Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042963 (vomiting)
 31,883 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Domestic kittens were used in four experiments to establish quantitative requirements for lysine and arginine. A purified L-amino acid diet (by calculation, 4,700 kcal metabolizable energy/kg diet) was employed throughout. Weight gain, gain:feed and nitrogen retention data of cats fed dietary lysine levels ranging from 0.48 to 1.92% suggested a requirement not exceeding 0.80%. The dietary arginine requirement for maximal gain was assayed at this level of lysine and found to be not greater than 0.83%. A dietary arginine level of 0.33% resulted in vomiting and extreme lethargy within 4 hours of ingestion.
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PMID:Lysine and arginine requirements of the domestic cat. 45 91

The mechanisms underlying ethylmalonic-adipic aciduria were studied in a 5-yr-old girl. Oxidation of radioactive substrates by cultured skin fibroblasts from the proband and asymptomatic family members was also determined and compared to that by normal fibroblasts and that by cells from a patient with glutaric aciduria type II. Feeding medium-chain triglycerides promptly induced vomiting and lethargy accompanied by a pronounced increase of urinary ethylmalonate. Significant increases of serum isovalerate and urinary isovalerylglycine were observed after leucine feeding, but urinary glutarate increased only slightly after lysine feeding. Thus, the results from clinical investigation remained equivocal as to whether pathways other than fatty acid oxidation were blocked in our patient. Oxidation of [1-(14)C]butyrate by cultured skin fibroblasts from the proband was reduced to 14% of control. In vitro oxidation of [2-(14)C]lysine and [2-(14)C]leucine was also reduced to 28 and 23% of control, respectively. Much more severe reduction in oxidation of these three substrates (3, 9, and 9%, respectively) was observed in glutaric aciduria type II cells. These results indicated that in the proband, degradative pathways of fatty acids, lysine, and leucine are blocked at the steps of butyryl-CoA, glutaryl-CoA, and isovaleryl-CoA dehydrogenases, respectively, as in the case of glutaric aciduria type II. Because activities of multiple acyl-CoA dehydrogenases are reduced, a deficiency of electron-transferring flavoprotein, which serves as a hydrogen-acceptor for these dehydrogenases, is postulated as the underlying mechanisms of these two diseases, but a genetic heterogeneity was indicated by significant differences in the residual activities in these two types of cells. The hypothesis of more than one mutant allele of an autosomal recessive gene was also suggested by the study on cells from asymptomatic members of the family.
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PMID:Ethylmalonic-adipic aciduria. In vivo and in vitro studies indicating deficiency of activities of multiple acyl-CoA dehydrogenases. 50 Aug 26

Congenital hyperdibasicaminoaciduria without cystinuria was detected in a mentally but not physically retarded boy. Plasma lysine and arginine were normal, whereas plasma ornithine was decreased. Although oral or intravenous loading tests could not be performed, the history without vomiting or diarrhea, and the normal physical development indicated an unimpaired intestinal transport of basic aminoacids. Our case could be a further mutant of this transport defect which concerns the renal tubuli only.
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PMID:[Congenital hyperlysin-arginin-ornithinuria in a mentally retarded child (author's transl)]. 91 28

An 18-year-old mentally and physically retarded boy, suffering from episodes of anorexia, vomiting, coma and convulsion which have been severer with advance in age, had periodic hyperammonemia, hyperlysinemia and homocitrullinuria. Blood cell arginase activity of the patient on normal diet was markedly reduced after an oral load of L-lysine. The oral loading tests of L-lysine revealed hyperammonemia, hyperlysinemia, hyperargininemia, hypercitrullinemia and homocitrullinuria. Etiology of metabolic error of our patient was discussed in reference to lysine-urea cycle.
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PMID:Clinical and biochemical studies on periodic hyperammonemia with hyperlysinemia and homocitrullinuria. 98 31

Lysinuric protein intolerance (LPI), an autosomal recessive defect of diamino acid transport, is characterized chemically by renal hyperdiaminoaciduria, especially lysinuria, and by impaired formation of urea with hyperammonemia after protein ingestion. Our 20 patients thrived during breast-feeding, but ingestion of cow's milk caused diarrhea and vomiting. When able to select their diet, they rejected all protein-rich foods. They were short staturated and had weak atrophic muscles, osteoporosis, hepatomegaly and often splenomegaly. Four patients were mentally retarded. Fifteen patients had leukocyte counts below 4,000/mm3, and 17 patients had platelet counts below 150,000/mm3. Serum lactate dehydrogenase activity was constantly increased, and transaminase and aldolase activities were often increased. In the infants' livers, changes were only revealed by electron microscopy: increased and vesicular smooth endoplasmic reticulum, and abundance of glycogen particles in the hepatocytes. In the older patients, light microscopy demonstrated clearly limited areas where hepatocytes had large pale cytoplasm and small pyknotic nuclei. The diamino acids lysine, arginine and ornithine had plasma concentrations only one-third to one-half the normal mean; the renal clearances were clearly increased. Oral diamino acid loading tests suggested impaired intestinal absorption. Urea is built in the liver through transformation of ornithine to arginine, and cleavage of arginine to ornithine and urea. The addition of ornithine to an intravenous I-alanine loading prevented the hyperammonemia and normalized the urea production. Therefore, the diet has been supplemented with arginine, and more protein has been added. This therapy has lead to a remarkable catch-up growth in some patients. The pathophysiology of LPI is explained. Because of defective intestinal absorption and incrased renal loss, the diamino acids have a low plasma concentration. Their transport from plasma to hepatocytes is also impaired, and the liver becomes deficient in ornithine. This retards the urea cycle, and leads to postprandial hyperammonemia and protein aversion. The presence of the transport defect in the hepatocytes distinguishes LPI from other hyperdibasicaminoacidurias.
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PMID:Lysinuric protein intolerance. 115 80

A patient with Addison's disease, treated with conventional hydrocortisone replacement, developed deep hyperpigmentation, headache and vomiting. Plasma adrenocorticotropin (ACTH) level was extremely high, showing abnormal diurnal rhythm. Suppression of ACTH with glucocorticoids was attenuated and the responses to ovine corticotropin-releasing hormone (oCRF) and lysine vasopressin (LVP) were absent. Magnetic resonance imaging (MRI) suggested an enlargement of the pituitary gland, while immunohistological examination of pituitary fragments obtained by transsphenoidal surgery revealed corticotroph hyperplasia without microadenoma. Postoperatively, plasma ACTH returned to normal and adequately responded to oCRF and LVP. Over the year since surgery, the symptoms have gradually improved and the patient has resumed normal activities.
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PMID:Inappropriate secretion of adrenocorticotropin from corticotroph hyperplasia in a case of Addison's disease. 217 Jul 22

The effect of repeated doses of 1.8 g lysine acetyl salicylic acid (LAS) i.v. on severe pain secondary to acute renal colic (ARC) was studied in 45 consecutive patients. Clinically acceptable analgesia was obtained in 65% of the cases. No additional pain relief was achieved with the combination of pethidine 100 mg i.v. + metoclopramide 10 mg, i.m. (narcotics). Pain relief occurred within five minutes in one third of the patients while in the rest within 30 minutes. Significant reduction of systolic blood pressure (mean +/- S.D.) 23.8 +/- 19.5, pulse rate (mean +/- S.D.) 19.5 +/- 10.1 and vomiting were noted in patients who had pain relief. The incidence of nausea has increased after LAS administration. No other side effects were observed. LAS might therefore be applied as a first-hand alternative to narcotics for the treatment of ARC.
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PMID:Lysine acetyl salicylic acid in acute renal pain. 250 91

A controlled randomized trial of WHO standard oral rehydration solution containing 50 g rice powder instead of 20 g/l glucose was conducted in 48 male children aged 3 months to 3 years. Both solutions contained 90 mM Na+, 20 mM K+, 80 mM C1- and 30 mm HC03-. Rice powder solution was prepared by boiling 50 g dry rice powder in several hundred m1 water, cooling, dissolving electrolytes, and adding water to 1 L. Children with dehydration clinically estimated at 10% body weight were initially treated with Ringers lactate iv until blood pressure and pulse normalized, then joined the rehydration study. Weight, fluid intake, urine output by condom drainage and gastrointestinal losses by weight of linen were recorded every 4 hours. 23 children in each group were successfully rehydrated determined clinically; 1 in each group required iv fluids for persistent vomiting. Patients on rice powder had a lower purge rate and fluid intake, and a significant decrease in stool frequency during the 1st 24 hours, mean 14.1 vs. 20 5. Rice contains 7-10% protein, is rich in glycine, lysine, leucine and isoleucine, and is 80% hydrolyzed to glucose. Futhermore, it is readily available and has been used traditionally in Indian homes.
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PMID:Rice powder oral rehydration solution as an alternative to glucose electrolyte solution. 329 74

A second child with a more severe deficiency of malonyl CoA decarboxylase is described. He is mildly mentally retarded and presented with vomiting, a seizure, hypoglycaemia and mild metabolic acidosis during a urinary tract infection. The urine contained increased amounts of malonic, methylmalonic, succinic, adipic, glutaric and suberic acids. Mitochondrial malonyl CoA decarboxylase activity in cultured fibroblast extracts was 4% of the mean control value. A high fat, low carbohydrate diet led to symptomatic hypoglycaemia, a moderate metabolic acidosis and excretion in the urine of large amounts of the same organic acids and 3-hydroxybutyrate. Only relatively small quantities of malonic, methylmalonic and succinic acid were excreted in the urine when the boy was fed an isocaloric low fat, high carbohydrate diet. Acute fat and lysine loads led to increased excretion of malonic acid in the urine without affecting the excretion of the other organic acids. Experience with this patient suggests that malonyl CoA decarboxylase serves an important function in the mitochondrion by preventing accumulation of malonyl CoA. The importance of the enzyme is best seen when fat is the main metabolic fuel. The mechanisms by which malonyl CoA produces its complex metabolic effects remain to be elucidated.
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PMID:Malonyl coenzyme A decarboxylase deficiency. Clinical and biochemical findings in a second child with a more severe enzyme defect. 370 68

Experiments were conducted with growing English Pointer puppies to examine the effects of ingesting excess lysine. A purified crystalline amino acid basal diet containing 0.40% L-arginine (the arginine requirement for maximal weight gain) and 0.91% L-lysine was fed in all assays. All diets were kept isonitrogenous by the addition of diammonium citrate, and lysine was supplied as L-lysine acetate. Both weight gain and gain/feed were reduced in the presence of 4% excess dietary lysine. However, 1 and 2% excess supplemental lysine had no effect on performance. In a second experiment, a growth response to supplemental arginine was obtained in the presence, but not in the absence, of a growth-depressing level of lysine (4%). Therefore, lysine appeared to depress growth by antagonizing arginine. The mechanism of the lysine-arginine antagonism was examined in a third experiment. Classic signs of arginine deficiency: orotic aciduria, depressed urea formation, hyperammonemia, a reduction in weight gain, and emesis were observed in puppies consuming excess lysine but not in their pair-fed controls. Excess lysine ingestion neither inhibited nor induced liver arginase, but it did result in a generalized amino aciduria early in the experiment. In addition, lysine did not appear to affect arginine absorption. Therefore, the mechanism behind the lysine-arginine antagonism in the dog remains to be elucidated.
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PMID:Antagonism of arginine by excess dietary lysine in the growing dog. 392 64


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