{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T22:00:26Z","timestamp":1790200826603,"version":"4.1.0"},"reference-count":17,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2024,8,30]],"date-time":"2024-08-30T00:00:00Z","timestamp":1724976000000},"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 Transplantation"],"published-print":{"date-parts":[[2024,11]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Liver transplantation (LT) normalizes fasting tolerance in glycogen storage disease type (GSD) 1b. However, reported outcomes post\u2010LT with respect to correction of neutropenia, infection risk and growth are varied. Sodium\u2010glucose cotransporter\u20102 (SGLT2) inhibitors have been recently shown to improve neutropenia in GSD1b patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In this single\u2010center retrospective study, we reviewed all children who underwent LT for GSD1b. Neutropenia, dose of granulocyte colony\u2010stimulating factor (G\u2010CSF), unplanned hospital attendance, anthropometrics, graft rejection, survival, and the effects of dapagliflozin were analyzed. Data from protocol biopsies obtained at 1, 5, and 10\u2009years post\u2010LT and immunosuppression levels were collected.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Eight children (6 female), all on G\u2010CSF pre\u2010transplant, underwent cadaveric LT for GSD1b at median age 3.6\u2009years (IQR 3.3\u20135.1) with mean follow\u2010up time of 10.3\u2009years (95% CI 7.5\u201313.1). Neutrophil count and G\u2010CSF requirement did not improve post\u2010LT. Although a reduction in unplanned hospital attendance due to infection (0.98 [95% CI 0.76\u20131.26] vs. 0.49 [95% CI 0.41 to 0.57] per person\u2010year, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.01) was observed, gastrointestinal complaints and graft dysfunction accounted for a similar hospitalization burden pre\u2010 versus post\u2010LT. Body mass index (BMI) reduced post\u2010LT (<jats:italic>Z<\/jats:italic>\u2010score 1.47 [95%CI 0.39\u20132.23] vs. 0.56 [95% CI \u22120.74 to 1.45], <jats:italic>p<\/jats:italic>\u2009=\u20090.02), with no significant change in height.<\/jats:p><jats:p>Although all children and grafts have survived, 75% of recipients developed rejection, despite adequate immunosuppression levels, with two children having been found to have developed significant fibrosis on their 5\u2010year protocol biopsy. Although dapagliflozin allowed cessation of G\u2010CSF, no improvement in neutrophil count was observed. Despite this, a reduction in gastrointestinal and infection\u2010related morbidity was noted following dapagliflozin.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Although LT normalizes fasting tolerance in GSD1b and reduces hospital attendance due to infection, morbidity from infection and gastrointestinal manifestations persist. Children in our cohort experienced high rates of rejection necessitating titration of immunosuppression to balance risk of infection against organ rejection. Future studies should investigate whether early introduction of SGLT2 inhibitors post\u2010LT impact morbidity in this group.<\/jats:p><\/jats:sec>","DOI":"10.1111\/petr.14839","type":"journal-article","created":{"date-parts":[[2024,8,30]],"date-time":"2024-08-30T09:31:08Z","timestamp":1725010268000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Outcomes of Pediatric Liver Transplantation in Glycogen Storage Disease Type 1b\u2014A Single\u2010Center Experience"],"prefix":"10.1111","volume":"28","author":[{"given":"Vivek","family":"Rajasekaran","sequence":"first","affiliation":[{"name":"Liver Unit (Including Small Bowel Transplantation) Birmingham Children's Hospital  Birmingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saikat","family":"Santra","sequence":"additional","affiliation":[{"name":"Department of Clinical Inherited Metabolic Disorders Birmingham Children's Hospital  Birmingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chayarani","family":"Kelgeri","sequence":"additional","affiliation":[{"name":"Liver Unit (Including Small Bowel Transplantation) Birmingham Children's Hospital  Birmingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lauren","family":"Johansen","sequence":"additional","affiliation":[{"name":"Liver Unit (Including Small Bowel Transplantation) Birmingham Children's Hospital  Birmingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suresh","family":"Vijay","sequence":"additional","affiliation":[{"name":"Department of Clinical Inherited Metabolic Disorders Birmingham Children's Hospital  Birmingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sreevidya","family":"Sreekantam","sequence":"additional","affiliation":[{"name":"Department of Clinical Inherited Metabolic Disorders Birmingham Children's Hospital  Birmingham 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UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Girish","family":"Gupte","sequence":"additional","affiliation":[{"name":"Liver Unit (Including Small Bowel Transplantation) Birmingham Children's Hospital  Birmingham 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