Jensen N F, Fiddler D S, Striepe V
Department of Anesthesiology, University of Iowa College of Medicine, Iowa City 52242.
Anesth Analg. 1995 Mar;80(3):591-9. doi: 10.1097/00000539-199503000-00028.
Four hereditary types of porphyria are now classified as acute porphyrias. Enzymatic defects result in accumulation of porphyrin precursors (usually ALA and PGB). The quantity of these precursors may be normal or slightly increased in latent periods but increase to toxic levels during a porphyric crisis. Iatrogenic induction of ALA synthetase by administration of certain triggers (classically barbiturates) is only one of several factors which contribute to porphyric crisis. Signs and symptoms of acute porphyric attack consist primarily of neurologic dysfunction, which occurs secondary to neurotoxicity of ALA or diminished intraneuronal heme levels. Appropriate anesthetic management of porphyria requires knowledge of the type of porphyria (acute vs non-acute), assessment of latent versus active (crisis) phase, awareness of clinical features of porphyric attack, and knowledge of safe pharmacologic intervention.
目前，四种遗传性卟啉病被归类为急性卟啉病。酶缺陷导致卟啉前体（通常是δ-氨基-γ-酮戊酸和卟胆原）积累。在潜伏期，这些前体的量可能正常或略有增加，但在卟啉危象期间会增加到有毒水平。通过给予某些诱因（经典的是巴比妥类药物）医源性诱导δ-氨基-γ-酮戊酸合成酶只是导致卟啉危象的几个因素之一。急性卟啉发作的体征和症状主要包括神经功能障碍，这是由于δ-氨基-γ-酮戊酸的神经毒性或神经元内血红素水平降低继发所致。对卟啉病进行适当的麻醉管理需要了解卟啉病的类型（急性与非急性）、评估潜伏期与活动期（危象）、了解卟啉发作的临床特征以及安全的药物干预知识。
