High-dose vitamin e lowers urine porphyrin levels in patients affected by porphyria cutanea tarda

Pinelli, A; Trivulzio, Silvio; Tomasoni, Livio; Bertolini, Boris; Pinelli, Giovanna

doi:10.1006/phrs.2002.0956

Cited by 13 publications

(5 citation statements)

References 24 publications

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“…Nox enzymes may be involved in the pathogenesis of PCT as it was discovered that the ROS, O 2 − and H 2 O 2 , are produced in PCT [17]. The ROS mediate the skin fibrosis in PCT and various antioxidants (vitamins C and E, lycopene, and beta-carotene) successfully inhibited porphyrin-induced cellular phototoxicity [17,77]. More research is needed to fully elucidate the involvement of Nox enzymes in the pathogenesis of PCT.…”

Section: The Role Of Nox In Skin Fibrosismentioning

confidence: 99%

NADPH oxidase enzymes in skin fibrosis: molecular targets and therapeutic agents

et al. 2013

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Fibrosis is characterized by the excessive deposition of extracellular matrix components eventually resulting in organ dysfunction and failure. In dermatology, fibrosis is the hallmark component of many skin diseases, including systemic sclerosis, graft versus host disease, hypertrophic scars, keloids, nephrogenic systemic fibrosis, porphyria cutanea tarda, restrictive dermopathy and other conditions. Fibrotic skin disorders may be debilitating and impair quality of life. There are few FDA-approved anti-fibrotic drugs; thus, research in this area is crucial in addressing this deficiency. Recent investigations elucidating the pathogenesis of skin fibrosis have implicated endogenous reactive oxygen species produced by the multicomponent nicotinamide adenine dinucleotide phosphate (NADPH) oxidase (Nox) enzyme complex. In this review we discuss Nox enzymes and their role in skin fibrosis. An overview of the Nox enzyme family is presented and their role in the pathogenesis of skin fibrosis is discussed. The mechanisms that Nox enzymes influence specific skin fibrotic disorders are also reviewed. Finally, we describe the therapeutic approaches to ameliorate skin fibrosis by directly targeting Nox enzymes with the use of statins, p47phox subunit modulators, or GKT137831, a competitive inhibitor of Nox enzymes. Nox enzymes can also be targeted indirectly via scavenging ROS with antioxidants. We believe that Nox modulators are worthy of further investigation and have the potential to transform the management of skin fibrosis by dermatologists.

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Section: The Role Of Nox In Skin Fibrosismentioning

confidence: 99%

NADPH oxidase enzymes in skin fibrosis: molecular targets and therapeutic agents

et al. 2013

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“…Substitution with high-dose vitamin C significantly lowered urinary eight carboxylised porphyrins in five patients [52]. However, proof of efficacy of vitamins in PCT is missing.…”

Section: Other Pharmacological Treatmentsmentioning

confidence: 99%

Therapy of porphyria cutanea tarda

Köstler¹,

Wollina²

2005

Expert Opinion on Pharmacotherapy

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Porphyria cutanea tarda (PCT) is the most common type of porphyria. There is an association of PCT with haemochromatosis, diabetes mellitus and hepatitis C infection. The basis of treatment of PCT consists of three elements: avoidance of triggering factors, iron depletion and porphyrin elimination. Alcohol and certain systemic medical drugs, such as oestrogens (or tuberculostatics), should be considered as triggering factors, and as far as possible, avoided. Other triggering factors, such as chronic haemodialysis in renal insufficiency, need a different approach. The hallmark in iron depletion is phlebotomy. Porphyrin elimination is achieved using low-dose chloroquin therapy. The treatment is safe and effective but has its limits in cases with haemochromatosis (HFE) gene mutations. Here iron depletion needs additional phlebotomy. In patients with chronic haemodialysis-associated PCT, chloroquine is ineffective. Erythropoietin, desferroxamine and small-volume phlebotomy have been employed to control the disease. Childhood PCT is very rare. No controlled studies are available, but published experience suggests that body weight-adapted chloroquine therapy or small volume phlebotomy might be useful.

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“…Also, Vitamin E is able to interact with the enzyme uroporphyrinogen decarboxylase involved in PCT to increase its activity. Patients treated with alpha-tocopherol (1 g/day) for one month showed a reduction in urinary porphyrins than that of untreated patients [86]. Szekely E. et al performed a randomized controlled study on 23 PCT patients treated with 200 mg/day of alpha-tocopherol acetate in conjunction with phlebotomy for eight weeks and demonstrated a decrease in porphyrins and an increase in sunlight exposure [87].…”

Section: Vitamin Ementioning

confidence: 99%

Nutrients and Porphyria: An Intriguing Crosstalk

Pierro

Granata

2020

IJMS

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Porphyria refers to a group of fascinating diseases from a metabolic and nutritional standpoint as it provides an example of how metabolic manipulation can be used for therapeutic purposes. It is characterized by defects in heme synthesis, particularly in the erythrocytes and liver. Specific enzymes involved in heme biosynthesis directly depend on adequate levels of vitamins and minerals in the tissues. Moreover, micronutrients that are required for producing succinyl CoA and other intermediates in the Krebs (TCA) cycle are indirectly necessary for heme metabolism. This review summarizes articles that describe the nutritional status, supplements intake, and dietary practices of patients affected by porphyria, paying special attention to the therapeutic use of nutrients that may help or hinder this group of diseases.

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High-dose vitamin e lowers urine porphyrin levels in patients affected by porphyria cutanea tarda

Cited by 13 publications

References 24 publications

NADPH oxidase enzymes in skin fibrosis: molecular targets and therapeutic agents

NADPH oxidase enzymes in skin fibrosis: molecular targets and therapeutic agents

Therapy of porphyria cutanea tarda

Nutrients and Porphyria: An Intriguing Crosstalk

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