Abstract
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.
Highlights
Porphyria is a group of hereditary metabolic defects in heme biosynthesis that involves eight functionally interlocked enzymes required for the conversion of glycine and succinyl-coenzyme A to heme [1]
The dietary pattern observed among porphyria patients was in line with current dietary trends
Porphyria patients are not required to follow a special diet and recommendations are based on prevailing dietary guidelines for the general population
Summary
Porphyria is a group of hereditary metabolic defects in heme biosynthesis that involves eight functionally interlocked enzymes required for the conversion of glycine and succinyl-coenzyme A to heme [1]. The selective blockage at specific enzymatic steps results in the overproduction and accumulation of heme intermediates such as 5-aminolevulinic acid (ALA), porphobilinogen (PBG) and uro, copro, or proto porphyrins. Porphyrias are classified as hepatic or erythropoietic according to the major site of expression of enzyme deficiency and overproduction of heme precursors [6]. Increased calorie intake has been reported to be negatively correlated with urinary PBG levels in AIP patients suggesting that the total energy intake affects the biochemical disease activity [10]. The changes in diet can alter the excretion of porphyrin-precursors in AHP patients. Especially considering the lack of toxicity of ALA and PBG [16], it should not be concluded that changes in diet are capable of affecting the clinical disease activity
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