Diet And Porphyria

[ironjustice]

Antioxidants restore protoporphyrinogen oxidase in variegate porphyria
patients
Miguel D. Ferrer1, Pedro Tauler1, Antoni Sureda1,2, Clara Palacín3,
Josep A. Tur1,2, Antoni Pons1,2,
Published online: 20 APR 2013
European Journal Of Clinical Investigation

Abstract
Background
Variegate porphyria (VP) is the result of decreased protoporphyrinogen
oxidase (PPOX) activity and results in the accumulation of porphyrins
and porphyrin precursors.
Our aims were to analyse the basal antioxidant defences and oxidative
damage markers and the effects of a diet supplementation with vitamins
E and C on the oxidant/antioxidant status and PPOX gene expression in
lymphocytes of variegate porphyria (VP) patients.
Materials and methods
Twelve women affected by VP and 12 control women participated in a
randomized and double-blind crossover study.
Each participant took either 50 mg/day vitamin E and 150 mg/day
vitamin C or a placebo for 6 months.
Results
Lymphocyte PPOX gene expression, together with catalase and
glutathione peroxidase activities, was reduced in VP women.
No differences were observed in the levels of malondialdehyde and
protein carbonyl derivatives.
Stimulated lymphocyte H2O2 production was higher in porphyric women.
Supplementation with antioxidant vitamins increased PPOX expression in
VP patients.
Glutathione reductase (GRd) and superoxide dismutase (SOD) activities
were higher in the treatment groups.
Conclusions
Lymphocytes from VP patients show reduced PPOX expression and present
a greater susceptibility to producing H2O2 and impaired H2O2
detoxifying mechanisms.
Supplementation with vitamins E and C restores PPOX expression in VP
patients and enhances GRd and SOD activity, suggesting the potential
benefits of a diet rich in vitamins E and C in these patients.

Keywords:Antioxidants;lymphocytes;oxidative stress;variegate porphyria

DOI: 10.1111/eci.12091

© 2013 Stichting European Society for Clinical Investigation Journal
Foundation. Published by John Wiley & Sons Ltd

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High-dose vitamin therapy as prophylaxis against porphyria cutanea
uremica.
Wimmershoff F, Gardlo K, Bolsen K, Ruzicka T, Fritsch C
Hautarzt. 2006 Mar ; 57(3): 228-36

50 patients with chronic renal failure undergoing hemodialysis with
or
without porphyria cutanea tarda (PCT)-like skin changes were
investigated. The total porphyrin amount in erythrocytes, plasma and
dialysate and the distribution of porphyrin metabolites in plasma and
dialysate were measured. In plasma, the group of patients with skin
changes (referred as PCU = porphyria cutanea uremica) showed
significantly increased uroporphyrin levels as compared to the
non-symptomatic group. In addition, significant differences
concerning
the ratio uro-/coproporphyrin in plasma were shown: non-symptomatic
patients with 0.87, as opposed to the PCU group with 3.7.
Considerable
differences between the level of vitamin ingestion were identified
between the groups. Patients with PCU took distinctly less vitamins
C,
E and B than patients without symptoms.

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