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Clinical features and rare complications in 132 patients with hepatic glycogenosis [PDF]
Orphanet Journal of Rare DiseasesBackground Glycogen storage diseases (GSDs) with liver involvement are classified into subtypes—types 0, Ia, and Ib; III, IV, VI, IX, and XIa, XIb, and XIc, depending on the deficient enzyme. Hypoglycemia and hepatomegaly (except type 0) are hallmarks of
Deniz Kor +7 more
doaj +6 more sources
Hepatic Glycogenosis: An Underdiagnosed Entity? [PDF]
Cureus, 2022 Hepatic glycogenosis (HG) is a rare complication of long-standing poorly controlled type 1 diabetes mellitus (T1DM), which is often misdiagnosed as non-alcoholic fatty liver disease (NAFLD). Despite the existence of several reports in the literature, it still is underrecognized, even among gastroenterologists.
Garcês Soares S +5 more
europepmc +4 more sources
Persistent hyperlactatemia in decompensated type I diabetes with hepatic glycogenosis and hepatomegaly: Mauriac syndrome: a case report [PDF]
Journal of Medical Case Reports, 2022 Background We describe a case of Mauriac syndrome, which is a rare complication of poorly controlled type I diabetes that combines glycogenosis, hepatomegaly, growth retardation with a Cushingoid appearance that is most often present in children but also
Waheed Dolip +3 more
doaj +3 more sources
Mauriac Syndrome: A Rare Hepatic Glycogenosis in Poorly Controlled Type 1 Diabetes [PDF]
GE: Portuguese Journal of Gastroenterology, 2019 Background: Hepatic glycogenosis (HG) is a complication of poorly controlled type 1 diabetes mellitus (T1DM), characterized by glycogen accumulation in hepatocytes.
Marta Patita +5 more
doaj +3 more sources
Acute Hepatitis due to Hepatic Glycogenosis After Insulin Overdose and Oral Glucose Administration in an Adolescent [PDF]
Journal of the Endocrine Society, 2021 Background Hepatic glycogenosis (HG) has been reported after intravenous (IV) dextrose administration to treat insulin overdose. We describe a case of HG in a patient with type 1 diabetes mellitus (T1DM) due to insulin overdose treated with oral glucose ...
Sabitha Sasidharan Pillai +2 more
exaly +3 more sources
Hepatic glycogenosis in type 1 diabetes mellitus mimicking Mauriac syndrome [PDF]
Korean Journal of Pediatrics, 2015 Hepatic glycogenosis in type 1 diabetes mellitus (DM) can be caused by poor glycemic control due to insulin deficiency, excessive insulin treatment for diabetic ketoacidosis, or excessive glucose administration to control hypoglycemia.
In Ah Jung +7 more
doaj +3 more sources
Journal of Clinical and Translational Endocrinology Case Reports, 2022 Unbalanced diabetes can lead to many complications related to insulin deficiency. Hepatocytic glycogenic overload, formerly known as Mauriac syndrome, is one of them.
M'harzi Soulaimane +8 more
doaj +4 more sources
Annals of Hepatology, 2018 Glycogen storage disease type I and glycogenic hepatopathy are the most common type of primary and secondary hepatic glycogenosis, with presenting common radiological features of hepatomegaly, hepatic signal, or density change.
Zhi-yuan Chen +2 more
doaj +5 more sources
Hepatic Glycogenosis: A Rare Complication of Type 1 Diabetes Mellitus. [PDF]
CureusType 1 diabetes mellitus (T1DM) is a common autoimmune pathology requiring lifelong insulin therapy. We report the case of a 12-year-old girl with T1DM admitted to Department C of the National Institute of Nutrition of Tunis for diabetic ketosis. She had suffered from T1DM for five years, with poor glycemic control (hemoglobin A1C = 10%) and poor ...
Jemai C +4 more
europepmc +4 more sources
Hepatic glycogenosis: An underdiagnosed complication of diabetes mellitus? [PDF]
World J Diabetes, 2015 Hepatic glycogenosis (HG) is characterized by excessive glycogen accumulation in hepatocytes and represents a hepatic complication of diabetes that particularly occurs in patients with longstanding poorly controlled type 1 diabetes (T1D). HG has been reported to be a very rare disease, although it is believed to be extremely underdiagnosed because it ...
Julián MT +5 more
europepmc +4 more sources