{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,7]],"date-time":"2026-05-07T18:48:37Z","timestamp":1778179717868,"version":"3.51.4"},"reference-count":11,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2025,12,22]],"date-time":"2025-12-22T00:00:00Z","timestamp":1766361600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2025,12,22]],"date-time":"2025-12-22T00:00:00Z","timestamp":1766361600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Hepatology Research"],"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background &amp; Aim<\/jats:title>\n                    <jats:p>Classic hepatic glycogen storage disease IV (GSD\u2010IV) is widely regarded as an irreversible disorder that causes fatal cirrhosis by the age of 5\u00a0years unless liver transplantation is performed. We describe the first patient with biopsy\u2010proven classic hepatic GSD\u2010IV who survived into middle adulthood without transplantation and showed histological reversal of cirrhosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case<\/jats:title>\n                    <jats:p>\n                      We followed an infant diagnosed at 11\u00a0months with hepatosplenomegaly, severe aminotransferase elevation, and near\u2010absent erythrocyte branching enzyme activity. Liver biopsies at ages 4 and 12\u00a0years confirmed polyglucosan\u2010laden METAVIR F4 cirrhosis, and two episodes of variceal bleeding were controlled endoscopically and surgically. Thereafter, without disease\u2010specific therapy, the aminotransferase level, platelet count, and portal hemodynamics gradually normalized. A third biopsy at age 43 showed complete disappearance of polyglucosan bodies and regression of fibrosis to F0, despite persistently minimal enzyme activity and compound heterozygous\n                      <jats:italic>GBE1<\/jats:italic>\n                      variants in trans: c.137A\u00a0&gt;\u00a0C p.(Gln46Pro) and the novel c.1340T\u00a0&gt;\u00a0C p.(Leu447Pro).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>This unprecedented case demonstrates that biochemical and histological remission is possible in classic hepatic GSD\u2010IV despite persistently deficient branching\u2010enzyme activity. Early, aggressive management of portal hypertension may therefore permit long\u2010term survival and full histological recovery, challenging the transplant\u2010first paradigm and supporting individualized, transplant\u2010sparing care.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/hepr.70084","type":"journal-article","created":{"date-parts":[[2025,12,22]],"date-time":"2025-12-22T16:50:49Z","timestamp":1766422249000},"page":"629-634","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Biopsy\u2010Proven Reversal of F4 Cirrhosis in Classic Hepatic Glycogen Storage Disease Type IV: A 42\u2010Year Follow\u2010Up Without Transplantation"],"prefix":"10.1111","volume":"56","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-0226-5981","authenticated-orcid":false,"given":"Masaaki","family":"Mino","sequence":"first","affiliation":[{"name":"Department of Gastroenterology Graduate School of Biomedical and Health Sciences Hiroshima University  Hiroshima Japan"},{"name":"Department of Gastroenterology and Hepatology National Kohnodai Medical Center Japan Institute for Health Security  Ichikawa Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nami","family":"Mori","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology Hiroshima Red Cross Hospital and Atomic\u2010Bomb Survivors Hospital  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yumi","family":"Shimomura","sequence":"additional","affiliation":[{"name":"Program of Biotechnology Graduate School of Integrated Sciences for Life Hiroshima University  Higashi\u2010Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Taiji","family":"Yamazoe","sequence":"additional","affiliation":[{"name":"Department of Liver Diseases The Research Center for Hepatitis and Immunology National Institute of Global Health and Medicine Japan Institute for Health Security  Ichikawa Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8492-5339","authenticated-orcid":false,"given":"Shintaro","family":"Takaki","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology Hiroshima Red Cross Hospital and Atomic\u2010Bomb Survivors Hospital  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daiki","family":"Miki","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology Graduate School of Biomedical and Health Sciences Hiroshima University  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4000-835X","authenticated-orcid":false,"given":"Keiji","family":"Tsuji","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology Hiroshima Red Cross Hospital and Atomic\u2010Bomb Survivors Hospital  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kazuko","family":"Hamamoto","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Hiroshima Red Cross Hospital and Atomic\u2010Bomb Survivors Hospital  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tokiko","family":"Fukuda","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Hamamatsu University School of Medicine  Hamamatsu Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hideo","family":"Sugie","sequence":"additional","affiliation":[{"name":"Faculty of Health and Medical Sciences Tokoha University  Hamamatsu Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7591-8287","authenticated-orcid":false,"given":"Masataka","family":"Tsuge","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology Graduate School of Biomedical and Health Sciences Hiroshima University  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1652-0743","authenticated-orcid":false,"given":"Shiro","family":"Oka","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology Graduate School of Biomedical and Health Sciences Hiroshima University  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,12,22]]},"reference":[{"issue":"1","key":"e_1_2_11_2_1","first-page":"11","article-title":"Familial Cirrhosis of the Liver With Storage of Abnormal Glycogen","volume":"5","author":"Andersen D. 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