戈谢病中的葡萄膜炎。
Uveitis in Gaucher disease.
作者信息
Dweck Altoon, Rozenman Jaacov, Ronen Suzy, Zimran Ari, Elstein Deborah
机构信息
Gaucher Clinic, Shaare Zedek Medical Center, Jerusalem, Israel.
出版信息
Am J Ophthalmol. 2005 Jul;140(1):146-7. doi: 10.1016/j.ajo.2004.12.081.
PURPOSE
Chronic uveitis has been previously reported in a patient with Gaucher's disease and improved with enzyme replacement therapy. This report describes the course of uveitis in two other patients with type I Gaucher's disease.
DESIGN
Observational case reports.
METHODS
Review of all patients in a large referral clinic for Gaucher's disease for incidence of uveitis.
RESULTS
Two patients with uveitis among 527 patients (0.4%) were found. Each had a different clinical outcome from the other and from the patient cited in the literature.
CONCLUSIONS
Both patients have mild Gaucher's disease, but one patient has uveitis well controlled for 10 years solely by local corticosteroids, whereas the other has suffered progression of uveitis despite enzyme replacement therapy.
目的
先前有报道称一名戈谢病患者患有慢性葡萄膜炎，经酶替代疗法后病情改善。本报告描述了另外两名Ⅰ型戈谢病患者的葡萄膜炎病程。
设计
观察性病例报告。
方法
回顾一家大型戈谢病转诊诊所所有患者的葡萄膜炎发病率。
结果
在527例患者中发现2例患有葡萄膜炎（0.4%）。这两名患者的临床结局彼此不同，也与文献中提及的患者不同。
结论
两名患者均患有轻度戈谢病，但其中一名患者仅通过局部使用皮质类固醇，葡萄膜炎病情得到良好控制达10年，而另一名患者尽管接受了酶替代疗法，葡萄膜炎仍有进展。
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