Glycogenic hepatopathy pdf files

Awareness of glycogenic hepatopathy should help the clinician to not treat lactic acidosis based on numbers and expose patients to unnecessary sepsis treatment in the right clinical setting. Glycogenic hepatopathy as a cause of severe deranged liver. Glycogenic hepatopathy is a rare but potentially reversible condition characterized by hepatomegaly and elevated liver enzymes occurring in poorly controlled type 1 diabetes mellitus patients and often requires a liver biopsy to confirm the diagnosis. Jan 25, 2010 liver test abnormalities vary significantly in glycogenic hepatopathy from normal to 10 times the upper limits of normal in some cases. We report a case of liver biopsyproven glycogenic hepatopathy gh in a. Clinical, laboratory, and pathological information are described. Glycogenic hepatopathy presents with abdominal pain, hepatomegaly, and transaminasemia as a result of increased glycogen formation and deposition in the setting of excess levels of insulin and glucose. While nonalcoholic fatty liver disease nafld is more common, glycogenic hepatopathy gh should be considered as a differential diagnosis, especially in patients with poorly controlled type 1 diabetes dm1.

Vacuolar hepatopathy can seem like a complicated topic. We herein report the first case of glycogenic hepatopathy. An 18yearold male with uncontrolled type 1 diabetes and recurrent diabetic ketoacidosis dka presented with abdominal distention and severe hyper. Case report we report a 28yearold female, previously diagnosed long lasting type. Pdf glycogenic hepatopathy in type 1 diabetes mellitus. Glycogenic hepatopathy as an unusual etiology of deranged. Glycogenic hepatopathy is a distinct clinicopathologic entity in which there is a pathologic overloading of hepatocytes with glycogen. This condition, characterized by elevated liver enzymes and hepatomegaly, is caused by irreversible. When the patient was 20, a diagnosis of celiac disease was made based on the presence of positive antitranglutaminase antibodies and a compati.

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. Pdf glycogenic hepatopathy is a rare and underrecognized complication in longstanding poorly controlled type 1 diabetes mellitus patients. We report a case of a 19yearold woman with a history of poorly controlled diabetes mellitus. Diabetes hepatopathy in dogs symptoms, causes, diagnosis.

Our patients diagnosis of glycogenic hepatopathy required a liver biopsy, which. 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. We present the case of a young man who was admitted with diabetic ketoacidosis in the setting of poorly controlled diabetes mellitus type 1 and. A similar condition was described by mauriac in 1930, characterized by growth retardation, hepatomegaly, cushingoid features, and delayed puberty 1. 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. Request pdf glycogenic hepatopathy background glycogenic hepatopathy gh is a disorder associated with uncontrolled diabetes mellitus, most commonly type 1, expressed as right. Please, select more pdf files by clicking again on select pdf files.

Hepatopathy definition of hepatopathy by medical dictionary. Abstract 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. Pdf glycogenic hepatopathy and nonalcoholic fatty liver. 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. The chart showing pdf series, word series, html series, scan qr codes. This is a pdf file of an unedited manuscript that has been accepted for publication. Glycogenic hepatopathy gh is excessive intrahepatic glycogen accumulation that manifests as hepatomegaly with transient elevation in serum. It appears as an acute relapsing hepatitis with reversible transaminase elevations secondary to excessive hepatic glycogen accumulation. Additional patient groups that can be affected by glycogenic hepatopathy include those with type ii diabetes mellitus, urea cycle defects, and drug effect. 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 glycogenichepatopathy. Department of pediatrics, far eastern memorial hospital, new taipei city, taiwan. Jul 23, 2019 congestive hepatopathy refers to hepatic manifestations attributable to passive hepatic congestion, as occurs in patients with rightsided heart failure.

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. View the article pdf and any associated supplements and figures for a period of 48 hours. Understanding vacuolar hepatopathy vh, a liver disease in dogs. Glycogenic hepatopathy and nonalcoholic fatty liver disease in type 1 diabetes patients article pdf available in pediatrics international 556. Diabeticglycogenic hepatopathy global events usa europe. Glycogenic hepatopathy is an underrecognised cause of. Canine vacuolar hepatopathy merck veterinary manual.

Hepatopathy unlike humans, liver disease is relatively common in cats and dogs, especially with advancing age. Glycogenic hepatopathy gh is a rare complication of the poorly controlled diabetes mellitus characterized by the transient liver dysfunction. A diagnosis of glycogenic hepatopathy was made after clinicopathological correlation. 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. Clinical resolution of glycogenic hepatopathy following. It is characterized by the development of significant transaminitis and marked hepatomegaly. We report a case of a 19yearold woman with a history of poorly controlled diabetes mellitus and frequent admissions for diabetic ketoacidosis, who presented with abdominal pain, nausea. 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. This condition, characterized by elevated liver enzymes and hepatomegaly, is caused by irreversible and excessive accumulation of glycogen in hepatocytes.

Imageofthemonth the bright liver of glycogenic hepatopathy. Future studies warranted to further assess for development of fibrosis in gh as it can progress to cirrhosis. Case report a rare case of persistent lactic acidosis in. The bright liver of glycogenic hepatopathy request pdf. Tweet pale, enlarged hepatocytes may be mistaken for. How to merge pdfs and combine pdf files adobe acrobat dc. An oe n underrecognized nding of glycogenic hepatopathy is lactic acidosis and hyperlactatemia. There are multiple etiologies, the most common being nonalcoholic fatty liver disease nafld. Gh is characterized by transient elevation of liver transaminase and hepatomegaly caused by reversible and excessive glycogen accumulation in hepatocytes. Glycogenic hepatopathy is a rare cause of high transaminase levels in type 1 diabetes mellitus. Fourteen cases were studied by stains, including hematoxylin and eosin, trichrome, periodic acidschiff, and periodic acidschiff with diastase.

Glycogenic hepatopathy is a rare cause of liver dysfunction that occurs in patients with 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 glycogen overload hepatocytes. 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. Glycogenic hepatopathy gh is an underdiagnosed complication ofuncontrolledtype 1 diabetes mellitus t1dm. Classically, it has been described in association with poorly controlled diabetes mellitus types i and ii in both pediatric and adult populations. Pdf on apr 1, 2015, ravi kant and others published glycogenic hepatopathy with persistent hepatomegaly in a patient with uncontrolled. Prolonged exposure to elevated hepatic venous pressure may lead to liver fibrosis and cirrhosis. As a service to our customers we are providing this early version of the manuscript.

Click add files and select the files you want to include in your pdf. Ethics documents guidelines for the manuscript publishing process. Jgim clinical vignettes druginduced liver injury in the. The bright liver of glycogenic hepatopathy sweetser 2010. Diabetic hepatopathy simple steatosis or nonalcoholic steatohepatitis are liver diseases associated with diabetes mellitus dm. Glycogenic hepatopathy is a rare and underecognized complication in longstanding poorly controlled type 1 diabetes mellitus patients. 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. 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 is a distinct entity from other causes of hepatomegaly and elevated liver enzymes in diabetics, such as nonalcoholic fatty liver disease.

Mauriac syndrome is a rare disorder that can present with the single feature of glycogenic hepatopathy in children and adults with poorly controlled diabetes mellitus. Clinical, biochemical, and histopathology features of. Select up to three search categories and corresponding keywords using the fields to the right. While the majority of the few studied cases on this. 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.

Clinical presentations can include abdominal pain, tender hepatomegaly, nausea and elevated transaminases. We conclude that glycogenic hepatopathy can cause hepatomegaly and significant transaminase elevations in individuals with type i diabetes mellitus. Glycogenic hepatopathy as a cause of severe deranged liver enzymes in a young patient with type 1 diabetes mellitus. Hashimotos thyroiditis was diagnosed when she was 16, and was treated with levothyroxine. Glycogenic hepatopathy associated with type 1 diabetes. Glycogenic hepatopathy gh is a rare complication of poorly controlled type 1 diabetes mellitus t1dm. Several types of vacuolar hepatopathy can occur in dogs with diabetes mellitus. Glycogenic hepatopathy gh is the hallmark pathological finding of mauriac syndrome, first described in 1930 in children with uncontrolled type 1 diabetes, delayed growth, cushingoid features, hypercholesterolemia, hepatomegaly, and elevated liver transaminases. Deranged liver function is a common finding among patients with diabetes mellitus. We present a 19yearold 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. Gh remains underrecognized, so that only around 50 adult cases of gh have been reported to.

Glycogenic hepatopathy gh is considered as a benign reversible condition. Glycogenic hepatopathy gh is an underrecognised complication of type 1 diabetes mellitus t1dm not controlled to target resulting in hepatomegaly and elevated liver transaminases. On liver pathology, there is an abundance of glycogen deposition, which is not associated with inflammation or fibrosis. It is predominantly seen in patients with longstanding type 1 diabetes mellitus and rarely reported in association with. See pathogenesis of liver injury in circulatory failure. This is also complicated by the fact that both entities are indistinguishable on liver ultrasound. Glycogenic hepatopathy as an unusual etiology of deranged li. Pdf a case of glycogenic hepatopathy in type 1 diabetes. A case of glycogenic hepatopathy developed in a patient with. Unusual glycogenic hepatopathy causing abnormal liver.

Pdf glycogenic hepatopathy with persistent hepatomegaly in a. Rearrange individual pages or entire files in the desired order. To change the order of your pdfs, drag and drop the files as you want. Elevated liver enzymes is a common clinical problem encountered in patients with diabetes mellitus dm. Gh is a relatively underrecognized condition seen in poorly controlled type 1 diabetes mellitus. The liver is an extremely important organ in the body, responsible for processing digested food components absorbed from the intestinal tract, cleansing and filtering the blood, breaking down the. Congestive hepatopathy radiology reference article. Patients are often asymptomatic but can present with abdominal pain, nausea and vomiting.

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. Deranged liver function is commonly encountered in patients with diabetes. In glycogenic hepatopathy, insulin presence and high levels of glucose increase livers glycogen load. Druginduced liver injury in the setting of glycogenic. Unusual glycogenic hepatopathy causing abnormal liver enzymes in a morbidly obese adolescent with well. Glycogenic hepatopathy associated with type 1 diabetes mellitus.

Merge pdf files combine pdfs in the order you want with the easiest pdf merger available. Passive congestion often coexists with reduced cardiac output, making their relative contributions to hepatic injury intertwined. 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. To help us understand it better, lets break it down. You can merge pdfs or a mix of pdf documents and other files. Pdf glycogenic hepatopathy gh is a rare complication of the poorly controlled diabetes mellitus characterized by the transient liver. Elevated lactate levels in patients with poorly regulated. Glycogenic hepatopathy is an under recognised condition, described as a pathological overloading of hepatocytes with glycogen in patients with poorly controlled type 1 diabetes mellitus. After the diagnosis was documented by liver biopsy, the. A case of glycogenic hepatopathy developed in a patient with newonset fulminant type 1 diabetes.

Glycogenic hepatopathy is excessive intrahepatic glycogen accumulation. We describe a presentation of glycogenic hepatopathy in a poorly controlled type i diabetic patient. Glycogenic hepatopathy in type 1 diabetes mellitus. This condition remains poorly recognized among clinicians, which may lead to expensive and unnecessary testing and delaying appropriate management. Glycogenic hepatopathy gh is a rare condition characterized by glycogen deposition in the liver in patients with. Canine vacuolar hepatopathy in dogs symptoms, causes. Fourteen cases were studied by stains, including hematoxylin and eosin, trichrome, periodic acid. 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. Glycogenic hepatopathy gh has been reported as a very rare and under recognized complication in longstanding poorly controlled type 1 diabetes t1d patients. Primary treatment of glycogenic hepatopathy is improved longterm blood glucose. Glycogenic hepatopathy as an unusual etiology of deranged liver function in a patient with type 1 diabetes. It is a rare complication of longstanding, poorly controlled type 1 diabetes mellitus. Liver biopsy showed features consistent with glycogenic hepatopathy gh.

We report the case of a 19yearold man with t1dm not controlled to target who presented with abdominal pain, hepatomegaly and deranged liver transaminases. Glycogenic hepatopathy gh is considered as a benign glycogenjc condition. 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. The combination of poorly controlled diabetes, acute liver injury with marked elevation in serum aminotransferases, and the characteristic histological changes on liver biopsy are diagnostic of glycogenic hepatopathy. The key feature is glycogen accumulation in the liver causing hepatomegaly and raised serum transaminases with wide fluctuations in both glucose and insulin levels. 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. Glycogenic hepatopathy gh is an underdiagnosed complication of uncontrolled type 1 diabetes mellitus t1dm. Glycogenic hepatopathy pdf new pdf download service. This is a case report on a 19yearold male case, diagnosed with glycogenic hepatopathy. Congestive hepatopathy resulting from passive hepatic congestion is caused by stasis of blood within the liver parenchyma due to compromise of hepatic venous drainage. This occurs as a result of excessive accumulation of glycogen in hepatocytes, manifesting as hepatomegaly and flares of elevation in transaminases 1, which in some cases. Case report a rare case of persistent lactic acidosis in the. The term glycogenic hepatopathy gh has been proposed by torbenson et al.

Hepatopathy the hepato part of the word, hepatopathy, refers to the liver and the pathy part refers to a disease. Refer to the help section for more detailed instructions. Pdf glycogenic hepatopathy as an unusual etiology of. Case report glycogenic hepatopathy in type 1 diabetes mellitus. Case report glucocorticoidinduced rapid development of. Glycogenlike vh is associated with typical or atypical hyperadrenocorticism or endogenous release of corticosteroids in response to chronic. Abstractglycogenic hepatopathy gh is a rare complication of type 1 diabetes. The diagnosis may be difficult, because laboratory and imaging tests are not pathognomonic. It dephosphorylates and activates glycogen synthase, which is the enzyme required to transform glucose 1phosphate into glycogen. Liver test abnormalities vary significantly in glycogenic hepatopathy from normal to 10 times the upper limits of normal in some cases. 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. Glycogenic hepatopathy gh is a rare complication of the poorly. More recently, it has been recognized that adolescents and adults with uncontrolled diabetes on insulin therapy may present with. Glycogenic hepatopathy gh is a rare complication of poorly controlled type 1 diabetes mellitus t1dm, and is characterized by elevated liver enzymes, hepatomegaly, and glycogen accumulation.

Click, drag, and drop to reorder files or press delete to remove any content you dont want. Periodic acid schiff stain for glycogen was positive in these hepatocytes. Glycogenic hepatopathy gh is a rare condition characterized by glycogen deposition in the liver in patients with poorlycontrolled type 1 dm. Glycogenic hepatopathy gh is a disorder associated with uncontrolled diabetes mellitus, most commonly type 1, expressed as right upper quadrant abdominal.

1380 1451 1131 360 1175 812 1290 1063 342 416 221 109 1555 1188 957 801 791 72 851 946 450 1593 479 779 253 731 1602 580 133 387 453 165 29 1172 113 112 275