中华实验眼科杂志
中華實驗眼科雜誌
중화실험안과잡지
CHINESE JOURNAL OF EXPERIMENTAL OPHTHALMOLOGY
2013年
6期
587-591
,共5页
张译心%戴艳丽%巩琰%黄厚斌%魏世辉
張譯心%戴豔麗%鞏琰%黃厚斌%魏世輝
장역심%대염려%공염%황후빈%위세휘
Leber遗传性视神经病变%基因携带者%视网膜神经纤维层厚度%黄斑%视网膜厚度%光学相干断层扫描%性别
Leber遺傳性視神經病變%基因攜帶者%視網膜神經纖維層厚度%黃斑%視網膜厚度%光學相榦斷層掃描%性彆
Leber유전성시신경병변%기인휴대자%시망막신경섬유층후도%황반%시망막후도%광학상간단층소묘%성별
Leber's hereditary optic neuropathy%Genetic carrier%Retinal nerve fiber layer thickness%Macula%Retinal thickness%Optical coherence tomography%Gender
背景 研究表明Leber遗传性视神经病变(LHON)的女性基因携带者眼底各象限视网膜神经纤维层厚度(RNFLT)有不同程度的增厚,但目前对其黄斑区视网膜厚度的变化却不清楚. 目的 应用光学相干断层扫描仪(OCT)测量LHON女性携带者RNFLT和黄斑区视网膜厚度的变化. 方法 纳入2011年9月至2012年10月在解放军总医院眼科就诊的经mtDNA检测确诊的来自5个家系的5例女性LHON患者及来自16个家系的18例女性携带者,并收集性别和年龄匹配的健康对照者25人25眼.所有入选者均行一般眼科检查和OCT检查,OCT扫描程序选择“视盘容积200×200”和“黄斑容积200×200”.记录视盘360°平均RNFLT、4个象限RNFLT、黄斑容积平均厚度及早期糖尿病视网膜病变治疗研究(ETDRS)栅格9个子区的黄斑区视网膜厚度,统计分析携带者与正常对照组和患者的差别.结果 与正常对照组相比,LHON女性携带者内子区颞侧、上方、鼻侧、下方黄斑区视网膜厚度均明显变薄,差异均有统计学意义(P=0.022、0.046、0.024、0.008),而黄斑容积平均厚度,中心子区和外子区颢侧、上方、鼻侧、下方黄斑区视网膜厚度以及平均、颞侧、上方、鼻侧、下方象限RNFLT的差异均无统计学意义(P=0.102、0.051、0.238、0.663、0.110、0.104、0.419、0.371、0.158、0.063、0.563).与LHON女性携带者相比,LHON女性患者除中心子区黄斑区视网膜厚度和鼻侧象限RNFLT差异均无统计学意义(P=0.388、0.580)外,黄斑容积平均厚度,内子区颞侧、上方、鼻侧、下方和外子区颢侧、上方、鼻侧、下方黄斑区视网膜厚度以及平均、颞侧、上方、下方象限RNFLT值均明显下降,差异均有统计学意义(P=0.000、0.000、0.000、0.007、0.002、0.002、0.000、0.000、0.040、0.000、0.016、0.000、0.000).结论 LHON女性携带者视盘周围RNFLT正常,但黄斑4个内子区黄斑区视网膜厚度显著变薄,中心子区和4个外子区黄斑区视网膜厚度正常.本研究首次发现LHON女性基因携带者黄斑区视网膜厚度异常,提示LHON黄斑部病变早于RNFLT的变化.
揹景 研究錶明Leber遺傳性視神經病變(LHON)的女性基因攜帶者眼底各象限視網膜神經纖維層厚度(RNFLT)有不同程度的增厚,但目前對其黃斑區視網膜厚度的變化卻不清楚. 目的 應用光學相榦斷層掃描儀(OCT)測量LHON女性攜帶者RNFLT和黃斑區視網膜厚度的變化. 方法 納入2011年9月至2012年10月在解放軍總醫院眼科就診的經mtDNA檢測確診的來自5箇傢繫的5例女性LHON患者及來自16箇傢繫的18例女性攜帶者,併收集性彆和年齡匹配的健康對照者25人25眼.所有入選者均行一般眼科檢查和OCT檢查,OCT掃描程序選擇“視盤容積200×200”和“黃斑容積200×200”.記錄視盤360°平均RNFLT、4箇象限RNFLT、黃斑容積平均厚度及早期糖尿病視網膜病變治療研究(ETDRS)柵格9箇子區的黃斑區視網膜厚度,統計分析攜帶者與正常對照組和患者的差彆.結果 與正常對照組相比,LHON女性攜帶者內子區顳側、上方、鼻側、下方黃斑區視網膜厚度均明顯變薄,差異均有統計學意義(P=0.022、0.046、0.024、0.008),而黃斑容積平均厚度,中心子區和外子區顥側、上方、鼻側、下方黃斑區視網膜厚度以及平均、顳側、上方、鼻側、下方象限RNFLT的差異均無統計學意義(P=0.102、0.051、0.238、0.663、0.110、0.104、0.419、0.371、0.158、0.063、0.563).與LHON女性攜帶者相比,LHON女性患者除中心子區黃斑區視網膜厚度和鼻側象限RNFLT差異均無統計學意義(P=0.388、0.580)外,黃斑容積平均厚度,內子區顳側、上方、鼻側、下方和外子區顥側、上方、鼻側、下方黃斑區視網膜厚度以及平均、顳側、上方、下方象限RNFLT值均明顯下降,差異均有統計學意義(P=0.000、0.000、0.000、0.007、0.002、0.002、0.000、0.000、0.040、0.000、0.016、0.000、0.000).結論 LHON女性攜帶者視盤週圍RNFLT正常,但黃斑4箇內子區黃斑區視網膜厚度顯著變薄,中心子區和4箇外子區黃斑區視網膜厚度正常.本研究首次髮現LHON女性基因攜帶者黃斑區視網膜厚度異常,提示LHON黃斑部病變早于RNFLT的變化.
배경 연구표명Leber유전성시신경병변(LHON)적녀성기인휴대자안저각상한시망막신경섬유층후도(RNFLT)유불동정도적증후,단목전대기황반구시망막후도적변화각불청초. 목적 응용광학상간단층소묘의(OCT)측량LHON녀성휴대자RNFLT화황반구시망막후도적변화. 방법 납입2011년9월지2012년10월재해방군총의원안과취진적경mtDNA검측학진적래자5개가계적5례녀성LHON환자급래자16개가계적18례녀성휴대자,병수집성별화년령필배적건강대조자25인25안.소유입선자균행일반안과검사화OCT검사,OCT소묘정서선택“시반용적200×200”화“황반용적200×200”.기록시반360°평균RNFLT、4개상한RNFLT、황반용적평균후도급조기당뇨병시망막병변치료연구(ETDRS)책격9개자구적황반구시망막후도,통계분석휴대자여정상대조조화환자적차별.결과 여정상대조조상비,LHON녀성휴대자내자구섭측、상방、비측、하방황반구시망막후도균명현변박,차이균유통계학의의(P=0.022、0.046、0.024、0.008),이황반용적평균후도,중심자구화외자구호측、상방、비측、하방황반구시망막후도이급평균、섭측、상방、비측、하방상한RNFLT적차이균무통계학의의(P=0.102、0.051、0.238、0.663、0.110、0.104、0.419、0.371、0.158、0.063、0.563).여LHON녀성휴대자상비,LHON녀성환자제중심자구황반구시망막후도화비측상한RNFLT차이균무통계학의의(P=0.388、0.580)외,황반용적평균후도,내자구섭측、상방、비측、하방화외자구호측、상방、비측、하방황반구시망막후도이급평균、섭측、상방、하방상한RNFLT치균명현하강,차이균유통계학의의(P=0.000、0.000、0.000、0.007、0.002、0.002、0.000、0.000、0.040、0.000、0.016、0.000、0.000).결론 LHON녀성휴대자시반주위RNFLT정상,단황반4개내자구황반구시망막후도현저변박,중심자구화4개외자구황반구시망막후도정상.본연구수차발현LHON녀성기인휴대자황반구시망막후도이상,제시LHON황반부병변조우RNFLT적변화.
Background Researches documented that retinal nerve fiber layer thickness (RNFLT) in unaffected carriers of Leber hereditary optic neuropathy (LHON) becomes thickened in different quadrants to different degrees.But the change of their macular thickness is still unclear.Objective This study was to clarify RNFLT and macular thickness by optical coherence tomography (OCT) in unaffected female carriers of LHON families.Methods Five female LHON patients (5 eyes) from 5 LHON families,eighteen unaffected female carriers (18eyes) from 18 LHON families and twenty-five age-matched healthy female controls (25 eyes) were included in this study.The patients and genetic carriers were diagnosed in PLA General Hospital from 2011 September to 2012 October.Regular ocular examination were performed followed by OCT measurement of retinas.The Optic Disc Cube 200×200 and Macular Cube 200×200 protocols were used during the OCT measurement.Average (360°) RNFLT,RNFLT at four quadrantic sections,cube average macular thickness and macular thickness of nine Early Treatment Diabetic Retinopathy Study (ETDRS) sub-areas were compared among the LHON genetic carriers,LHON patients and normal controls.Results Compared to the normal control group,significant reduced values were seen in temporal,superior,nasal and inferior side of sub-area macular thickness in the LHON female carriers (P=0.022,0.046,0.024,0.008).In addition,but no significant differences were found in cube average thickness,central subarea macular thickness,temporal,superior,nasal and inferior side of lateral sub-area macular thickness,average RNFLT,and temporal,superior,nasal and inferior quadrant RNFLT between the LHON female carriers and normal controls (P=0.102,0.051,0.238,0.663,0.1 10,0.104,0.419,0.371,0.158,0.063,0.563).Compared to the unaffected female carrier group,female patients showed significant reductions in cube average macular thickness,temporal,superior,nasal and inferior side of sub-area macular thickness,temporal,superior,nasal and inferior side of lateral sub-area mac ular thickness,average R NFLT and temporal,superior,and inferior quadrant RNFLT (P =0.000,0.000,0.000,0.007,0.002,0.002,0.000,0.000,0.040,0.000,0.016,0.000,0.000) except for the central subarea macular thickness and nasal quadrant RNFLT (P=0.388,0.580).Conclusions Unaffected LHON female carriers show a normal peripapillary RNFLT,but the macular thickness at medial sub-area is thinner.This first report offers an information of macular structure change in unaffected LHON female carriers,which suggest that macular damage appears prior to RNFLT change.