Glycogen storage disease type I natural history, complications and prognosis: Difference between revisions
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==Natural History== | ==Natural History== | ||
*If left untreated, patients with GSD I dies in infancy or childhood of overwhelming [[hypoglycemia]] and [[acidosis]]. | |||
*Surviving individuals have stunted physical growth and delayed puberty due to chronically low insulin levels. | |||
*Mental retardation as a result of severe and recurrent hypoglycemia is considered preventable with appropriate treatment. | |||
==Complications== | ==Complications== | ||
[[Hepatic]] complications have been serious in some patients. [[Adenomas]] of the liver can develop in the second decade or later, with a small chance of later [[malignant]] transformation to [[hepatoma]] or [[hepatic carcinomas]] (detectable by [[alpha-fetoprotein]] [[screening]]). Several children with advanced hepatic complications have improved after liver transplantation. | [[Hepatic]] complications have been serious in some patients. [[Adenomas]] of the liver can develop in the second decade or later, with a small chance of later [[malignant]] transformation to [[hepatoma]] or [[hepatic carcinomas]] (detectable by [[alpha-fetoprotein]] [[screening]]). Several children with advanced hepatic complications have improved after liver transplantation. |
Revision as of 16:07, 31 October 2017
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
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Overview
Natural History
- If left untreated, patients with GSD I dies in infancy or childhood of overwhelming hypoglycemia and acidosis.
- Surviving individuals have stunted physical growth and delayed puberty due to chronically low insulin levels.
- Mental retardation as a result of severe and recurrent hypoglycemia is considered preventable with appropriate treatment.
Complications
Hepatic complications have been serious in some patients. Adenomas of the liver can develop in the second decade or later, with a small chance of later malignant transformation to hepatoma or hepatic carcinomas (detectable by alpha-fetoprotein screening). Several children with advanced hepatic complications have improved after liver transplantation.
Additional problems reported in adolescents and adults with GSD I have included hyperuricemic gout, pancreatitis, and chronic renal failure. Despite hyperlipidemia, atherosclerotic complications have been infrequently reported.
Prognosis
With diagnosis before serious harm occurs, prompt reversal of acidotic episodes, and appropriate long-term treatment, most children will be healthy. With exceptions and qualifications, adult health and life span may also be fairly good, although lack of effective treatment before the mid-1970s has limited our long-term information.