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2018 ; 10
(2
): 172-185
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Glycogenic hepatopathy: A narrative review
#MMPMID29527255
Sherigar JM
; Castro J
; Yin YM
; Guss D
; Mohanty SR
World J Hepatol
2018[Feb]; 10
(2
): 172-185
PMID29527255
show ga
Glycogenic hepatopathy (GH) is a rare complication of the poorly controlled
diabetes mellitus characterized by the transient liver dysfunction with elevated
liver enzymes and associated hepatomegaly caused by the reversible accumulation
of excess glycogen in the hepatocytes. It is predominantly seen in patients with
longstanding type 1 diabetes mellitus and rarely reported in association with
type 2 diabetes mellitus. Although it was first observed in the pediatric
population, since then, it has been reported in adolescents and adults with or
without ketoacidosis. The association of GH with hyperglycemia in diabetes has
not been well established. One of the essential elements in the pathophysiology
of development of GH is the wide fluctuation in both glucose and insulin levels.
GH and non-alcoholic fatty liver disease (NAFLD) are clinically
indistinguishable, and latter is more prevalent in diabetic patients and can
progress to advanced liver disease and cirrhosis. Gradient dual-echo MRI can
distinguish GH from NAFLD; however, GH can reliably be diagnosed only by liver
biopsy. Adequate glycemic control can result in complete remission of clinical,
laboratory and histological abnormalities. There has been a recent report of
varying degree of liver fibrosis identified in patients with GH. Future studies
are required to understand the biochemical defects underlying GH, noninvasive,
rapid diagnostic tests for GH, and to assess the consequence of the fibrosis
identified as severe fibrosis may progress to cirrhosis. Awareness of this entity
in the medical community including specialists is low. Here we briefly reviewed
the English literature on pathogenesis involved, recent progress in the
evaluation, differential diagnosis, and management.