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Query: UMLS:C0015695 (fatty liver)
 13,941 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Alcoholic liver disease presents a wide spectrum of clinical manifestations ranging from mild asymptomatic fatty liver to alcoholic hepatitis and severe life-threatening liver failure with ascites, hemorrhaging esophageal varices, and encephalopathy. Although still poorly understood, the mechanism of this injury is probably the result of numerous direct toxic and metabolic effects of alcohol on the hepatocyte. Therapy consists primarily of abstinence and supportive care. However, several newer treatments are actively being studied. These include prednisolone, anabolic steroids, glucagon and insulin, propylthiouracil, and cyanidanol. Colchicine is promising as an agent to inhibit fibrosis. Complications of cirrhosis, including ascites and variceal hemorrhage, are the result of end stage disease. A return to old techniques of ascitic fluid management suggests that therapeutic large-volume paracentesis with albumin infusion is a safe and effective form of therapy. Variceal hemorrhage is best treated with sclerotherapy, vasoconstrictors, and balloon tamponade. Little has been done to alter the ultimately dismal prognosis and long-term survival of alcoholic liver disease.
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PMID:Alcoholic liver disease. 222 93

We treated nine infants who unexpectedly developed shock, seizures, and fever, followed by diarrhea, consumption coagulopathy, and hepatorenal dysfunction. Despite vigorous treatment, three infants died and all except one of the six survivors have severe residual neurologic abnormalities. Postmortem findings included cerebral edema, white matter petechial hemorrhages, gastrointestinal hemorrhages, and fatty liver. These clinicopathologic features are similar to those previously described in 10 infants as being due to hemorrhagic shock and encephalopathy, except for the presence of fatty liver in our patients. Based on the combined experience of 19 infants, we propose diagnostic criteria for hemorrhagic shock and encephalopathy that may facilitate recognition and differentiation from other shock syndromes in infancy.
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PMID:Hemorrhagic shock and encephalopathy. Clinical definition of a catastrophic syndrome in infants. 240 86

A previously healthy 35-year-old woman was seen at 37 weeks' gestation with a 10-day history of fever, vomiting, diarrhea and malaise. Serum laboratory findings included elevation of serum bilirubin and AST, prolongation of serum prothrombin time and a positive monospot. A tentative diagnosis of acute fatty liver of pregnancy was made, and a healthy male infant was delivered by emergency cesarean section because of fetal distress. Over the subsequent 3 days, acute progressive oliguric renal failure, disseminated intravascular coagulation, hypoglycemia requiring intravenous dextrose infusion and pancreatitis developed; her mental status progressed to stage III encephalopathy. Quantitative computed tomography estimated the liver volume to be 770 cm3. The decision to proceed with orthotopic liver transplantation was made on the basis of progressive clinical deterioration despite aggressive support and because of her small liver size. After transplant, the patient's multisystem failure rapidly reversed. Histopathological examination of the native liver demonstrated predominantly zone 3 microvesicular steatosis with characteristic ultrastructural changes consistent with acute fatty liver of pregnancy. Southern blot analysis for Epstein-Barr virus DNA was negative. We conclude that orthotopic liver transplantation should be considered for the small group of patients with fulminant hepatic failure associated with acute fatty liver of pregnancy who manifest signs of irreversible liver failure despite delivery of the fetus and aggresive supportive care.
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PMID:Fulminant hepatic failure caused by acute fatty liver of pregnancy treated by orthotopic liver transplantation. 240 63

In a group of 1,025 cases of alcoholic liver the authors detected 28 cases (2.73%) of acute alcoholic hepatitis on the basis of the morphologic examination, 75% were males, mean age 45 +/- 18 years. The mean amount of alcohol consumed daily was 106.7 g for a mean duration of 19.78 years. The hepatic lesional substrate was hepatic steatosis and cirrhosis in 7 cases (25%) and steatofibrosis in the other 14 cases (50%). The triggering factors were the abuse of alcohol in 27 cases and a pneumonia in the last case. The disease was characterized by fever, encephalopathy, hepatomegaly, hepatocytolytic-cholestatic syndrome. The evolution was dictated by the gravity of the background hepatic lesional substrate.
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PMID:[Acute alcoholic hepatitis]. 257 74

The liver in an infant or child is as liable to the same pathologies afflicting the adult liver but with certain differences in prevalence and causes. Genetic disorders are more likely to present in the paediatric age group where many involve metabolic processes such as galactosemia, phenylketonuria, glycogen storage disease and others. Many of these present in the newborn period. However, neoplasms and hamartomas also present in the newborn period, such as congenital neuroblastoma with an enormously enlarged liver, hepatoblastoma and haemangioma. The latter may present with intractable cardiac failure as a result of considerable shunting of blood. Acquired liver lesions often present in the newborn period or early infancy and this includes hepatitis and biliary atresia. The difficulties in the differentiation of the two lesions will be discussed together with the management of biliary atresia. As the child grows older, Reyes encephalopathy with microvesicular fat in the liver is not uncommon. The pathophysiology of Reyes encephalopathy as seen locally will be described. The choledochal cyst with direct (Caroli's disease) or indirect effect on the liver will be described. Problems of childhood portal hypertension as well as congenital hepatic fibrosis will be described. Hemosiderosis of the liver is chiefly seen in homozygous beta-thalassaemia patients who have been kept alive with repeated blood transfusions. Amoebic and pyogenic hepatitis, fatty liver due to protein malnutrition, biliary ascariasis, etc, which are common in tropical and subtropical countries are rarely seen now in Singapore children.
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PMID:Paediatric liver disorders in Singapore. 346 38

A boy suffering from recurrent episodes of acute encephalopathy and hepatic steatosis died at 40 months of age. The symptoms started when he was 13 months old and he appeared completely normal in the intervals. Pertinent biologic findings were as follows: slight labile hypoglycemia and hyperammonemia having no direct correlation with neurologic derangement, no elevation of ammonia levels in loading tests, complete failure to generate ketones and the absence of organic aciduria during a fast, normal plasma carnitine levels and normal activity of long and medium chain acyl-CoA-dehydrogenase in skin fibroblasts. Pertinent autopsy findings were marked steatosis of liver and renal tubular cells with many foamy histiocytes in bone marrow. An error in metabolic pathways, particularly a derangement in lipid metabolism, was considered.
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PMID:[Acute encephalopathy and recurrent hepatic steatosis with normal long and medium chain fatty acyl-CoA-dehydrogenase activity]. 361 69

Reye's syndrome (RS) is generally considered a childhood disease. We report our experience with RS in adults in the metropolitan Milwaukee area. Reye's syndrome was diagnosed in seven 18- to 46-year-old adults. The diagnostic criteria were as follows: viral prodrome followed by vomiting and encephalopathy without focal neurological signs, normal cerebrospinal fluid values, increased levels of serum aminotransferases (transaminase), prolonged prothrombin time, elevated blood ammonia levels, and characteristic microvesicular fatty liver and mitochondrial changes. None of the patients was hypoglycemic. The diagnosis of RS was entertained in 22 but confirmed in only seven patients. In cases of non-Reye's encephalopathy, drug ingestion presented as one of the most difficult differential diagnostic problems, which also included alcohol abuse, collagen vascular disease, and hepatitis B surface antigenemia. Clinical jaundice, distinctly uncommon in RS, was present in only one patient who presented to us in stage V coma. In adults, RS is more difficult to diagnose and should be suspected more frequently in patients with unexplained altered behavior following a viral illness and vomiting. Liver biopsy can be performed safely and is usually mandatory in adults. Patients with RS diagnosed during stage I or II coma and treated experienced an uneventful recovery.
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PMID:Reye's syndrome in adults. Diagnostic considerations. 380 May 31

A case is reported of acute fatty liver of pregnancy with maternal and foetal survival inspite of jaundice, encephalopathy, renal failure, gastro-intestinal haemorrhage and serious coagulation problems. The acute fatty liver is a rare disease of unknown aetiology which occurs during the third trimester of pregnancy. Early diagnosis based on liver biopsy obtained by the transjugular route, immediate delivery of the foetus and intensive care improve an otherwise fatal prognosis.
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PMID:[Acute fatty liver of pregnancy]. 381 47

To evaluate the alterations of plasma catecholamines in chronic and acute liver diseases and their complications: hepatic encephalopathy (grade 1-4), ascites, deranged metabolism, and circulatory alterations, we measured the concentrations of norepinephrine, epinephrine, and dopamine in plasma in 49 patients with cirrhosis of the liver, in 2 patients with fulminant hepatitis B, in 2 patients with acute gestational fatty liver, and in 11 patients with fatty liver. We examined 21 healthy controls. The norepinephrine concentrations in patients with cirrhosis were raised and reached the highest values in hepatic coma grade 4. As well patients with fulminant hepatitis B had excessive high norepinephrine concentrations. The epinephrine concentrations were not significantly raised in patients with toxic cirrhosis and in patients with posthepatitic cirrhosis without encephalopathy. In hepatic coma grade 4 in patients with cirrhosis and fulminant hepatitis they reached again the highest values. Patients with acute gestational fatty liver had only slightly increased, and patients with fatty liver had normal catecholamine concentrations in plasma.
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PMID:[Plasma catecholamine levels in liver disease]. 406 Aug 3

A 4-year-old girl with juvenile rheumatoid arthritis developed fever, protracted vomiting, disturbance of consciousness and decorticate posture following the administration of salicylate. There were elevated levels of transaminases in serum, hyperammonemia and a fatty liver. However, the fatty droplets were different electronmicroscopically from that of Reye's syndrome. This observation emphasizes the importance of electronmicroscopic observation of the liver in the differential diagnosis between Reye's syndrome and aspirin-induced encephalopathy, because the clinico-pathological findings of intoxication are so similar.
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PMID:Simulated Reye's syndrome and salicylate therapy. 646 45


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