Cripps D J, Goldfarb S S
Br J Dermatol. 1978 Mar;98(3):349-54. doi: 10.1111/j.1365-2133.1978.tb06163.x.
Observations on liver pathology and function were made on 12 patients with erythropoietic protoporphyria (EPP). Needle liver biopsies in 3 of 11 patients showed mild portal or periportal fibrosis without evidence of abnormal liver function. One patient (Case 12), died from hepatic cirrhosis and failure at the age of 11 years, the youngest yet reported. He was not suspected of pending liver disease until 3 months before death. The quantity of protoporphyrin present in his liver was approximately 5.75% of total liver weight. Fatal liver disease in EPP has occurred in 13 patients, 7 male and 6 female, in which the mean age of death was 38 years. The pathology of the liver in EPP is characteristic and is described.
对12例红细胞生成性原卟啉病（EPP）患者的肝脏病理和功能进行了观察。11例患者中的3例经肝脏穿刺活检显示轻度门脉或门脉周围纤维化，但肝功能无异常。1例患者（病例12），11岁时死于肝硬化和肝功能衰竭，这是迄今报道的最年轻病例。直到死亡前3个月才怀疑他患有潜在的肝脏疾病。其肝脏中存在的原卟啉量约占肝脏总重量的5.75%。EPP患者发生致命性肝脏疾病的有13例，其中男性7例，女性6例，平均死亡年龄为38岁。描述了EPP患者肝脏的特征性病理表现。
