急性卟啉病的发病机制。
Pathogenesis of acute porphyria.
作者信息
Yeung Laiwah A C, Moore M R, Goldberg A
机构信息
Faculty of Medicine, University of Alberta, Edmonton, Canada.
出版信息
Q J Med. 1987 May;63(241):377-92.
The pathogenesis of the clinical manifestations of acute porphyria has been considered in the light of their pathological changes and their aberrations of the haem biosynthetic pathway. These manifestations may be explained almost entirely upon a neurogenic basis. A number of hypotheses have been considered to explain the clinical, pathological and biochemical features. Of these hypotheses two seem more impressive: (i) the neurological manifestations may be explained by a deficiency of haem in neural tissues; (ii) the porphyrin precursor 5-aminolaevulinic acid (ALA) may have in addition specific pharmacological activity.
急性卟啉病临床表现的发病机制已根据其病理变化及血红素生物合成途径的异常进行了探讨。这些表现几乎完全可以用神经源性基础来解释。人们考虑了多种假说来解释其临床、病理和生化特征。在这些假说中，有两种似乎更令人关注：（i）神经组织中血红素缺乏可解释神经学表现；（ii）卟啉前体5-氨基酮戊酸（ALA）可能还具有特定的药理活性。
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