Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

I treated a patient who had amyloidosis with predominantly hepatic involvement and portal hypertension. The main clinical features were hepatomegaly, gross ascites, proteinuria, and elevated alkaline phosphatase levels. Despite permanganate-sensitive AA protein being present in the biopsy specimen, none of the recognized disease entities associated with secondary amyloidosis were found. A review of the literature and the mechanism of portal hypertension in amyloidosis is given. It is suggested that elevated portal pressures may be of greater importance in the pathogenesis of ascites in amyloidosis than has been appreciated.
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PMID:Hepatic amyloidosis. An unusual cause of ascites and portal hypertension. 649 30

Liver disease related to alpha-1-antitrypsin deficiency occurs only in Pi ZZ homozygous children. Eleven per cent of Pi ZZ infants present with prolonged neonatal cholestasis. In our group, 25 of 45 Pi ZZ infants with prolonged neonatal cholestasis presented with later cirrhosis. Persistence of jaundice beyond the sixth month of age, early development of splenomegaly, persistence of hard hepatomegaly and liver function abnormalities, and early portal fibrosis have a poor prognostic significance. The most severe course occurs in infants with an early histologic pattern of paucity of interlobular bile ducts. Portal hypertension was present in 19 of 25 children presenting with cirrhosis; 8 of 25 Pi ZZ children with cirrhosis died during childhood. Long-term protein-restricted diet and portal systemic shunts were helpful in treatment of four Pi ZZ children with cirrhosis; however, the long-term course in Pi ZZ children with cirrhosis is unpredictable.
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PMID:alpha-1-Antitrypsin deficiency. 660 4

The authors report 15 cases of nodular regenerative hyperplasia (NRH) of the liver observed in 10 women and 5 men during a 9 year period. Gastrointestinal bleeding due to ruptured esophageal varices revealed the liver disease in 11 cases. Hepatomegaly and splenomegaly were noted in 9 cases and ascites in 7. Anicteric cholestasis was demonstrated in 10 cases. Another disease, e. g. myelofibrosis and monoclonal gammapathy, was present in 11 patients. In 10 patients, portal diversion was performed; outcome being favorable with a follow-up of one to six years. The analysis of these cases and of the 113 previously published reports calls for the following comments: 1) In most cases, NRH is characterized by small-sized hepatocytic nodules scattered throughout the entire liver with no surrounding fibrosis; however this histological pattern may vary somewhat, with adjacent normal zones being found adjacent to typical cirrhotic fibrosis; although a precise morphometric study was not performed in our patients, obstruction of the tiny branches of intrahepatic portal veins was not observed. 2) Histological diagnosis of NRH is difficult and in most cases requires surgical biopsy specimens and specific coloration of the reticulin network. 3) NRH must be considered as a new cause of intrahepatic (sinusoidal or presinusoidal) portal hypertension and/or of chronic anicteric cholestasis. 4) A number of various conditions may be associated with NRH, the most frequent being Felty's syndrome and myeloproliferative disorders. 5) The pathogenesis of NRH remains unknown. 6) Portal diversion generally has a favorable outcome in this disease.
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PMID:[Nodular regenerative hyperplasia of the liver. Study of 15 cases and review of the literature]. 662 10

Hepatic vein thrombosis (Budd-Chiari Syndrome) is a rare disorder resulting from obstruction to the outflow of blood from the liver. The characteristic pathologic findings are intense congestion most pronounced around the terminal hepatic venules, cell necrosis, and a scant inflammatory reaction. Major clinical manifestations include hepatomegaly, right upper quadrant abdominal pain, and ascites. Disorders associated with hepatic vein thrombosis include those associated with a thrombotic tendency, such as polycythemia vera and paroxysmal nocturnal hemoglobinuria. Use of oral contraceptive agents probably increases tendency to develop hepatic vein thrombosis. Biochemical tests of the liver are of little value. The hepatic scan may be useful in suggesting the diagnosis with a marked decrease in uptake of isotope over affected areas of the liver. The diagnosis is confirmed by inferior vena caval and attempted hepatic venous catheterization. An associated thrombosis of the inferior vena cava is frequently found. Therapy in hepatic vein thrombosis is directed towards correction, in so far as possible, the underlying disorder, prevention of further extention of thrombosis, and management of ascites. Side-to-side portacaval or mesocaval shunt operation may convert the portal vein into an effective hepatic outflow vessel and reduce intrahepatic pressure and decrease injury. Mesoatrial shunts have proven useful in a few patients with hepatic vein thrombosis and either an inferior vena caval thrombosis or a marked pressure gradient from the abdomen to the thorax from ascites and an hypertrophied caudate lobe of the liver. For patients with extensive hepatocellular disease and portal hypertension, hepatic transplantation would seem to offer the best chance for survival.
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PMID:Hepatic vein thrombosis (Budd-Chiari syndrome). 669 73

Children who have abnormalities of the inferior vena cava (IVC) appear with diverse signs and symptoms. Those with congenital IVC malformations have symptoms related to associated cardiac disease. Those with IVC compression or obstruction may have ascites, hepatomegaly, edema of the lower extremities, and/or signs of portal hypertension. Sonography of the IVC has become a routine part of the evaluation of congenital heart disease and also part of the examination of patients with abdominal masses. Sonography should be the initial imaging modality in children at high risk for caval disorders. Previously used diagnostic procedures such as the inferior venacavogram should now be used only as a secondary investigative tool.
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PMID:Noninvasive evaluation of the inferior vena cava. The value of sonography. 670 74

Alcohol inhibits the secretion of protein from the liver. Chronic abuse results in intrahepatic accumulation of export-type proteins and decreased plasma levels. These effects appear to be mediated by acetaldehyde, an oxidation product of ethanol. Acetaldehyde is capable of interfering with the assembly of microtubules, a component of the cytoskeleton, the integrity of which is required for normal secretion. Protein retention and cytoskeletal alterations may contribute to manifestations of alcoholic liver disease, such as hepatomegaly, ballooning of the hepatocyte, portal hypertension, and development of Mallory bodies.
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PMID:Effects of alcohol on hepatic transport of proteins. 704 72

Hepatomegaly after chronic alcohol consumption results from an increase in cell size and not in cell number. About 50--60% of the increase in liver weight is accounted for by an increase in intracellular water, while extracellular water remains constant. Therefore, a substantial reduction in the ratio of extracellular to intracellular water occurs. Intracellular potassium can osmotically account for 40--50% of the excess water retained in the hepatocytes. It is proposed that an increase in hepatocyte size after chronic alcohol consumption compresses vascular-sinusoidal pathways. This results, after a threshold in cell size is exceeded, in increased intrahepatic and portal pressure. Possible factors responsible for the threshold are proposed. By applying the concept that animal cells act as osmometers, a new in vitro model has been developed to study the relationship between cell enlargement and portal pressure. In this model, the existence of a threshold and the generation of portal hypertension associated with hepatocyte enlargement have been demonstrated. In humans with alcoholic liver disease, a threshold in hepatocyte size enlargement (1600--1700 micrometer 2) before pressures were increased was also observed. In these patients, a strong correlation was also found between hepatocyte size and intrahepatic pressure. The same correlation occurs regardless of the presence or absence of cirrhosis, therefore suggesting that a major determinant of portal hypertension in cirrhosis is cell size and not the existence of nodules of fibrous septa. The higher portal pressures found in cirrhotics may be explained by the fact that these patients have larger hepatocytes.
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PMID:Alcohol-induced hepatomegaly: pathogenesis and role in the production of portal hypertension. 708 88

Twenty-five patients with enzymatically confirmed Gaucher disease were selected for study of hepatic complications of the disorder. Hepatomegaly was noted clinically and confirmed by radioisotopic scan in all but 1 patient. The majority had abnormalities of serum transaminase, alkaline phosphatase, and sulfobromophthalein (BSP) clearance. Three patients had evidence of portal hypertension. Light microscopic examination of liver showed some variation in the pattern of the disease. Gaucher cells were present in all specimens. In 5 patients the distribution was in scattered foci throughout the liver lobule. In 14 patients there was prominent centrilobular accumulation of Gaucher cells. Extensive replacement of liver by storage cells and cirrhosis was documented in 3 patients. No patient was found to have amyloid deposits. The severity of hepatic involvement correlated with the occurrence of other severe complications of the disorder. The wide range of liver abnormalities in Gaucher disease should be considered in evaluating patients for participation in experimental therapeutic trials.
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PMID:Gaucher disease: hepatic abnormalities in 25 patients. 712 31

Hepato-splenic scintigraphy with 99mTc-S-colloid was performed in twelve patients with bacterial endocarditis. These images showed that the size of the liver varied from normal to a severe hepatomegaly, depending on the presence of congestive heart failure. Intrahepatic distribution of the radiocolloid was slighty irregular in all cases. The spleen was conspicuously enlarged, and showed irregular distribution of the radiopharmaceutical. In some cases intrasplenic concentration defects caused by infarcts, abscesses or cysts, were observed. The relative uptake of radiocolloid by the spleen, varied from hypo- to hyperconcentration according to the degree of lymphoid hyperplasia caused by the infection. In two cases, both with congestive heart failure, concentration of the radiocolloid was evident in the bone marrow. The scintigraphic pattern observed in these patients with bacterial endocarditis can be easily differentiated from that caused by only congestive heart failure, which is similar to the observed in patients with cirrhosis of the liver and/or portal hypertension.
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PMID:[Hepato-splenic scintigraphy in finding indications of bacterial endocarditis. Preliminary report]. 719 44

A 27-year-old patient, originally from Martinique, presented with a progressive hepatic granulomatosis with hepatomegaly, splenomegaly, and non-icteric cholestasis, associated with bronchial granulomatosis lesions. The sarcoidosis regressed rapidly after high doses (60 mg/day) of prednisone. Portal hypertension developed later and provoked a severe hematemesis from rupture of esophageal varices. Signs of pulmonary arterial hypertension were then observed, and the diagnosis confirmed by pressure tests after catheterization, and angiography. A portocaval shunt caused the esophageal varices to subside, but the pulmonary arterial hypertension, resistant to corticotherapy, was rapidly fatal. In the case reported, the pulmonary arterial hypertension, independent of any parenchymatous lesion, was attributed to fibrosis of the arterial walls. The association of portal and pulmonary arterial hypertension with sarcoidosis is a very rare occurrence, and the pathogenesis of this association remains a debatable subject.
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PMID:[Sarcoidosis and portal and pulmonary arterial hypertension: a case report (author's transl)]. 722 52


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