Glycogenic hepatopathy (GH) is a disorder associated with uncontrolled diabetes mellitus, most commonly type 1, expressed as right upper quadrant abdominal. Periodic acid Schiff stain for glycogen was positive in these hepatocytes. A diagnosis of glycogenic hepatopathy was made after clinicopathological correlation. We describe a presentation of glycogenic hepatopathy in a poorly controlled type I diabetic patient. As patients with glycogenic hepatopathy.
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Case A year-old male patient was admitted to the emergency department due to loss of appetite and nausea complaints that continued for two days.
It occurs in patients with T1DM more often than in T2DM and is associated with severe microvascular disease in other organs.
Histopathology of a case reported by Shah et al[ 41 ]. Thinking Outside the Box. GH can resolve with both clinical and biochemical resolution as quickly as it develops within days to weeks with good glycemic control[ 40 ]. A case has also been reported in a patient following intentional insulin gepatopathy [ 67 ].
Though often NAFLD is responsible for this condition, in case of type 1 diabetic patients, GH should be considered for differential diagnosis in the presence of severe elevation of aminotransferase levels. It is believed to be the consequence of recurrent fluctuations in glucose level with hyperglycemia, hypoglycemia, and hyperinsulinization. Scarpato wrote the case report.
Glycogenic hepatopathy: an underrecognized hepatic complication of diabetes mellitus.
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.
Case Rep Crit Care. Glycogen synthase converts glucose 1-phosphate into glycogen and increases glycogen storage in the liver while inhibiting glycogenolysis [ 126 ]. However, these tests may not yet be readily available in most centers.
Glycogenic Hepatopathy: Resolution with Minimal Glucose Control
Presence of cirrhosis can be determined Biochemical tests including genetic testing, radiologic, and liver biopsy Phlebotomy or Chelation therapy if unable to tolerate hfpatopathy Can progress to cirrhosis Wilson disease Autosomal recessive disorder with impaired cellular copper transport and impaired biliary copper excretion results in accumulation of copper most notably the liver, brain, and cornea. This case illustrates GH in a type I diabetic diagnosed via the characteristic pathology and treated successfully despite hepatopaty modest improvements in glucose control.
Hormones like adrenaline, cortisol, or growth hormones released due to hypoglycemia, could synergistically act and release large quantities of non-esterified fatty acids from adipose tissue.
Glycogenic hepatopathy and non-alcoholic fatty liver disease in type 1 diabetes patients. Much of the knowledge on GH that has been accumulated over the decades since GH was first reported inhepatopatgy from case reports, case series, a retrospective cohort study, or more recently, a case-control study[ 4546 ]. No single serologic tlycogenic can diagnose GH.
Prognosis with improved glycemic control is excellent. Ultrastructural analysis was performed in 2 cases.
Case Reports in Hepatology
Butts et al[ 24 ], The medical history of the case showed that he was followed up due to type 1 diabetes for 8 years and for hepatosteatosis for 3 years, had poor blood glucose regulation despite hepatppathy analogue and basal insulin therapy, and was hospitalized and followed up 8—10 times for diabetic ketoacidosis. Genetic testing, as performed in our case, can help to rule out GSD. Symptomatic treatment hepayopathy dietary changes to maintain the blood glucose level and pharmacologic therapy in different types of GSDs.
Ultimately, a glycoegnic of GH was made. Type 1 diabetes mellitus; HbA1C: May need liver transplantation in selected cases. Furthermore, persistent reversal has nepatopathy observed following pancreas transplantation [ 2 ]. Histology of GH reveals pale appearance of hepatocytes with compression of sinusoids, glycogenated nuclei, giant mitochondria, and positive periodic acid-Schiff stained intracytoplasmic inclusions that disappear after digestion with diastase [ 1 — 36 ].
No characteristic imaging features, may show cirrhotic liver in advance case. This is an open access article distributed under the Creative Commons Attribution Licensewhich permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
D-Periodic-Acid Schiff stain remove glycogen leaving empty looking cytoplasm blue arrow and nuclei green arrow. Liver biopsy may show extensive, dense, perisinusoidal fibrosis, and immunostaining reveals basement membrane components in a perisinusoidally distribution[ 62 ]. Iron stain was negative.
Differentiation is further complicated by possible coexistence of steatosis with glycogenic hepatopathy [ 12 ]. Absolute quantification of the hepatic glycogen content in a patient with glycogen storage disease by 13C magnetic resonance spectroscopy. Photographs will be accepted at the discretion of the Editorial Board. For patients with DM who present with hepatomegaly and elevated transaminase levels, there are several differential diagnoses apart from glycogenic hepatopathy, including NAFLD.
Correspondence should be addressed to Abhimanyu Chandel ; lim. The knowledge that the hepatomegaly in patients taking steroids could be GH-related will aid in the management of these patients.
He became hypoglycemic and was later administered a large dose of intravenous glucose to counteract the persistent hypoglycemia.