{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,9]],"date-time":"2026-01-09T13:23:35Z","timestamp":1767965015314,"version":"3.49.0"},"reference-count":0,"publisher":"Oxford University Press (OUP)","issue":"2","content-domain":{"domain":["eje.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2021,8,1]]},"abstract":"The aim of the study is to find possible explanations for vanishing juvenile hypoglycemia in growth hormone receptor deficiency (GHRD) in human patients and animal models. We reviewed parameters of glucose metabolism in distinct age groups into two human cohorts (Israeli and Ecuadorian) of Laron syndrome (LS) patients, a mouse model (Ghr<\/jats:italic>-KO mouse) and provided additional data for a porcine model (GHR<\/jats:italic>-KO pig). Juvenile hypoglycemia is a common symptom of GHRD and vanishes in adulthood. In the Israeli cohort, developing metabolic syndrome is associated with decreasing insulin sensitivity, insulinopenia and glucose intolerance, and increasing glucose levels with age. In the Ecuadorian patients and both animal models, insulin sensitivity is preserved or even enhanced. Alterations in food intake and energy consumption do not explain the differences in glucose levels; neither is the accumulation of body fat associated with negative effects in the Ecuadorian cohort nor in the animal models. A reduced beta-cell mass and resulting insulin secretory capacity is common and leads to glucose intolerance in Ghr<\/jats:italic>-KO mice, while glucose tolerance is preserved in Ecuadorian patients and the GHR<\/jats:italic>-KO pig. In human patients and the GHR<\/jats:italic>-KO pig, a simultaneous occurrence of normoglycemia with the onset of puberty is reported. Reduced gluconeogenesis in GHRD is discussed to cause juvenile hypoglycemia and a counter-regulatory stimulation of gluconeogenesis can be hypothesized. A coherent study assessing endogenous glucose production and beta-cell capacity in the hypoglycemic and normoglycemic age group is needed. This can be performed in GHR<\/jats:italic>-KO pigs, including castrated animals.<\/jats:p>","DOI":"10.1530\/eje-21-0013","type":"journal-article","created":{"date-parts":[[2021,5,28]],"date-time":"2021-05-28T17:33:46Z","timestamp":1622223226000},"page":"R35-R47","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-7","source":"Crossref","is-referenced-by-count":13,"title":["MECHANISMS IN ENDOCRINOLOGY: Transient juvenile hypoglycemia in growth hormone receptor deficiency \u2013 mechanistic insights from Laron syndrome and tailored animal models"],"prefix":"10.1093","volume":"185","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3022-5983","authenticated-orcid":true,"given":"Arne","family":"Hinrichs","sequence":"first","affiliation":[{"name":"1Chair for Molecular Animal Breeding and Biotechnology, Gene Center and Department of Veterinary Sciences, LMU Munich, Munich, Germany"},{"name":"2Center for Innovative Medical Models (CiMM), LMU Munich, Oberschlei\u00dfheim, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5866-4577","authenticated-orcid":true,"given":"Simone","family":"Renner","sequence":"additional","affiliation":[{"name":"1Chair for Molecular Animal Breeding and Biotechnology, Gene Center and Department of Veterinary Sciences, LMU Munich, Munich, Germany"},{"name":"2Center for Innovative Medical Models (CiMM), LMU Munich, Oberschlei\u00dfheim, Germany"},{"name":"3German Center for Diabetes Research (DZD), Neuherberg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4681-6668","authenticated-orcid":true,"given":"Martin","family":"Bidlingmaier","sequence":"additional","affiliation":[{"name":"4Endocrine Laboratory, Medizinische Klinik und Poliklinik IV, Klinikum der Universit\u00e4t M\u00fcnchen, Munich, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4561-2177","authenticated-orcid":true,"given":"John J","family":"Kopchick","sequence":"additional","affiliation":[{"name":"5Edison Biotechnology Institute Heritage College of Osteopathic Medicine and Molecular and Cellular Biology Program, Department of Biomedical Sciences, Ohio University, Athens, Ohio, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0430-9510","authenticated-orcid":true,"given":"Eckhard","family":"Wolf","sequence":"additional","affiliation":[{"name":"1Chair for Molecular Animal Breeding and Biotechnology, Gene Center and Department of Veterinary Sciences, LMU Munich, Munich, Germany"},{"name":"2Center for Innovative Medical Models (CiMM), LMU Munich, Oberschlei\u00dfheim, Germany"},{"name":"3German Center for Diabetes Research (DZD), Neuherberg, Germany"},{"name":"6Laboratory for Functional Genome Analysis (LAFUGA), Gene Center, LMU Munich, Munich, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","container-title":["European Journal of Endocrinology"],"original-title":[],"link":[{"URL":"https:\/\/eje.bioscientifica.com\/view\/journals\/eje\/185\/2\/EJE-21-0013.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/eje.bioscientifica.com\/downloadpdf\/journals\/eje\/185\/2\/EJE-21-0013.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,2,14]],"date-time":"2023-02-14T16:16:15Z","timestamp":1676391375000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ejendo\/article\/185\/2\/R35\/6654375"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,8,1]]},"references-count":0,"journal-issue":{"issue":"2"},"URL":"https:\/\/doi.org\/10.1530\/eje-21-0013","relation":{},"ISSN":["0804-4643","1479-683X"],"issn-type":[{"value":"0804-4643","type":"print"},{"value":"1479-683X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,8,1]]}}}