{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T17:40:41Z","timestamp":1791222041842,"version":"4.1.0"},"reference-count":24,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2026,5,25]],"date-time":"2026-05-25T00:00:00Z","timestamp":1779667200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,5,25]],"date-time":"2026-05-25T00:00:00Z","timestamp":1779667200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["RC2DK122533"],"award-info":[{"award-number":["RC2DK122533"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R24AI162637"],"award-info":[{"award-number":["R24AI162637"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>\n                    Shwachman\u2010Diamond syndrome (SDS) is an inherited bone marrow failure syndrome characterized by neutropenia and pancreatic insufficiency. The current understanding of immune function in SDS is limited. We performed a retrospective study of the US\u2010based Shwachman\u2010Diamond Syndrome Registry (SDSR) to characterize the immunological profile of patients with SDS. Data were obtained from chart review of patients with biallelic mutations in\n                    <jats:italic>SBDS<\/jats:italic>\n                    enrolled on the SDSR (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u2009223) or followed clinically at participating institutions with local IRB approval (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u20096). Immunological data were available from 409 time points on 98 individuals with SDS at healthy time points. We identified limited immune deficits, largely quantitative changes in the B\u2010cell compartment in a subset of patients with SDS including low absolute B cells in 31% of patients and low IgM in 30% of patients. Patients with SDS with low B cells may be at increased risk for sinopulmonary, skin or soft tissue infections particularly if they also have low IgG levels. These data support a baseline immunological evaluation, including a full blood count, lymphocyte subpopulations and immunoglobulin levels, for patients with SDS. However, it is reasonable to reserve more detailed assessments for patients with recurrent or severe infections.\n                  <\/jats:p>","DOI":"10.1111\/bjh.70553","type":"journal-article","created":{"date-parts":[[2026,5,26]],"date-time":"2026-05-26T03:32:08Z","timestamp":1779766328000},"page":"180-188","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Beyond neutropenia: Immunological evaluation of patients with Shwachman\u2010Diamond syndrome"],"prefix":"10.1111","volume":"209","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8105-7011","authenticated-orcid":false,"given":"Nicholas J.","family":"Gloude","sequence":"first","affiliation":[{"name":"Division of Pediatric Hematology Oncology, Department of Pediatrics, Rady Children's Hospital San Diego University of California San Diego  San Diego California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karyn","family":"Brundige","sequence":"additional","affiliation":[{"name":"Dana\u2010Farber and Boston Children's Cancer and Blood Disorders Center Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Loveless","sequence":"additional","affiliation":[{"name":"Division of Bone Marrow Transplantation and Immune Deficiency Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ian","family":"Atkinson","sequence":"additional","affiliation":[{"name":"Dana\u2010Farber and Boston Children's Cancer and Blood Disorders Center Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sharat","family":"Chandra","sequence":"additional","affiliation":[{"name":"Division of Bone Marrow Transplantation and Immune Deficiency Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"},{"name":"Department of Pediatrics University of Cincinnati College of Medicine  Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard","family":"Cooper","sequence":"additional","affiliation":[{"name":"Division of Bone Marrow Transplantation and Immune Deficiency Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine","family":"Coyne","sequence":"additional","affiliation":[{"name":"Dana\u2010Farber and Boston Children's Cancer and Blood Disorders Center Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire","family":"Dusa","sequence":"additional","affiliation":[{"name":"Division of Bone Marrow Transplantation and Immune Deficiency Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashley","family":"Galvin","sequence":"additional","affiliation":[{"name":"Dana\u2010Farber and Boston Children's Cancer and Blood Disorders Center Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy","family":"Geddis","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Hematology\/Oncology, Cancer and Blood Disorders Center Seattle Children's Hospital  Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margret","family":"Joos","sequence":"additional","affiliation":[{"name":"Dana\u2010Farber and Boston Children's Cancer and Blood Disorders Center Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maggie","family":"Malsch","sequence":"additional","affiliation":[{"name":"Dana\u2010Farber and Boston Children's Cancer and Blood Disorders Center Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8919-5243","authenticated-orcid":false,"given":"Thomas","family":"Michniacki","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, C. 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