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vervolgens moet de concentratie worden bepaald van de abnormale galzuurmetabolieten die worden gesynthetiseerd.
the concentrations of the abnormal bile acid metabolites synthesised subesquently should be determined.
Última atualização: 2017-04-26
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ook moet de concentratie worden bepaald van de abnormale galzuurmetabolieten die bij 3β--hydroxy--Δ5
the concentrations of the abnormal bile acid metabolites synthesised in 3β-hydroxy-Δ5-c27-steroid oxidoreductase deficiency (3β, 7α-dihydroxy- and
Última atualização: 2017-04-26
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gelijktijdig met het verdwijnen van atypische galzuurmetabolieten treedt er een consequente daling en normalisering van leverenzymen in het serum op.
concurrent with the disappearance of atypical bile acid metabolites, there is a consistent reduction and normalization in serum liver enzymes.
Última atualização: 2017-04-26
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de laagste dosis cholzuur die de galzuurmetabolieten effectief vermindert tot zo dicht mogelijk bij nul, dient te worden gekozen.
the lowest dose of cholic acid that effectively reduces the bile acid metabolites to as close to zero as possible should be chosen.
Última atualização: 2017-04-26
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massaspectrometrische analyse van urine tijdens cholzuurtherapie laat de aanwezigheid van cholzuur en een opvallende daling of zelfs volledige eliminatie van de toxische galzuurmetabolieten zien.
mass spectrometry analysis of urine during cholic acid therapy shows the presence of cholic acid and a marked reduction, or even complete elimination of the toxic bile acid metabolites.
Última atualização: 2017-04-26
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de rationele basis voor behandeling bestaat uit herstel van de galzuurafhankelijke component van de galafvoer wat herstel van de galsecretie en eliminatie van toxische metabolieten via de gal mogelijk maakt, remming van de aanmaak van de toxische galzuurmetabolieten door negatieve feedback op cholesterol-7α-hydroxylase (het snelheidsbepalende enzym in de galzuursynthese) en verbetering van de voedingsstatus van de patiënt door correctie van intestinale malabsorptie van vetten en in vet oplosbare vitaminen.
the rational basis for treatment consists of restoration of the bile acid- dependent component of bile flow enabling restoration of biliary secretion and biliary elimination of toxic metabolites; inhibition of the production of the toxic bile acid metabolites by negative feedback on cholesterol 7α-hydroxylase, which is the rate-limiting enzyme in bile acid synthesis; and improvement of the patient’s nutritional status by correcting intestinal malabsorption of fats and fat-soluble vitamins.
Última atualização: 2017-04-26
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