{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,11]],"date-time":"2026-09-11T02:37:29Z","timestamp":1789094249403,"version":"build-2803163510"},"reference-count":25,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2022,1,19]],"date-time":"2022-01-19T00:00:00Z","timestamp":1642550400000},"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":["Scand J Immunol"],"published-print":{"date-parts":[[2022,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background and objectives<\/jats:title>\n                    <jats:p>Glucose\u20106\u2010phosphate catalytic subunit 3 (G6PC3) deficiency is characterized by severe congenital neutropenia with recurrent pyogenic infections, a prominent superficial venous pattern and cardiovascular and urogenital malformations caused by an alteration of glucose homeostasis, with increased endoplasmic reticulum stress and cell apoptosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We reviewed our patients with G6PC3 deficiency diagnosed along the last decade in Mexico; we also searched the PubMed\/Medline database for the terms (\u2018G6PC3 deficiency\u2019 OR \u2018Dursun syndrome\u2019 OR \u2018Severe congenital neutropenia type 4\u2019), and selected articles published in English from 2009 to 2020.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>We found 89 patients reported from at least 14 countries in 4 continents. We describe five new cases from Mexico. Of the 94 patients, 56% are male, 48% from Middle East countries and none of them had adverse reactions to live vaccines; all presented with at least 1 severe infection prior to age 2. Seventy\u2010five per cent had syndromic features, mainly atrial septal defect in 55% and prominent superficial veins in 62%.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>With a total of 94 patients reported in the past decade, we delineate the most frequent laboratory and genetic features, their treatment and outcomes, and to expand the knowledge of syndromic and non\u2010syndromic phenotypes in these patients.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/sji.13136","type":"journal-article","created":{"date-parts":[[2021,12,29]],"date-time":"2021-12-29T04:25:40Z","timestamp":1640751940000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Severe congenital neutropenia due to G6PC3 deficiency: Case series of five patients and literature review"],"prefix":"10.1111","volume":"95","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2936-3679","authenticated-orcid":false,"given":"Natalia","family":"Velez\u2010Tirado","sequence":"first","affiliation":[{"name":"Clinical Immunology Cl\u00ednica del Country  Bogot\u00e1 Colombia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco Antonio","family":"Yamazaki\u2010Nakashimada","sequence":"additional","affiliation":[{"name":"Clinical Immunology Service at the National Institute of Pediatrics  Mexico City Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Enrique","family":"Lopez Valent\u00edn","sequence":"additional","affiliation":[{"name":"Allergy Department Hospital del Ni\u00f1o de Toluca  Toluca Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Armando","family":"Partida\u2010Gaytan","sequence":"additional","affiliation":[{"name":"Fundacion Mexicana Para Ni\u00f1os Con Inmunodeficiencias, AC  Mexico City Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Selma C.","family":"Scheffler\u2010Mendoza","sequence":"additional","affiliation":[{"name":"Clinical Immunology Service at the National Institute of Pediatrics  Mexico City Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Genny M.","family":"Chaia Semerena","sequence":"additional","affiliation":[{"name":"Hospital Angeles Metropolitano  Mexico City Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arist\u00f3teles","family":"Alvarez\u2010Cardona","sequence":"additional","affiliation":[{"name":"Unidad de investigacion en Inmunologia Clinica y Alergia Aguascalientes  Aguascalientes Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcos Alejandro","family":"Su\u00e1rez Guti\u00e9rrez","sequence":"additional","affiliation":[{"name":"Unidad de investigacion en Inmunologia Clinica y Alergia Aguascalientes  Aguascalientes 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