Abstract
The porphyrias are a group of disorders of heme metabolism that result from partial defects in the several enzymes that control heme biosynthesis. Accumulation of porphyrins or porphyrin precursors in several different patterns results from these defects and biochemically characterizes each specific syndrome. Patterns of cutaneous photosensitivity and associated systemic symptoms among the several porphyrias result from the types of porphyrins or precursors accumulated in each.
摘要
卟啉病是一组血红素代谢紊乱疾病，由控制血红素生物合成的几种酶的部分缺陷引起。这些缺陷导致卟啉或卟啉前体以几种不同模式蓄积，这在生化上表征了每种特定综合征。几种卟啉病中的皮肤光敏性模式及相关全身症状，是由每种疾病中蓄积的卟啉或前体类型所致。
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