{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T05:18:58Z","timestamp":1789017538699,"version":"build-2803163510"},"reference-count":46,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2018,7,10]],"date-time":"2018-07-10T00:00:00Z","timestamp":1531180800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Health Labour Sciences Research Grants for Research on Intractable Disease","award":["H29-015"],"award-info":[{"award-number":["H29-015"]}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatrics International"],"published-print":{"date-parts":[[2018,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>\n                      Shwachman\u2013Diamond syndrome (\n                      <jats:styled-content style=\"fixed-case\">SDS<\/jats:styled-content>\n                      ) is a rare multisystem disorder associated with exocrine pancreatic insufficiency. The present study reports the results of a nationwide survey and a systematic review on\n                      <jats:styled-content style=\"fixed-case\">SDS<\/jats:styled-content>\n                      to develop consensus guidelines for intractable diarrhea including\n                      <jats:styled-content style=\"fixed-case\">SDS<\/jats:styled-content>\n                      .\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      Questionnaires were sent to 616 departments of pediatrics or of pediatric surgery in Japan in a nationwide survey. A second questionnaire was sent to doctors who had treated\n                      <jats:styled-content style=\"fixed-case\">SDS<\/jats:styled-content>\n                      patients and included questions on clinical information. Additionally, a systematic review was performed using digital literature databases to assess the influence of medical (i.e. non\u2010surgical) treatment on\n                      <jats:styled-content style=\"fixed-case\">SDS<\/jats:styled-content>\n                      prognosis.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Answers were received from 529 institutions (85.9%), which included information on 24 patients with\n                      <jats:styled-content style=\"fixed-case\">SDS<\/jats:styled-content>\n                      (median age, 10.4\u00a0years; male,\n                      <jats:italic>n<\/jats:italic>\n                      \u00a0=\u00a015) treated from January 2005 to December 2014. Although 75% of patients received pancreatic enzyme replacement therapy, there was no significant association between treatment and prognosis. Systematic review identified one clinical practice guideline, two case series, eight case reports and 26 reviews. Patient information from those studies was insufficient for meta\u2010analysis.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      The rarity of\n                      <jats:styled-content style=\"fixed-case\">SDS<\/jats:styled-content>\n                      makes it difficult to establish evidence\u2010based treatment for\n                      <jats:styled-content style=\"fixed-case\">SDS<\/jats:styled-content>\n                      . According to the limited information from patients and published reports, medical treatment for malabsorption due to\n                      <jats:styled-content style=\"fixed-case\">SDS<\/jats:styled-content>\n                      should be performed to improve fat absorption and stool condition, but it is not clear whether this treatment improves the prognosis of malabsorption.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/ped.13601","type":"journal-article","created":{"date-parts":[[2018,5,27]],"date-time":"2018-05-27T05:24:41Z","timestamp":1527398681000},"page":"719-726","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Shwachman\u2013Diamond syndrome: Nationwide survey and systematic review in 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