Current Perspectives and Unmet Needs of Primary Immunodeficiency Care in Asia Pacific

Background: The Asia Pacific Society for Immunodeficiencies (APSID) conducted nine primary immunodeficiency (PID) Schools in 5 years since inauguration to provide PID care training for early career physicians in Asia Pacific, a region with divergent needs in PID resources and training. Objective: To identify differences in PID patient care resource and training needs across Asia Pacific and propose a corresponding action plan. Methods: The Human Development Index (HDI) indicates the degree of socio-economic development in each country/region. Information related to investigations and learning issues were extracted from the abstracts and personal statements from all Schools and mapped onto resource and training needs. Correlations between HDI and country/region-specific parameters were tested by two-tailed Pearson correlation. Results: A total of 427 abstracts were received in nine Schools between 2015 and 2020, predominantly on immunodeficiencies affecting cellular and humoral immunity. Genetic confirmation was described in 61.8% of abstracts, and its absence negatively correlated with HDI (r = −0.696, p = 0.004). Essential immunologic and genetic tests were not available in 25.4 and 29.5% of abstracts, respectively, and their absence negatively correlated with HDI (r = −0.788, p < 0.001; r = −0.739, p = 0.002). HDI positively correlated with average testing level (r = 0.742, p = 0.002). Cases from medium-HDI countries/regions focused on learning how to investigate a patient for PIDs in cases of severe or atypical infections, whereas those from very-high-HDI countries/regions, from which most faculty members originated, listed hematopoietic stem cell transplantation and gene therapy, newborn screening, and research as learning issues more frequently. Conclusion: There are unique HDI-related PID resource and training needs in each country/region. APSID proposes HDI group-specific strategies to improve PID care and education in her member countries/regions. Further quantitative analysis of needs in PID care in Asia Pacific is needed for lobbying governments to increase their support for PID care and research.

[1]  A. Uitterlinden,et al.  Rapid Low-Cost Microarray-Based Genotyping for Genetic Screening in Primary Immunodeficiency , 2020, Frontiers in Immunology.

[2]  Jeffrey R. Whiteaker,et al.  Multiplexed Proteomic Analysis for Diagnosis and Screening of Five Primary Immunodeficiency Disorders From Dried Blood Spots , 2020, Frontiers in Immunology.

[3]  J. Casanova,et al.  Human Inborn Errors of Immunity: 2019 Update on the Classification from the International Union of Immunological Societies Expert Committee , 2020, Journal of Clinical Immunology.

[4]  A. Rawat,et al.  Current status and prospects of primary immunodeficiency diseases in Asia , 2019, Genes & diseases.

[5]  Leung,et al.  Review of a Decade of International Experiences in Severe Combined Immunodeficiency Newborn Screening Using T-cell Receptor Excision Circle , 2020 .

[6]  M. V. van Zelm,et al.  Editorial: Primary Immunodeficiencies Worldwide , 2020, Frontiers in Immunology.

[7]  Raphael Scheible,et al.  The NEW ESID online database network , 2019, Bioinform..

[8]  Julie Wang,et al.  Newborn Screening for Severe Combined Immunodeficiency and T–Cell Lymphopenia in California, 2010–2017 , 2019, Pediatrics.

[9]  A. Rawat,et al.  Flow Cytometry for Diagnosis of Primary Immune Deficiencies—A Tertiary Center Experience From North India , 2019, Front. Immunol..

[10]  S. Ehl,et al.  The European Society for Immunodeficiencies (ESID) Registry Working Definitions for the Clinical Diagnosis of Inborn Errors of Immunity. , 2019, The journal of allergy and clinical immunology. In practice.

[11]  Y. Kodera,et al.  Advances in hematopoietic stem cell transplantation in the Asia-Pacific region: the second report from APBMT 2005–2015 , 2019, Bone Marrow Transplantation.

[12]  H. Koo,et al.  Allogeneic Hematopoietic Cell Transplantation in Patients with Primary Immunodeficiencies in Korea: Eleven-Year Experience in a Single Center , 2018, Journal of Clinical Immunology.

[13]  R. Uppuluri,et al.  Hematopoietic Stem Cell Transplantation for Primary Immunodeficiency Disorders: Experience from a Referral Center in India , 2018, Indian Pediatrics.

[14]  Smita Y. Patel,et al.  The United Kingdom Primary Immune Deficiency (UKPID) registry 2012 to 2017 , 2018, Clinical and experimental immunology.

[15]  R. Kobayashi,et al.  Dried Blood Spots, an Affordable Tool to Collect, Ship, and Sequence gDNA from Patients with an X-Linked Agammaglobulinemia Phenotype Residing in a Developing Country , 2018, Front. Immunol..

[16]  S. Reda,et al.  Allergy and immunology in Africa: Challenges and unmet needs. , 2017, The Journal of allergy and clinical immunology.

[17]  Tong-Xin Chen,et al.  Family History of Early Infant Death Correlates with Earlier Age at Diagnosis But Not Shorter Time to Diagnosis for Severe Combined Immunodeficiency , 2017, Front. Immunol..

[18]  Joel E. Oestreich UNITED NATIONS DEVELOPMENT PROGRAMME , 2000 .

[19]  A. Rawat,et al.  Primary Immunodeficiency Disorders in India—A Situational Review , 2017, Front. Immunol..

[20]  M. González-Serrano,et al.  Latin American challenges with the diagnosis and treatment of primary immunodeficiency diseases , 2017, Expert review of clinical immunology.

[21]  M. Pac,et al.  Comprehensive activities to increase recognition of primary immunodeficiency and access to immunoglobulin replacement therapy in Poland , 2016, European Journal of Pediatrics.

[22]  Ppw,et al.  Addressing Training Needs and Professional Advancement : A Postgraduate Study Day for Subspecialty Training , 2016 .

[23]  J. Franco,et al.  Current state and future perspectives of the Latin American Society for Immunodeficiencies (LASID). , 2015, Allergologia et immunopathologia.

[24]  M. van der Burg,et al.  TREC Based Newborn Screening for Severe Combined Immunodeficiency Disease: A Systematic Review , 2015, Journal of Clinical Immunology.

[25]  Smita Y. Patel,et al.  The United Kingdom Primary Immune Deficiency (UKPID) Registry: report of the first 4 years' activity 2008–2012 , 2014, Clinical and experimental immunology.

[26]  Y. Lau,et al.  Considerations for Primary Immune Deficiency Disorders in Asia , 2014 .

[27]  Y. Lau,et al.  Endemic infections in Southeast Asia provide new insights to the phenotypic spectrum of primary immunodeficiency disorders. , 2013, Asian Pacific journal of allergy and immunology.

[28]  B. Knoppers,et al.  Whole-genome sequencing in health care , 2013, European Journal of Human Genetics.

[29]  W. Al-Herz,et al.  Combined immunodeficiency: the Middle East experience. , 2013, The Journal of allergy and clinical immunology.

[30]  Y. Lau,et al.  Improving care, education, and research: the Asian primary immunodeficiency network , 2011, Annals of the New York Academy of Sciences.

[31]  A. Fischer,et al.  Building networks for immunodeficiency diseases and immunology training , 2008, Nature Immunology.

[32]  A. Heinz,et al.  Postgraduate training for young psychiatrists—experience of the Berlin Summer School , 2006, European Psychiatry.

[33]  J. Puck,et al.  Development of population-based newborn screening for severe combined immunodeficiency. , 2005, The Journal of allergy and clinical immunology.

[34]  L. Notarangelo,et al.  Diagnostic Criteria for Primary Immunodeficiencies , 1999 .

[35]  A. Schäffer,et al.  Genetic linkage of hyper-IgE syndrome to chromosome 4. , 1999, American journal of human genetics.