2023
DOI: 10.1016/j.jaip.2022.10.003
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The Middle East and North Africa Diagnosis and Management Guidelines for Inborn Errors of Immunity

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Cited by 37 publications
(14 citation statements)
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“…All available patients with known genetic defects in the B cell development and differentiation stages were included from the cases registered in the national IEI registry at Children’s Medical Center Hospital in Iran. The diagnosis of IEI was established based on the updated clinical diagnostic criteria recommended by the European Society for Immunodeficiencies (ESID) [ 17 ] and the Middle East and North Africa Diagnosis and Management Guidelines for IEI [ 18 ]. Genes involved in different stages of B lymphocyte differentiation, as well as intrinsic and mixed extrinsic and intrinsic genes, were categorized according to Amirifar et al [ 19 ].…”
Section: Methodsmentioning
confidence: 99%
“…All available patients with known genetic defects in the B cell development and differentiation stages were included from the cases registered in the national IEI registry at Children’s Medical Center Hospital in Iran. The diagnosis of IEI was established based on the updated clinical diagnostic criteria recommended by the European Society for Immunodeficiencies (ESID) [ 17 ] and the Middle East and North Africa Diagnosis and Management Guidelines for IEI [ 18 ]. Genes involved in different stages of B lymphocyte differentiation, as well as intrinsic and mixed extrinsic and intrinsic genes, were categorized according to Amirifar et al [ 19 ].…”
Section: Methodsmentioning
confidence: 99%
“…Emerging genomics platforms now offer the promise of identifying previously elusive IEI genetic lesions [144][145][146], but also demand the development of novel bioinformatics pipelines, data infrastructures, and analytical strategies. All of these considerations highlight the urgent need for flexible, evolving and realistic demographic-appropriate guidelines, as well as acknowledging and addressing the systemic biases currently found in our data [147]. Finally, regardless of the assays chosen, there is no replacement for the precise clinical and immune phenotyping needed to help navigate the complexities of the IEI landscape.…”
Section: Many Of Our Index Patients Did Not Present With the Most 'Ca...mentioning
confidence: 99%
“…She did not have any liver test or imaging abnormality before HSCT. She was on antimicrobial prophylaxis (trimethoprim/sulfamethoxazole [TMP-SMZ], acyclovir, and fluconazole) for infection since first month as suggested by the guideline 3 . Family history was remarkable with a cousin with SCID and 2 siblings who died in the neonatal period.…”
Section: Casementioning
confidence: 99%
“…She was on antimicrobial prophylaxis (trimethoprim/sulfamethoxazole [TMP-SMZ], acyclovir, and fluconazole) for infection since first month as suggested by the guideline. 3 Family history was remarkable with a cousin with SCID and 2 siblings who died in the neonatal period. Her postnatal course was complicated with pneumonia at fifth day of life which was managed with gentamicin and ampicillin.…”
Section: Casementioning
confidence: 99%