title: Diagnosis of hepatic glycogenosis in poorly controlled type 1 diabetes mellitus.
abstract: Hepatic glycogenosis (HG) in type 1 diabetes is a underrecognized complication. Mauriac firstly described the syndrome characterized by hepatomegaly with altered liver enzymes, growth impairment, delay puberty and Cushingoid features, during childhood. HG in adulthood is characterized by the liver disorder (with circulating aminotransferase increase) in the presence of poor glycemic control (elevation of glycated hemoglobin, HbA1c levels). The advances in the comprehension of the metabolic pathways driving to the hepatic glycogen deposition point out the role of glucose transporters and insulin mediated activations of glucokinase and glycogen synthase, with inhibition of glucose-6-phosphatase. The differential diagnosis of HG consists in the exclusion of causes of liver damage (infectious, metabolic, obstructive and autoimmune disease). The imaging study (ultrasonography and/or radiological examinations) gives information about the liver alterations (hepatomegaly), but the diagnosis needs to be confirmed by the liver biopsy. The main treatment of HG is the amelioration of glycemic control that is usually accompanied by the reversal of the liver disorder. In selected cases, more aggressive treatment options (transplantation) have been successfully reported.
authors.affiliation: Stefania Giordano, Antonio Martocchia, Lavinia Toussan, Manuela Stefanelli, Francesca Pastore, Antonio Devito, Marcello G Risicato, Luigi Ruco, Paolo Falaschi, Geriatric Unit, "Sapienza" University of Rome, Faculty of Medicine and Psychology, S. Andrea Hospital, 00189 Rome, Italy.
authors.forename: Stefania
authors.initials: S
authors.lastname: Giordano
authors.affiliation: Stefania Giordano, Antonio Martocchia, Lavinia Toussan, Manuela Stefanelli, Francesca Pastore, Antonio Devito, Marcello G Risicato, Luigi Ruco, Paolo Falaschi, Geriatric Unit, "Sapienza" University of Rome, Faculty of Medicine and Psychology, S. Andrea Hospital, 00189 Rome, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Martocchia
authors.affiliation: Stefania Giordano, Antonio Martocchia, Lavinia Toussan, Manuela Stefanelli, Francesca Pastore, Antonio Devito, Marcello G Risicato, Luigi Ruco, Paolo Falaschi, Geriatric Unit, "Sapienza" University of Rome, Faculty of Medicine and Psychology, S. Andrea Hospital, 00189 Rome, Italy.
authors.forename: Lavinia
authors.initials: L
authors.lastname: Toussan
authors.affiliation: Stefania Giordano, Antonio Martocchia, Lavinia Toussan, Manuela Stefanelli, Francesca Pastore, Antonio Devito, Marcello G Risicato, Luigi Ruco, Paolo Falaschi, Geriatric Unit, "Sapienza" University of Rome, Faculty of Medicine and Psychology, S. Andrea Hospital, 00189 Rome, Italy.
authors.forename: Manuela
authors.initials: M
authors.lastname: Stefanelli
authors.affiliation: Stefania Giordano, Antonio Martocchia, Lavinia Toussan, Manuela Stefanelli, Francesca Pastore, Antonio Devito, Marcello G Risicato, Luigi Ruco, Paolo Falaschi, Geriatric Unit, "Sapienza" University of Rome, Faculty of Medicine and Psychology, S. Andrea Hospital, 00189 Rome, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Pastore
authors.affiliation: Stefania Giordano, Antonio Martocchia, Lavinia Toussan, Manuela Stefanelli, Francesca Pastore, Antonio Devito, Marcello G Risicato, Luigi Ruco, Paolo Falaschi, Geriatric Unit, "Sapienza" University of Rome, Faculty of Medicine and Psychology, S. Andrea Hospital, 00189 Rome, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Devito
authors.affiliation: Stefania Giordano, Antonio Martocchia, Lavinia Toussan, Manuela Stefanelli, Francesca Pastore, Antonio Devito, Marcello G Risicato, Luigi Ruco, Paolo Falaschi, Geriatric Unit, "Sapienza" University of Rome, Faculty of Medicine and Psychology, S. Andrea Hospital, 00189 Rome, Italy.
authors.forename: Marcello G
authors.initials: MG
authors.lastname: Risicato
authors.affiliation: Stefania Giordano, Antonio Martocchia, Lavinia Toussan, Manuela Stefanelli, Francesca Pastore, Antonio Devito, Marcello G Risicato, Luigi Ruco, Paolo Falaschi, Geriatric Unit, "Sapienza" University of Rome, Faculty of Medicine and Psychology, S. Andrea Hospital, 00189 Rome, Italy.
authors.forename: Luigi
authors.initials: L
authors.lastname: Ruco
authors.affiliation: Stefania Giordano, Antonio Martocchia, Lavinia Toussan, Manuela Stefanelli, Francesca Pastore, Antonio Devito, Marcello G Risicato, Luigi Ruco, Paolo Falaschi, Geriatric Unit, "Sapienza" University of Rome, Faculty of Medicine and Psychology, S. Andrea Hospital, 00189 Rome, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Falaschi
keywords: Glucokinase; Glucose transporters; Glucose-6-phosphatase; Glycogen; Glycogen synthase; Hepatic glycogenosis; Hepatomegaly; Insulin; Type 1 diabetes mellitus