{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,8]],"date-time":"2026-07-08T18:49:55Z","timestamp":1783536595619,"version":"3.55.0"},"reference-count":26,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2025,9,17]],"date-time":"2025-09-17T00:00:00Z","timestamp":1758067200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. pediatr. gastroenterol. nutr."],"published-print":{"date-parts":[[2025,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>To investigate potential mechanisms of muscle wasting in children with chronic cholestatic liver disease (CCLD).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      This is a cross\u2010sectional study comparing estimated skeletal muscle mass (eSMM)\n                      <jats:italic>z<\/jats:italic>\n                      \u2010score, assessed by whole body dual energy X\u2010ray absorptiometry (DXA), in children with CCLD versus healthy controls. Relationships between eSMM\n                      <jats:italic>z<\/jats:italic>\n                      \u2010score and serum growth hormone (GH), insulin\u2010like growth factor 1 (IGF\u20101), myostatin, and ammonia were analyzed by Spearman correlation and multiple linear regression. Myopenia was defined as eSMM\n                      <jats:italic>z<\/jats:italic>\n                      \u2010score\u2009\u2264\u2009\u22122.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      DXA was obtained in 15 children with CCLD (eight biliary atresia, four Alagille syndrome, one alpha\u20101\u2010antitrypsin deficiency, one bile salt export protein deficiency, one idiopathic cirrhosis; mean age 12.1\u2009\u00b1\u20093.9 years) and 19 controls (mean age 11.5\u2009\u00b1\u20093.9 years). Although growth and muscle mass (eSMM\n                      <jats:italic>z<\/jats:italic>\n                      \u2010score) were similar between CCLD and controls, children with CCLD had significantly higher GH levels (2.7\u2009\u00b1\u20094.0\u2009ng\/mL vs. 0.5\u2009\u00b1\u20090.6\u2009ng\/mL,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.04) and lower IGF\u20101\n                      <jats:italic>z<\/jats:italic>\n                      \u2010score (\u22120.9\u2009\u00b1\u20090.8 vs. 0.0\u2009\u00b1\u20090.5,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.01), indicating GH resistance in CCLD. Children with CCLD and myopenia had significantly higher GH levels than those without myopenia (median 7.9 [0.5\u201313.0]\u2009ng\/mL vs. 0.3 [0.0\u20134.6]\u2009ng\/mL,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.04), with a trend toward a moderate inverse correlation between GH and eSMM\n                      <jats:italic>z<\/jats:italic>\n                      \u2010score in CCLD (rho\u2009=\u2009\u22120.48,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.07).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      GH resistance is prevalent in CCLD, even in older children with relatively mild liver disease severity and preserved growth. The relationship between eSMM\n                      <jats:italic>z<\/jats:italic>\n                      \u2010score and GH indicates a potential role for GH resistance in the development of myopenia in CCLD.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/jpn3.70210","type":"journal-article","created":{"date-parts":[[2025,9,17]],"date-time":"2025-09-17T07:26:36Z","timestamp":1758093996000},"page":"1390-1399","source":"Crossref","is-referenced-by-count":1,"title":["Growth hormone resistance in children with chronic cholestatic liver disease and reduced skeletal muscle mass"],"prefix":"10.1002","volume":"81","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3061-1449","authenticated-orcid":false,"given":"Julia M.","family":"Boster","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Pediatric Liver Center, Digestive Health Institute and Section of Pediatric Gastroenterology, Hepatology &amp; Nutrition; Children's Hospital Colorado University of Colorado School of Medicine Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kelly","family":"Gilmartin","sequence":"additional","affiliation":[{"name":"Children's Hospital Colorado Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhaoxing","family":"Pan","sequence":"additional","affiliation":[{"name":"University of Colorado School of Medicine Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7433-4095","authenticated-orcid":false,"given":"Ronald J.","family":"Sokol","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Pediatric Liver Center, Digestive Health Institute and Section of Pediatric Gastroenterology, Hepatology &amp; Nutrition; Children's Hospital Colorado University of Colorado School of Medicine Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2523-6389","authenticated-orcid":false,"given":"Shikha S.","family":"Sundaram","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Pediatric Liver Center, Digestive Health Institute and Section of Pediatric Gastroenterology, Hepatology &amp; Nutrition; Children's Hospital Colorado University of Colorado School of Medicine Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,9,17]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.3390\/cells10010129"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.molmet.2021.101245"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0168-8278(02)00049-1"},{"issue":"1","key":"e_1_2_8_5_1","first-page":"73","article-title":"Resistance to growth hormone in children with chronic liver disease","volume":"1","author":"Bucuvalas JC","year":"1997","journal-title":"Pediatr Tranplant"},{"issue":"1","key":"e_1_2_8_6_1","first-page":"76","article-title":"The insulin\u2010like growth factor and binding protein axis in children with end\u2010stage liver disease before and after orthotopic liver transplantation","volume":"2","author":"Holt RI","year":"1998","journal-title":"Pediatr Tranplant"},{"issue":"9","key":"e_1_2_8_7_1","first-page":"3277","article-title":"The effect of short stature, portal hypertension, and cholestasis on growth hormone resistance in children with liver disease","volume":"84","author":"Holt RI","year":"1999","journal-title":"J Clin Endocrinol Metab"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpendo.00652.2013"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2021.01.025"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/jcsm.12262"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00535-019-01605-6"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.2147\/HMER.S348888"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/lt.26027"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/lt.26205"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/petr.13996"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000002333"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13539-011-0042-6"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2014-0793"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcn\/84.5.1014"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1097\/HC9.0000000000000293"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1097\/LVT.0000000000000520"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/liv.15689"},{"key":"e_1_2_8_23_1","first-page":"512","article-title":"Natural history of liver disease in a large international cohort of children with alagille syndrome: results from the GALA study","author":"Vandriel SM","year":"2022","journal-title":"Hepatology"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00018-014-1689-x"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1002\/jcsm.13737"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40659-023-00436-3"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1002\/jcsm.13522"}],"container-title":["Journal of Pediatric Gastroenterology and Nutrition"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jpn3.70210","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,12,1]],"date-time":"2025-12-01T17:44:30Z","timestamp":1764611070000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/jpn3.70210"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,9,17]]},"references-count":26,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2025,12]]}},"alternative-id":["10.1002\/jpn3.70210"],"URL":"https:\/\/doi.org\/10.1002\/jpn3.70210","archive":["Portico"],"relation":{},"ISSN":["0277-2116","1536-4801"],"issn-type":[{"value":"0277-2116","type":"print"},{"value":"1536-4801","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,9,17]]}}}