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Review of Primary Immunodeficiency Diseases (PIDs): Attention and Awareness is Critical for Proper Management

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Pranab Karmaker1, Anila Pasha1, Mamudul Hasan Razu1, Uttam Kumar Barua2, A F M Nazmus Sadat1 and Mala Khan1
» doi: 10.48047/ecb/2023.12.12.206


Primary immunodeficiency diseases (PIDs) are diverse genetic disorders mainly responsible for severe and recurrent infectious diseases such as ear infections, sinus infections, pneumonias, deep skin or organ abscesses, persistent thrush in mouth etc. PIDs may also be responsible for many life threatening syndromes such as DiGeorge syndrome, Wiskott-Aldrich syndrome, Ataxia-Telangiectasia, Ivemark syndrome, polyendocrinopathy syndrome etc. Defects occurring at any stage of development, differentiation and maturation of innate immune and adaptive immune cells may be responsible for such PIDs as they failed to perform their defensive function. Latest publication of the International Union of Immunological Societies (IUIS) in 2022 described 485 inborn errors of immunity under 10 broad classification. The increasing nature of PIDs observed in different medically developed countries is alarming for the rest of the world. Though the disease is directly related to the immune system which is developed at an early stage after birth, it may have a correlation with the under-five year’s mortality rate of several regions. This scenario is observed in most of the developing and under developed countries of Asia and Africa region. It was observed from a literature survey that there are a number of authentic and flexible techniques for rapid identification of the PIDs suspects. In this context, JMF suggested 10 warning signs for PIDs may be used for preliminary screening out the PIDs suspects from the general group of population with the help of concerned parents and relatives. Earlier the disease was rarely diagnosed due to its wide genetic level variation in the genome, however, improvement in the diagnostic apparatus such as flow-cytometer, genomic PCRs, gene scan analysis, RT-PCR, next-generation sequencing (NGS) techniques make this disease understandable to the medical world. Not only that, nowadays there are many authentic treatment options such as HSCT, BMT, Ig etc. developed for PIDs sufferers. In developing and underdeveloped countries, PID is still being neglected by health professionals. Therefore, a proposal of combined treatment strategy for preventing this diseases morbidity is necessary.

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