alport综合征眼部临床表现分析

《alport综合征眼部临床表现分析》由会员上传分享，免费在线阅读，更多相关内容在应用文档-天天文库。

1、Alport综合征眼部临床表现分析：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，黄斑周围斑点2例。结论：眼部异常不是Alport综合征诊断的必

2、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oldhealthyladyittedforsepticarthritisintherightknee.Tpexaminationrevealedevidenceofinflammationinbothanteriorandposteriorsegments.Thereultiplechorioretinallesionsonbothfundi.Synovialfluidaspirationoftherightkneeandblood

3、culturegreicantibiotics.Herfinalvisualacuityitisinanimmunopetentyoungpatientpresentingptmanagementcarryafavorablevisualprognosis.　　KEYeningitis,endocarditis,infectionofurinaryorgastrointestinaltractandostmoncausesofendogenousendophthalmitis,onsourceincludespharyngitis,pneumonia,pyelonephritisandin

4、traabdominalabscess[1].Septicarthritisisanunmonprimarysourceofinfection[2].U6hourly.Sheunderission.Onedaylater,sheplainedblurredvisioninbotheyes.Itilddisfortandredness.Hoa,skindisease,underlyingmedicalillnessorconsumptionofimmunosuppressivemedications.Visualacuitypexaminationofbothanteriorandposter

5、iorsegmentsrevealedmoderateinflammation.Thereultiplesmallicexaminationrevealedalethargicandfebrilepatient.Herrightkneeandtenderinflexionposition.Otherjointsandsystemicexaminationsarkable.Thereyeliticchangesorgasshadoerouspuscells.Bothsynovialfluidandbloodculturesgrem/firsthour.Collagenscreeningsand

6、otherinfectiveide8hourlyinbotheyes,andaddedontointravenousciprofloxacin200mg12hourly.Sheshoaticclinicalimprovementaaintainedforanotheronemonth.Herfinalvisualacuitymaryofvisualouteinendogenousendophthalmitisrelatedtosepticarthritisreportedintheliterature19962009（略）　　DISCUSSION　　Septicarthritisdenot

7、esasaprimarysourceorconitantsiteofinfectioninendogenousendophthalmitis.Itaccounts8%ofendogenousendophthalmitisin2largecaseseries[1,2].Table1illustratesthepatientsriskfactorsandpotentialmicroorganismcausing