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词汇 Erythropoietic uroporphyria
分类 英语词汇 英语翻译词典
释义

Erythropoietic uroporphyria

中文百科

紫质症 Porphyria

(重定向自Erythropoietic uroporphyria)

紫质症英语:Porphyria,又称噗瑳症紫质症吡咯紫质症)是一组因为人体内的紫质(Porphyrin)等物质异常累积所造成的身体病变。人体以紫质为前驱物,透过特定酶的催化作用来制造血基质,一旦酶无法正常作用,紫质等物质便会越积越多,最终引致紫质症。紫质症是一种罕见疾病,临床统计估计发生机率约30万分之1,目前仍无治愈方法,只能针对紫质症的病征进行治疗。

紫质症的成因有先天遗传也有后天造成,因此可广义的分类为急性或缓发性两类,又或根据紫质积聚的地方分类为肝脏性及皮肤性两类。两种分类的范围大致相同,但以遗传性病例为多,有研究显示此病和1号染色体异常有关。紫质症可引致皮肤或脑神经方面的问题。此外,在临床及病理学上亦有一种病征与紫质症完全相同,但病发因由完全不同的病症,称为假性紫质症或伪紫质症(pseudoporphyria)。这种假性的紫质沉着病征可以透过进行血浆或尿液胞体紫质检查分辨出来。

英语百科

Porphyria 紫质症

(重定向自Erythropoietic uroporphyria)
Heme synthesis—note that some reactions occur in the cytoplasm and some in the mitochondrion (yellow)
George III in his coronation robes
Mary Stuart c.1578.
Maria I of Portugal in a c. 1790s portrait attributed to Giuseppe Troni or Thomas Hickey.

The porphyrias( poor-FEAR-e-uh) are a group of rare diseases in which chemical substances called porphyrins accumulate with high metabolism. The body requires porphyrins to produce heme, which carries oxygen in the blood. But in the porphyrias, there is a deficiency (inherited or acquired) of the enzymes that transform the various porphyrins into others, leading to abnormally high levels of one or more of these substances. This manifests with neurological symptoms or skin problems, or occasionally both.

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