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Deficiency of glucose\u20106\u2010phosphatase impairs the processes of gluconeogenesis and glycogenolysis by preventing conversion of glucose\u20106\u2010phosphate to glucose. Clinical features include fasting hypoglycemia, lactic acidosis, hypertriglyceridemia, hyperuricemia, hepatomegaly, and development of hepatocellular adenomas (HCAs) with potential for malignant transformation. Additionally, patients with GSD Ia often exhibit short stature, in some instances due to growth hormone (GH) deficiency. Patients with short stature caused by GH deficiency typically receive GH injections. Here, we review the literature and describe a female with GSD Ia who had short stature, failure of growth progression, and suspected GH deficiency. This patient received GH injections from ages 11 to 14\u2009years under careful monitoring of an endocrinologist and developed HCAs during that time. To date, there is no reported long\u2010term follow up data on patients with GSD Ia who have received GH therapy, and therefore the clinical outcomes post\u2010GH therapy are unclear.<\/jats:p>","DOI":"10.1002\/jmd2.12381","type":"journal-article","created":{"date-parts":[[2023,8,18]],"date-time":"2023-08-18T23:58:42Z","timestamp":1692403122000},"page":"303-311","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Development of hepatocellular adenomas in a patient with glycogen storage disease Ia treated with growth hormone therapy"],"prefix":"10.1002","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0351-0642","authenticated-orcid":false,"given":"David G.","family":"Jackson","sequence":"first","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics Duke University Medical Center  Durham North Carolina 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