{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T02:54:41Z","timestamp":1772160881839,"version":"3.50.1"},"reference-count":21,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2022,12,19]],"date-time":"2022-12-19T00:00:00Z","timestamp":1671408000000},"content-version":"vor","delay-in-days":18,"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":[[2022,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>There is an increased demand for hematopoietic stem cell transplant (HSCT) to treat various diseases including combined immunodeficiencies (CID), with limited worldwide availability. Variables affecting the decision regarding CID patients' prioritization for HSCT are not known. We aimed to determine general, clinical, and immunologic factors associated with the higher risk of early death (\u22646\u00a0months after diagnosis) in untransplanted CID patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Data collection was done retrospectively from five centers and included general patients' information, and clinical and laboratory variables. Inclusion criteria were untransplanted patients who are either dead or alive with a follow\u2010up period \u22656\u00a0months after diagnosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Two hundred and thirty\u2010six CID patients were reported by participating centers, of whom 111 were included in the study with a cumulative follow\u2010up period of 278.6\u2009years. Seventy\u2010two patients died with the median age of death of 10.5\u2009months. 35.1% of the patients succumbed within 6\u2009months after the diagnosis. Having a history of\n                      <jats:italic>Candida<\/jats:italic>\n                      infections, sepsis or hepatomegaly was associated with an increased risk of early death. None of the other general or clinical variables was associated with such risk. Bivariate analysis of lymphocyte subsets showed that patients with the following counts: CD3\n                      <jats:sup>+<\/jats:sup>\n                      \u00a0&lt;\u2009100, CD4\n                      <jats:sup>+<\/jats:sup>\n                      \u00a0&lt;\u2009200, CD8\n                      <jats:sup>+<\/jats:sup>\n                      \u00a0&lt;\u200950, or CD16\n                      <jats:sup>+<\/jats:sup>\n                      CD56\n                      <jats:sup>+<\/jats:sup>\n                      &lt;200\u2009cells\/\u03bcl had increased risk of early death. In adjusted analysis, increased risk of early death was observed among patients with CD3\n                      <jats:sup>+<\/jats:sup>\n                      count &lt;100 cells\/\u03bcl.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>\n                      Combined immunodeficiencies patients with a history of\n                      <jats:italic>Candida<\/jats:italic>\n                      infections, sepsis, hepatomegaly, or severe T\u2010lymphopenia should be given priority for HSCT to avoid early death.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/pai.13901","type":"journal-article","created":{"date-parts":[[2022,12,19]],"date-time":"2022-12-19T09:31:17Z","timestamp":1671442277000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Predictors of early death risk among untransplanted patients with combined immunodeficiencies affecting cellular and humoral immunity: A multicenter report"],"prefix":"10.1111","volume":"33","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4244-4489","authenticated-orcid":false,"given":"Waleed","family":"Al\u2010Herz","sequence":"first","affiliation":[{"name":"Department of Pediatrics, College of Medicine Kuwait University  Kuwait City Kuwait"},{"name":"Allergy and Clinical Immunology Unit, Pediatric Department Al\u2010Sabah Hospital  Kuwait City Kuwait"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3099-4424","authenticated-orcid":false,"given":"Ali H.","family":"Ziyab","sequence":"additional","affiliation":[{"name":"Department of Community Medicine and Behavioral Sciences, Faculty of Medicine Kuwait University  Kuwait City Kuwait"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mehdi","family":"Adeli","sequence":"additional","affiliation":[{"name":"Division of Immunology and Allergy Sidra Medicine and Hamad Medical Corporation  Doha Qatar"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tariq","family":"Al Farsi","sequence":"additional","affiliation":[{"name":"Department of Pediatric Allergy and Clinical Immunology The Royal Hospital  Muscat Oman"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Suleiman","family":"Al\u2010Hammadi","sequence":"additional","affiliation":[{"name":"College of Medicine Mohammed Bin Rashid University for Medicine and Health Sciences  Dubai United Arab Emirates"},{"name":"Al Jalila Children's Hospital  Dubai United Arab Emirates"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amna Ali","family":"Al Kuwaiti","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Tawam Hospital  Al\u2010Ain United 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