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)

Congenital carbamyl phosphate synthetase deficiency was diagnosed by liver biopsy in a 13-year-old girl, alpha-Keto analogues of essential amino acids have been shown to spare nitrogen by reducing urea formation; hence, they were given to this patient in the hope of reducing hyperammonemia and improving protein tolerance. After intravenous infusion of the keto analogues of valine, leucine, isoleucine, methionine and phenylalanine, the corresponding plasma amino acids, including alloisoleucine and tyrosine, rose sharply. Twenty-four hours later, fasting plasma ammonia had fallen from the preinfusion value of 0.050 to 0.028 mM. Protein intake was kept at 0.5 g per kilogram for two weeks. Addition of keto acids by mouth reduced plasma ammonia and alanine to normal or near normal levels. Seizures and episodes of vomiting and lethargy decreased in frequency. Urinary nitrogen decreased, suggesting that nitrogen balance improved. These data indicate that keto acids may be useful in the treatment of congenital hyperammonemia.
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PMID:Treatment of carbamyl phosphate synthetase deficiency with keto analogues of essential amino acids. 16 4

Two children are described who suffered from episodes of metabolic acidosis and progressive mental and motor deterioration. The patients showed periodic elevation of blood lactate, pyruvate and alanine, which was accompanied by vomiting, hypotonia or convulsions. The concentrations of lactate and pyruvate in cerebrospinal fluid were found to be increased. Liver biopsies revealed a decrease in pyruvate carboxylase activity and normal pyruvate decarboxylase activity. No inhibitor of TPP-ATP phosphoryl transferase was detected in urine from the patients. These findings suggest that congenital lactic acidosis due to pyruvate carboxylase deficiency is probably a different disease entity from Leigh's encephalomyelopathy. A possible mechanism of brain damage caused by a defect in pyruvate carboxylase is postulated.
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PMID:Congenital lactic acidosis due to pyruvate carboxylase deficiency: absence of an inhibitor of TPP-ATP phosphoryl transferase. 20 66

During a total population survey of viral hepatitis in the London Boroughs of Hounslow, Richmond and Ealing, 784 patients were seen in three years from 1 March 1972 to 28 February 1975. A diagnosis of viral hepatitis was accepted in 489. The annual incidence was 24 per 100 000. 455 of the patients were tested for the hepatitis B surface antigen (HBsAg) by a radioimmunoassay technique and 93 (20%) of these were positive. The majority of the patients with type B hepatitis were in their third or fourth decades. None was under the age of 16. The male to female ratio among patients with hepatitis B was 2 to 1 in those under the age of 30 and 5 to 1 in those aged 30 and over. The seasonal distribution of viral hepatitis showed a peak in the spring, solely from an increased incidence of non-B hepatitis, and a second, smaller peak in the autumn. There was no appreciable clustering of patients except for one local outbreak in a housing estate during the first year affecting mainly children going to the same primary school, and their parents. Patients with hepatitis B had a longer pre-icteric illness (p less than 0.05), greater duration of jaundice (p less than 0.001) and higher peak levels of serum bilirubin (p less than 0.0005) and serum alanine amino transferase (A1T) (p less than 0.03) than patients with non-B hepatitis. The finding of the surface antigen was also associated with a higher frequency of skin rash (p less than 0.0005) and a greater duration of arthralgia (p less than 0.03). Among the HBsAg negative patients the incidence of arthralgia increased with age (p less than 0.0005). Abdominal pain (p less than 0.005) and vomiting (p less than 0.005) were more common in the young. The injection experience of patients with hepatitis B showed a high proportion of 'non-therapeutic' exposure such as drug addiction. Significantly more HBsAg positive men were single than in the local community (p less than 0.001) or among the HBsAg negative men (p less than 0.01). There was no significant difference between the proportions of single women among the antigen positive and negative patients. Many of the HBsAg positive single men were either known to be or strongly suspected of being homosexual. The ad subtype of the HBsAg was found more often in males (p less than 0.01), particularly over the age of 30. All eight drug addicts tested for subtype were ay, as were two non-addicted female consorts. The association between addiction and ay subtype was highly significant in the males (p less than 0.001). The ad subtype was found in all 11 of the admitted homosexual HBsAg positive men and in all but one of the 17 strongly suspected of being homosexual.
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PMID:A three-year survey of viral hepatitis in West London. 71 74

A 5-year old boy with intermittent sopor, severe vomiting and ataxia since 2 1/2 years old was described. During attack period the patient showed elevation of blood pyruvate and alanine and also cerebrospinal-fluid pyruvate, whereas during the time free from attack only blood pyruvate was increased. Deficiency of pyruvate decarboxylase was found in patient's leukocyte and in cultured skin fibroblasts. Addition of thiamine in assay medium did not correct the enzyme activity. Oral administration of citrate seemed to be most effective and during the treatment only a mild attack was once observed.
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PMID:Citrate treatment in a patient with pyruvate decarboxylase deficiency. 98 28

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

Three residents of New Jersey suddenly developed vomiting, abdominal pain, and watery diarrhea 12 h after ingesting five to 13 hand-picked Amanita phalloides-type mushrooms. The group was led by an amateur mycologist who had been eating hand-picked mushrooms without complications for 20 years. The patients developed mild to severe acute hepatic injury, with a peak serum aspartate aminotransferase level ranging from 133 to 826 Karmen U/L, and a peak serum alanine amino-transferase level ranging from 163 to 1176 Karmen U/L. One of the patients developed a transient severe coagulopathy, but no one developed renal failure or hepatic coma, and all rapidly improved. These three cases demonstrate that mycetismus can occur in the heavily urbanized northeastern United States, that consuming hand-picked mushrooms even under the guidance of an experienced amateur may be dangerous, and that Amanita phalloides ingestion produces a biphasic illness with early gastrointestinal symptoms and subsequent hepatic injury.
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PMID:Gastrointestinal and hepatic effects of Amanita phalloides ingestion. 147 67

Studies of "improved" oral rehydration solutions, in which glucose polymers (starch) derived from rice or other cereals were added to improve cotransport of sodium and to promote sodium and water absorption, have been reported. These solutions were administered to decrease diarrhea volume and duration, reduce vomiting, and replace volume loss in stools. In clinical trials of children and adults with high-output diarrhea, such as in cases of cholera, the use of cereal-based oral rehydration solutions (ORS) compared with glucose-based ORS produced significant (20% to 53%) reductions in stool volumes. In one study the duration of diarrhea was shortened by 30%. In noncholera diarrhea in children, cereal-based ORS was as effective as glucose-based ORS. Although the amino acid transport systems were intact in patients with cholera, the addition of glycine to glucose-based or rice-based ORS did not reduce stool volume or duration of diarrhea. The exception was alanine, which reduced stool output and ORS requirements. More research is needed to determine the optimal mix of starch, amino acids, oligopeptides, and proteins that would utilize the absorptive active transport systems maximally to reduce fluid losses and duration of diarrhea.
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PMID:Cereal-based oral rehydration therapy. I. Clinical studies. 200 59

In a multicenter study of 128 patients treated with ciprofloxacin (mean daily dosage, 982 mg per day; mean duration of treatment, 8.9 days) for a variety of infections, 48 were microbiologically proven. Of these, bacteriologic cure and/or improvement resulted in 93% of cases. For all 128 infections clinical cure and/or improvement resulted in 93.8% of cases. Twenty-nine (23.8%) of all infections were classified as chronic. Overall, there were 3/128 (2.3%) adverse reactions (ADRs); one case each of diarrhea, malaise, and nausea/vomiting. None were related definitely to ciprofloxacin therapy. Therapy with ciprofloxacin was discontinued in two (1.6%) of 128 patients because of adverse gastrointestinal (GI) effects. One patient elected to continue ciprofloxacin therapy despite mild GI side effects.
Ala Med 1989 Feb
PMID:Clinical experience with ciprofloxacin: analysis of a multicenter study. 264 74

Nineteen evaluable patients with advanced malignancy were treated with recombinant methionyl human interleukin-2 (Ala 125), 5 days per week by intravenous bolus. Patients were entered in five groups at starting doses ranging from 0.05 to 2.56 x 10(6) U/m2. Doses were escalated weekly as tolerated toward a potential maximal dose of 11.6 x 10(6) U/m2. Maximal tolerated dose was 3.84 x 10(6) U/m2. Dose-limiting toxicity included fatigue, rigors, nausea/vomiting, fever, and diarrhea. Other toxicities included hyperesthesias, arthralgias/myalgias, rash, fluid retention, balanitis, and mild confusion. Leukocytosis, including granulocytosis, eosinophilia, and mild lymphocytosis, was observed, as was rare mild thrombocytopenia. No partial or complete response occurred. T1/2 alpha averaged 13.4 min, with interleukin-2 detectable 2 h after doses of greater than or equal to 2.56 x 10(6) U/m2. Three patients developed anti-IL-2 antibodies without demonstrable clinical significance.
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PMID:Systemic administration of recombinant methionyl human interleukin-2 (Ala 125) to cancer patients: clinical results. 278 63

Benzoate and phenylacetate improve prognosis in inherited urea cycle enzyme deficiencies by increasing waste nitrogen excretion as amino acid acylation products. We studied metabolic changes caused by these substances and their pharmacokinetics in a biochemically different urea cycle disorder, lysinuric protein intolerance (LPI), under strictly standardized induction of hyperammonemia. Five patients with LPI received an intravenous infusion of 6.6 mmol/kg L-alanine alone and separately with 2.0 mmol/kg of benzoate or phenylacetate in 90 min. Blood for ammonia, serum urea and creatinine, plasma benzoate, hippurate, phenylacetate, phenylacetylglutamine, and amino acids was obtained at 0, 120, 180, and 270 min. Urine was collected in four consecutive 6-h periods. Alanine caused hyperammonemia: maximum increase 107, 28-411 microM (geometric mean, 95% confidence interval); ammonia increments were nearly identical after alanine + benzoate (60, 17-213 microM) and alanine + phenylacetate (79, 13-467 microM) (NS). Mean plasma benzoate was 6.0 mM when extrapolated to the end of alanine + benzoate infusions; phenylacetate was 4.9 mM at the end of alanine + phenylacetate. Transient toxicity (dizziness, nausea, vomiting) occurred in four patients at the end of combined infusions, and we suggest upper therapeutic plasma concentrations of 4.5 mM for benzoate and 3.5 mM for phenylacetate. Benzoate and phenylacetate then decreased following first-order kinetics with t1/2S of 273 and 254 min, respectively. Maximal plasma hippurate (0.24, 0.14-0.40 mM) was lower than maximal phenylacetylglutamine (0.48, 0.22-1.06 mM, p = 0.008).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Waste nitrogen excretion via amino acid acylation: benzoate and phenylacetate in lysinuric protein intolerance. 309 49


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