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成人黄斑中心凹卵黄样营养不良患者中心凹下组织切除术后黄斑转位的光镜和电镜观察

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世界核心医学期刊文摘（眼科学分册） 2005年第1期1卷 40-41页

作者：张晓光 Eckardt C Eckardt U Groos S Aug enklinik der Stadtischen Kliniken Frankfurt a .M.-H chstGotenstras se 6-865929Frankfurt am MainGermany

Purpose:To correlate the functional results of maculartranslocation(MT )in a patient suffering from an adult-onset foveomacular vitellif orm dystrophy(AFVD)with the microscopic findings of the surgically removed subfoveal retinal pigment epithelium(RPE).Methods:A78-year-old woman with AFVD underwe nt MT with 360°retinotomy 3-4months after loss of r eading *** of the vitelliform material was lost during surgery;the subfoveal tissue was excised,fixed in aldehydes,postfixed in reduced OSO4and embedded in epoxy *** sections were stained with toluidin e blue for light mi-croscopy(LM)and thin sections with uranyl acetate and lead citrate for transmission electron microscopy(TEM).Results:Postoperatively,the pati ent developed a retinal detachment complicated by proliferative vitreoretinopathy(PVR)requiring two additional vitreoretinal procedures before finally the silicone oil coul d be ***-ty-two months after MT the distance v isual acuity was un-changed at 0.2;the near visual acuit y had improved from less than 0.1before MT to *** retina was completely *** and TEM revealed seriou s alterations in-dicative of a breakdown of the outer l ayer of the ***:Through the present sin gle case it is not possible to determine whether MT cou ld be a therapeutic approach in patients with *** m ost important cause for the limited postoperative visual improvement seems to be a primary injury of the foveal function due to the *** is supported by the extensive subfoveal degeneration and necrosis affectin g not only the RPE cells but also their basement membrane and the interposed basal laminar deposits.

关键词：黄斑中心凹转位术切除术光镜营养不良患者视网膜色素细胞视网膜脱离近视力术后视力远视力

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1型肌强直性营养不良患者的有氧训练

世界核心医学期刊文摘（神经病学分册） 2005年第9期1卷 11-12页

作者： rngreen M.C. Olsen D.B. Vissing J. 袁海峰 Department of Neurology 2082 National University Hospital Rigshospitalet Blegdamsvej 9 DK-2100 Copenhagen Denmark Dr.

The effect of 12 weeks of aerobic training on a cycle ergometer was studied in 12 patients with myotonic dystrophy. Efficacy was evaluated by cycle testing an d muscle morphology before and after training. Patients increased their maximal oxygen uptake by 14%, the maximal workload by 11%, muscle fiber diameter incre ased significantly, and creatine kinase did not increase with training. The stud y indicates that aerobic training is safe and can improve fitness effectively in patients with myotonic dystrophy.

关键词：肌强直营养不良患者有氧训练最大摄氧量形态学检查工作负荷肌纤维直径训练效果

眩光敏感度与专业司机的安全:1例视网膜电图阴性的视锥视杆营养不良患者

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世界核心医学期刊文摘（眼科学分册） 2005年第11期1卷 14-15页

作者： Jgle H. Besch D. 陈立军 University Eye Hospital Department of Pathophy siology of Vision and Neuro-ophthalmology Schleichstrasse 12-16 D-72076 Tü bingen Germany

Background: To obtain a driver’s licence certain requirements for visual acui ty and visual field have to be fulfilled. Mesopic contrast and glare sensitivity are not regularly tested and are not crucial to passing the driving test. Case report: We report a case of a 39-year-old professional bus driver whose only c omplaint was increased glare sensitivity. After he had been involved in four tra ffic accidents, ophthalmological investigations revealed binocular annular scoto mata and night blindness, leading to the diagnosis of rod-cone dystrophy. Discu ssion: Enhanced glare sensitivity is a common complaint in elderly people or peo ple with the beginnings of cataract but may also represent an initial symptom of a retinal disorder. It is therefore advisable for traf-fic safety if drivers w ith such complaints undergo a complete ophthalmological investigation including visual field testing.

关键词：视网膜电图视杆眩光营养不良患者视锥中心视力视网膜疾病初发白内障环形暗点眼部检查

胃肠外营养支持对老年慢性阻塞性肺疾病急性加重期合并营养不良患者肺功能的改善

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山西医药杂志 2020年第4期49卷 430-432页

作者：祝建宙陈小玲徐迅科上海申园康复医院内科

慢性阻塞性肺疾病(chronic obstructive pul-monary diseases,COPD)是临床常见病变,我国人口老龄化加快导致临床老年COPD患者增多。老年COPD患者通常存在营养不良,严重影响病情和患者机体免疫功能,使其呼吸系统防御机制减弱,甚至损伤重... 详细信息

慢性阻塞性肺疾病(chronic obstructive pul-monary diseases,COPD)是临床常见病变,我国人口老龄化加快导致临床老年COPD患者增多。老年COPD患者通常存在营养不良,严重影响病情和患者机体免疫功能,使其呼吸系统防御机制减弱,甚至损伤重要器官;营养不良、感染、机体免疫和COPD患者肺功能相互影响,一旦发生营养不良则容易形成恶性循环[1]。据不完全统计,临床20%~60%的COPD患者存在营养不良,而老年患者更高[2]。胃肠外营养支持是目前干预营养不良患者的重要方法之一,本研究分析其对老年COPD急性加重期合并营养不良患者肺功能的影响,报告如下。

关键词：胃肠外营养支持营养不良患者 COPD患者慢性阻塞性肺疾病肺功能呼吸系统防御机制

老年慢性阻塞性肺疾病并发自发性气胸的营养不良患者的饮食护理

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中国乡村医药 2014年第7期21卷 66-67页

作者：胡建兰陈瑾浙江金华市中医院呼吸内科 321017

老年慢性阻塞性肺疾病(COPD)营养不良发生率高,自发性气胸是其常见并发症。2011年5月至2012年12月,我科收治了21例老年COPD并发自发性气胸的营养不良患者,经积极治疗和护理,效果满意。现将饮食护理体会总结如下:1临床资料所有病例均符... 详细信息

老年慢性阻塞性肺疾病(COPD)营养不良发生率高,自发性气胸是其常见并发症。2011年5月至2012年12月,我科收治了21例老年COPD并发自发性气胸的营养不良患者,经积极治疗和护理,效果满意。现将饮食护理体会总结如下:1临床资料所有病例均符合我国2007年修订的慢性COPD诊治指南中的诊断标准,并经影像学检查确诊。21例中男15例,

关键词：自发性气胸营养不良患者影像学检查气胸患者中男少食多餐参芪粥诊断标准脾胃虚弱调补

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世界核心医学期刊文摘（眼科学分册） 2005年第1期1卷 59-60页

作者：陈立军 Lorenz B Wabbels B Wegscheider E Dept.Paediatr.Ophthalmol .O. U-niversity of Reg ensburg Reg ensbur g Germany.Dr.

Purpose Fundus autofluorescence is due to accumulation of lipofuscin in the retinal pigment ep ithelium(RPE)resulting from incomplete digestion of N-retinylidene-phosphatidyl-ethanolamine from shed photorecep tor outer segment *** in autofluorescence reflects changes in lipofuscin content of the *** on both alleles of RPE65result in absent or largely decrease d formation of rhodopsin,due to a defect in alltrans retinol is omerization in the *** could therefore b e *** study was conducted to evaluate fundus autofl uorescence in patients with early-onset severe retinal dystrophy(EOSRD,or ear-ly-onset rod-cone dystrophy)associated with mutations on both alleles of *** Case se *** and controls Ten 10-to 55-year-old p atients with EOSRD and compound heterozygous or homozy gous mutations in *** comparison,6heterozygous parents and 2patients with other forms of EOSRD we re *** Participants underwent,in addition to standard clinical and electrophysiological examination,autofluores-cence imaging using a confocal scanning laser *** of the patients were als o examined by optical coherence tomography(OCT)to evaluate the status of retinal *** in7patients have been reported previously;the other pati ents were investigated by polymerase chain reaction-single-strand conformation poly-morphism and direct sequencing for mutations in RPE65and lecithin retinol acyltransfera se(LRAT).Main outcome measures Fundus autofluorescence a nd *** Ab-sent or minimal autofluorescence wa s found in all patients with compound heterozygous or homozygous *** was normal in the heterozygous *** was present in 2children with EOSRD not associated with mutations in RPE65or LRAT,another gene involved in retinol *** coher-ence tomography in younger patients revealed an intraretinal appearance similar to that of

关键词：自体荧光 RPE65 基因突变早发性营养不良患者视杆细胞扫描激光检眼镜光感受器脂褐素聚合酶链反应

有氧锻炼可有效提高面肱肌营养不良患者的运动能力

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世界核心医学期刊文摘（神经病学分册） 2005年第8期1卷 62-62页

作者： Olsen D.B. φrngreen M.C. Vissing J. 朱光明 Copenhagen Muscle Res. Center 7652 National University Hospital Rigshospitalet Blegdamsvej 9 DK-2100 CopenhagenDenmark Dr.

Exercise programs have been shown to increase strength and endurance in patien ts with myopathic disorders. The authors investigated the effect of aerobic trai ning in patients with facioscapulohumeral dystrophy (FSHD). Twelve weeks of lowi ntense aerobic exercise improved maximal oxygen uptake and workload with no sign s of muscle damage. The authors conclude that aerobic training is a safe method to increase exercise performance in patients with FSHD.

关键词：肱肌有氧锻炼营养不良患者最大摄氧量肌肉损伤肌肉力量工作负荷肌病

低热量胃肠外营养治疗普外营养不良患者的疗效分析

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临床医药文献电子杂志 2018年第72期5卷 14-14页

作者：孙晖高邮市人民医院

目的分析低热量胃肠外营养治疗普外营养不良患者的临床疗效观察。方法现随机选取我院2014年1月;018年7月普外科营养不良患者共70例,按照随机数字表法分成对照组35例、实验组35例,进行对照试验。对照组进行常规的营养支持进行治疗,实验... 详细信息

目的分析低热量胃肠外营养治疗普外营养不良患者的临床疗效观察。方法现随机选取我院2014年1月;018年7月普外科营养不良患者共70例,按照随机数字表法分成对照组35例、实验组35例,进行对照试验。对照组进行常规的营养支持进行治疗,实验组进行低热量胃肠外营养支持进行治疗,将两组患者的营养指标进行比对。结果对照组患者的总蛋白（g/L）、血清前蛋白（mg/L）、血清白蛋白（g/L）均低于实验组,组间差异明显,具有统计学意义（P营养不良患者采用低热量胃肠外营养治疗能够有效缓解病情发展,并且使病情得到改善,值得临床推广和应用。

关键词：营养不良患者低热量胃肠外营养治疗疗效

在特殊类型锥细胞营养不良患者的锥细胞cGMP磷酸二脂酶γ亚基因中发现一种G替代C的替代物

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世界核心医学期刊文摘（眼科学分册） 2005年第5期1卷 60-60页

作者： Piri N. Danciger M. 邢咏新

To identify genes responsible for cone dystrophies and determine the functiona l consequences of their underlying mutations. Case-control study. Two hundred f orty unrelated patients diagnosed with cone dystrophy, cone-rod dystrophy, macu lar dystrophy, macular degeneration, or Stargardt disease, 95 control individual s, and 2 unrelated families with a distinctive type of cone dystrophy. The DNAs of the 240 probands were screened for sequence variants in the PDE6H gene (that encodes the inhibitory γ-subunit of cone cyclic guanosine monophosphate [cGMP ] -phosphodiesterase [PDE]) by single-strand conformation polymorphism electro ph oresis. The effect of a nucleotide substitution in the DNA of a patient on gene expression efficiency was analyzed by in vitro transcrip-tion/translation. Cone -specific gene variants, fundus, visual field and electroretinogram (ERG) findi ngs, and protein synthesis efficiency. We found a heterozygous G to C substituti on in the 5′untranslated region (UTR) of the PDE6H gene in the DNA of a patient with a distinctive form of cone dystrophy, her sibling, and their father. This rare form of disease is very different in manifestation from other cone dystroph ies and has been described as “cone dystrophy with nyctalopia and supernormal r od responses,”“cone dystrophy with supernormal scotopic ERGs”and “supernorma l and delayed rod ERG syndrome.”Among the 240 patients that we studied, only 1 proband had the G to C variant. Furthermore, none of the 95 controls used in thi s study had this nucleotide change. We also determined that the PDE6H variant wa s not present in another family affected with this particular type of cone dystr ophy. Because the 5’UTR of mRNAs plays a critical role in the regulation of pro tein synthesis, we determined the effect of the G to C change in this process. B y use of in vitro transcription/translation experiments, we demonstrated that th is substitution could lead to an increase in PDE6H gene expression. Our result

关键词：磷酸二脂酶 γ亚基营养不良患者黄斑营养不良黄斑变性基因突变光感受器体外观察先证者视网膜电图