THE LIVER IN PROTOPORPHYRIA
THE LIVER IN PROTOPORPHYRIA
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DOI：
10.1002/hep.1840080235
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发表时间：
1988-03-01
期刊：
影响因子：
13.5
通讯作者：
BLOOMER, JR
中科院分区：
文献类型：
--
作者：
BLOOMER, JR
1961年，Magnus和同事（1）报告了一名35岁男性的病例，他在暴露于阳光下后皮肤严重瘙痒和肿胀长达27年。尿中卟啉和卟啉衍生物的排泄正常，但红细胞和粪便中含有大量的原卟啉。作者建议将这种“迄今未描述的红细胞生成性疾病”命名为红细胞生成性原卟啉症。《柳叶刀》是一本发行广泛的杂志，几位胃肠病学家/肝病学家可能看到了文章的标题。有些人甚至可能读了总结，只是松了一口气，这不是一种卟啉症，它将进入不明腹痛的鉴别诊断。此外，肝脏不是代谢紊乱的部位，这种紊乱将被归类为红细胞生成性疾病，而不是肝脏疾病。然而，乌云很快就出现在地平线上。1963年，Porter和Lowe（2）报告了一例6岁男性患者，其临床和生化特征为原卟啉症，同时伴有肝脾肿大和肝脏生化异常。在脾切除术时，他被发现有肝硬化。两年后，Cripps和Scheuer（3）描述了5例原卟啉症患者肝活检标本的组织学发现。四个活检显示局灶性沉积的色素含有原卟啉，和门静脉纤维化存在于两个。雨终于在1968年降临，当时巴恩斯和同事描述了一名42岁的男子，他有长期的光敏性病史，出现黄疸和肝肿大，随后迅速恶化并死亡。验尸报告显示病人患有原卟啉症。
In 1961, Magnus and coworkers (1) reported the case of a 35-year-old man who for 27 years had had intense itching and swelling of his skin after exposure to sunlight. Urinary excretion of porphyrins and porphyrin precur-sors was normal, but the red blood cells and stool contained increased amounts of protoporphyrin. The authors proposed the name erythropoietic protoporphyria for this “hitherto undescribed erythropoietic condition.” t Lancet is a widely distributed journal, and several gastroenterologists/hepatologists probably saw the title of the article. Some may have even read the summary, only to breathe a sigh of relief that this was not a type of porphyria which would enter into the differential diagnosis of obscure abdominal pain. Moreover, the liver was not the site of the metabolic disturbance, and the disorder would be classified as erythropoietic and not hepatic.Dark clouds soon began to appear on the horizon, however. In 1963, Porter and Lowe (2) reported a 6-yearold male with clinical and biochemical features of protoporphyria who also had hepatosplenomegaly and abnormal liver biochemistries. At the time of splenectomy, he was found to have a cirrhotic liver. Two years later, Cripps and Scheuer (3) described the histological findings in liver biopsy specimens from five patients with protoporphyria. Four of the biopsies showed focal deposits of pigment containing protoporphyrin, and portal fibrosis was present in two. The rain finally came in 1968, when Barnes and coworkers (4) described a 42-year-old man with a long history of photosensitivity who developed jaundice and hepatomegaly, followed by a rapid downhill course and death (4). Postmortem examination indicated that the patient had protoporphyria.