Nutritional assessment of patients affected by porphyria variegata.

ABSTRACT

AIMS: To describe the nutritional status and dietary practices of patients affected by porphyria variegata, paying special attention to the consumption of nutrients that may help or hinder the condition, and to assess the compliance with prevalent nutritional recommendations. METHODS: Cross-sectional study. 24 individuals affected by porphyria variegata (16 females and 8 males; mean age 46.8 (SD 19.5) years) from the Balearic Islands (Spain) recruited through the Balearic Porphyria Association. Dietary questionnaires (7-day dietary record and a semi-quantitative food frequency questionnaire), socio-demographic, lifestyle and health status interviews and anthropometric measurements were carried out. RESULTS: According to current recommendations for the Spanish population and to specific recommendations for porphyria patients, the daily intake of saturated fat (13.2% of total energy), sugars (16.9% of total E), cholesterol (387 mg) and alcoholic beverages (1.1 servings per day) were too high whereas the consumption of total carbohydrates (43.5% of total E), vitamin E (69.5% of RDI), beta-carotene (63.1% of RDI) and vitamin D (42.4% of RDI) was lower than recommended. CONCLUSIONS: Dietary pattern observed among porphyria variegata patients was in line with current dietary trends in the Balearic Islands. Nutritional recommendations to these individuals for the management of porphyria are poorly met. It is necessary to translate these recommendations into food-based dietary guidelines based on prevailing dietary patterns.