Case report a rare case of persistent lactic acidosis in the. Fortunately, the transaminase elevations are reversible. Additional patient groups that can be affected by glycogenic hepatopathy include those with type ii diabetes mellitus, urea cycle defects, and drug effect. Color doppler reveals irregularly branching neovascularity. Glycogenic hepatopathy gh has been reported as a very rare and under recognized complication in longstanding poorly controlled type 1 diabetes t1d patients. Reported are the clinical and pathologic features of glycogenic hepatopathy, a pathologic overloading of hepatocytes with glycogen that is associated with poorly controlled diabetes mellitus.
Sep 01, 2012 glycogenic hepatopathy in type 1 diabetes mellitus lin, yu. Patients are often asymptomatic but can present with abdominal pain, nausea and vomiting. Druginduced liver injury in the setting of glycogenic. This case report illustrates the fact that glycogenic hepatopathy may relapse, and. Vacuolar hepatopathy vh is a commonly diagnosed canine liver syndrome in which hepatocytes become markedly distended with cytosolic glycogen with or without discrete membranebound lipid inclusions. We conclude that glycogenic hepatopathy can cause hepatomegaly and significant transaminase elevations in individuals with type i diabetes mellitus. How it works each grade level comes with two music books a repertoire book, and a rcm piano syllabus book songs designed to. Diabetes hepatopathy in dogs symptoms, causes, diagnosis. The bright liver of glycogenic hepatopathy the bright liver of glycogenic hepatopathy sweetser, seth. Clinically, gh cannot be distinguished from nonalcoholic fatty liver disease nafld by history, physical examination or.
It is a rare complication of longstanding, poorly controlled type 1. Jgim clinical vignettes druginduced liver injury in the. In addition to the ebook, youll get occasional updates on new lessons and videos, and tips to help you out on your piano journey. Liver test abnormalities vary significantly in glycogenic hepatopathy from normal to 10 times the upper limits of normal in some cases. To identify scientific studies and to deepen the knowledge on dh, and to evaluate the prevalence of dh in the main published studies in medical literature. The marked elevation in aminotransferases in our patient is much greater than described in the literature, 1 raising the question of whether the clinical presentation is solely related to glycogenic hepatopathy. Glycogenic hepatopathy gh is an underdiagnosed complication of uncontrolled type 1 diabetes mellitus t1dm. Dec 11, 2012 glycogenic hepatopathy was first described by mauriac, in 1930, in diabetic children as part of a syndrome that can occur without the syndromal features in adults with type 1 diabetes. Case report glycogenic hepatopathy in type 1 diabetes mellitus. Glycogenic hepatopathy gh is a disorder associated with uncontrolled diabetes mellitus, most commonly type 1, expressed as right upper quadrant abdominal pain, hepatomegaly and increased liver enzymes.
This activation stimulates glycogen storage in the liver. After the diagnosis was documented by liver biopsy, the. Jan 25, 2010 liver test abnormalities vary significantly in glycogenic hepatopathy from normal to 10 times the upper limits of normal in some cases. Glycogenic hepatopathy is excessive intrahepatic glycogen accumulation. Glycogenic hepatopathy in type 1 diabetes mellitus glycogenic hepatopathy in. This is a distinct entity from other causes of hepatomegaly and elevated liver enzymes in diabetics, such as nonalcoholic fatty liver disease.
Glycogenic hepatopathy gh remains underrecognized in adults as most clinicians mistake it for the more common. May 01, 2020 glycogenic hepatopathy pdf glycogenic hepatopathy gh is a disorder associated with uncontrolled diabetes mellitus, most commonly type 1, expressed as right upper quadrant abdominal. Congestive hepatopathy radiology reference article. To help us understand it better, lets break it down. The liver biopsy typically shows numerous swollen and palestaining hepatocytes on hematoxylin and eosin stains with excess glycogen accumulation demonstrated by periodic acidschiff stains. Several types of vacuolar hepatopathy can occur in dogs with diabetes mellitus. Glycogenic hepatopathy and mauriac syndrome kirstens. The diagnosis may be difficult, because laboratory and imaging tests are not pathognomonic. When the patient was 20, a diagnosis of celiac disease was made based on the presence of positive antitranglutaminase antibodies and a compati. Congestive hepatopathy resulting from passive hepatic congestion is caused by stasis of blood within the liver parenchyma due to compromise of hepatic venous drainage. We report a case of a 19yearold woman with a history of poorly controlled diabetes mellitus. Glycogenic hepatopathy gh is a quite unknown cause of elevated transaminases in type 1 diabetic patients with poor glycemic control characterized by.
Fourteen cases were studied by stains, including hematoxylin and eosin, trichrome, periodic acidschiff, and periodic acidschiff with diastase. It is a rare complication of longstanding, poorly controlled type 1 diabetes mellitus. Clinical, biochemical, and histopathology features of. In glycogenic hepatopathy, insulin presence and high levels of glucose increase livers glycogen load. Liver dysfunction and corresponding clinical signs and. It is characterized by the development of significant transaminitis and marked hepatomegaly. Glycogenic hepatopathy in type 1 diabetes mellitus, liver. Our patients diagnosis of glycogenic hepatopathy required a liver biopsy, which. Request pdf glycogenic hepatopathy background glycogenic hepatopathy gh is a disorder associated with uncontrolled diabetes mellitus, most commonly type 1, expressed as right.
Jan 31, 2020 lantus sliding scale pdf in slidingscale insulin therapy, the dose is based on your blood sugar level. Imageofthemonth the bright liver of glycogenic hepatopathy. Glycogenic hepatopathy gh remains underrecognized in adults as most clinicians mistake it for the more common hepatic abnormality associated with uncontrolled diabetes mellitus in this age group, nonalcoholic fatty liver disease. Understanding vacuolar hepatopathy vh, a liver disease. The chart showing pdf series, word series, html series, scan qr codes. Glycogenic hepatopathy gh is a disorder associated with uncontrolled diabetes mellitus, most commonly type 1, expressed as right upper quadrant abdominal. Gh is characterized by transient elevation of liver transaminase and hepatomegaly caused by reversible and excessive glycogen accumulation in hepatocytes. Feb 01, 2010 the bright liver of glycogenic hepatopathy the bright liver of glycogenic hepatopathy sweetser, seth. Glycogenic hepatopathy pdf glycogenic hepatopathy gh is a disorder associated with uncontrolled diabetes mellitus, most commonly type 1, expressed as right upper quadrant. Lantus sliding scale pdf in slidingscale insulin therapy, the dose is based on your blood sugar level. As patients with glycogenic hepatopathy often have nonspecific complaints, diagnosis tends to be delayed and laboratory and imaging data are often indistinguishable from nonalcoholic fatty liver disease.
Imaging findings of congestive hepatopathy1 congestive hepatopathy ch refers to hepatic abnormalities that result from passive hepatic venous congestion. The bright liver of glycogenic hepatopathy, hepatology. Pdf glycogenic hepatopathy gh is a rare complication of the poorly controlled diabetes mellitus characterized by the transient liver. Glycogenic hepatopathy results from the pathologic accumulation of excess glycogen within the liver and is most commonly associated with poorly controlled type i diabetes mellitus.
Glycogenic hepatopathy gh is a rare complication of the poorly controlled. Gh is characterized by transient elevation of liver transaminase and hepatomegaly caused by reversible and excessive glycogen accumulation in. Although the vacuoles in the liver are most often filled with glycogen, a polysaccharide that provides carbohydrate storage for most animals, they can also be filled with fat, water, or waste. Electromyography was normal no neuropathy, or myopathy. Vacuoles occur when liver cells become enlarged and take on another cell, either a glycogen molecule, or a combination of glycogen and. Oct 12, 2019 nonetheless, a subclinical hypothyroidism was revealed. Clinical vignettes druginduced liver injury in the setting of glycogenic hepatopathy valmiki maharaj, md1, matthew fitz, md1, and xianzdong ding, md phd2 1department of medicine, loyola university stritch school of medicine, maywood, il, usa. Tweet pale, enlarged hepatocytes may be mistaken for. Pdf glycogenic hepatopathy as an unusual etiology of. Glycogenic hepatopathy associated with type 1 diabetes mellitus. The bright liver of glycogenic hepatopathy, hepatology 10. Case report glycogenic hepatopathy in type 1 diabetes mellitus muratatmaca, 1 rifkiucler, 1 mehmetkartal, 2 ismetseven, 2 muratalay, 1 irfanbayram, 3 andsehmusolmez 4 department of endocrinology and metabolism, yuzuncu yil university faculty of medicine, van, turkey. Vacuoles occur when liver cells become enlarged and take on another cell, either a glycogen molecule, or a combination of glycogen and lipid molecules most common with diabetes.
On liver pathology, there is an abundance of glycogen deposition, which is not associated with inflammation or fibrosis. Glycogenlike vh is associated with typical or atypical hyperadrenocorticism or endogenous release of corticosteroids in response to chronic. This condition, characterized by elevated liver enzymes and hepatomegaly, is caused by irreversible and excessive accumulation of glycogen in hepatocytes. If you do not see its contents the file may be temporarily unavailable at the journal website or you do not have a pdf plugin installed and enabled in your browser. Periodic acid schiff stain for glycogen was positive in these hepatocytes. She had poor glycaemic control, with a recent serum hba1c value of 12. A liver biopsy performed in the occasion of the second hospitalization allowed to rule out.
It appears as an acute relapsing hepatitis with reversible transaminase elevations secondary to excessive hepatic glycogen accumulation. In front of an uncontrolled diabetes type 1, hepatomegaly, glycogenic hepatopathy and persistent hyperlactatemia, a diagnosis of mauriac syndrome was made. Glycogenic hepatopathy is a rare cause of high transaminase levels in type 1 diabetes mellitus. Glycogenic hepatopathy pdf new pdf download service.
Hepatopathy definition of hepatopathy by medical dictionary. Abstractglycogenic hepatopathy gh is a rare complication of type 1 diabetes. We describe a presentation of glycogenic hepatopathy in a poorly controlled type i diabetic patient. You have a marvelous accomplishment for which you are to be congratulated on a job well done. A case of glycogenic hepatopathy developed in a patient. Stavros thoroughly explains the physics of breast ultrasound and the download and read free online breast ultrasound a. Hepatopathy the hepato part of the word, hepatopathy, refers to the liver and the pathy part refers to a disease. Glycogenic hepatopathy as an unusual etiology of deranged liver function in a patient with type 1 diabetes.
We report a case of a 19yearold woman with a history of poorly controlled diabetes mellitus and frequent admissions for diabetic ketoacidosis. A diagnosis of glycogenic hepatopathy was made after clinicopathological correlation. Glycogen hepatopathy gh is a rare complication of type 1 diabetes mellitus that leads to an abnormal accumulation of glycogen in the hepatocytes. This is a case report on a 19yearold male case, diagnosed with glycogenic hepatopathy. Glycogenic hepatopathy is a rare cause of liver dysfunction that occurs in patients with poorly controlled diabetes mellitus. Prolonged exposure to elevated hepatic venous pressure may lead to liver fibrosis and cirrhosis. Glycogenic hepatopathy associated with type 1 diabetes mellitus as. Glycogenic hepatopathy was first described by mauriac, in 1930, in diabetic children as part of a syndrome that can occur without the syndromal features in adults with type 1 diabetes. The bright liver of glycogenic hepatopathy sweetser 2010. Case report a rare case of persistent lactic acidosis in. Future studies warranted to further assess for development of fibrosis in gh as it can progress to cirrhosis.
Glycogenic hepatopathy in type 1 diabetes mellitus glycogenic hepatopathy in type 1 diabetes mellitus lin, yu. Glycogenic hepatopathy in type 1 diabetes mellitus glycogenic hepatopathy in type 1 diabetes mellitus. Glycogenic hepatopathy associated with type 1 diabetes. Jul 09, 2015 glycogenic hepatopathy gh remains underrecognized in adults as most clinicians mistake it for the more common hepatic abnormality associated with uncontrolled diabetes mellitus in this age group, nonalcoholic fatty liver disease. Shun the politics that consumes peoples and devote your efforts to the wherewithal of their sustenance and enrichment. Congestive hepatopathy refers to hepatic manifestations attributable to passive hepatic congestion, as occurs in patients with rightsided heart failure. The term glycogenic hepatopathy gh has been proposed by torbenson et al. Glycogenic hepatopathy is a rare and underecognized complication in longstanding poorly controlled type 1 diabetes mellitus patients. A liver biopsy confirmed the diagnosis of glycogenic hepatopathy and the patient improved with diabetes control. Hepatic biopsy showed a hepatic glycogen overload with fibrous frame. Vacuolar hepatopathy can seem like a complicated topic. Canine vacuolar hepatopathy in dogs symptoms, causes. It dephosphorylates and activates glycogen synthase, which is the enzyme required to transform glucose 1phosphate into glycogen. Case report glycogenic hepatopathy in type 1 diabetes.
Hashimotos thyroiditis was diagnosed when she was 16, and was treated with levothyroxine. Hepatopathy definition at, a free online dictionary with pronunciation, synonyms and translation. Passive congestion often coexists with reduced cardiac output, making their relative contributions to hepatic injury intertwined. See pathogenesis of liver injury in circulatory failure. Clinically, gh cannot be distinguished from nonalcoholic fatty liver. A case of glycogenic hepatopathy developed in a patient with. Canine vacuolar hepatopathy merck veterinary manual. Elevated lactate levels in patients with poorly regulated.
The bright liver of glycogenic hepatopathy sweetser. It is recognized that glycogenic hepatopathy can present without the complete features of mauriac syndrome,3 as in our patient. The key feature is glycogen accumulation in the liver causing hepatomegaly and raised serum transaminases with wide fluctuations in both glucose and insulin levels. Canine vacuolar hepatopathy is a disorder of the liver in which small fluidfilled cavities referred to as vacuoles develop in the liver of your dog. Case report a rare case of persistent lactic acidosis in the icu. This is also complicated by the fact that both entities are indistinguishable on liver ultrasound. Glycogenic hepatopathy gh is considered as a benign glycogenjc condition. The clinical presentation typically includes varying degrees. Clinical resolution of glycogenic hepatopathy following.