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【medical-news】硬皮病的未解之谜
In Brief:
Scleroderma, an autoimmune disease, tends to afflict middle-age women and can affect many parts of the body, inside and out.
Lung disease, the biggest killer of scleroderma patients, is the main focus of research today..
Doctors have a growing arsenal of proven and potential treatments, some of which are risky and the subjects of current research, including stem cell transplants and powerful but toxic cancer drugs. 本人已认领该文编译，48小时后若未提交译文，请其他战友自由认领。 The Unfolding Mystery of Scleroderma
硬皮病的未解之谜
In Brief:
摘要：
Scleroderma, an autoimmune disease, tends to afflict middle-age women and can affect many parts of the body, inside and out.
硬皮病，一种自身免疫疾病，多发于中年妇女，可以影响到躯体内外的多个部位。
Lung disease, the biggest killer of scleroderma patients, is the main focus of research today..
肺病——硬皮病患者的最大杀手——成为近年的主要研究焦点。
Doctors have a growing arsenal of proven and potential treatments, some of which are risky and the subjects of current research, including stem cell transplants and powerful but toxic cancer drugs.
对于硬皮病，医生拥有了越来越多确诊和有潜在治疗作用的手段，其中部分具有一定的风险性，近期的研究对象包括干细胞移植和强效但有毒性的肿瘤药物。
Like many autoimmune ailments, scleroderma remains a great unknown. Despite decades of research, the cause of this rare and complicated disease has yet to be discovered. But the good news is that doctors have a pretty clear understanding of how scleroderma progresses — a natural history, they call it — and are better than ever at extending and easing their patients’ lives.
和许多自身免疫性疾病一样，硬皮病还是一个很大的谜团。尽管经过了数十年的研究，这种罕见的复杂疾病病因仍不清楚。但好在医生对于硬皮病的发展过程——被称为自然过程——了解比较充分，而且在延长患者生命并提高生存质量的方法上有了长足的发展。
“Lots of patients and lots of doctors used to consider it a ‘black box’ disease, a complete mystery, with little that could be done,” said Dr. Philip J. Clements of the University of California, Los Angeles, who is a scleroderma specialist. “Now there’s a body of evidence that tells us what to watch out for, and when.”
“许多的患者和医生曾将其看作是一个神秘的疾病，完全是个未解之谜，而且对它束手无策，”洛杉矶加州大学的Dr. Philip J. Clements说，他是一位硬皮病专家。“而现在有大量的证据可以告诉我们在什么阶段我们需要提防哪些问题。”
Experts now know, for example, that the gradual hardening of tissues and blood vessels that is a hallmark of scleroderma usually starts on the hands and face, with skin thickening, pitted scars and cool, pale fingertips among the earliest symptoms. Damage can then progress inward to internal organs, though the course varies widely from patient to patient. Of the 10,000 cases diagnosed among Americans each year, mainly women, a small subset will die quickly. But many others are able to manage their condition with a variety of treatments and have normal life expectancies.
例如，专家们现在知道，逐渐硬化的组织和血管作为硬皮病的特征，常常在手部和面部开始发生，伴随着皮肤变厚、出现凹痕和变凉、指端苍白等早期症状。损害过程随后逐渐向深部器官发展，但该过程在不同病人表现出较大差异。美国每年有10000例左右确诊患者，多数为女性，其中一小部分会迅速死亡。但是其他多数人可以通过多种治疗手段控制病程并享有通常的预期寿命。
Doctors also now know that if a patient’s internal organs are going to be affected as well as the skin, that is likely to happen in the first four or five years of the disease. So early diagnosis and close monitoring of the heart, lungs and kidneys are vitally important.
医生现在也知道了如果患者的内脏器官将象皮肤一样受到侵袭，这一过程一般会在疾病的前4到5年发生。因此早期确诊和对心脏、肺和肾脏的密切监测是性命攸关的。
They have also learned that steroids, once viewed as a cure-all for immune disorders, can worsen the effects of scleroderma, especially in the kidneys, and should be used with caution.
他们也了解了曾一度被认为是免疫疾病万灵丹的类固醇类可能是硬皮病的症状更加恶化，特别是对肾脏，因此应该慎用。
“Learning which drugs to avoid was itself a big step,” said Dr. John Varga, the Gallagher Professor of Medicine at Northwestern University and chairman of the Medical Advisory Board for the Scleroderma Foundation, a nonprofit group that sponsors research and support for patients and families.
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作者:admin@医学,生命科学 2010-10-30 05:11
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