Tornaghi G, Vitali L
Minerva Med. 1979 Mar 10;70(12):831-44.
Porphyria is making increasing demands on the attention of clinicians and research worker. An account is given of hepatic forms, since these have recently come into prominence on account of recent advances in the understanding of their metabolic, diagnostic and therapeutic aspects. A description of the physiopathology of porphyrin metabolism is followed by an examination of the incidence, genetic features, aetiology, pathogenesis, pathological anatomy, symptomatology, diagnosis, prognosis, and treatment of each form. Particular attention is devoted to intermittent acute and cutanea tarda porphyria, since these are more commonly encountered in practice. Personal experience gathered in a large series of cases of cutanea tarda porphyria is presented.
卟啉症越来越需要临床医生和研究人员的关注。本文介绍了肝性卟啉症,因为鉴于最近在其代谢、诊断和治疗方面的理解取得进展,这些类型最近受到了关注。在描述卟啉代谢的生理病理学之后,对每种类型的发病率、遗传特征、病因、发病机制、病理解剖、症状学、诊断、预后和治疗进行了研究。特别关注间歇性急性卟啉症和迟发性皮肤卟啉症,因为这些在实践中更常见。本文介绍了在大量迟发性皮肤卟啉症病例中积累的个人经验。