Analyse colorimétrique des céto-hexoses et de l'inuline par la réaction à l'acide thiobarbiturique conditions de la réaction

Zender, Raphael; Falbriard, A

doi:10.1016/0009-8981(66)90300-7

Cited by 19 publications

(14 citation statements)

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“…During the in vivo studies the antiepileptic medication was continued. Fructose in plasma was analyzed by a colorimetric method using thiobarbituric acid (8). It should be mentioned that additional in vivo studies were not allowed by the parents.…”

Section: Methodsmentioning

confidence: 99%

D-Glyceric Acidemia: An Inborn Error Associated with Fructose Metabolism

et al. 1987

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ABSTRACT. A mentally retarded girl with epileptic sei-CASE REPORT zures is described. urinary organic acid screening ievealed a massive excretion of glyceric acid, a normally barely detectable metabolite. Hyperglycinemia was not observed. Capillary gas chromatography of the 0-acetylated (-)-menthyl ester of urinary glyceric acid showed the substance to have the D-configuration. The urinary D-glycerate excretion remained unaltered after an oral load with 200 mg/kg L-serine, but oral loading with fructose (1 g/kg) or dihydroxyacetone (1 g/kg) caused a sharp increase of the Dglycerate excretion. Treatment with a diet moderately restricted in fructose led to some clinical improvement as judged by subjective criteria. The metabolic lesion is thought to be located at some step of the fructose catabolic pathway, possibly at the level of hepatic triokinase deficiency. (Pediatr Res 21: 502-506, 1987) Abbreviations GAPD, glyceraldehyde-3-phosphate dehydrogenase NKH, nonketotic hyperglycinemia TMS, trimethylsilyl Hb, hemoglobin D-Glyceric acidemia seems to be an extremely rare inborn error of metabolism. To date only three isolated cases have been reported (1-3). The clinical presentation of the patients varied: the patient described by Wadman et al. (2) had a chronic metabolic acidosis and only a moderate developmental delay, whereas the patients described by Brandt et al. (I) and Grandgeorge et al. (3) showed all characteristic symptoms of NKH such as extreme hypotonia, deep mental retardation, myoclonic jerks, and grand ma1 seizures. Brandt's patient not only had the clinical signs of NKH, but actually had elevated levels of glycine in his plasma and urine. The concentration of D-glycerate in urine of the previously described patients ranged from 1-1 16 mmol/liter.A clear-cut enzyme defect leading to D-glyceric acidemia has not yet been found, although K$lvraa et al. (4) found evidence for a decreased activity of D-glycerate dehydrogenase in the fresh leukocytes of Brandt's patient. Enzyme studies were not performed in the other two patients.Herein we describe a patient with ~-glyceric acidemia. Results of biochemical investigations as well as of clinical studies, designed to obtain more insight in the metabolic defect, will be given.Received June 2, 1986; accepted December 19, 1986. Correspondence Dr. M. Duran, University Children's Hospital, Het Wilhelmina Kinderziekenhuis, Nieuwe Gracht 137, 3512 LK Utrecht, The Netherlands.The proband B., the first child of healthy, nonrelated parents, was born at home after an uneventful gestation of 38 wk; her birth weight was 2800 g. There were signs of perinatal asphyxia: crying started late. Apgar scores were not recorded. The mother's next pregnancy ended with a spontaneous abortion at 1 1 wk; the third pregnancy resulted in the birth of a child with spina bifida who died in the neonatal period. Subsequently two healthy children were born.In the first weeks of life the patient was noted to be hypotonic and she had severe feeding difficulties. Motor development was slo...

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Section: Methodsmentioning

confidence: 99%

D-Glyceric Acidemia: An Inborn Error Associated with Fructose Metabolism

et al. 1987

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“…Blood samples were obtained at the beginning and end of each collection period. PAH and inulin were analyzed by colorimetric techniques (16,17). The clearance of PAH was corrected assuming a PAH extraction of 0.9 (18).…”

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confidence: 99%

Effect of Low Dose Dopamine on Hemodynamic and Renal Function in Children

et al. 1989

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ABSTRACT. The purpose of the study was to investigate the effect of low doses of dopamine in children. Fourteen cases were studied after open heart surgery. Cardiac output and renal parameters were determined under baseline conditions and under continuous infusion of dopamine 2.5 and 5 ~g/kg/min. During the control period cardiac index was 2.62 f 0.19 L/min/m2, renal plasma flow was decreased at 269 f 41 mL/min/1.73 m2, GFR was 86.6 2 9.2 mL/min/ 1.73 m2, and filtration fraction was elevated at 37.1 f 1.9%. Plasma concentration of aldosterone correlated with the filtration fraction. At 5 pg/kg/min dopamine increased significantly cardiac output, renal plasma flow, and to a lesser extent GFR, thus decreasing the filtration fraction. At 2.5 pg/kg/min dopamine, increased renal plasma flow only in patients older than 5 y and had no effect on the other parameters. The increase of cardiac output in response to dopamine was abolished by propanolol pretreatment. By contrast, the hemodynamic renal response to dopamine was not altered by P-blockade. These results indicate that 5 wg/kg/min of dopamine could prevent renal failure after open heart surgery in children by increasing renal blood flow and attenuating renal compensatory mechanisms. (Pediatr Res 26:200-203,1989) Abbreviation PAH, para-aminohippurateIn the presence of reduced cardiac output, dopamine is frequently used for both its positive cardiac inotropic and its renal vasodilatory effects. A considerable amount of data supports its use in adults (1-5) but hemodynamic and renal effect of dopamine have not yet been assessed in children. Some animal (6-8) and clinical studies (9) suggest that differences in response to dopamine exist between adults and pediatric patients.After open heart surgery, cardiac output and systemic pressure are often depressed. Renal failure develops in 3 to 20% of patients depending on the criteria used and the patient population. It markedly influences the ultimate prognosis (10-1 5).Our study was designed to test the effect of a continuous infusion of dopamine, 2.5 and 5.0 pg/kg/min, on hemodynamic and renal function in children after open heart surgery. renal function before the operation. The group consisted of eight girls and six boys aged 18 mo to 15 y. The clinical characteristics and the preoperative plasma creatinine concentrations are given in Table I. All the patients had normal urinalysis with negative or trace proteinuria. Before the operation, six children with tetralogy of Fallot were treated with 1 to 4 mg/kg/day of propranolol to prevent hypoxic spells; the drug was stopped 8 h before the beginning of the operation. The surgery was performed with cardiopulmonary bypass, deep hypothermia (1 8 to 24"C), and moderate hemodilution (20 to 30%).Cold chemical cardioplegia was used to obtain asystole. When cardiopulmonary bypass was discontinued, blood was infused until the left atrial pressure reached 9.5 to 12.5 mm Hg, and a single dose of furosemide (1 mg/kg) was given.Method. After the operation, a Swan-Ganz catheter (5 F...

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“…The method 26,27 was developed recently in one of our laboratories for the detection and quantification of D-fructose 6-phosphate. 19 We found that TBA reacts similarly with Allu6P 28 which, following dehydration and dephosphorylation in the presence of hydrogen chloride, gives rise to 5-hydroxymethylfurfural, which in turn reacts with TBA to yield the characteristic yellow adduct.…”

Section: Kinetic Assaysmentioning

confidence: 99%

d-Ribose-5-Phosphate Isomerase B from Escherichia coli is Also a Functional d-Allose-6-Phosphate Isomerase, While the Mycobacterium tuberculosis Enzyme is Not

Roos

Mariano

Kowalinski

et al. 2008

Journal of Molecular Biology

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SummaryInterconversion of D-ribose 5-phosphate and D-ribulose 5-phosphate is an important step in the pentose phosphate pathway. Two unrelated enzymes with D-ribose-5-phosphate isomerase activity were first identified in Escherichia coli, RpiA and RpiB. In this organism, the essential 5-carbon sugars were thought to be processed by RpiA, while the primary role of RpiB was suggested instead to be interconversion of the rare 6-carbon sugars, D-allose 6-phosphate and D-allulose 6-phosphate. In Mycobacterium tuberculosis, where only an RpiB is found, the 5-carbon sugars are believed to be the enzyme's primary substrates. Here we present kinetic studies examining the D-allose-6-phosphate isomerase activity of the RpiBs from these two organisms, and show that only the E. coli Keywords: Ribose-5-phosphate isomerase; allose-6-phosphate isomerase; rare sugar; Xray crystallography; Rv2465c. A C C E P T E D M A N U S C R I P T ACCEPTED MANUSCRIPT

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Analyse colorimétrique des céto-hexoses et de l'inuline par la réaction à l'acide thiobarbiturique conditions de la réaction

Cited by 19 publications

References 1 publication

D-Glyceric Acidemia: An Inborn Error Associated with Fructose Metabolism

D-Glyceric Acidemia: An Inborn Error Associated with Fructose Metabolism

Effect of Low Dose Dopamine on Hemodynamic and Renal Function in Children

d-Ribose-5-Phosphate Isomerase B from Escherichia coli is Also a Functional d-Allose-6-Phosphate Isomerase, While the Mycobacterium tuberculosis Enzyme is Not

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