Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0015695 (fatty liver)
 13,941 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Three groups of freshly calved dairy cows suffering from fatty liver syndrome of different stages were examined for serum cholic acid (SCA) levels to assess the value of SCA-determination as a diagnostic approach to fatty liver syndrome. A. Comparison of the SCS levels between cows with and without ketonuria: Cows with ketonuria (n = 13) and with elevated plasma ASAT activity, indicating a moderate liver damage, showed a rise of their mean SCA level to 35.3 (+/- 14.9) mumol/l. Freshly calved cows without ketonuria (n = 10) had at the same time a mean SCA level of 17.9 (+/- 6.4) mumol/l, falling practically into the physiological range. The SCA level increased above the physiological range in 10 of the 13 cases with ketonuria, in seven of them even more than twice. B. Interrelationship between SCA level and hepatic lipid content: Increase in hepatic total lipid (TL) was always associated with SCA-elevation. The mean SCA level was 55 (+/- 22.0) mumol/l in cases of severe fatty liver (TL = 200-280 g/kg), and 39.5 (+/- 6.0) mumol/l in the moderate form (TL = 180-200 g/kg) of the syndrome. C. Peripartal SCA levels of cows with fatty liver: Clinically healthy cows (n = 6) with ketonuria and an elevation of serum ASAT and FFA concentrations had a mean SCA level of 35-45 mumol/l. One cow of this group, which developed acute fatty liver syndrome and died within the period of study, showed an extreme SCA level of 100 mumol/l in it's terminal stage.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Cholic acid levels in the serum of dairy cows with metabolically-caused liver diseases]. 204 69

The overall rate of inactive ovaries diagnosed by two consecutive rectal examinations was 8.5 per cent for 7751 lactations. Inactive ovaries had an inverse association with increasing parity and were directly associated with twinning, retained placenta, primary metritis and high milk yield after calving in heifers. No independent associations were established with stillbirth, a low milk yield in the last 120 days before calving, a long dry period, ketonuria or a high serum glutamate oxaloacetate transaminase activity in the first week after calving. The presence of inactive ovaries in the previous lactation was a significant predictor of the trait. Cows with either metritis or inactive ovaries were at a greater independent risk of not conceiving within 150 days after calving and the combined risk associated with both factors was higher than their sum. It is concluded that while damage to the uterus repeatability and an energy shortage after calving are responsible for ovarian inactivity, this trait was not associated with fatty liver. Such an association could be due to an indirect causal relationship, the outcome of post parturient uterine diseases which are associated independently with overfeeding before calving and inactive ovaries.
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PMID:Inactive ovaries in high-yielding dairy cows before service: aetiology and effect on conception. 366 May 46

The effects of late pregnancy on metabolic fuels, liver composition, gluconeogenesis, and nitrogen metabolism have been examined in fed and fasted rats. Plasma free fatty acid (FFA) and immunoreactive insulin (IRI) are greater and glucose and ketones are lower in fed 19-day pregnant than they are in agematched virgin rats. A 48 hr fast elicits greater increases in FFA and ketones and more profound reductions in glucose in the pregnant rats and obliterates the differences in IRI. Fetal weight is not modified by such fasting but maternal weight losses exceed that of the nongravid rats. Livers from rats 19 days pregnant contain more and larger hepatocytes. Per mumole hepatic deoxyribonucleic acid (DNA)-phosphorus, water and protein are more abundant, whereas glycogen is unaffected. Livers from fed pregnant rats contain more lipid phosphorus and less neutral lipid fatty acid. After a 48 hr fast, hepatic steatosis supervenes in gravid animals due to accumulated neutral fat. The contents of hepatic acetyl-coenzyme A (CoA) and citric acid are not different in fed pregnant and virgin rats but are greater in the pregnant rats after fasting. Formation of glucose-(14)C and glycogen-(14)C from administered pyruvate-(14)C are the same in fed pregnant and virgin rats, but greater in the pregnant ones after a 24 or 48 hr fast. Pregnancy does not affect creatinine excretion, and urinary urea is not different in fed pregnant, virgin, and postpartum animals. Contrariwise, more nitrogen, potassium, and phosphorus are excreted by the pregnant animals during a 2 day fast. The increment in urinary nitrogen is due largely to urea on the 1st day, whereas heightened ammonia accounts for half the increase on the 2nd and correlates with the enhanced ketonuria. Muscle catabolism, gluconeogenesis, and diversion to fat are activated more rapidly and to a greater degree when food is withheld during late gestation in the rat. These catabolic propensities are restrained in the fed state. The capacity for "accelerated starvation" may confer survival benefit upon an intermittently eating mother in the presence of a continuously feeding fetus.
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PMID:Carbohydrate metabolism in pregnancy. VI. Plasma fuels, insulin, liver composition, gluconeogenesis, and nitrogen metabolism during late gestation in the fed and fasted rat. 535 39

Some inherited metabolic disorders (IMDs) can cause significant complications during pregnancy, affecting the mother and/or the fetus. Although it appears that only a minority of IMDs have these effects, experience is still being acquired. For some disorders, patients will not have reached child-bearing age. Pregnancies in this group of patients will increase as the management of IMDs in childhood and adolescence improves. Clinicians should be aware of potential complications and consider carefully how best to manage these conditions. Ideally, patients should be followed up in adult life by a specialized clinical team, which can implement a planned approach to conception and pregnancy, but often this is not possible. For disorders where the risk of complications is well established (e.g. phenyl-ketonuria), optimal treatment may lead to a good fetal and maternal outcome. It is important also to consider the possibility of an IMD being present in fetuses of pregnancies that are affected by non-immune hydrops, maternal HELLP syndrome (haemolysis, elevated liver enzymes and low platelets) or acute fatty liver of pregnancy.
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PMID:Pregnancy and inherited metabolic disorders: maternal and fetal complications. 1222 50

We report a new case of hereditary hepatic glycogen synthase (GS) deficiency (MIM 240600) in a French Canadian girl referred at 7 years of age for a family history of hyperlipidemia. Her initial evaluation incidentally revealed fasting hypoglycemia and ketonuria after a 10-hr fast with normal growth, development, and physical examination. Additional biochemical findings included fasting hypoalaninemia with elevated plasma branched chain amino acids and postprandial hyperlactatemia. Liver glycogen synthase activity was reduced. Unlike most other reported patients, we observed on three different occasions an increase in fasting plasma glucose levels after glucagon administration during episodes of hypoglycemia. At 13 years of age, her growth and intellect are normal; however, she still has hypoglycemia after 18 hr of fasting. From our patient's course and a review of the literature, we conclude: (A) Usual modes of presentation of GS deficiency are non-specific symptoms after overnight fasting (7/17), incidental findings (3/17), or positive family history (7/17); (B) Most patients maintain normal growth (8/11) and intellectual abilities (12/15); (C) Fasting hypoglycemia (17/17) and reduced liver glycogen content (9/9) are constant features; (D) Biochemical findings also include postprandial hyperlactatemia (13/13), fasting hyperketonemia (12/12), and fasting hypoalaninemia (8/9); (E) Glucagon response following fasting hypoglycemia is usually reduced or absent (7/8) but can be repeatedly present (1/8); (F) Liver steatosis is frequent (6/6). Although rare, GS deficiency results in a characteristic biochemical profile that, if recognized, should lead promptly to its diagnosis.
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PMID:Long-term follow-up of a new case of liver glycogen synthase deficiency. 1279 86

A 4-year-old child was found dead in her house. She had been suffering from insulin-dependent diabetes mellitus (IDDM) for 3 years. She had been admitted to hospital three times, and attended the hospital once a month. Her glycaemic control remained poor. Autopsy findings revealed a remarkably fatty liver and lack of beta cells in the pancreatic islets of Langerhans. The laboratory reported ketoacidosis, ketonuria, glycosuria, and high levels of vitreous glucose and ketones. Her father had been absent for the 3 days before her death, and the mother should have fed her. Her mother was found drunk on the floor. No detailed history was available because of the mother's death, but the probability of the mother's omission of the insulin injection was suggested. Poor control of IDDM might have also been closely connected to general neglect by the mother. This case was considered child abuse.
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PMID:Child death related to insulin omission by mother. 1533 42

Congenital lack of proopiomelanocortin (POMC) causes obesity and glucocorticoid deficiency. The responses of Pomc-/- and wild-type mice to the administration of corticosterone were compared. In study 1, mice were given corticosterone-supplemented water (CORT) for 10 days, resulting in plasma CORT levels within the physiological range, with partial suppression of hypothalamic corticotropin-releasing hormone expression to a similar degree between genotypes. Body weight, fat mass, and food intake increased in CORT-treated Pomc-/- but not wild-type mice. CORT increased plasma insulin levels 50-fold in Pomc-/- versus 14-fold in wild-type mice (P < 0.01) and increased hypothalamic agouti-related protein (AgRP) expression by more than 200% in Pomc-/- versus 40% in wild type (P < 0.05). In study 2, mice were given CORT from weaning, and Pomc-/- but not wild-type mice developed hyperglycemia, ketonuria, and hepatic steatosis by 8-12 weeks. Thus, Pomc-/- mice are hypersensitive to the adverse metabolic effects of glucocorticoids. Additionally, as the levels of plasma CORT achieved, especially in study 1, were not grossly supraphysiological, we conclude that glucocorticoid deficiency may afford Pomc-/- mice some protection from the full adverse consequences of melanocortin deficiency. This may occur through a mechanism involving the suppression of AgRP by the hypoadrenal state.
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PMID:Proopiomelanocortin-deficient mice are hypersensitive to the adverse metabolic effects of glucocorticoids. 1604 91

Diabetic ketoacidosis (DKA) is an acute severe complication of diabetes and characterized by a complex disordered metabolic state due to an absolute or relative insulin deficiency, leads to hyperglycaemia, ketoacidosis and ketonuria. DKA can cause sudden unexpected death and often yields minimal and/or subtle autopsy findings or a negative autopsy and the diagnosis mainly depends upon biochemical analysis of body fluids. This communication highlights the role of Armanni-Ebstein lesion, hepatic steatosis, biochemical analysis and second generation anti-psychotic drugs in 25 adult cases of fatal diabetic ketoacidosis. The study recognises and reconfirms that fatal DKA occurs in both type I and II diabetes. The macroscopic autopsy features observed in this study are non-specific and do not guide the pathologist towards the diagnosis of fatal DKA. Once other possibilities have been excluded, the Armanni-Ebstein lesion alone or the combination of hepatic steatosis and Armanni-Ebstein lesion in an otherwise negative autopsy of a sudden unexpected death should raise the suspicion of DKA as the cause of death and indicate biochemical analysis of body fluids. Our findings also remind forensic pathologists to search for fatal DKA in sudden unexpected death with a negative autopsy, where there is a history of second generation anti-psychotic treatment.
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PMID:The role of the Armanni-Ebstein lesion, hepatic steatosis, biochemical analysis and second generation anti-psychotic drugs in fatal diabetic ketoacidosis. 2391 Aug 54