Considerations for Primary Immune Deficiency Disorders in Asia

Lee, Pamela; Lau, Yu‐Lung

doi:10.1016/b978-0-12-405546-9.00055-8

Cited by 4 publications

(5 citation statements)

References 66 publications

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“…Madkaikar et al reported 159 PID cases from a tertiary care center in India in 2013 followed by a more recent report describing 778 registered patients. Frequencies of PIDs were as follows: diseases of immune dysregulation (23.1%), phagocytic defects (21.3%) and predominant antibody deficiency Other registries were reported from Oman (140 patients with high proportion of patients with congenital defects of phagocyte 35%) [44], Taiwan (215 patients with 50.6% molecular diagnosis) [13], Malaysia (150 patients) [43,45], Thailand (67 patients) [46], Jordan (53 patients) [47], Singapore (39 patients) [48], UAE (30 patients) [49] and Bangladesh (13 patients) [50].…”

Section: Article Highlightsmentioning

confidence: 99%

“…In 2013, Ehlayel et al have reported a single-center registry of 131 pediatric PID patients from Qatar with the lowest number of classical PID diseases and only 23.7% predominant antibody deficiency (prevalence of PID of 1.1 per 100000 children, genetic diagnosis of 36.6%, parental consanguinity of 61.1%)[40]. Although a cohort from Hong Kong also reported 107 genetically solved PID patients, the current number of patients from this national registry is not updated and spans back to 2014 with 180 reported patients[41][42][43]. Rhim et al have reported 152 PID patients from Korea up to 2012 with an estimated prevalence of 1.1 per 100000 children with the highest rate of males to females with PID in Asia (~3.6)[34].…”

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confidence: 99%

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Global systematic review of primary immunodeficiency registries

Abolhassani

Azizi

Sharifi

et al. 2020

Expert Review of Clinical Immunology

100

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Introduction: During the last 4 decades, registration of patients with primary immunodeficiencies (PID) has played an essential role in different aspects of these diseases worldwide including epidemiological indexes, policymaking, quality controls of care/life, facilitation of genetic studies and clinical trials as well as improving our understanding about the natural history of the disease and the immune system function. However, due to the limitation of sustainable resources supporting these registries, inconsistency in diagnostic criteria and lack of molecular diagnosis as well as difficulties in the documentation and designing any universal platform, the global perspective of these diseases remains unclear. Areas covered: Published and unpublished studies from January 1981 to June 2020 were systematically reviewed on PubMed, Web of Science and Scopus. Additionally, the reference list of all studies was hand-searched for additional studies. This effort identified a total of 104614 registered patients and suggests identification of at least 10590 additional PID patients, mainly from countries located in Asia and Africa. Molecular defects in genes known to cause PID were identified and reported in 13852 (13.2% of all registered) patients. Expert opinion: Although these data suggest some progress in the identification and documentation of PID patients worldwide, achieving the basic requirement for the global PID burden estimation and registration of undiagnosed patients will require more reinforcement of the progress, involving both improved diagnostic facilities and neonatal screening.

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Section: Article Highlightsmentioning

confidence: 99%

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confidence: 99%

Global systematic review of primary immunodeficiency registries

Abolhassani

Azizi

Sharifi

et al. 2020

Expert Review of Clinical Immunology

100

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“…In Asia, the mortality rate of patients with IEI between birth and five years old is approximately 15 of 1000 live births. 5 Infectious susceptibility is typically associated with the type of immune system defect. 2 The oral cavity is protected by immune cells and antibodies from the circulation, lymphoid organs and mucosal immunity.…”

Section: Introductionmentioning

confidence: 99%

“…Inborn errors of immunity are life‐threatening. In Asia, the mortality rate of patients with IEI between birth and five years old is approximately 15 of 1000 live births 5 . Infectious susceptibility is typically associated with the type of immune system defect 2 .…”

Section: Introductionmentioning

confidence: 99%

Orodental health status of patients with inborn errors of immunity

Chaiboonyarak,

Chantarangsu,

Gavila

et al. 2023

Int J Paed Dentistry

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BackgroundVarious orodental problems affect patients with inborn errors of immunity (IEI), but there are limited studies on these issues.AimTo study orodental status and its confounding factors in patients with IEI.DesignCaries, enamel defects, gingival, and soft tissue conditions were examined. Data on patient characteristics, dental hygiene habits, dental attendance, and household income were collected. Statistical analysis and logistic regression were performed.ResultsForty‐five participants with a mean age of 9.20 ± 6.41 years were included. Almost all participants had gingivitis (42 of 45; 93.3%), whereas a small number had periodontitis (five of 45; 11.1%). Calculus was found in 33 (73.3%) and caries in 30 (66.7%). Mucosal ulcers, enamel defects, and candidiasis were observed in 23 of 45 (51.1%), 16 of 43 (37.2%), and six of 43 (14.0%), respectively. Chances of having caries, moderate‐to‐severe gingivitis, periodontitis, calculus, and ulcers increased with age. Taking antibiotics in the last two months increased the risk of caries by five times. Lower income increased the risk of calculus deposit by nine times.ConclusionGingivitis, calculus, caries, and mucosal ulcers were the most common orodental findings in patients with IEI. Antibiotics increased the risk of caries, and low‐income children had higher calculus accumulation.

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“…There exist large differences in various facets of human development such as health, standard of living, and education across Asia Pacific, as reflected by the United Nations Development Program 2019 Human Development Index (HDI), which result in significant differences in the level of care available for patients with rare diseases in each country/region (4). Countries/regions of very high HDIs such as Hong Kong and Japan have adequate expertise and resources for the diagnosis and management of PIDs along with structured pediatric and adult immunology training programs, whereas these are lacking in countries with medium HDIs such as Myanmar and Cambodia (5).…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2020

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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.

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Considerations for Primary Immune Deficiency Disorders in Asia

Cited by 4 publications

References 66 publications

Global systematic review of primary immunodeficiency registries

Global systematic review of primary immunodeficiency registries

Orodental health status of patients with inborn errors of immunity

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

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