Devars du Mayne J F, Deybach J C, Phung L, Likforman J, Tenhunen R, Cerf M, Nordmann Y
Presse Med. 1986 Oct 4;15(33):1673-6.
The effectiveness of 2 hematins administered by intravenous infusion was compared in acute intermittent porphyria. Judging from subjective symptoms (abdominal pain), clinical improvement was complete and constant. There was a rapid decrease in urinary excretion of porphyrins precursors, with a clearer response of delta-aminolevulinic acid than of porphobilinogen. Urine levels of uroporphyrin often returned to normal after two infusions. The drugs were very well tolerated. Provided it is administered early, before neurological complications develop, treatment with hematin completely relieves abdominal symptoms and suppresses most of the biochemical changes associated with hepatic porphyria.
在急性间歇性卟啉症中比较了两种通过静脉输注给药的血红素的有效性。从主观症状(腹痛)判断,临床改善是完全且持续的。卟啉前体的尿排泄量迅速下降,δ-氨基乙酰丙酸的反应比胆色素原更明显。经两次输注后,尿卟啉水平通常恢复正常。这些药物耐受性良好。如果在神经并发症出现之前尽早给药,血红素治疗可完全缓解腹部症状,并抑制与肝卟啉症相关的大多数生化变化。