2004
DOI: 10.1055/s-2004-44906
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Die ketogene Diät in den deutschsprachigen Ländern im Jahr 2003: eine Standortbestimmung

Abstract: In contrast to the general restraint towards the ketogenic diet in Europe, our data supports its effectiveness as the treatment of choice for GLUT1-deficiency syndrome und pyruvate-dehydrogenase-complex-deficiency. In children with refractory epilepsy, the ketogenic diet matched the effect of most anticonvulsants and was well tolerated. These data and two workshops resulted in recommendations for the use of the ketogenic diet in children as a basis for a general diagnostic and therapeutic standards to compare … Show more

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Cited by 23 publications
(6 citation statements)
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“…During fasting or the KD D-BHB concentrations typically rise to levels between 0.5–5 mmol/L and up to 8 mmol/L during starvation [18]. Elevated KB levels have been shown to be well tolerated for extended periods of time (up to several years [19,20,21,22,23,24,25,26,27,28,29,30,31,32]).…”
Section: Introductionmentioning
confidence: 99%
“…During fasting or the KD D-BHB concentrations typically rise to levels between 0.5–5 mmol/L and up to 8 mmol/L during starvation [18]. Elevated KB levels have been shown to be well tolerated for extended periods of time (up to several years [19,20,21,22,23,24,25,26,27,28,29,30,31,32]).…”
Section: Introductionmentioning
confidence: 99%
“…Several strategies have been employed to treat PDC-deficient patients, with variable and often limited success [18] , [19] , [20] , [21] . They include three major therapies (and often in some combination) [5] , [8] : (i) the use of a ketogenic diet to provide ketone bodies as an alternate fuel for brain metabolism (by-passing PDC reaction) [18] , [20] , [21] , [22] , [23] , [24] , [25] (ii) supplementation of high doses of thiamine, presumably to meet the increased Km requirement for thiamine pyrophosphate associated with some PDHA1 mutations and/or for enzyme stability [26] , [27] , [28] , [29] , [30] , [31] , and (iii) administration of dichloroacetate which is known to inhibit PDH kinases decreased blood cerebrospinal fluid lactate concentrations in a large number of PDC-deficient children [19] , [32] . Beneficial effect of phenylbutyrate on residual ‘active’ PDC activity was shown in skin fibroblast cell lines from PDC-deficient patients caring PDHA1 missense mutations [33] , [34] .…”
Section: Introductionmentioning
confidence: 99%
“…Unfortunately, none of these approaches have been evaluated in a controlled manner with a sufficient number of subjects or duration to establish their efficacy. Several studies suggest that an early postnatal implementation of a ketogenic diet severely restricted in its carbohydrate content may be beneficial; however, this remains to be further evaluated [18] , [20] , [21] , [22] , [36] . The efficacy of ketogenic substrates was tested using a zebrafish model for PDC deficiency (due to deletion of the dihydrolipoamide acetyltransferase gene, Dlat ) [36] .…”
Section: Introductionmentioning
confidence: 99%
“…In contrast to other possible causes of neonatal lactic acidosis, there is currently no fully satisfying treatment available for patients with PDH deficiency [ 5 ]. However, ketogenic diet (KD) has been shown to positively influence the outcome of patients with PDH deficiency as it provides an alternative energy source and thereby bypasses the detrimental energy deprivation [ 2 , 6 ]. In case PDH deficiency underlies neonatal lactic acidosis, early initiation of KD supposedly correlates with improved long-term outcome [ 7 ].…”
Section: Introductionmentioning
confidence: 99%