Glycogenic hepatopathy gh has been reported as a very rare and under recognized complication in longstanding poorly controlled type 1 diabetes t1d patients. They contain their normal structures, and there are no. Several types of vacuolar hepatopathy can occur in dogs with diabetes mellitus. An often underrecognized finding of glycogenic hepatopathy is lactic acidosis and hyperlactatemia. Imageofthemonth the bright liver of glycogenic hepatopathy. Case report a rare case of persistent lactic acidosis in the. We report a patient who presented with predominant elevation in alkaline phosphatase and. Passive congestion often coexists with reduced cardiac output, making their relative contributions to hepatic injury intertwined. Case report glycogenic hepatopathy in 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. 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. Congestive hepatopathy hepatic and biliary disorders. This condition, characterized by elevated liver enzymes and hepatomegaly, is caused by irreversible. Sparing of portal tracts the portal tracts appear normal in glycogenic hepatopathy.
See pathogenesis of liver injury in circulatory failure. Glycogenic hepatopathy is an underrecognised cause of. 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. 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. Congestive hepatopathy radiology reference article. Glycogenic hepatopathy is a rare condition that causes significant hepatomegaly and elevated liver enzyme levels in uncontrolled type 1. Gh by combining with thorough clinical evaluation of. Pdf a case of glycogenic hepatopathy in type 1 diabetes. A case of glycogenic hepatopathy developed in a patient with newonset fulminant type1 diabetes. Background mauriac syndrome is characterised by growth failure, cushingoid appearance and hepatomegaly which occurs in patients with insulin dependent diabetes and was first described shortly after the introduction of insulin as a treatment for the condition. What it means is that there is more glycogen than normal being stored in liver cells.
Abstractglycogenic hepatopathy gh is a rare complication of type 1 diabetes. Glycogenic hepatopathy is a rare and underrecognized complication of poorly controlled diabetes mellitus. Glycogenic hepatopathy is a rare cause of liver dysfunction that occurs in patients with poorly controlled diabetes mellitus. Progressive vh merges into the classic hepatic syndrome associated with the. 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. 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. Hepatopathies in children and adolescents with type 1. We will be providing unlimited waivers of publication charges for accepted articles related to covid19. We describe the clinical, biochemical, and histopathology profile of a cohort of patients with glycogenic hepatopathy. Gh is characterized by transient elevation of liver transaminase and hepatomegaly caused by reversible and excessive glycogen accumulation in hepatocytes. Canine vacuolar hepatopathy in dogs symptoms, causes. More recently, it has been recognized that adolescents and adults with uncontrolled diabetes on insulin therapy may present with. 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. Gh usually presents with abdominal pain and elevated transaminases.
Tight glycemic control glyckgenic intensive insulin therapy provides full remission of clinical, laboratory, and histological abnormalities 4. A rare case of persistent lactic acidosis in the icu. 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. After the diagnosis was documented by liver biopsy, the. Clinical, biochemical, and histopathology features of.
Torbenson m, chen yy, brunt e, cummings ow, gottfried m, jakate s et al. Glycogenlike vh is associated with typical or atypical hyperadrenocorticism or endogenous release of corticosteroids in response to chronic. 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, a syndrome characterized by hepatomegaly and increased liver transaminases in patients with type 1 diabetes, has not been well characterized in adults. Canine vacuolar hepatopathy digestive system merck. Nonetheless, a subclinical hypothyroidism was revealed. Hashimotos thyroiditis was diagnosed when she was 16, and was treated with levothyroxine. The diagnosis may be difficult, because laboratory and imaging tests are not pathognomonic. Glycogenic hepatopathy pdf glycogenic hepatopathy gh is a disorder associated with uncontrolled diabetes mellitus, most commonly type 1, expressed as right upper quadrant. Cn101596230a a kind of pharmaceutical composition for. A case of glycogenic hepatopathy developed in a patient with. 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. 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. Diabetes hepatopathy in dogs symptoms, causes, diagnosis.
By performing a controlled exposure of an amphibian model to endocrine disruptors eds at concentrations within the range of safe drinking water, we provide evidence of the role played by these widespread contaminants in amphibian population decline through metabolic disruption. We read with great interest the paper by asada et al. It appears as an acute relapsing hepatitis with reversible transaminase elevations secondary to excessive hepatic glycogen accumulation. An unusual presentation of glycogenic hepatopathy with. 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, vomiting, and hepatomegaly. Unexpected metabolic disorders induced by endocrine.
An 18yearold male with uncontrolled type 1 diabetes and recurrent diabetic ketoacidosis dka presented with abdominal distention and severe hyper. Glycogenic hepatopathy gh is a rare complication of the poorly controlled diabetes mellitus characterized by the transient liver dysfunction. Murata f, horie i, ando t, isomoto e, hayashi h, et al. Glycogenic hepatopathy associated with type 1 diabetes mellitus. The gross pathological appearance of a liver affected by chronic passive congestion is speckled like a grated nutmeg kernel. Glycogenic hepatopathy associated with type 1 diabetes. Glycogenic hepatopathy in type 1 diabetes mellitus. An underrecognized hepatic complication of diabetes mellitus. Glycogenic hepatopathy, an underdiagnosed cause of relapsing. We are committed to sharing findings related to covid19 as quickly and safely as possible. Glycogenic hepatopathy, an underdiagnosed cause of. Glycogenlike vh is associated with typical or atypical hyperadrenocorticism or endogenous.
Pdf glycogenic hepatopathy gh has been reported as a very rare and under. Glycogenic hepatopathy as a cause of severe deranged liver. Department of pediatrics, far eastern memorial hospital, new taipei city, taiwan. Glycogenic hepatopathy is a rare but potentially reversible condition characterized by hepatomegaly and elevated liver enzymes occurring in poorly controlled. They contain their normal structures, and there are no portal inflammatory cell infiltrates. We describe a yearold male with a history of poorly controlled type 1 diabetes mellitus who presented with hepatomegaly and severe transaminase flares. Glycogenic hepatopathy gh is considered as a benign glycogenjc condition. It is characterized by excessive hepatic glycogen storage as first described in 1930 by mauriac as a part of a syndrome comprising growth retardation, delayed puberty, and cushingoid features in young patients with type 1 diabetes 1. Pdf glycogenic hepatopathy gh is a rare complication of the poorly controlled diabetes mellitus characterized by the transient liver. The bright liver of glycogenic hepatopathy request pdf. Glycogenic hepatopathy gh is a quite unknown cause of elevated transaminases in type 1 diabetic patients with poor glycemic control characterized by. 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. Glycogenic hepatopathy gh is an underrecognised complication of type 1 diabetes mellitus t1dm not controlled to target resulting in hepatomegaly and elevated liver transaminases. Children typically present with abdominal pain, possibly mediated by distension of the liver capsule by what is often massive hepatomegaly.
The background hepatocytes are diffusely swollen and filled with glycogen. Glycogen like vh is associated with typical or atypical hyperadrenocorticism or endogenous. The present invention relates to a kind of pharmaceutical composition for the treatment of chronic viral hepatitis b, liver cirrhosis, hepatic fibrosis, this drug main will be made by the radix astragali, radix notoginseng, stirfry carapax trionycis, herb gynostemmae pentaphylli, raw material of chinese medicine, the conventional medicine preparation be can make, chronic viral hepatitis b. Glycogenic hepatopathy in type 1 diabetes mellitus lin. Electromyography was normal no neuropathy, or myopathy. See also overview of vascular disorders of the liver. Case report a rare case of persistent lactic acidosis in the icu.
If there is subsequent hepatic fibrosis the term cardiac cirrhosis may be used. Glycogenic hepatopathy is a rare cause of high transaminase levels in type 1 diabetes mellitus. It is predominantly seen in patients with longstanding type 1 diabetes mellitus and rarely reported in association with. Glycogenic hepatopathy gh is a rare cause of serum transaminase elevations in type 1 diabetes mellitus. Glycogenic hepatopathy is excessive intrahepatic glycogen accumulation. 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. Four cases of type 1 diabetes mellitus showing sharp serum. The bright liver of glycogenic hepatopathy sweetser 2010. Dumping syndrome is proposed to be a cause of hepatocellular glycogenosis, the latter representing a form of acquired glycogenic hepatopathy. On liver pathology, there is an abundance of glycogen deposition, which is not associated with inflammation or fibrosis. The liver biopsy typically shows numerous swollen and pale.
It is important to recognize and distinguish glycogen hepatopathy from non alcoholic steatohepatitis as it impacts the management and longterm prognosis. Objective to describe the clinical features, histological findings and outcome of young people with glycogenic hepatopathy, the hepatic. Periodic acid schiff stain for glycogen was positive in these hepatocytes. View the article pdf and any associated supplements and figures for a period of 48 hours. Fourteen cases were studied by stains, including hematoxylin and eosin, trichrome, periodic acid. Diabeticglycogenic hepatopathy global events usa europe. The clinical presentation typically includes varying degrees. Congestive hepatopathy is diffuse venous congestion within the liver that results from rightsided heart failure usually due to a cardiomyopathy, tricuspid regurgitation, mitral insufficiency, cor pulmonale, or constrictive pericarditis.
Jul 23, 2019 congestive hepatopathy refers to hepatic manifestations attributable to passive hepatic congestion, as occurs in patients with rightsided heart failure. When the patient was 20, a diagnosis of celiac disease was made based on the presence of positive antitranglutaminase antibodies and a compati. Glycogenic hepatopathy is a rare condition that causes significant hepatomegaly and elevated liver enzyme levels in uncontrolled type 1 diabetic 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, liver glycogenosis, mauriac syndrome, type 1 diabetes mellitus. Metabolic evaluation confirmed the diagnosis of dumping syndrome, and appropriate dietary management led to sustained resolution of symptomatology and hypertransaminasemia. 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. Druginduced liver injury in the setting of glycogenic. We report a case of a 19yearold woman with a history of poorly controlled diabetes mellitus. The portal tracts appear normal in glycogenic hepatopathy.
Congestive hepatopathy includes a spectrum of hepatic derangements that can occur in the setting of rightsided heart failure and its underlying causes. Pdf glycogenic hepatopathy as an unusual etiology of. Glycogen hepatopathy caused by corticosteroid administration to a dog. 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 a disorder associated with uncontrolled diabetes mellitus, most commonly type 1, expressed as right upper quadrant abdominal. The key feature is glycogen accumulation in the liver causing hepatomegaly and raised serum transaminases with wide fluctuations in both glucose and insulin levels. The bright liver of glycogenic hepatopathy sweetser. 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.
This condition, characterized by elevated liver enzymes and hepatomegaly, is caused by irreversible and excessive accumulation of glycogen in hepatocytes. Glycogenic hepatopathy gh is a rare condition first described in the context of mauriac syndrome in 1930, in a set of clinical features including hepatomegaly, growth retardation and cushingoid characteristics in children with poorly controlled type 1 diabetes mellitus t1dm. We conclude that glycogenic hepatopathy can cause hepatomegaly and significant transaminase elevations in individuals with type i diabetes mellitus. Augmented reality ar technology is used to reconstruct threedimensional 3d images of hepatic and biliary structures from computed tomography and magnetic resonance imaging data, and to superimpose the virtual images onto a view of the surgical field. Vacuolar hepatopathy is not really a disease on its own, but rather it is a way to describe how the liver cells look.
Hepatopathy definition of hepatopathy by medical dictionary. In front of an uncontrolled diabetes type 1, hepatomegaly, glycogenic hepatopathy and persistent hyperlactatemia, a diagnosis of mauriac syndrome was made. A diagnosis of glycogenic hepatopathy was made after clinicopathological correlation. This is a case report on a 19yearold male case, diagnosed with glycogenic hepatopathy. Glycogenic hepatopathy gh is an underdiagnosed complication of uncontrolled type 1 diabetes mellitus t1dm. We herein report the first case of glycogenic hepatopathy. In frogs exposed throughout their life cycle, this disruption induces a metabolic syndrome characteristic of a. This is also complicated by the fact that both entities are indistinguishable on liver ultrasound. Glycogenic hepatopathy gh is a rare complication of the poorly controlled diabetes mellitus characterized by the transient liver dysfunction with elevated liver. 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. Congestive hepatopathy, is liver dysfunction due to venous congestion, usually due to congestive heart failure. Glycogenic hepatopathy pdf new pdf download service.
View enhanced pdf access article on wiley online library html view download pdf for offline viewing. Case report glycogenic hepatopathy in type 1 diabetes mellitus. In glycogenic hepatopathy, insulin presence and high levels of glucose increase livers glycogen load. It is a reversible condition, with good glycemic control, and is not known to progress to fibrosis. It dephosphorylates and activates glycogen synthase, which is the enzyme required to transform glucose 1phosphate into glycogen. 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. Clinical, laboratory, and pathological information are described. Glycogenic hepatopathy is a rare and underecognized complication in longstanding poorly controlled type 1 diabetes mellitus patients. Hepatic biopsy showed a hepatic glycogen overload with fibrous frame. We report a patient who presented with predominant elevation in alkaline phosphatase and liver biopsy showing bridging fibrosis, which is an unusual presentation of glycogenic hepatopathy. Future studies warranted to further assess for development of fibrosis in gh as it can progress to cirrhosis.
Augmented reality technology for preoperative planning and. It is recognized that glycogenic hepatopathy can present without the complete features of mauriac syndrome, 3 as in our patient. 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. This entity should be distinct from other causes of hepatomegaly and elevated liver enzymes, in diabetic patients, such as nonalcoholic fatty liver disease. Liver biopsy from a patient with glycogenic hepatopathy shows diffuse enlargement of hepatocytes with pale, wispy cytoplasm and prominent cytoplasmic membranes. Case report glucocorticoidinduced rapid development of. Primary treatment of glycogenic hepatopathy is improved longterm blood glucose control. By the action of this enzyme, one glucose can combine with glycogen.
For a long time, this case had been misdiagnosed as nonalcoholic fatty liver disease nafld, which is more common in t2dm and which may at least in part lead to liver fibrosis. Diabetic hepatopathy simple steatosis or nonalcoholic steatohepatitis are liver diseases associated with diabetes mellitus dm. 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. Clinical presentations can include abdominal pain, tender hepatomegaly, nausea and elevated transaminases. 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. Pdf a case of glycogenic hepatopathy developed in a patient with. Jan 25, 2010 it is recognized that glycogenic hepatopathy can present without the complete features of mauriac syndrome, 3 as in our patient. Dogs with zone 3 degenerative hepatopathy concurrent with a psva usually respond poorly to surgical or ameroid shunt attenuation. It is characterized by the development of significant transaminitis and marked hepatomegaly. Canine vacuolar hepatopathy merck veterinary manual. Glycogenic hepatopathy is characterized by abnormal glycogen accumulation in hepatocytes. Table of contents 2020 case reports in medicine hindawi. Glycogen hepatopathy in a yearold male with type 1.
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 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. Glycogenic hepatopathy and mauriac syndrome kirstens. Glycogenic hepatopathy gh is a rare condition characterized by glycogen deposition in the liver in patients with poorlycontrolled type 1 dm. It is a rare complication of longstanding, poorly controlled type 1 diabetes mellitus.
A small subset of dogs may have copperassociated hepatopathy rhodanine staining and quantified tissue copper 1,200 ppm. Learn about the veterinary topic of canine vacuolar hepatopathy. The diagnosis requires liver biopsy to confirm characteristics pathological findings. Additional patient groups that can be affected by glycogenic hepatopathy include those with type ii diabetes mellitus, urea cycle defects, and drug effect. Dumping syndrome, a cause of acquired glycogenic hepatopathy. Patients are often asymptomatic but can present with abdominal pain, nausea and vomiting.
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