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

Five cases of acute fatty liver of pregnancy are described. These are the only recognized cases of this disorder occurring in a 2 year period in Western Australia. Clinical and laboratory features are presented. There was no maternal death. Of the six babies, there were three intrauterine deaths, including the only set of twins. All the babies were male. Vomiting in the third trimester was the chief presenting feature in all cases, often accompanied by a systemic illness with malaise and tiredness. Extreme polydipsia was noted as a prominent symptom in all cases. The combination of moderately abnormal liver function tests, extreme leucocytosis with other blood film abnormalities, hypoglycaemia, impaired renal function, coagulopathy and gross elevation of uric acid level is regarded as highly suggestive of the diagnosis. Features of a preeclamptic illness were present in several cases. Three of the patients have since had uneventful pregnancies. The constellation of clinical and laboratory features is sufficiently characteristic to allow accurate clinical diagnosis in most cases of this disorder. The chances of both maternal and fetal survival are enhanced by early diagnosis allowing intervention in the form of prompt delivery of the infant.
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PMID:Acute fatty liver of pregnancy: clinical features and diagnosis. 321 85

The metabolic effects of ethanol are due to a direct action of ethanol or its metabolites, changes in the redox state occurring during its metabolism, and modifications of the effects of ethanol by several nutritional factors. Ethanol causes hyperglycemia or hypoglycemia depending whether or not glycogen stores are adequate, inhibits protein synthesis, and results in a fatty liver and elevations in serum triglyceride levels. Increases in serum lactate, results from the increased reduced nicotinamide-adenine dinucleotide/nicotinamide-adenine dinucleotide + (NADH/NAD+) ratio, and hyperuricemia probably occurs owing to the increased turnover of adenine nucleotides after ethanol ingestion. Ethanol decreases thiamine absorption and decreases the enterohepatic circulation of folate. Acetaldehyde, the major metabolite of ethanol, increases the degradation of pyridoxal 5'-phosphate by displacing it from its binding protein and making it susceptible to hydrolysis by membrane-bound alkaline phosphatase. Chronic ethanol administration also results in decreased vitamin A stores and reduced bone mass and blood levels of 25-hydroxyvitamin D. The mechanism whereby ethanol affects these vitamins and their associated enzymes is unknown.
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PMID:The effect of ethanol and its metabolites on carbohydrate, protein, and lipid metabolism. 329 39

There are now nine inherited diseases that have been identified in the pathway of mitochondrial fatty acid oxidation, including LCAD, MCAD, SCAD, and HMG-CoA lyase deficiencies, two forms each of CPT and MAD deficiencies and an incompletely characterized disorder of primary carnitine deficiency. The varied range of clinical manifestations in this new group of diseases should attract the attention not only of general pediatricians (coma, hypoglycemia) but also of pediatric subspecialists in neurology (myopathy), cardiology (cardiomyopathy), and gastroenterology (fatty liver), as well as genetics and metabolism. The presenting features of the genetic defects in fatty acid oxidation fit well with the concept that fatty acid oxidation plays a major role in energy production during prolonged fasting and in working cardiac and skeletal muscle. Life-threatening episodes of coma and hypoglycemia induced by fasting are a common presenting feature in most of the fatty acid oxidation disorders (MCAD, LCAD, and HMG-CoA lyase deficiencies, the infantile form of CPT deficiency, the mild form of MAD deficiency, and in some cases of primary carnitine deficiency). The hypoglycemia in these disorders is most easily explained by the inability of affected patients to use fatty acids as a fuel as a substitute for glucose. It should be stressed, however, that the coma in these disorders may occur from direct toxic effects of fatty acids or fatty acid intermediates before plasma glucose concentrations reach hypoglycemic levels. Severe disturbances of muscle function are a feature in several of the disorders; hypertrophic cardiomyopathy and chronic skeletal muscle weakness occur in both the mild and severe forms of MAD deficiency, in primary carnitine deficiency, and in some patients with LCAD deficiency. In contrast, patients with the adult form of CPT deficiency have normal muscle strength but are prone to episodes of painful rhabdomyolysis induced by prolonged exercise. These manifestations presumably reflect the requirement of working cardiac and skeletal muscle for energy supplied from fatty acid oxidation. In two of the disorders, SCAD deficiency and the severe form of MAD deficiency, chronic CNS toxicity is a dominant feature. The severe effects on the brain in these two disorders may reflect the fact that short-chain fatty acids more readily cross the blood-brain barrier than longer-chain fatty acids.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:New genetic defects in mitochondrial fatty acid oxidation and carnitine deficiency. 331 4

The cause of death (besides dehydration) for 140 diarrhoeal patients who died in hospital following rehydration was determined by autopsy examination. Children under 5 years comprised 74% of the patients. Diarrhoeal pathogens were identified as Shigella spp. in 27%, enterotoxigenic Escherichia coli in 17%, Entamoeba histolytica in 16%, Campylobacter jejuni in 12%, Salmonella spp. in 4%, Vibrio cholerae in 4%, and Giardia lambliain 4% of cases. The most frequent underlying causes of death were colitis in 44% and pneumonia in 38%. The most frequent immediate causes of death were septicaemia in 27%, hypoglycaemia in 9%, and hypokalaemia in 9%; multiple causes of death were present in 89% of cases. Kwashiorkor or marasmus was present in 59% and fatty degeneration of the liver was detected in 61% of cases. It is concluded that, in susceptible children, diarrhoeal pathogens produce destructive inflammation in the intestine and cause death or contribute to it by provoking disease in other tissues, especially septicaemia and fatty liver, or by combining these effects with antecedent or concomitant conditions, especially pneumonia and malnutrition.
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PMID:Causes of death in diarrhoeal diseases after rehydration therapy: an autopsy study of 140 patients in Bangladesh. 349 47

Treatment of rats with mirex (40 ppm in diet) caused hypoglycemia, liver enlargement, and inhibition of adrenal corticosteroid-synthesizing enzyme activity. At toxic dosages (20,000 ppm mirex in diet, which has a lethal toxicity-50 [LT-50] of ten days) poisoned female rats showed severe hypoglycemia, fatty liver, adrenal hyperplasia, hypophagia, lipid mobilization, and body weight (bw) loss. A 50 micrograms/kg intraperitoneal (IP) dose of 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD) in male rats caused similar effects two days posttreatment. Hypoglycemia could be overcome by prednisone (which also inhibited adrenocorticoid-synthesizing enzyme activities) but not by streptozotocin treatment, indicating that hypoglycemia may be related to glucocorticoid deficiency resulting from inhibition of their synthesis and not by direct effects on pancreatic beta-cells. Glucocorticoid deficiency could also cause increased release of adrenocorticoid hormone (ACTH), which may enhance fat mobilization caused by hypophagia.
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PMID:Significance of mirex-caused hypoglycemia and hyperlipidemia in rats. 350 72

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

Acute fatty liver is a rare complication of pregnancy occurring in approximately one in 13,000 deliveries. This disease has been associated with high maternal and fetal mortality rates in the past. Advances in medical care and early recognition and delivery are credited with recent reductions in the maternal and fetal loss seen with this disease. Maternal death is associated with hypoglycemia, hepatic failure, or coagulopathy. The etiology of fetal mortality is not well delineated. Reported is a patient with histologically proved acute fatty liver who developed fetal distress during therapy for coagulopathy. Review of the fetal monitor tracing in conjunction with examination of the placenta have led us to propose that uteroplacental insufficiency may be the cause for fetal distress and fetal wastage in fatty liver of pregnancy.
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PMID:Acute fatty liver of pregnancy: etiology of fetal distress and fetal wastage. 380 31

The metabolic effects of ethanol are due to a direct action of ethanol or its metabolites, changes in the redox state occurring during its metabolism, and modifications of the effects of ethanol by nutritional factors. Ethanol causes hyperglycemia or hypoglycemia depending on whether glycogen stores are adequate, inhibits protein synthesis, and results in fatty liver and in elevations in serum triglyceride levels. Increases in high-density lipoprotein cholesterol after ethanol ingestion may explain the lower risk of myocardial infarction and death from coronary disease after moderate drinking. Increases in serum lactate, resulting from the increased NADH/NAD+ ratio, and hyperuricemia, most likely the result of increased turnover of adenine nucleotides, are common transient effects of ethanol ingestion. Causes of vitamin deficiencies in alcoholism are decreased dietary intake, decreased intestinal absorption, and alterations in vitamin metabolism. Ethanol decreases thiamine absorption and decreases the enterohepatic circulation of folate. Acetaldehyde increases the degradation of pyridoxal 5'-phosphate by displacing it from its binding protein and making it susceptible to hydrolysis by membrane-bound alkaline phosphatase. Ethanol decreases hepatic vitamin A concentration and its conversion to active retinal, and modifies renal metabolism of vitamin D.
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PMID:Metabolic effects of alcohol. 388 Dec 85

A maternal survivor of idiopathic fatty liver, with the characteristic histological appearance on liver biopsy, is reported. Profound hypoglycaemia was the major complication in this patient and it is suggested that more attention should be given to the blood glucose in this disease. The consideration of future pregnancy in this patient is discussed.
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PMID:Idiopathic acute fatty liver of pregnancy. 548 32

Alcoholism is associated with increased mortality from violent and nonviolent causes. The increase in nonviolent deaths is usually ascribed, at least in part, to "cirrhosis." In the majority of these deaths this implies fatty liver rather than true Laennec's cirrhosis. Studies of sudden nonviolent deaths illustrate the largely unrecognized and frequent occurrence of sudden death with autopsy findings limited solely to fatty liver. The mechanism(s) of these sudden fatty liver deaths is unknown. Several attractive theories attribute such deaths to ethanol withdrawal induced hypoglycemia or hypomagnesemia, pulmonary fat embolization from fatty liver, or other facets of the alcohol withdrawal syndrome, including ethanol dependent maladaptive derangements of neurotransmitters. All the theories of fatty liver death remain essentially untested, however, owing to uncontrolled postmortem conditions and the lack of awareness of fatty liver deaths within the scientific community.
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PMID:Fatty liver and sudden death. A review. 610 25


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