{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T07:53:12Z","timestamp":1793173992351,"version":"4.5.0"},"reference-count":78,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2024,1,17]],"date-time":"2024-01-17T00:00:00Z","timestamp":1705449600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2024,1,17]],"date-time":"2024-01-17T00:00:00Z","timestamp":1705449600000},"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":["Liver International"],"published-print":{"date-parts":[[2024,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background and Aims<\/jats:title>\n                    <jats:p>To systematically review the literature for reports on Wolcott\u2010Rallison syndrome, focusing on the spectrum and natural history, genotype\u2010phenotype correlations, patient and native liver survival, and long\u2010term outcomes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>PubMed, Livio, Google Scholar, Scopus and Web of Science databases were searched. Data on genotype, phenotype, therapy, cause of death and follow\u2010up were extracted. Survival and correlation analyses were performed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Sixty\u2010two studies with 159 patients met the inclusion criteria and additional 30 WRS individuals were collected by personal contact. The median age of presentation was 2.5\u2009months (IQR 2) and of death was 36\u2009months (IQR 50.75). The most frequent clinical feature was neonatal diabetes in all patients, followed by liver impairment in 73%, impaired growth in 72%, skeletal abnormalities in 59.8%, the nervous system in 37.6%, the kidney in 35.4%, insufficient haematopoiesis in 34.4%, hypothyroidism in 14.8% and exocrine pancreas insufficiency in 10.6%. Episodes of acute liver failure were frequently reported. Liver transplantation was performed in six, combined liver\u2010pancreas in one and combined liver\u2010pancreas\u2010kidney transplantation in two individuals. Patient survival was significantly better in the transplant cohort (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u2009.0057). One\u2010, five\u2010 and ten\u2010year patient survival rates were 89.4%, 65.5% and 53.1%, respectively. Liver failure was reported as the leading cause of death in 17.9% of cases. Overall survival was better in individuals with missense mutations (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u2009.013).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Wolcott\u2010Rallison syndrome has variable clinical courses. Overall survival is better in individuals with missense mutations. Liver\u2010 or multi\u2010organ transplantation is a feasible treatment option to improve survival.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/liv.15834","type":"journal-article","created":{"date-parts":[[2024,1,17]],"date-time":"2024-01-17T08:05:20Z","timestamp":1705478720000},"page":"811-822","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Natural history of\n                    <scp>Wolcott\u2010Rallison<\/scp>\n                    syndrome: A systematic review and follow\u2010up study"],"prefix":"10.1111","volume":"44","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6787-4939","authenticated-orcid":false,"given":"Denise","family":"Aldrian","sequence":"first","affiliation":[{"name":"Department of Paediatrics I Medical University of Innsbruck  Innsbruck Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clemens","family":"Bochdansky","sequence":"additional","affiliation":[{"name":"Department of Paediatrics I Medical University of Innsbruck  Innsbruck Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna M.","family":"Kavallar","sequence":"additional","affiliation":[{"name":"Department of Paediatrics I Medical University of Innsbruck  Innsbruck Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"Mayerhofer","sequence":"additional","affiliation":[{"name":"Department of Paediatrics I Medical University of Innsbruck  Innsbruck Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Asma","family":"Deeb","sequence":"additional","affiliation":[{"name":"Paediatric Endocrinology Division Sheikh Shakhbout Medical City, Khalifa University  Abu Dhabi United Arab Emirates"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abdelhadi","family":"Habeb","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Prince Mohamed bin Abdulaziz Hospital, National Guard Health Affairs  Madinah Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Romera\u00a0Rabasa","sequence":"additional","affiliation":[{"name":"Department of Pediatric Anesthesia Gregorio Mara\u00f1\u00f3n University Hospital  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anuradha","family":"Khadilkar","sequence":"additional","affiliation":[{"name":"Hirabai Cowasji Jehangir Medical Research Institute Jehangir Hospital  Pune India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ahmet","family":"U\u00e7ar","sequence":"additional","affiliation":[{"name":"Department of Pediatric Endocrinology &amp; Diabetes University of Health Sciences, \u015ei\u015fli Hamidiye Etfal Health Practices &amp; Research Centre  Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Birgit","family":"Knoppke","sequence":"additional","affiliation":[{"name":"University Children's Hospital Regensburg (KUNO) University Medical Center Regensburg  Regensburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dimitrios","family":"Zafeiriou","sequence":"additional","affiliation":[{"name":"1st Department of Pediatrics, Hippokratio General Hospital Aristotle University  Thessaloniki Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariarosaria","family":"Lang\u2010Muritano","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Diabetology and Children's Research Centre University Children's Hospital  Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohamad","family":"Miqdady","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology Sheikh Khalifa Medical City  Abu Dhabi United Arab Emirates"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sylvia","family":"Judmaier","sequence":"additional","affiliation":[{"name":"Department of Paediatrics LKH Hochsteiermark\/Standort Leoben  Leoben Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0711-687X","authenticated-orcid":false,"given":"Valeri\u00e9","family":"McLin","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Gynecology and Obstetrics, Swiss Pediatric Liver Center University of Geneva  Geneva Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Viktoriya","family":"Furdela","sequence":"additional","affiliation":[{"name":"Department of Pediatrics I. 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