Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0162275 (ketonuria)
 553 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A review if presented of the use of low-dose insulin infusion in the management of 58 episodes of severe diabetic hyperglycaemia. Neutral insulin in a dosage of 2-4 units per hour is infused via a paediatric giving set to achieve a sustained physiological elevation of insulin levels. This method is safe, simple and rapidly effective in lowering the blood glucose level, the mean rate of fall (62 mg/100 ml/hr, or 11% per hour) being unaffected by prior insulin therapy, acidosis or ketonuria. Classification of the hyperglycaemia as ketoacidotic or hyperosmolar is unnecessary before insulin therapy is instituted, as the relative decline in glucose level is the same in the hyperosmolar non-ketotic group as in the others. Proven infection significantly lowers the rate of fall of glucose level. Hypoglycaemia and hypokalaemia are rare during low-dose infusion. Early and adequate replacement with potassium phosphate is recommended, oral potassium supplements being continued for several days. Bicarbonate therapy is rarely indicated in the management of acidosis. No patient had cerebral oedema during treatment, and one elderly patient with extensive pneumonia and empyema died during the infusion. It is suggested that continuation of low-dose insulin infusion, together with 5% dextrose solution, after the plasma glucose level reaches 200 mg/100 ml, may hasten the clearance of ketones, preventing relapse.
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PMID:Routine use of low-dose intravenous insulin infusion in severe hyperglycaemia. 99 52

Two groups of rats were given, ad libitum, diets containing 20% fat for 4-5 weeks. The fat consisted of either long chain (LCT) or medium chain triglycerides (MCT). A third group of rats was given a low fat control diet. No difference in weight increase could be found between LCT and MCT rats. Total lipids and cholesterol concentrations in the plasma were the same whatever the diet. The high fat diets caused an increase in hepatic acetyl-CoA, citrate, malate and ketone body concentrations. Concentration of ketone bodies in the blood, however, decreased. No ketonuria occurred in any case. Hepatic levels of adenosine 5'-triphosphate, adenosine 5'-monophosphate and inoganic phosphate seemed higher in MCT rats than in LCT rats. The levels of these substrates were increased in both groups compared with the control group. LCT rats had hepatic (NS) and blood (HS) lactate and pyruvate concentrations below the corresponding figures for control rats. Hepatic and blood lactate concentrations in MCT rats were at least equal to or slightly higher (NS) than those of control rats. Hepatic glucose and glycogen and blood glucose concentrations were higher in rats given the high fat diet.
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PMID:Influence of a long or medium chain triglyceride diet on intermediary hepatic metabolism of the rat. 122 67

Investigations of renal function have been done in healthy ewes in different stages of reproduction, and after food restriction, as well as in ewes sick with ketosis, hypocalcemia, rumen acidosis and different nephropathies. The determination was based on the estimated weight dependent endogenous creatinine excretion (E). A reference population of 56 healthy non or early pregnant ewes (before day 120 of pregnancy) was used as control. Late pregnancy (121.-149. day, n = 14) and lactation (n = 14) lead to higher renal creatinine-clearance (approximately GFR). Food restriction in all stages of pregnancy was followed by lower plasma concentrations of potassium, calcium, magnesium and glucose, and a reduced fractional excretion of potassium, calcium and magnesium. Pregnancy and especially food restriction caused a marked rise of the 3-OH-hydroxybutyrate values in the plasma and a significant acetonuria. After withdrawal of feed in lactating sheep, in opposition to pregnant sheep, the plasma values of phosphate were higher. In ketotic sheep (n = 43) a failure of renal function could not be demonstrated, the pronounced acetonuria could be explained by acetonemia. In hypocalcemia (n = 23) disturbance of creatinine-clearance and tubular reabsorption of sodium, potassium, glucose and 3-OH-butyrate could be seen. Glucosuria and acetonuria were caused by increased plasma concentrations and reduced tubular reabsorption. In rumen acidosis (n = 10) disturbances of low degree of GFR and reabsorption could be seen, glucosuria was mainly due to hyperglycemia.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Clinical studies of kidney function in sheep. II. Effect of pregnancy, lactation and feed restriction and metabolic diseases on kidney function]. 772 May 46

When glucose utilisation is impaired due to decreased insulin effect, ketones are produced by the liver from free fatty acids to supply an alternate source of energy. This adaptation may be associated with severe metabolic acidosis and tends to occur in patients with type I (insulin-dependent) diabetes mellitus. In addition, hypovolemia is an almost invariable finding with marked hypoglycemia and is primarily induced by the associated glucosuria. Ketoacidosis stimulates both the central and peripheral chemoreceptors controlling respiration, resulting in alveolar hyperventilation (Kussmaul's respiration). With the ensuing fall in pCO2 the patient tries to raise the extracellular pH. A fruity odor of acetone on the patient's breath sometimes suggests that ketoacidosis is present. The classical triad of symptoms associated with hyperglycemia are polyuria, polydipsia, and weight loss. Circulatory insufficiency with hypotension is not uncommon due to the marked fluid loss and acidemia. In more severely affected patients, neurologic abnormalities may be seen, including lethargy, seizures or coma. Some patients also have marked vomiting and abdominal pain. The history and physical examination may provide important clues to the presence of uncontrolled diabetes mellitus. Once suspected, the diagnosis can be easily confirmed by measuring the plasma glucose concentration. Glucosuria and ketonuria can be semiquantitatively detected with reagent sticks. Blood gas analysis and anion gap give objective information as to the severity of the metabolic acidosis. Therapy must be directed toward each of the metabolic disturbances: hyperosmolality, ketoacidosis, hypovolemia and potassium, and phosphate depletion. The mainstays of therapy are the administration of low-dose insulin and volume repletion.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Ketoacidotic diabetic metabolic dysregulation: pathophysiology, clinical aspects, diagnosis and therapy]. 817 67

Two sisters with fructose-1,6-diphosphatase deficiency are reported. They presented with ketonuria, elevated plasma transaminase activity and severe metabolic acidosis during hypoglycaemic crises, which resembled Reye syndrome. Intravenous fructose tolerance tests provoked severe hypoglycaemia and metabolic acidosis. Fructose-1,6-diphosphatase activities in both peripheral leukocytes and cultured lymphocytes were below the limit of detection. Urinary organic acid analysis during crises revealed markedly increased excretion of lactate, ketone bodies, glycerol and glycerol-3-phosphate. We newly identified other glycolytic intermediates, glyceraldehyde, 3-phosphoglycerate and fructose-1,6-diphosphate, in the urine during hypoglycaemic attacks or after fructose tolerance tests. Identification of such compounds may be useful in the early diagnosis of this disease.
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PMID:Urinary sugar phosphates and related organic acids in fructose-1,6-diphosphatase deficiency. 841 1

The activities of either the mitochondrial or cytosolic glycerol phosphate dehydrogenase (mGPD, cGPD) plus that of glycerol kinase are required for the use of glycerol in aerobic metabolism and gluconeogenesis. A knockout mouse lacking mGPD has reduced body weight and fertility but shows remarkably normal liver and muscle metabolite levels. The BALB/cHeA mouse strain, which lacks cGPD, breeds well and is phenotypically normal, although it demonstrates metabolite abnormalities in certain tissues. Crosses were made between these two strains, and mice were generated that lacked both dehydrogenases. These mice, although active and nursing well for several days, failed to grow, and usually died within the first week. Liver glycerol phosphate levels were elevated 30-fold, whereas liver ATP, ADP, and AMP levels were reduced by 30-40%. Plasma glycerol was elevated 30- to 50-fold to 30-50 mm, and urine glycerol exceeded 0.45 m (4% w/v). GPD-deficient mice were hypoglycemic, had a 50% increase in plasma free fatty acids, and developed ketonuria within the first day of life. Uncoupling protein-1 mRNA in brown adipose tissue was reduced 60%. These mice share some features of both glycerol kinase deficiency and hereditary fructose intolerance, suggesting the phenotype may be due to the combined effects of the loss of a gluconeogenic substrate, the osmotic effects of glycerol, and the metabolic effects of the accumulation of a phosphorylated metabolite.
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PMID:Lethal hypoglycemic ketosis and glyceroluria in mice lacking both the mitochondrial and the cytosolic glycerol phosphate dehydrogenases. 1209

The safety of contemporary volatile anesthetic agents with respect to kidney function is well established, and growing evidence suggests that volatile anesthetics even protect against ischemic nephropathy. However, studies examining effects of volatile anesthetics on kidney function frequently demonstrate transient proteinuria and glycosuria following exposure to these agents, although the cause of these findings has not been thoroughly examined. We describe the case of a patient who underwent a neurosurgical procedure, then experienced glycosuria without hyperglycemia that resolved within days. Following a second neurosurgical procedure, the patient again developed glycosuria, now associated with ketonuria. Further examination demonstrated nonalbuminuric proteinuria in conjunction with urinary wasting of phosphate and potassium, indicative of proximal tubule impairment. We suggest that transient proximal tubule impairment may play a role in the proteinuria and glycosuria described following volatile anesthetic exposure and discuss the relationship between these observations and the ability of these agents to protect against ischemic nephropathy.
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PMID:Case report: proximal tubule impairment following volatile anesthetic exposure. 2641 76