Brugsch J, Müller H
MMW Munch Med Wochenschr. 1979 Mar 30;121(13):457-8.
On the basis of many years of examination and supervision of 26 patients with acute intermittent porphyria three different types of excretion behavior in the urine can be distinguished. 1. After porphyric crises the urinary porphyrin content is completely normalized. 2. Even after the crisis the excretion levels for porphyrins remain very high. 3. The urinary porphyrins return to normal levels but the increases in precursors such as delta-aminolevulinic acid and porphobilinogen still persist. 4. In the diagnosis true acute coproporphyria and increased of excretion of precursors must be differentiated as well as increased coproporphyrin resulting from liver diseases, exogenic, toxic influences and secondary disorders of porphyrin metabolism.
基于多年来对26例急性间歇性卟啉病患者的检查和监测，可区分出三种不同类型的尿液排泄行为。1. 卟啉病发作后，尿卟啉含量完全恢复正常。2. 即使在发作后，卟啉的排泄水平仍非常高。3. 尿卟啉恢复到正常水平，但δ-氨基乙酰丙酸和胆色素原等前体物质的增加仍然持续存在。4. 在诊断中，必须区分真正的急性粪卟啉病以及前体物质排泄增加的情况，还有由肝脏疾病、外源性毒性影响和卟啉代谢继发性紊乱导致的粪卟啉增加。
