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)

Urine ketone levels were measured in patients receiving peripheral amino acid solutions, and the results were correlated with changes in nitrogen balance. Thirty well-nourished patients who were to undergo cystectomy were placed on liquid, noncarbohydrate diets 3 days before operation, and no oral intake was allowed until 7 days after operation. Crystalline amino acid (1.3 to 1.5 gm/kg/day) solutions were infused continuously from 3 days before to 7 days after operation. Blood was obtained 3 days before and 3, 7, and 10 days after operation; 24-hour urine outputs were determined daily. Qualitative urine acetone levels were determined four times daily. During the infusion period, 14 (47%) patients developed ketonuria (group I); 16 patients did not (group II). The mean serum glucose levels ranged from 99 to 107 mg/dl in group I and from 108 to 113 mg/dl in group II (P less than 0.05). The mean serum transferrin level decreased after operation to 117 mg/dl in group I and 97 mg/dl in group II. The mean cumulative adjusted nitrogen balance was -24 +/- 8 gm in group I and -47 +/- 9 gm in group II (P less than 0.05). No patient developed sepsis. Qualitative testing of urinary ketones correlated with significant alterations in blood urea nitrogen, serum glucose, transferrin, and cumulative adjusted nitrogen balance. The bedside determination of urinary ketones may be useful in assessing a patient's adaptation to peripheral amino acid infusions.
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PMID:Adaptation to amino acid infusion in patients undergoing operation. 683 5

The purpose of these experiments was to determine if augmented renal ammoniagenesis in chronic metabolic acidosis could increase the negative nitrogen balance during prolonged fasting. To explore this question, rats and rabbits were fasted for up to 10 days because acidosis would markedly augment ammonium excretion in the rat but not in the rabbit. Since the ketoacidosis of fasting was mild in both species (less than 2 mM) and ketonuria virtually absent, a hydrochloric acid load was given to stimulate renal ammoniagenesis. Under these conditions, nitrogen balance was significantly more negative during acidosis in the rat but not in the rabbit. This increment in nitrogen excretion appeared as ammonium with no detectable difference in urea nitrogen excretion. Therefore, it appears that if more nitrogen is excreted as ammonium, net protein breakdown increases to furnish the substrate for ammoniagenesis rather than reducing the excretion of the other nitrogenous waste component urea. The implications of these findings will be discussed.
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PMID:Role of acidosis in the protein wasting of fasting in the rat and the rabbit. 707 21

A case of adult Reye's syndrome is described. A previously healthy 17-year-old man developed convulsions 2 days after resolution of an upper respiratory infection with parainfluenza virus type 3. During the preceding infection, he took aspirin. On admission, he was drowsy. There was no focal signs. Cranial CT scan was unremarkable. A lumbar puncture revealed an opening pressure of 180 mm H2O; the cerebrospinal fluid was acellular with normal protein level. Serum chemistry showed elevated transaminase activities and normal bilirubin level. Blood ammonia level was high; urea and citrulline levels were abnormally low. These abnormalities disappeared later, suggesting transient cysfunction of mitochondrial urea-cycle enzymes. Free and acyl carnitine levels were unremarkable. Both metabolic acidosis and ketonuria were absent. Thus, a variety of aminoacidurias and organic acidemias are unlikely. All these findings meet diagnostic criteria for Rye's syndrome proposed by the CDC of the USA. Status epilepticus was treated with intravenous infusion of thiamylal sodium. He was treated with hypertonic glucose solution and osmotic diuretic. Three months after the onset of the illness, his convulsions were controlled only with zonisamide, clonazepam, and carbamazepine. He had motor dysfunctions. This case is unique in that a patient with adult Reye's syndrome and status epilepticus favorably recovered.
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PMID:[A case of adult Reye's syndrome with favorable outcome despite status epilepticus]. 904 59

Nausea and vomiting are both common in early pregnancy. Most cases are mild and do not require treatment. However, persistent vomiting and severe nausea can progress to hyperemesis if the woman is unable to maintain adequate hydration, and fluid and electrolyte as well as nutritional status are jeopardised. Hyperemesis gravidarum is a diagnosis of exclusion, characterised by prolonged and severe nausea and vomiting, dehydration, ketosis and bodyweight loss. Investigation may show hyponatraemia, hypokalaemia, a low serum urea level, metabolic hypochloraemic alkalosis and ketonuria. The haematocrit is raised and the specific gravity of the urine is increased. There may be associated liver function test abnormalities and abnormal thyroid function tests, with biochemical thyrotoxicosis with raised free thyroxine levels and/or suppressed thyroid-stimulating hormone levels. The pathophysiology of hyperemesis is poorly understood. Various hormonal, mechanical and psychological factors have been implicated. Studies have demonstrated a direct relationship between the severity of hyperemesis, the degree of biochemical hyperthyroidism and the levels of human chorionic gonadotrophin (hCG). Management of hyperemesis should include hospitalisation, intravenous fluid and electrolyte replacement, thiamine (vitamin B1) supplementation, use of conventional antiemetics and psychological support. Most patients improve spontaneously with the help of the above measures without long term sequelae. Conventionally, antiemetics are not usually prescribed, especially before 12 weeks gestation, except for women with hyperemesis. This reluctance relates to fears which are often unfounded concerning the teratogenic effects of antiemetics. Severe hyperemesis, refractory to conventional management with intravenous fluids and antiemetics is a rare, miserable and disabling condition, associated with multiple hospital admissions, time away from work and the family, and psychological morbidity. If inadequately or inappropriately treated, it may cause Wernicke's encephalopathy, central pontine myelinolysis and death. In extreme cases, women may request, or their obstetricians recommend, termination of the pregnancy. There are uncontrolled data supporting a beneficial effect of corticosteroids in these women, and a randomised placebo-controlled trial is currently in progress.
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PMID:Treatment of nausea and vomiting in pregnancy. When should it be treated and what can be safely taken? 970 51

Methods to microencapsulate enzyme, cells, and genetically engineered cells have been described in this article. More specific examples of enzyme encapsulation include the microencapsulation of xanthine oxidase for Lesch-Nyhan disease; phenylalanine ammonia lyase for pheny, ketonuria and microencapsulation of multienzyme systems with cofactor recycling for multistep enzyme conversions. Methods for cell encapsulation include the details for encapsulating hepatocytes for liver failure and for gene therapy. This also includes the details of a novel two-step method for encapsulation of high concentrations of smaller cells. Another new approach is the detailed method of the encapsulation of genetically engineered Escherichia coli DH5 cells for lowering urea, ammonia, and other metabolites in kidney or, liver failure and other diseases.
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PMID:Procedures for microencapsulation of enzymes, cells and genetically engineered microorganisms. 1143 13

There are almost one hundred inborn errors of metabolism which can start in the neonatal period, but less than 20 are amenable to treatment. In general, an extremely evocative clinical setting is the course of a full-term baby born after normal pregnancy and delivery who, after an initial symptom-free period deteriorates relentlessly for no apparent reason and does not respond to symptomatic therapy. Investigations routinely performed in all sick neonates yield normal results. Emergency treatment must be undertaken in parallel with investigations. Five main presentations can be observed: a neurologic deterioration 'intoxication' type mostly suggests maple syrup urine disease, methylmalonic, propionic, isovaleric acidaemias and urea cycle disorders. Isolated seizures is the revealing symptom of pyridoxine-responsive and folinic acid responsive seizures. A jaundice or a liver failure suggest galactosaemia, fructosaemia, tyrosinaemia type I (after 3 weeks), phosphomannoisomerase deficiency or bile acid synthesis defects. Cardiac failure and heartbeat disorders should first suggest mitochondrial fatty acid oxidation (FAO) disorders. Persistent hypoglycaemia is the presenting sign of glyco/gluconeogeneis defects, hyperinsulinism and FAO disorders. The first line investigation relies upon the collection at the same time of a few samples including blood gases electrolytes, prothrombin time, transaminases, ammonia and lactic acid, and the search for ketonuria. The storage of plasma, urine and blood (on filter paper) is an important element in the diagnosis. The utilization of these samples should be carefully planned after taking advice from specialists in inborn errors.
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PMID:Clinical approach to inherited metabolic disorders in neonates: an overview. 1206 34

This study examines the association of pre-surgical clinical parameters in cows with left displacement of the abomasum (LDA) with the probability of cure. Data from 466 cases of LDA in which omentopexy was performed were analysed. Cows were classified in three categories according to the outcome of treatment. Cows in the first category were cured, i.e. sent back to the farm for further milk production. Cows in the second category were sent for slaughter because of an unfavourable prognosis concerning productivity. Cows in the third category either died or were salvaged because of poor prognosis concerning survival. Factors were examined for their possible influence on the outcome of treatment by comparing the values of successfully treated cows to those cows that either died or had to be sent for slaughter. Factors that were associated with a favourable prognosis were a short duration of disease, an undisturbed general condition, good appetite, normal faeces, a higher body weight, lower haematocrit, haemoglobin and erythrocyte counts, lower urea, AST and bilirubin, and higher sodium, potassium and chloride concentrations compared with cows with an unfavourable prognosis. Haemoconcentration was associated with higher AST, bilirubin and urea and with low sodium, potassium and chloride. Acetonuria was associated with increased AST and bilirubin and low urea. Low sodium and potassium concentrations were associated with an impaired general condition. It is concluded that a thorough clinical examination with special emphasis on general condition, liver function and dehydration status is of great importance in determining the prognosis of abdominal surgery in cows with LDA.
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PMID:Survival of dairy cows after surgery to correct abomasal displacement: 2. Association of clinical and laboratory parameters with survival in cows with left abomasal displacement. 1548 66

The association between female fetal sex and hyperemesis gravidarum is well established in European and North American populations. The association between female fetuses and severity of hyperemesis remains uncertain. A retrospective study based on case notes review of 166 Asian women hospitalised for hyperemesis was performed. Female fetuses were significantly associated with hyperemesis in our population (P= 0.004, OR 1.6, 95% CI 1.2-2.2) as well as associated with severe ketonuria and high urea. When both severe ketonuria and high urea were present at initial hospital admission for hyperemesis, 83% (95% CI 66-93) of the fetuses were female.
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PMID:The fetal sex ratio and metabolic, biochemical, haematological and clinical indicators of severity of hyperemesis gravidarum. 1670 19

Effects of feeding a dry glycerin product (minimal 65% of food grade glycerol, dry powder) to 39 multiparous Holstein dairy cows (19 control and 20 glycerin-supplemented; lactation number = 2.2 +/- 1.3 SD) on feed intake, milk yield and composition, and blood metabolic profiles were investigated. Dry glycerin was fed at 250 g/d as a top dressing (corresponding to 162.5 g of glycerol/d) to the common lactating total mixed ration from parturition to 21 d postpartum. Individual milk was sampled from 2 consecutive milkings weekly and analyzed for components. Blood was sampled from the coccygeal vein at 4, 7, 14, and 21 (+/-0.92, pooled SD) d in milk and analyzed for urea nitrogen, glucose, insulin, nonesterified fatty acids, and beta-hydroxybutyrate. Urine was tested for the acetoacetate level weekly by using Ketostix. Average feed intake, milk yield and components, blood metabolites, and serum insulin concentrations were not affected by dry glycerin supplementation. Glycerin-supplemented cows experienced a more positive energy status (higher concentrations of plasma glucose, lower concentrations of plasma beta-hydroxybutyrate, and lower concentrations of urine ketones), which was observed during the second week of lactation, suggesting that energy availability may have been improved. This glucogenic effect of dry glycerin did not result in an increase in feed intake or milk yield during the first 3 wk of lactation, likely because of the relatively less negative energy status of cows transitioning into lactation. The tendency toward higher milk yield for glycerin-supplemented cows during wk 6 of lactation (52 vs. 46 kg/d) after the supplementation period (dry glycerin was terminated at wk 3 of lactation) suggested a potential benefit of dry glycerin on subsequent milk production, perhaps through changes in metabolism, which requires further investigation.
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PMID:Effects of feeding dry glycerin to early postpartum Holstein dairy cows on lactational performance and metabolic profiles. 1802 60

Background. Prolonged obstructed labour is a major cause of maternal and perinatal morbidity and mortality especially in the developing countries of the world, where the incidence is high. These complications are partly attributed to the metabolic and electrolyte derangements that are often associated with this problem. It is, therefore, important to evaluate the metabolic and electrolyte changes of these patients in a rural community in a developing country. Objective. To compare the electrolyte changes, maternal, and perinatal outcomes in patients with prolonged obstructed labour with that of normal labour in General Hospital Ikot Ekpene, Akwa Ibom State, Nigeria. Patients and Methods. This is a prospective cross-sectional case control study conducted in the Labour Ward of the General Hospital Ikot Ekpene to compare the electrolyte levels and perinatal outcome of 95 pregnant women who had prolonged labour with 105 women who had normal labour within the same period. Main Outcome Measures. Electrolyte changes, ketonuria, maternal complications, and perinatal outcome. Results. The majority of women with prolonged labour (91.6%) had major surgical interventions requiring anaesthesia. Perinatal death occurred in 12.6%, and a major life-threatening maternal complications (including two deaths) occurred in 13.7% of those with prolonged labour compared to 2.9% (with no death) in those with normal labour. Significant abnormal electrolyte changes included hyperkalemia, high urea, and creatinine as well as low bicarbonate levels were recorded. Metabolic abnormality was shown by ketonuria in 91.1% of the patients compared to 1.9% in women with normal labour. Conclusion. Women with prolonged labour in Ikot Ekpene have significant electrolyte and metabolic changes which impact adversely on the maternal and perinatal outcomes of the pregnancy. Effort should be made to correct these electrolyte and metabolic abnormalities during resuscitation of the woman in order to reduce the complications associated with such derangements.
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PMID:Study of electrolyte changes in patients with prolonged labour in ikot ekpene, a rural community in niger delta region of Nigeria. 2331 80


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