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Hepatocellular adenoma and metabolic balance in patients with type Ia glycogen storage disease.

Abstract：

:Glycogen storage disease type I (GSD I) is a metabolic disorder resulting from defects in the glucose-6-phosphatase system. Approximately 75% of adolescent and adult patients develop hepatocellular adenomas, which can lead to considerable morbidity and mortality. The pathogenesis of adenomas is unclear and the risk of developing adenomas in treated patients is uncertain. The objective of this study was to determine whether metabolic imbalance was related to the occurrence of adenomas in patients with GSD I, and to determine what specific biochemical pathways were involved. We performed a 1:1 case-control retrospective study; cases were GSD I patients with adenomas and controls were GSD I patients without adenomas. Controls and cases were matched according to age at diagnosis, age at adenoma detection, and gender. We investigated biochemical abnormalities indicative of metabolic balance and exogenous factors potentially related to the onset of adenomas in the two groups. We detected no significant differences in dietetic treatment, compliance to treatment, or biochemical parameters related to metabolic balance between the two groups. In conclusion, we were unable to identify any significant differences in metabolic balance between GSD I patients who developed adenomas and those who did not.

journal_name

Mol Genet Metab

journal_title

Molecular genetics and metabolism

authors

Di Rocco M,Calevo MG,Taro' M,Melis D,Allegri AE,Parenti G

doi

10.1016/j.ymgme.2007.10.134

subject

Has Abstract

pub_date

2008-04-01 00:00:00

pages

398-402

issue

4

eissn

1096-7192

issn

1096-7206

pii

S1096-7192(07)00574-4

journal_volume

93

pub_type

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