Gregor A, Kostrzewska E, Tarczyńska-Nosal S, Stachurska H
Poradni i Pracowni Badań nad Porfiria Instytutu Hematologii i Transfuzjologii w Warszawie.
Pol Tyg Lek. 1994;49(12-13):284-6.
Fluorescence spectra of plasma porphyrin were measured in 6 patients with the acute intermittent porphyria (AIP), 30 patients with variegate porphyria (PV), 2 patients with hereditary coproporphyria, 2 patients with porphyria cutanea tarda, and in 6 patients with erythropoietic protoporphyria (EPP). It was found that the excitation and emission wavelengths at which maximum fluorescence is seen may help to diagnose and differentiate PV and EPP. In patients with AIP spectrum characteristic for porphyria of such a type was noted in all patients during the attack of disease and in only 33% of patients in remission. Fluorescence spectrum was normal in asymptomatic family members. In variegate porphyria spectrum with a characteristic maximum of fluorescence was noted in all patients during an attack and remission, and 62% of the asymptomatic family members.
对6例急性间歇性卟啉病(AIP)患者、30例混合型卟啉病(PV)患者、2例遗传性粪卟啉病患者、2例迟发性皮肤卟啉病患者以及6例红细胞生成性原卟啉病(EPP)患者的血浆卟啉荧光光谱进行了测量。结果发现,出现最大荧光的激发和发射波长可能有助于诊断和区分PV和EPP。在AIP患者中,所有患者在疾病发作期间均呈现出该类型卟啉病的光谱特征,而在缓解期仅有33%的患者呈现此特征。无症状家庭成员的荧光光谱正常。在混合型卟啉病中,所有发作期和缓解期患者以及62%的无症状家庭成员均呈现出具有特征性最大荧光的光谱。