Mooyaart B R, de Jong G M, van der Veen S, Driessen L H, Beukeveld G J, Grond J, Gips C H
Dermatologica. 1986;173(3):120-30. doi: 10.1159/000249233.
A standardized hepatological investigation was performed in 9 unselected patients with erythropoietic protoporphyria (EPP). The aim of this study was to detect early liver involvement due to EPP and to determine the significance of several diagnostic procedures. Scintigraphy revealed slight enlargement of liver and spleen in all cases. Light microscopic examination of liver tissue in 7 patients showed protoporphyrin deposition in 4 and signs of fibrosis in 3 cases. Cirrhosis was not found. Electron microscopical examination (EM) of all 7 cases was negative and needle-shaped crystals were not found. Therefore we regard EM of little diagnostic value in the detection of early liver involvement. The literature on fatal and asymptomatic cases of liver involvement in EPP is discussed with emphasis on possible predictive and provocative factors. A proposal for EPP patients intending to reduce possible risk factors is made.
对9例未经挑选的红细胞生成性原卟啉病（EPP）患者进行了标准化的肝脏检查。本研究的目的是检测EPP所致的早期肝脏受累情况，并确定几种诊断程序的意义。闪烁扫描显示所有病例肝脏和脾脏均有轻度肿大。7例患者肝脏组织的光镜检查显示，4例有原卟啉沉积，3例有纤维化迹象。未发现肝硬化。所有7例的电子显微镜检查（EM）均为阴性，未发现针状晶体。因此，我们认为EM在检测早期肝脏受累方面诊断价值不大。本文讨论了EPP患者肝脏受累的致命和无症状病例的文献，重点讨论了可能的预测和激发因素。并针对EPP患者提出了降低可能危险因素的建议。
