Glycogen Storage in the Liver in Diabetes Mellitus

Rw, Evans; Tr, Littler; Hs, Pemberton

doi:10.1136/jcp.8.2.110

Cited by 32 publications

(9 citation statements)

References 12 publications

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“…It has since been recognized that glycogen loading and hepatic disease may be present in the absence of the extrahepatic manifestations described earlier by Mauriac (2). This process has variably been referred to as hepatic glycogenosis (3), liver glycogenosis (4), liver glycogen storage (5,6), diabetes mellitus-associated glycogen storage hepatomegaly (7), and most recently glycogenic hepatopathy (8).…”

Section: Discussionmentioning

confidence: 99%

Complete Reversal of Glycogen Hepatopathy With Pancreas Transplantation: Two Cases

et al. 2007

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Glycogen hepatopathy is the pathological overloading of hepatocytes with glycogen that is associated with poorly controlled diabetes mellitus. Clinically, it presents with abdominal discomfort, tender hepatomegaly and elevated transaminases. In this report, we describe our experience with two cases of type I diabetes mellitus and glycogen hepatopathy. The patients underwent isolated pancreas transplantation, following which, we have been able to demonstrate complete histological resolution of glycogen hepatopathy associated with control of glucose metabolism.

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Section: Discussionmentioning

confidence: 99%

Complete Reversal of Glycogen Hepatopathy With Pancreas Transplantation: Two Cases

et al. 2007

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“…Despite some previously mentioned studies showing that glycogen storage is decreased in persons with type 1 diabetes, there are numerous case reports of excessive glycogen storage in type 1 diabetes patients [103][104][105][106][107][108][109][110][111][112], some of the cases also had hepatic steatosis. The diabetes of all the patients that were examined due to palpable hepatomegaly was poorly controlled.…”

Section: Cases Of Excessive Glycogen Storage In Type 1 Diabetesmentioning

confidence: 99%

Hepatic Steatosis in Type 1 Diabetes

Regnell

Lernmark

2011

Rev Diabet Stud

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■ AbstractIslet autoimmunity in type 1 diabetes results in the loss of the pancreatic β-cells. The consequences of insulin deficiency in the portal vein for liver fat are poorly understood. Under normal conditions, the portal vein provides 75% of the liver blood supply. Recent studies suggest that non-alcoholic fatty liver disease (NAFLD) may be more common in type 1 diabetes than previously thought, and may serve as an independent risk marker for some chronic diabetic complications. The pathogenesis of NAFLD remains obscure, but it has been hypothesized that hepatic fat accumulation in type 1 diabetes may be due to lipoprotein abnormalities, hyperglycemia-induced activation of the transcription factors carbohydrate response element-binding protein (ChREBP) and sterol regulatory element-binding protein 1c (SREBP-1c), upregulation of glucose transporter 2 (GLUT2) with subsequent intrahepatic fat synthesis, or a combination of these mechanisms. Novel approaches to non-invasive determinations of liver fat may clarify the consequences for liver metabolism when the pancreas has ceased producing insulin. This article aims to review the factors potentially contributing to hepatic steatosis in type 1 diabetes, and to assess the feasibility of using liver fat as a prognostic and/or diagnostic marker for the disease. It provides a background and a case for possible future studies in the field.

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“…The patient reported here seems similar to the patients of EvANs et al (1955), and perhaps those of HAXSSEN (1936), although he is older than any diabetic previously reported to have a large liver because of excessive deposition of glycogen. The low renal threshold for glucose made management of his diabetes difficult and he developed severe hyperglycaemia four times.…”

Section: Discussionmentioning

confidence: 51%

Glycogen-laden hepatomegaly in diabetes

Middleton

Hockaday

1965

Diabetologia

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Summary.A 43-year old man is described who had been treated with insulin for diabetes for 18 years. For the last 3 years he had had nightly hypoglycaemie attacks. He was then found to have an enlarged liver, biopsy of which showed normal architecture but a markedly increased glycogen content. After his insulin was changed so that he no longer developed hypoglycaemia, his liver returned to normal size and normal glycogen content. Previous reports of glycogen-laden hepatomegaly are reviewed, and its mechanism is discussed, with special reference to its reversibility when hypoglycaemia is avoided.Rdsumd. Les auteurs rapportent l'observation d'un hemme ~gd de 43 ans traitd par l'insuline depuis 18 ans. Pendant les 3 derni@res anndes il souffrait d'attaques nocturnes d'hypoglyc6mie. On constata une augmentation du foie dent une biopsie dgmontra une architecture normale, mais des ddpSts considdrablement 61cvds de glyeog6ne. Apr6s abolition des hypoglycdmies par changement d'insuline, le volume et le contenu de glycog6ne du foie rcdevinrent normaux. Des rapports ant6rieurs d'hdpatomdgalie par d6p6t glycogdnique sent passds en revue ; le m6canisme de cette hdpatom6galie et particuli6rement sa reversibilitd, si l'hypoglyc6mie est 6vitde, est discut@. Zusammenfassung. Bericht fiber einen 43-j/~hrigenMann, dcr wegen seines Diabetes 18 Jahre lang mit Insulin behandelt wurde. In den letzten 3 Jahren hatte er n/ichtliche hypoglyk/imische Anf/ille. Die Untersuchung ergab eine vergrSBcrte Leber, deren bioptischc Untersuchung eine normale Struktur, abcr einen dcutlich erhShten Glykogengehalt erkennen liel]. Nachdem das Insulin gewechselt wurde, sodal] I-Iypoglyk/imien nicht mehr auftraten, bildete sich die LebervergrSl~erung zurfick. Sie enthielt jetzt normale Mengen von Glykogen. Uber frfihere Angaben der ,,glycogen-laden hepatomegaly" wird berichtet, ihr Entstehungsmechanismus diskutiert, wobei besonders die Reversibilitat des Zustandes berficksichtigt wird, wenn ttypoglykamien vermieden werden.

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Glycogen Storage in the Liver in Diabetes Mellitus

Cited by 32 publications

References 12 publications

Complete Reversal of Glycogen Hepatopathy With Pancreas Transplantation: Two Cases

Complete Reversal of Glycogen Hepatopathy With Pancreas Transplantation: Two Cases

Hepatic Steatosis in Type 1 Diabetes

Glycogen-laden hepatomegaly in diabetes

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