{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T01:18:03Z","timestamp":1787015883329,"version":"build-2736575974"},"reference-count":56,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2019,5,15]],"date-time":"2019-05-15T00:00:00Z","timestamp":1557878400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Allergy Immunology"],"published-print":{"date-parts":[[2019,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Hyper\u2010IgE syndromes (HIES) are distinct diseases characterized by recurrent cutaneous and lung infections, eczema, and elevated serum IgE level.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>In this study, clinical manifestations, immunologic findings, and genetic studies of all patients with HIES in the Iranian national registry database were evaluated.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      A total of 129 HIES patients with a median age of 14.0 (9.0\u201024.0) years were followed up for a total of 307.8 patient\u2010years. Genetic studies showed heterozygous\n                      <jats:italic>STAT3<\/jats:italic>\n                      mutations in 19 patients and homozygous\n                      <jats:italic>DOCK8<\/jats:italic>\n                      mutation in 16 patients. The mean of National Institutes of Health score in STAT3\u2010deficient patients was higher than in patients with\n                      <jats:italic>DOCK8<\/jats:italic>\n                      mutation (\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.001). It was shown that the presence of pneumatocele and hematologic complication were significantly frequent in STAT3\u2010deficient cases compared to patients with DOCK8 deficiency (\n                      <jats:italic>P\u00a0<\/jats:italic>\n                      =\u00a00.001 and\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.002, respectively). Moreover, the median IgE serum levels were higher in patients with\n                      <jats:italic>STAT3<\/jats:italic>\n                      gene mutation than in patients with\n                      <jats:italic>DOCK8<\/jats:italic>\n                      gene mutation (\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.02). The eosinophils\u2019 count was enhanced in patients with DOCK8 deficiency than in patients with\n                      <jats:italic>STAT3<\/jats:italic>\n                      gene defects (\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.02).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>\n                      Specific molecular study of\n                      <jats:italic>STAT3<\/jats:italic>\n                      and\n                      <jats:italic>DOCK8<\/jats:italic>\n                      mutations in patients with HIES clinical phenotype could help the physician to definitively characterize the disease. Since HIES showed the highest rate of unsolved combined immunodeficiency, investigation of other genetic and environmental factors could also help in understanding the mechanism of remaining patients as well as providing strategy into therapeutic modalities.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/pai.13043","type":"journal-article","created":{"date-parts":[[2019,2,25]],"date-time":"2019-02-25T23:36:31Z","timestamp":1551137791000},"page":"469-478","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":21,"title":["The first cohort of Iranian patients with hyper immunoglobulin E syndrome: A long\u2010term follow\u2010up and genetic analysis"],"prefix":"10.1111","volume":"30","author":[{"given":"Mahmood","family":"Tavassoli","sequence":"first","affiliation":[{"name":"Department of Immunology, School of Medicine Isfahan University of Medical Sciences Isfahan 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