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首页> 外文期刊>Amyloid: the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis >Recurrent hepatic hematoma due to familial lysozyme amyloidosis resolves with conservative management
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Recurrent hepatic hematoma due to familial lysozyme amyloidosis resolves with conservative management

机译:家族性溶菌酶淀粉样变性引起的复发性肝血肿可通过保守治疗解决

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摘要

Lysozyme (ALys) amyloidosis (Online Mendelian Inheritance in Man 153450), first identified in 1993 by Pepys et al., is a very rare autosomal dominant hereditary non-neuropathic amyloidosis [1]. Lysozyme, which is the amyloidogenic precursor, is a ubiquitous bacteriolytic enzyme that catalyzes the hydrolysis of certain mucopolysaccharides of bacterial cell walls. It is synthesized by hepatocytes but also macrophages throughout the body and is found in liver, spleen, lung, kidney, plasma, saliva, and tears. The phenotype of this disease is variable and may be complicated by spontaneous rupture of hepatic hematoma which can be life threatening [2-5].
机译:溶菌酶(ALys)淀粉样变性病(在线孟德尔遗传学(Man 153450),1993年由Pepys等人首次发现)是一种非常罕见的常染色体显性遗传性非神经性淀粉样变性病[1]。溶菌酶是产生淀粉样蛋白的前体,是一种普遍存在的溶菌酶,可催化细菌细胞壁某些粘多糖的水解。它是由肝细胞合成的,也是全身巨噬细胞合成的,存在于肝,脾,肺,肾,血浆,唾液和眼泪中。该疾病的表型是可变的,并可能因可能威胁生命的肝血肿的自然破裂而复杂化[2-5]。

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