Corino‐andrade disease (familial amyloidotic polineuropathy type I) in Spain: Urological and andrological disorders

Corino‐andrade disease (familial amyloidotic polineuropathy type I) in Spain: Urological and andrological disorders
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西班牙的 Corino‐andrade 病(I 型家族性淀粉样变神经病):泌尿科和男科疾病

DOI:
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发表时间:
1997
影响因子:
2
通讯作者:
M. P. Albacete
M. P. Albacete
中科院分区:
医学3区
文献类型:
--
作者:
G. Hita Villaplana;E. H. Rosino;P. L. Cubillana;L. Egea;P. M. Pertusa;M. P. Albacete

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在我们医院,我们跟踪了一组家族性淀粉样多发性神经病(FAP),I型患者。这种疾病的特征是进行性敏感性运动和自主性多发性神经病。FAP I的淀粉样纤维含有突变的甲状腺素运载蛋白(TTR)分子。超过90%的TTR产生发生在肝脏中。因此,肝移植治疗已被证明是有用的。我们所有的病人都接受了这种治疗。
In our hospital, we have followed a group of patients with familial amyloidotic polyneuropathy (FAP), type I. This disease is characterized by a progressive sensitive‐motor and autonomic polyneuropathy. The amyloid fibrils of FAP I contain a mutant transthyretin (TTR) molecule. More than 90% of TTR production occurs in the liver. Thus, therapy with liver transplantation has proved useful. All our patients received this treatment.
DOI: 10.1016/s0021-9258(17)39100-7
发表时间: 1985-09
期刊: The Journal of biological chemistry
影响因子: --
作者:
D. Soprano;J. Herbert;K. Soprano;E. Schon;D. Goodman
通讯作者: D. Soprano;J. Herbert;K. Soprano;E. Schon;D. Goodman