Systematic Review of Primary Immunodeficiency Diseases in Malaysia: 1979–2020
Introduction: Primary immunodeficiency diseases (PIDs) are under-reported in Malaysia. The actual disease frequency of PID in this country is unknown due to the absence of a national patient registry for PID.Objective: This systematic review aimed to determine the prevalence rates of PID cases diagn...
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doaj-3f347761153846ef98b31174d13725192020-11-25T03:40:06ZengFrontiers Media S.A.Frontiers in Immunology1664-32242020-08-011110.3389/fimmu.2020.01923564575Systematic Review of Primary Immunodeficiency Diseases in Malaysia: 1979–2020Intan Juliana Abd Hamid0Nur Adila Azman1Andrew R. Gennery2Ernest Mangantig3Ilie Fadzilah Hashim4Zarina Thasneem Zainudeen5Primary Immunodeficiency Diseases Group, Regenerative Medicine Cluster, Institut Perubatan and Pergigian Termaju, Universiti Sains Malaysia, Kepala Batas, MalaysiaDepartment of Biomedical Science, Universiti Islam Antarabangsa, Kuantan, Pahang, MalaysiaSir James Spence Professor of Child Health, Translational and Clinical Research Institute, Newcastle University, Great North Children's Hospital, Newcastle upon Tyne, United KingdomPrimary Immunodeficiency Diseases Group, Regenerative Medicine Cluster, Institut Perubatan and Pergigian Termaju, Universiti Sains Malaysia, Kepala Batas, MalaysiaPrimary Immunodeficiency Diseases Group, Regenerative Medicine Cluster, Institut Perubatan and Pergigian Termaju, Universiti Sains Malaysia, Kepala Batas, MalaysiaPrimary Immunodeficiency Diseases Group, Regenerative Medicine Cluster, Institut Perubatan and Pergigian Termaju, Universiti Sains Malaysia, Kepala Batas, MalaysiaIntroduction: Primary immunodeficiency diseases (PIDs) are under-reported in Malaysia. The actual disease frequency of PID in this country is unknown due to the absence of a national patient registry for PID.Objective: This systematic review aimed to determine the prevalence rates of PID cases diagnosed and published in Malaysia from 1st of January 1979 until 1st of March 2020. It also aimed to describe the various types of PIDs reported in Malaysia.Method: Following the development of a comprehensive search strategy, all published literature of PID cases from Malaysia was identified and collated. All cases that fulfilled the International Union of Immunological Societies (IUIS) classification diagnosis were included in the systematic review. Data were retrieved and collated into a proforma.Results: A total of 4,838 articles were identified and screened, with 34 publications and 119 patients fulfilling the criteria and being included in the systematic review. The prevalence rate was 0.37 per 100,000 population. In accordance with the IUIS, the distribution of diagnostic classifications was immunodeficiencies affecting cellular and humoral immunities (36 patients, 30.3%), combined immunodeficiencies with associated or syndromic features (21 patients, 17.6%), predominant antibody deficiencies (24 patients, 20.2%), diseases of immune dysregulation (13 patients, 10.9%), congenital defects in phagocyte number or function (20 patients, 16.8%), defects in intrinsic and innate immunity (4 patients, 3.4%), and autoinflammatory disorders (1 patient, 0.8%). Parental consanguinity was 2.5%. Thirteen different gene mutations were available in 21.8% of the cases.Conclusion: PIDs are underdiagnosed and under-reported in Malaysia. Developing PID healthcare and a national patient registry is much needed to enhance the outcome of PID patient care.https://www.frontiersin.org/article/10.3389/fimmu.2020.01923/fullprimary immunodeficiencyinborn error of immunityepidemiologyprevalenceMalaysia |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Intan Juliana Abd Hamid Nur Adila Azman Andrew R. Gennery Ernest Mangantig Ilie Fadzilah Hashim Zarina Thasneem Zainudeen |
spellingShingle |
Intan Juliana Abd Hamid Nur Adila Azman Andrew R. Gennery Ernest Mangantig Ilie Fadzilah Hashim Zarina Thasneem Zainudeen Systematic Review of Primary Immunodeficiency Diseases in Malaysia: 1979–2020 Frontiers in Immunology primary immunodeficiency inborn error of immunity epidemiology prevalence Malaysia |
author_facet |
Intan Juliana Abd Hamid Nur Adila Azman Andrew R. Gennery Ernest Mangantig Ilie Fadzilah Hashim Zarina Thasneem Zainudeen |
author_sort |
Intan Juliana Abd Hamid |
title |
Systematic Review of Primary Immunodeficiency Diseases in Malaysia: 1979–2020 |
title_short |
Systematic Review of Primary Immunodeficiency Diseases in Malaysia: 1979–2020 |
title_full |
Systematic Review of Primary Immunodeficiency Diseases in Malaysia: 1979–2020 |
title_fullStr |
Systematic Review of Primary Immunodeficiency Diseases in Malaysia: 1979–2020 |
title_full_unstemmed |
Systematic Review of Primary Immunodeficiency Diseases in Malaysia: 1979–2020 |
title_sort |
systematic review of primary immunodeficiency diseases in malaysia: 1979–2020 |
publisher |
Frontiers Media S.A. |
series |
Frontiers in Immunology |
issn |
1664-3224 |
publishDate |
2020-08-01 |
description |
Introduction: Primary immunodeficiency diseases (PIDs) are under-reported in Malaysia. The actual disease frequency of PID in this country is unknown due to the absence of a national patient registry for PID.Objective: This systematic review aimed to determine the prevalence rates of PID cases diagnosed and published in Malaysia from 1st of January 1979 until 1st of March 2020. It also aimed to describe the various types of PIDs reported in Malaysia.Method: Following the development of a comprehensive search strategy, all published literature of PID cases from Malaysia was identified and collated. All cases that fulfilled the International Union of Immunological Societies (IUIS) classification diagnosis were included in the systematic review. Data were retrieved and collated into a proforma.Results: A total of 4,838 articles were identified and screened, with 34 publications and 119 patients fulfilling the criteria and being included in the systematic review. The prevalence rate was 0.37 per 100,000 population. In accordance with the IUIS, the distribution of diagnostic classifications was immunodeficiencies affecting cellular and humoral immunities (36 patients, 30.3%), combined immunodeficiencies with associated or syndromic features (21 patients, 17.6%), predominant antibody deficiencies (24 patients, 20.2%), diseases of immune dysregulation (13 patients, 10.9%), congenital defects in phagocyte number or function (20 patients, 16.8%), defects in intrinsic and innate immunity (4 patients, 3.4%), and autoinflammatory disorders (1 patient, 0.8%). Parental consanguinity was 2.5%. Thirteen different gene mutations were available in 21.8% of the cases.Conclusion: PIDs are underdiagnosed and under-reported in Malaysia. Developing PID healthcare and a national patient registry is much needed to enhance the outcome of PID patient care. |
topic |
primary immunodeficiency inborn error of immunity epidemiology prevalence Malaysia |
url |
https://www.frontiersin.org/article/10.3389/fimmu.2020.01923/full |
work_keys_str_mv |
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