Glycogenic Hepatopathy: Resolution with Minimal Glucose Control

Chandel, Abhimanyu; Scarpato, Brittany; Camacho, Jeanette; McFarland, Miles; Mok, Shaffer

doi:10.1155/2017/7651387

Cited by 11 publications

(7 citation statements)

References 9 publications

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“…In contrast, our second patient showed improvement in his sugar control, which was followed by a resolution of his hepatomegaly and liver enzyme elevation, as well as improvement in his growth. This is consistent with the findings of many studies that reported remission of clinical and laboratory abnormalities in patients with glycogenic hepatopathy with optimization of glucose control ( 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 ). The negative effects of poor metabolic control on weight and height were observed in both patients.…”

Section: Discussionsupporting

confidence: 92%

Glycogenic hepatopathy in children with poorly controlled type 1 diabetes mellitus

Abu

Lim

Nor

2021

Clin Pediatr Endocrinol

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. Mauriac syndrome is a rare and underdiagnosed complication of type 1 diabetes mellitus (T1DM). It is characterized by growth retardation, delayed puberty, Cushingoid features, hepatomegaly, and increased transaminase levels. The term glycogenic hepatopathy has been used to describe patients with poorly controlled T1DM and glycogen overload in the hepatocytes but without all the features of Mauriac syndrome. Although rare, glycogenic hepatopathy is reported to be the main cause of hepatomegaly in young patients with T1DM. We report two cases of glycogenic hepatopathy in children with poorly controlled T1DM. Both children had hepatomegaly, elevated liver enzyme levels, and elevated lactate levels. A liver biopsy confirmed the diagnosis of glycogenic hepatopathy in both patients. In conclusion, hepatomegaly with elevated liver enzymes, negative infective and metabolic screenings and persistently elevated plasma lactate levels should raise the suspicion of glycogenic hepatopathy in poorly controlled T1DM. Early diagnosis and improvement in glycemic control are the mainstays of treatment, which can prevent long-term complications.

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Section: Discussionsupporting

confidence: 92%

Glycogenic hepatopathy in children with poorly controlled type 1 diabetes mellitus

Abu

Lim

Nor

2021

Clin Pediatr Endocrinol

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“…After GH diagnosis, the treatment should aim the intense glycemic control as this is considered the backbone of its management 15 34 44. However, resolution of GH has also been described after minimal glucose control 34 45. The disease has a benign course with excellent prognosis as after adherence to strict glycemic control and carbohydrate-restricted diet the liver enzymes normalise and symptoms disappear within a few weeks as also observed in our patient.…”

Section: Discussionsupporting

confidence: 69%

Glycogenic hepatopathy as a cause of severe deranged liver enzymes in a young patient with type 1 diabetes mellitus

et al. 2019

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Glycogenic hepatopathy (GH) is a rare complication of poorly controlled type 1 diabetes mellitus (T1DM). We present a 19-year-old woman with T1DM and autoimmune thyroiditis who admitted to our department because of abrupt onset intermittent abdominal pain in the right upper quadrant accompanied by laboratory evidence of acute anicteric hepatitis. Physical examination revealed significant hepatomegaly but the common imagining studies were negative. Following exclusion of common causes of acute hepatitis and because of the presence of smooth muscle antibodies in a young female patient with already established two autoimmune diseases, a liver biopsy was performed in order to exclude the potential presence of autoimmune hepatitis. However, liver histology showed typical findings of GH. Intense treatment targeting strict glycemic control resulted in normalisation of liver biochemistry. This case underlines that GH should be considered as a rare cause of acute hepatitis in T1DM patients with poor glycemic control.

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“…Although, in our patient, and many other published cases, the glycemic control may not always correlate directly with elevation of liver tests. In fact, like our patient whose transaminases improved despite improvements in glycohemoglobin levels, there is still much to be known about the degree of improved glycemic control needed for the resolution of GH [9]. In one study, two patients treated with pancreas transplantation were noted to have a complete reversal of their GH, inducing an insulin-independent, euglycemic state [10].…”

Section: Discussionmentioning

confidence: 83%

Glycogen Hepatopathy: A Reversible yet Relapsing Cause of Hepatitis in Type 1 Diabetics

Azhar

Hammami

Musmar

et al. 2021

Cureus

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Glycogen hepatopathy (GH), a rare glycogen storage disease caused by genetic or acquired overactivation of hepatic glycogen synthesis enzymes, can mimic non-alcoholic fatty liver disease (NAFLD). We describe a case of biopsy-proven GH in an adult with type 1 diabetes mellitus (DM).A 33-year-old Honduran woman with a 25-year history of type 1 DM complicated by gastroparesis, multiple episodes of diabetic ketoacidosis (DKA) and hypoglycemia, and recurrent pancreatitis was referred for abnormal liver enzymes. Family history was negative for liver disease. There was no history of alcohol or recreational drug use. Patients' medications included insulin and thyroxine. Physical exam showed hepatomegaly but no stigmata of chronic liver disease. Aspartate aminotransferase (AST) and alanine aminotransferase (ALT) had ranged from 100's to over 7000 U/L while alkaline phosphatase (ALP) was elevated to over 400 IU/L. Albumin, total bilirubin, platelets, international normalized ratio (INR), eosinophils, viral hepatitis panel, antinuclear antibody (ANA), smooth muscle antibody (Ab), anti-liverkidney microsomal (LKM) Ab, celiac serologies, ceruloplasmin, alpha 1 antitrypsin, iron studies, and acetaminophen levels were all normal. An abdominal ultrasound showed "fatty liver" and an atrophic pancreas. CT abdomen showed hepatomegaly. The common bile duct (CBD) was found to be normal on endoscopic ultrasound (EUS) and magnetic resonance cholangiopancreatography (MRCP). A liver biopsy was pursued eventually, demonstrating glycogenotic hepatocytes.GH is frequently misdiagnosed as NAFLD, a more common liver disease that occurs in association with diabetes While GH is known to be reversible, NAFLD has been known to progress to advanced liver disease, ranging from cirrhosis to hepatocellular carcinoma. Definite diagnosis often requires liver biopsy because of overlapping clinical and radiographical pictures. Elevation of both glucose and insulin levels in the setting of fragile DM control is thought to play a role via overstimulation of glycogen synthesis. Recommended treatment is stable "tight" glycemic control; pancreatic transplantation has resulted in sustained GH remission in two case reports. The required degree of stability and tightness of glucose control is not yet known. An increased awareness of GH is needed in an attempt to prevent delay in diagnosis, in a condition with an otherwise unknown incidence.

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Glycogenic Hepatopathy: Resolution with Minimal Glucose Control

Cited by 11 publications

References 9 publications

Glycogenic hepatopathy in children with poorly controlled type 1 diabetes mellitus

Glycogenic hepatopathy in children with poorly controlled type 1 diabetes mellitus

Glycogenic hepatopathy as a cause of severe deranged liver enzymes in a young patient with type 1 diabetes mellitus

Glycogen Hepatopathy: A Reversible yet Relapsing Cause of Hepatitis in Type 1 Diabetics

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