alport综合征眼部临床表现分析

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1、Alport综合征眼部临床表现分析作者：徐建敏，张士胜，张琼，周颖明，朱彩红，葛健，王玲【摘要】　　目的：总结Alport综合征的临床表现，尤其是眼部特征。方法：回顾性分析32例被确诊为Alport综合征患者的内科、耳鼻喉科和眼科检查结果。结果：患者30例(93.7%)有疾病家族史。所有患者均有不同程度的肾脏病变：18例(56.3%)有肾功能衰竭，4例(12.5%)肾功能不全，10例(31.3%)血尿。患者20例(62.5%)有感音神经性耳聋。患者13例(40.6%)有眼部异常表现，其中5例(15.6%)为典型性改变：前圆锥晶体3例

2、，黄斑周围斑点2例。结论：眼部异常不是Alport综合征诊断的必需条件，但因其典型的眼科表现应当引起眼科医师的注意，以便早期诊断治疗。【关键词】Alport综合征；前圆锥晶状体；黄斑斑点　　AbstractA20yearoldhealthyladywasadmittedforsepticarthritisintherightknee.Twoweeksaftertheonsetofjointpain,shecomplainedprogressiveblurringofvisioninbotheyes.Slitlampexamin

3、ationrevealedevidenceofinflammationinbothanteriorandposteriorsegments.Thereweremultiplechorioretinallesionsonbothfundi.Synovialfluidaspirationoftherightkneeandbloodculturegrew7Staphylococcusaureus.Sherespondedwelltotopicalandsystemicantibiotics.Herfinalvisualacuitywas6

4、/6inbotheyes.Thiscaseillustratesararepresentationofbilateralendogenousendophthalmitisinanimmunocompetentyoungpatientpresentingwithaconcurrentsepticarthritis.Earlydiagnosisandapromptmanagementcarryafavorablevisualprognosis.　　KEYWORDS:septicarthritis;endogenousendophtha

5、lmitis;immunocompetent　　INTRODUCTION　　Endogenousendophthalmitisisrelativelyarareconditionbutcarriessevereandpotentiallyblindingocularinfection.Itiscausedbyavarietyofinfectiousagents,namelybacteriaorfungalthatreachestheeyefromtheprimarysiteviaahaematogenousspread.Mening

6、itis,endocarditis,infectionofurinaryorgastrointestinaltractandwoundarethemostcommoncausesofendogenousendophthalmitis,whileotherlesscommonsourceincludespharyngitis,pneumonia,pyelonephritisandintraabdominalabscess[1].Septicarthritisisanuncommonprimarysourceofinfection[2]

7、.Wereportacaseofbilateral7endogenousendophthalmitiswithsepticarthritisofrightkneeastheprimarysourceofinfectioninayoungimmunocompetentlady.Earlydiagnosisandpromptmanagementisessentialtopreserveafavorablevisualoutcomeinthisuncommoncondition.　　CASEREPORT　　A20yearoldimmu

8、nocompetentladywasadmittedforswellingintherightkneeandfeverfortwoweeksduration.Shewasdiagnosedassepticarthritisinther