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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This report deals with a case of double gammopathy (IgM-kappa, IgG-lambda) with Crow-Fukase syndrome, which developed into primary macroglobulinemia four years after the diagnosis. In May 1980, a 74-year-old woman was admitted to the hospital because of a rapid progression of peripheral neuropathy. The patient was diagnosed as having Crow-Fukase syndrome from the following data: albumin-cytologic dissociation of cerebrospinal fluid, peripheral edema, diffuse hyperpigmentation of the skin, diabetic glucose intolerance, serum double gammopathy (IgM-kappa, IgG-lambda) and hepatomegaly. The administration of prednisolone yielded the improvement of neuropathy. In December 1984, serum IgM level was increased from 104 mg/dl to 3,025 mg/dl. Plasma cells in the bone marrow increased in the percentage from 5.6% to 18.4%, and then Bence Jones protein (kappa type) was excreted in the urine. No antibody activity to myelin antigens was detected in the serum. The patient died of cerebral infarction in 1985. At postmortem examination, lymphomatous involvement was found in the jejunum. At the immunohistological examination of the tumor specimens, the morphology and the distribution of IgM- and IgG-positive cells corresponded to that of kappa- and lambda-positive cells, respectively. A small number of cells containing both kappa and lambda light chains were also demonstrated. It seems likely that IgM (kappa)- and IgG (lambda)-positive cells were derived from the common precursor cells.
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PMID:[Progression from Crow-Fukase syndrome with double gammopathy (IgM-kappa, IgG-lambda) to primary macroglobulinemia]. 194 31

Histiocytosis X rarely disseminates in an adult. The authors describe an unusual patients who presented with multiple areas of cutaneous and bone involvement. During the course of his disease he developed massive hepatomegaly. Aggregates of vacuolated histiocytes were found on liver biopsy. He subsequently developed diabetes mellitus complicated by ketoacidosis. Both his hepatomegaly and diabetes resolved spontaneously. No pancreatic nor pituitary abnormalities were identified. The combination of histiocytosis X, hepatomegaly, and diabetes mellitus has not been previously reported. The medical literature is reviewed with an emphasis on disseminated histiocytosis X in adults and the mechanism of glucose intolerance in liver disease.
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PMID:Reversible hepatomegaly and diabetes mellitus in an adult with disseminated histiocytosis X. 218 Feb 96

Abdominal ultrasonography was performed on 35 adult patients with proven cystic fibrosis. Thirty-three patients showed pancreatic abnormalities. The most marked ultrasonographic features were increased parenchymal echogenicity, atrophy, non-visualisation of the duct and cyst formation. There was no correlation between these features and the severity of the pulmonary disease, the age of the patient, weight or glucose intolerance. Abnormalities of the biliary tract were demonstrated in nine (26%) patients and were associated with poor nutritional status. Multivariate analysis revealed a significant association between the following: hepatomegaly, increased liver echogenicity, splenomegaly, biliary disease; secondly, between lung function and serum albumin. Ultrasonography is useful in showing organ morphology but not in assessing disease severity.
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PMID:Cystic fibrosis: ultrasonographic findings in the pancreas and hepatobiliary system correlated with clinical data and pathology. 390 96

A patient who abstained from alcohol consumption but who had asymptomatic chronic progressive hepatomegaly, mild disturbance of liver function tests and hepatitis resembling alcoholic hepatitis (nonalcoholic steatohepatitis) developed glucose intolerance several years after the hepatitis was diagnosed. The patient had a family history of both diabetes and chronic liver disease. A lesion resembling alcoholic hepatitis in a patient who denies alcohol consumption, may be diabetic or pre-diabetic in aetiology and such a patient should be followed up with glucose tolerance tests.
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PMID:Diabetic hepatitis preceding the onset of glucose intolerance. 398 46

The features of 41 proven or suspected cases of pancreatic glucagonoma and one possible case of renal glucagonoma have been reviewed. Glucagonoma is one form of islet cell neoplasm and involves pancreatic alpha cells. It may occur more frequently in women and is more likely to be malignant than insulinoma. Patients may present with glucose intolerance, an erythematous, eczematous dermatitis, glossitis, stomatitis, vaginitis and unexplained weight loss. Anemia, hypoproteinemia, hypoaminoacidemia and hypolipidemia may also be present. Malignant glucagonoma metastasizes frequently to liver. An evaluation for possible glucagonoma may be considered in a patient with the characteristic eczematous dermatitis, glossitis or stomatitis and glucose intolerance, an unusual or atypical history of diabetes mellitus, or hepatomegaly with other characteristics of glucagonoma. Initial evaluation may include measurement of fasting plasma glucagon concentration, and an oral glucose tolerance test with measurements of plasma glucose and glucagon levels. Extreme fasting hyperglucagonemia, and a paradoxical rise in plasma glucagon concentrations after glucose ingestion should strongly suggest the presence of glucagonoma. Radiographic demonstration of pancreatic glucagonoma is best carried out by celiac arteriography. Surgical excision of the tumor is the treatment of choice. Nonresectable lesions may respond to chemotherapy with streptozotocin. Treatment for the various dermatologic or metabolic complications of glucagonoma which include glucose intolerance, hypoproteinemia, hypocholesterolemia and anemia may not be satisfactory. Glucose intolerance is usually mild and may be adequately treated with dietary or insulin therapy. Rarely, glucagonoma with massive destruction of the pancreas or other factors may induce severe glucose intolerance. In contrast, the anemia, skin rash, and hypoproteinemia do not respond to conservative therapies tested thus far. Glucagonoma is a model for studying the importance of glucagon in causing the hyperglycemia of diabetes mellitus. Study of patients with glucagonoma does suggest that glucagon has some role in the etiology of hyperglycemia in diabetic states; however, as in studies on diabetes, investigations on glucagonoma do not demonstrate that glucagon has a primary role in producing severe glucose intolerance.
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PMID:Clinical and metabolic aspects of glucagonoma. 698 81

Various metabolic studies were performed in a patient with the idiopathic Fanconi syndrome in whom constant ketonuria suggested that organic acidemia might contribute to the metabolic acidosis. Glucose intolerance with a diminished insulin release was found after PO or IV glucose loads and after glucagon administratio. An insulinopenic "diabetes-like" state has not previously been described in such patients. The patient had impaired galactose-glucose interconversion, elevated blood lactate levels, reduced pyruvate levels, and an increased lactate:pyruvate ratio. Hepatomegaly and hypoglycemia were not present, and liver and muscle biopsies revealed no enzymatic evidence of glycogenosis. The erythrocyte UDP galactose transferase activity was normal. The patient failed to convert fructose to glucose and had a rise in blood lactate after ethanol administration. Further studies revealed no production of glucose after alanine or glycerol administraion, each test being associated with elevated blood lactate levels and, after alanine, an increased lactate:pyruvate ratio. The lactate:pyruvate ratio was elevated after glucagon administration with increased lactate and reduced pyruvate concentrations.
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PMID:Abnormalities of carbohydrate metabolism in idiopathic Fanconi syndrome. 738 41

Primary hemochromatosis is characterized by a specific pattern of clinical manifestations. It includes liver disease with hepatomegaly, glucose intolerance, e.g. diabetes, hyperpigmentation oft the skin, impotence/ amenorrhea, arthropathy, cardiomyopathy and fatigue. Laboratory investigation reveals significantly elevated serum ferritin and transferrin saturation with iron. The diagnosis is confirmed by liver biopsy and quantitative determination of elevated liver iron content. Wilson's disease represents a copper storage disease. Prominent clinical features are hepatomegaly and splenomegaly. Neurological alterations and detection of Kayser-Fleischer corneal rings are typical. In the acute initial phase the often young patients present with Coombs-negative hemolysis. Psychiatric alterations, cardiomyopathy, arthropathy, nephropathy, as well as thrombocytopenia and leucopenia are other clinical features. Laboratory parameters of Wilson's disease include low serum ceruloplasmin and serum copper. There is an elevated urinary copper excretion and elevated serum free copper concentration. The diagnosis is confirmed by liver biopsy with quantitative determination of an elevated liver copper content.
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PMID:[Current diagnosis: hereditary metabolic diseases of the liver (primary hemochromatosis, Wilson disease)]. 898 78

We report a case of POEMS syndrome with various endocrine dysfunctions. A 49-year-old man was admitted to our hospital for pretibial edema and general fatigue. He had weakness of the lower extremities, hepatomegaly, monoclonal protein (IgG-lambda type), impotence, pigmentation and hypertrichosis. Endocrinological examination revealed impaired glucose tolerance, primary hypothyroidism and hypogonadotropic hypogonadism. After three months of treatment with corticosteroids, he showed an improvement in gonadotropin secretion, but no considerable change in the secretion of the other hormones. To our knowledge, this is the first case that showed an improvement in gonadotropin secretion as a result of corticosteroid therapy in POEMS syndrome.
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PMID:Improvement in gonadotropin secretion after corticosteroid therapy in a case of POEMS syndrome. 979 Feb 78

Insulin resistant metabolic syndrome is a major clinical disorder including hyperlipidaemia, hypertension, impaired glucose tolerance and/or type 2 diabetes and central obesity, which are well established cardiovascular risk factors. We report the case of a 61-year-old woman who developed severe hypercholesterolaemia and hypertriglyceridaemia after liver transplantation. In her forties she had hypertension, mixed hyperlipidaemia, mild hyperglycaemia and moderate abdominal obesity, suggesting the presence of the metabolic syndrome. She had liver enzyme elevation and severe steatosis and hepatomegaly at ultrasonography. At age 52, cryptogenic liver cirrhosis was diagnosed and rapidly progressing liver failure developed. In 1992 she underwent liver transplantation. Seven years after transplant the patient had abdominal obesity, high blood pressure, marked hypercholesterolaemia, hypertriglyceridaemia and moderate elevation of alanine aminotransferase. She also had impaired glucose tolerance and markedly increased basal and post-glucose load plasma insulin levels. Steatohepatitis was demonstrated by serial liver biopsies. This is the first case that reports the recurrence of the metabolic syndrome following liver transplantation. We postulate that metabolic syndrome may have promoted fatty liver and subsequent progression to end stage liver disease. We also stress the need for careful management of the metabolic syndrome in order to decrease the long-term risk for cardiovascular disease.
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PMID:Recurrence of insulin resistant metabolic syndrome following liver transplantation. 1254 3

Liver diseases are frequently associated with disorders of the carbohydrate metabolism--impaired glucose tolerance, hyperinsulinaemia, insulin resistance. Impaired glucose tolerance is due in particular to impaired glucose uptake in the splanchnic area and periphery. Glucose production by the liver is normal, similarly as insulin secretion. Its reduced clearance leads to hyperinsulinaemia with subsequent down regulation of insulin receptors and the development of insulin resistance. In diabetic subjects hepatomegaly is frequent, most frequently associated with steatosis of the liver. It correlates with the degree of obesity rather than the type of hepatic lesion. It is reversible for a long time and develops into cirrhosis only when combined with other factors, in particular alcoholism or infection with hepatotropic viruses. Cirrhosis of the liver is also more frequent in diabetic subjects. Treatment with antidiabetics is discussed, attention is drawn to new types of biguanides, which may have some advantages. Cholelithiasis is in diabetic subjects three times more frequent and leads more frequently to serious, in particular inflamatory, complications with an adverse course. Therefore cholecystectomy should be contemplated. According to contemporary views it seems that diabetic hepoatopathy proper does not exist. The authors draws practical conclusions for the everyday work of physicians.
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PMID:[Diabetes mellitus and the liver]. 1563 90


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