淀粉样变性

Objective To analyze the clinical characteristics of patients with cardiac amyloidosis.

目的 探讨心脏淀粉样变性的临床特点。

Methods Clinical features of 25 patients with primary systemic amyloidosis admitted to our hospital since 1991 were retrospectively analyzed.

回顾性分析了本院1991年以来收治的25例原发性系统性淀粉样变性患者的临床特征。

Congo red dye test was positive and pathological consequence was hepatic and renal amyloidosis.

肝穿及肾穿病理见大量粉红色淀粉样物质沉积，刚果红染色，病理结论为肝、肾淀粉样变性。

Amyloidosis cutis dyschromica is a rare distinct type of primary cutaneous amyloidosis characterized by the presence of widespread hypopigmented as well as hyperpigmented macules. We herein report a 25-year-old male showing diffuse hyperpigmentation as with hypopigmented spots over his whole body but sparing his lace, hands and feet from the age of 8 years.

色素异常性皮肤淀粉样变性症是原发性皮肤淀粉样变性症的一个罕见而独特的亚型，其特徵是在皮肤出现广泛的色素沉著及色素脱失的斑点；我们在此报告一位25岁男性自八岁起在全身皮肤出现广泛的色素沉著及色素脱失约斑点，病灶并未侵犯其脸部、手掌、及脚掌。

In patients with biopsy-proven cardiac amyloidosis, late gadolinium enhancement frequently occurs in a peculiar pattern. On the basis of the gold standard, EMB, noninvasive CMRI can be used to diagnose or rule out cardiac amyloidosis with good sensitivity and excellent specificity in a clinical routine setting.

在心肌活检证实为淀粉样变性的患者中，钆延迟增强扫描常常会以特异的方式出现，在此金标准基础上，EMB和非侵入性CMRI可用于诊断或排除心肌淀粉样变性，仅有较高的敏感性和优越的特异性。

Amylin plays a critical role in islet amyloidosis and in the deelopment of β-cell dysfunction in patients with diabetes; howeer, the inolement of amylin in renal amyloidosis has not been studied.

胰淀素在糖尿病患者的胰岛淀粉样变性及β-细胞机能异常过程中起关键作用；然而尚未研究过胰淀素在肾脏淀粉样变性中的作用。

Since the heart, kidney, liver and nervous system are the most commonly involved organs, amyloidosis should be considered if any clinical manifestations of hydremic nephritis, cardiac and hepatic disease, and abnormalities of the nervous system are found.

心脏、肾脏、肝脏及神经系统是淀粉样变性相对常见的累及部位，对于病因不明肾病综合征患者、任何无法解释心脏、肝脏及神经系统症状的患者都应考虑到淀粉样变性。

Amyloidosis is characterized by slow deposition over years of increasing amounts of an amorphous proteinaceous material in one or more tissues.

图示淀粉样变性，特征是不定形的蛋白性质物质多年慢慢沉积在一个或更多的组织中，见在暗红心肌纤维间有浅粉红色淀粉样沉积物。

Liver or kidney aspirate examination and specific dyeing is necessary in patients suspected with hepatic amyloid degeneration.

结论对临床上高度怀疑为肝淀粉样变性的患者，作肝肾活体组织检查及特异性组织学染色，可证实为肝淀粉样变性。

We aimed to reassess renal amyloidosis in kidney biopsies with a focus on possibly misclassified or unclassified cases and changes in the prevalence of different amyloid types.

本研究的目的就是再评价肾活检标本中的淀粉样变性，重点关注那些可能被错误分类或无法分类的病例，以及不同类型淀粉样物发病率的变化。

With Death guitarist Schuldiner adopting vocal duties, the band made a major impact on the scene.

随着死亡的吉他手Schuldiner接受主唱的职务，乐队在现实中树立了重要的影响。

But he could still end up breakfasting on Swiss-government issue muesli because all six are accused of nicking around 45 million pounds they should have paid to FIFA.

不过他最后仍有可能沦为瑞士政府&议事餐桌&上的一道早餐，因为这所有六个人都被指控把本应支付给国际足联的大约4500万英镑骗了个精光。