Porphyrin Patterns in Different Types of Porphyria

[摘要] Porphyrins from 38 cases of different types of porphyria were separated chromatographically and analyzed. Patients with different types of porphyria eliminated porphyrins with different patterns. Simple paper chromatography of the methyl ester of the total porphyrin of a urine sample revealed a chromatographic pattern of the porphyrins characteristic of the type of porphyria. The relative abundance of the heptacarboxylic and uroporphyrins in a sample also differentiated porphyria cutanea tarda from acute intermittent or congenital porphyria. The isomer compositions of the porphyrins were also disclosed. While one type of isomer might dominate one type of porphyria, the magnitude of predominance was by no means unanimous, or even reversed, among the porphyrins from uro- through the intermediates to coproporphyrins. For example, in cutanea tarda, Type I isomer dominates uro- and pentacarboxylic porphyrins, and Type Ill isomer dominates the hepta-, hexa- and coproporphyrins. The complexity of the enzymatic reaction involved in the decarboxylation of the porphyrins is thus indicated.

[学科分类] 过敏症与临床免疫学