Glycogen storage disease I and hepatocellular tumours - PubMed (original) (raw)

Review

. 1993:152 Suppl 1:S63-70.

doi: 10.1007/BF02072092.

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Review

Glycogen storage disease I and hepatocellular tumours

L Bianchi. Eur J Pediatr. 1993.

Abstract

From 50 published cases of hepatocellular adenoma (HCA) in glycogen storage disease, type I (GSD I) some characteristic features may be deduced: 1. The male:female ratio was 2:1. This sharply contrasts to HCA of other origin which shows a strong female preponderance. 2. The histology of adenomas largely corresponded to other adenomas, except for the appearance of Mallory bodies, accompanied by neutrophilic inflammation and a peculiar lamellar fibrosis. This observation is of particular interest because Mallory bodies have so far not been described in adenomas but are a well established feature in hepatocellular carcinoma of any aetiology. 3. Adenomas had a tendency to regress after continuous nocturnal intragastric feeding, although not all cases responded favourably. 4. Ten cases of hepatocellular carcinoma (HCC) are recorded in GSD I in literature, whereby in half of them transition from HCA into HCC seems likely. 5. The similarity in clinical presentation and evolution with oestrogen-induced tumours is striking. Pathogenesis of adenoma formation in GSD I is not understood. Experimental evidence and the clinical observation of regression after correction of the metabolic imbalance suggest three possible candidate mechanisms: (1) a glucagon/insulin imbalance; (2) cellular glycogen overload; and (3) proto-oncogene activation. Evidence in favour of these three mechanisms from experimental studies and observations in humans are briefly reviewed.

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References

    1. Proc Natl Acad Sci U S A. 1975 Mar;72(3):1157-60 - PubMed
    1. Surg Gynecol Obstet. 1975 Dec;141(6):843-58 - PubMed
    1. Pediatrics. 1955 Dec;16(6):785-800 - PubMed
    1. Ann Intern Med. 1963 Apr;58:654-63 - PubMed
    1. Arzneimittelforschung. 1964 Jul;14:805-14 - PubMed

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