| Arch Pediatr. 1999 Apr;6(4):421-6. |
| Leigh syndrome and leukodystrophy due to partial succinate dehydrogenase deficiency: regression with riboflavin.[Pubmed: 10230482] |
Succinate dehydrogenase (SDH) deficiency is rare. Clinical manifestations can appear in infancy with a marked impairment of psychomotor development with pyramidal signs and extrapyramidal rigidity.
METHODS AND RESULTS:
A 10-month-old boy developed severe neurological features, evoking a Leigh syndrome; magnetic resonance imaging showed features of leukodystrophy. A deficiency in the complex II respiratory chain (succinate dehydrogenase [SDH]) was shown. The course was remarkable by the regression of neurological impairment under treatment by riboflavine. The delay of psychomotor development, mainly involving language, was moderate at the age of 5 years.
CONCLUSIONS:
The relatively good prognosis of this patient, despite severe initial neurological impairment, may be due to the partial enzyme deficiency and/or riboflavine administration. |
| Food Chem Toxicol. 2014 May;67:65-71. |
| Riboflavine (vitamin B-2) reduces hepatocellular injury following liver ischaemia and reperfusion in mice.[Pubmed: 24560968] |
Riboflavine has been shown to exhibit anti-inflammatory and antioxidant properties in the settings of experimental sepsis and ischaemia/reperfusion (I/R) injury.
METHODS AND RESULTS:
We investigated the effect of riboflavine on normothermic liver I/R injury. Mice were submitted to 60 min of ischaemia plus saline or riboflavine treatment (30 μmoles/kg BW) followed by 6 h of reperfusion. Hepatocellular injury was evaluated by aminotransferase levels, reduced glutathione (GSH) content and the histological damage score. Hepatic neutrophil accumulation was assessed using the naphthol method and by measuring myeloperoxidase activity. Hepatic oxidative/nitrosative stress was estimated by immunohistochemistry. Liver endothelial and inducible nitric oxide synthase (eNOS/iNOS) and nitric oxide (NO) amounts were assessed by immunoblotting and a chemiluminescence assay. Riboflavine significantly reduced serum and histological parameters of hepatocellular damage, neutrophil infiltration and oxidative/nitrosative stress. Furthermore, riboflavine infusion partially recovered hepatic GSH reserves and decreased the liver contents of eNOS/iNOS and NO.
CONCLUSIONS:
These data indicate that riboflavine exerts antioxidant and anti-inflammatory effects in the ischaemic liver, protecting hepatocytes against I/R injury. The mechanism of these effects appears to be related to the intrinsic antioxidant potential of riboflavine/dihydroriboflavin and to reduced hepatic expression of eNOS/iNOS and reduced NO levels, culminating in attenuation of oxidative/nitrosative stress and the acute inflammatory response. |
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