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Biomedical subjects

H Wick

Publications and source records attributed to H Wick.

At least 37 records · Page 2Linked to original sources
PubMed1985

Biotinidase deficiency associated with renal loss of biocytin and biotin.

Clinical and biochemical investigations in six patients with congenital biotinidase deficiency are presented. The time course of biotin depletion in relation to carboxylase activities and clinical onset of symptoms was studied after withdrawal of biotin supplementation. Renal biotin clearance studies were performed in patients and controls. Renal loss of biocytin and biotin itself are shown to be a major cause for the increased biotin requirement in patients with congenital biotinidase deficiency.

Amidohydrolases↗
PubMed1984

Comparison of the effect of semisynthetic human insulin and porcine insulin on glucose kinetics, plasma free fatty acid and amino acid levels in man.

The effect of semisynthetic human insulin on hepatic glucose output, peripheral glucose clearance, plasma levels of C-Peptide, free fatty acids and amino acids was compared with purified pork insulin using the glucose clamp technique. 8 normal overnight-fasted subjects received intravenous infusions of either human or porcine insulin at 20 mU/m2.min(-1) during 120 min achieving plasma insulin levels of approximately equal to 50 mU/l. Plasma glucose levels were maintained at euglycaemia by variable rates of glucose infusion. Hepatic glucose production measured by continuous infusion of 3-(3) H-glucose was similarly suppressed by both insulins to rates near zero. The metabolic clearance rate of glucose increased during infusion of human insulin by 120%, C-peptide levels decreased by 41% and plasma FFA concentrations fell by 74%. The respective changes during infusion of pork insulin were similar, 118%, 48% and 72%. Both insulins decreased the plasma levels of branched-chain amino acids, tyrosine, phenylalanine, methionine, serine and histidine similarly. Thus, the results demonstrate that semisynthetic human and porcine insulin are aequipotent with respect to suppression of hepatic glucose output, stimulation of glucose clearance, inhibition of insulin secretion, lipolysis and proteolysis.

Aged↗
PubMed1979

[The importance of amino acids].

Complete AS mixtures which permit prolonged parenteral feeding are known. It is possible to achieve normal growth even in nursing infants with such solutions (reference should be made to the contributions of Professor Riccour and Professor Schärli). On the other hand the composition of an AS solution of maximum biological value, e.g. for the growing body, is not yet known. Improvements in this respect can be envisaged although they are relevant only to certain patients. Changes in the method administration of AS may affect the biological value. Continuous administration throughout the 24 hours by-passing the portal vein, as is usual today, must considerably impair the capacity of the liver to regulate the pattern of plasma amino acids. Possibly, an approximation to physiological proportions is possible and useful through intermittent administration. Further progress in this aspect of parenteral feeding can undoubtedly be expected.

Amino Acids↗
PubMed1979

Congenital defect in intracellular cobalamin metabolism resulting in homocystinuria and methylmalonic aciduria. II. Biochemical investigations.

Biochemical investigations are reported in an infant with methylmalonic aciduria and homocystinuria who died at 4 months of age. Postmortem analysis of liver obtained 2 weeks after the child was treated with vitamin B12 revealed deficient activity of both cobalamin dependent enzymes: N5-methyltetrahydrofolate: homocysteine methyltransferase (requiring Me-Cbl), and methylmalonyl CoA mutase (requiring Ado-Cbl). MMA-CoA mutase activity could be restored to normal in vitro by added Ado-Cbl, but MeTHF-HCy transferase activity was not significantly enhanced by addition of Me-Cbl. Though the serum total cobalamin was normal, total cobalamin in liver and kidney was abnormally low. In the kidney Me-Cbl and Ado-Cbl were disproportionally decreased whereas in the liver only Ado-Cbl was significantly reduced. This suggests that at least some of the CN-Cbl administered was converted to the coenzymes in liver which would explain the reduction of MMA- and HCy-excretion during therapy. The results show 1. that this infant suffered from a congenital defect in one of the steps of intracellular cobalamin metabolism or transport common to the synthesis of both coenzymes, 2. that life-long treatment with vitamin B12 (OH-Cbl) may be of value in similar cases, particularly if given early in the course of the disease.

5-Methyltetrahydrofolate-Homocysteine S-Methyltran↗
PubMed1979

Congenital defect in intracellular cobalamin metabolism resulting in homocysteinuria and methylmalonic aciduria. I. Case report and histopathology.

The case is described of an infant who suffered from progressive, severe dystrophy, hemolytic and megaloblastic anemia, hematuria, proteinuria and slight uremia. He died at 4 months of age following two acute episodes of heart failure. Abnormally increased excretion of methylmalonate and homocystine was detected by our screening program for metabolic disorders. Amino acid analyses showed that the plasma and urine levels of methionine were very low whereas those of cystathionine were raised. Vitamin B12 deficiency, malabsorption or abnormal cobalamin transport were excluded by a normal serum total cobalamin and normal transcobalamins. These findings suggested a congenital error of cobalamin metabolism. Treatment with vitamin B12 resulted in a biochemical though not a clinical response. Postmortem examination revealed severe vascular lesions with changes in the kidney characteristic of thrombotic microangiopathy supporting a diagnosis of hemolytic-uremic syndrome. It is assumed that the elevated plasma homocysteine induced the vascular lesions by causing detachment of endothelium.

Blood Vessels↗
PubMed1978

An improved method for preparation of samples for the simultaneous assay of some antiepileptic drugs by gas-liquid chromatography.

A new simple extraction method, e.g., for subsequent quantitative determination of some antiepileptic drugs by GLC is described. HCl and an internal standard are added to 100 microliter of plasma or any biological fluid. This mixture is applied directly to an Extrelut column. Lipophilic substances (i.e. the antiepileptic drugs diphenylhydantoin, primidone and phenobarbital) are then extracted into 20 ml of ether and the solvent is evaporated to dryness. Direct GLC analysis of the on-column methylated drugs is performed using a P-N-detector.

Barbiturates↗
PubMed1977

Thiamine dependency in a patient with congenital lacticacidaemia due to pyruvate dehydrogenase deficiency.

A patient with congenital lactic acidosis, muscular hypotonia and severe ataxia is reported. The aetiology of his disease was found to be a deficiency of pyruvate dehydrogenase (E.C. 4.1.1.1.). Thiamine treatment (1.8 g/day) was successful in correcting biochemical and clinical symptoms. The mechanism of its action is probably based on activation of pyruvate dehydrogenase through interference in the physiologic regulation.

Alanine↗
PubMed1977

Diastereoisomeric N-tetrahydrofurfurylnoroxymorphones with opioid agonist--antagonist properties.

The two diastereoisomeric N-tetrahydrofurfurylnoroxymorphones and their hydrochlorides 1a and 1b have been prepared and studied pharmacologically; The N-(R)-tetrahydrofurfuryl derivative 1a proved to be an opioid agonist--antagonist and the N-(S)-tetrahydrofurfuryl derivative 1b a pure antagonist. As an analgesic, 1a is 25 times more potent than morphine, but it does not show morphine-like side effects in mice. In withdrawn morphine-dependent rhesus monkeys, 1a only partially suppresses abstinence. Its therapeutic ratio is exceptionally favorable compared with those of morphine and pentazocine. As antagonists, 1a and 1b have comparable potencies of 0.25 and 0.20 of that of nalorphine, respectively, in vivo. In vitro, however, 1a is 28 times (guinea pig ileum) or 6.5 times (mouse vas deferens) more potent than 1b. The antagonist properties of 1a and 1b were not anticipated according to known structure--activity relationships.

Analgesia↗
PubMed1977

Effect of graded biotin levels in the diet on liver pyruvate carboxylase of chicks fed ad libitum and after starvation.

Decreasing levels of biotin in the diet from 200 mug/kg to 0 mug/kg reduced the pyruvate carboxylase activity per g liver and in the whole liver of chicks to 17% of the normal activity. Oral supplementation with 3 mg biotin per chick increased the enzyme activity within 24 hours back to normal. In animals well supplemented with biotin (200 mug/kg diet) 24 hours of starvation increased the per g liver pyruvate carboxylase activity. When the pyruvate carboxylase activities were related to the whole liver, no effect of starvation was found.

Animals↗
PubMed1976

Congenital methylmalonic acidemia: a variant of the B12 'non-responsive' form with evidence for reduced affinity of methylmalonyl-CoA mutase for its B12-coenzyme.

Methylmalonate metabolism was investigated in fibroblasts and leukocytes of two unrelated patient with a B12-nonresponsive type of congenital methylmalonic acidemia. Intact fibroblasts from both patients showed a defective metabolism of methyl-14 c-malonate to 14CO2, whereas no such defect was found in their intact peripheral leukocytes. In disrupted fibroblasts, the conversion of methylmalonyl coenzyme A to succinyl coenzyme A was markedly reduced but was completely normalized by the addition of 5'-deoxyadenosylcobalamin (AdoCb1; 10(-5) mol/l), the specific coenzyme of methylmalonyl coenzyme A mutase. Assays with decreasing concentrations of AdoCbl (10(-5)-10(-11) mol/l) suggested a reduced affinity of the mutase apoenzyme for its coenzyme, implicating yet another variant of this heterogeneous disease.

Amino Acid Metabolism, Inborn Errors↗
PubMed1976

[Cholinergic contraction of the dog urinary bladder by electrical stimulation of the n. pelvicus and the ligamenta lateralia nerves and its affection by atropine and scopolaminebutylbromide (author's transl)].

As is well known contraction of the urinary bladder induced by electrical stimulation of the N. pelvicus-hypogastric plexus in the dog can only be diminished by atropine but not completely abolished. The result of stimulation can, however, be quantitatively abolished by the quaternary ammonium base hyoscine-n-butylbromide (scopolaminebutylbromide, Buscopan). Apart from antimuscarine-like peripheral effects, Buscopan also possesses, in contrast to atropine, antinicotine-like ganglion-blocking properties. Since Buscopan possesses only anticholinergic properties the contraction triggered off by stimulation of the nerve can be considered as being purely cholinergic. In the collateral ligaments of the urinary bladder of the dog there is another nervous system which when electrically stimulated contracts the bladder. Depending on the dosage the effect of stimulation can be diminished or abolished by atropine as well as by Buscopan. The result of stimulation can be diminished by hexamethonium and increased by eserine (physostigmine). Because of the effect of atropine-Buscopan (inhibition) and eserine (increase) this nervous system can be characterised pharmacologically as cholinergic.

Animals↗