
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        14
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      "name": "Kamarul Azhar Mohd Razali"
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    {
      "affs": [
        15
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      "name": "Norzila Mohamed Zainudin"
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    {
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        16
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      "name": "Siti Mardiana Mohamad"
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    {
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        7
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      "name": "Zarina Thasneem Zainudeen"
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    {
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        7
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      "name": "Ilie Fadzilah Hashim"
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    {
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        8,
        9
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      "name": "Khairoon Nisa Mohamed Nashrudin"
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    {
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      "name": "Nik Khairulddin Nik Yusoff"
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    {
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      "name": "Marina Md Sham"
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    {
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      "name": "Sangeetha Sinniah"
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      "name": "Mohd Shahrir Mohamed Said"
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    {
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        18
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      "name": "Sabeera Begum Kader Ibrahim"
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    {
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      "name": "Nik Zainal Abidin Nik Ismail"
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    {
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      "name": "Mohd Suhaimi Ab Wahab"
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    {
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      "name": "Rus Anida Awang"
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      "name": "Thiyagar Nadarajaw"
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      "name": "Florence Bakon"
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      "name": "Alia Zainudin"
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      "name": "Zulkifli Ismail"
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  "bibtex": "https://research.draconitic.com/paper/28c4ydg1r5g7w.bib",
  "cited_by": 0,
  "doi": "10.1007/s10875-026-02037-7",
  "fulltext_url": "https://europepmc.org/articles/PMC13727841",
  "id": "28c4ydg1r5g7w",
  "issns": [
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    "1573-2592"
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  "landing_url": "https://europepmc.org/article/MED/42972176",
  "language": "eng",
  "licence": "CC-BY-NC-ND",
  "licence_label": "CC BY-NC-ND",
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  "native_subjects": [
    [
      "heading",
      "Review"
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      "2403|Immunodeficiency and Autoimmune Disorders"
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  "pdf_url": "https://europepmc.org/articles/PMC13727841?pdf=render",
  "pmcid": "PMC13727841",
  "pmid": "42972176",
  "published": "2026-08-05",
  "publisher": null,
  "ris": "https://research.draconitic.com/paper/28c4ydg1r5g7w.ris",
  "sources": [
    {
      "first_seen": "2026-11-04T15:20:49Z",
      "native_id": "MED:42972176",
      "source": "europepmc",
      "url": "https://europepmc.org/article/MED/42972176"
    },
    {
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      "native_id": "PMC13727841",
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      "url": "https://doi.org/10.1007/s10875-026-02037-7"
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  "subjects": [
    {
      "field": "biology",
      "origin": "openalex:2403|Immunodeficiency and Autoimmune Disorders",
      "subfield": "immunology"
    }
  ],
  "title": "A Review of Data from the Inborn Errors of Immunity Database in Malaysia: Clinical, Demographic, and Epidemiological Patterns Over Three Decades (1987-2016).",
  "title_text": "A Review of Data from the Inborn Errors of Immunity Database in Malaysia: Clinical, Demographic, and Epidemiological Patterns Over Three Decades (1987-2016).",
  "type": "peer-reviewed",
  "updated_at": "2026-11-07T12:20:57Z",
  "url": "https://research.draconitic.com/paper/28c4ydg1r5g7w",
  "venue": "Journal of clinical immunology",
  "versions": []
}