以直立性低血压为主要表现的Shy-Drager综合征42例分析

该文探讨Shy-Drager综合征的临床表现,以便早期诊断治疗。方法 :回顾性分析经临床诊断的42例Shy-Drager综合征患者的相关资料。结果 :Shy-Drager综合征患者临床表现为直立性低血压42例,卧立位压差平均为36/22mmHg(收缩压差/舒张     

自主神经功能障碍型多系统萎缩临床诊断及治疗

目的:通过对自主神经功能障碍型多系统萎缩(Shy-Drager综合征,SDS)的临床和影像学特征、治疗方法和预后的研究,从而探讨SDS的早期诊断和治疗.方法:采用回顾性分析方法,收集我院神经科2005-06至2010-07诊断为SDS患者37例,男性22例(59.5%),女性15例(40.5%).对37例SDS的特点及其他临床和影像学特征进行综合分析,尤其是直立性低血压的血压变化和伴发症状.结果:37例SDS患者临床以直立性低血压(30例,81.1%)、头昏或晕厥(29例,78.4%)、睡眠呼吸暂停综合征(28例,75.7%)常见.头颅计算机断层摄影术(27例,73.0%)和核磁共振成像(29例,78.4%)异常阳性率高.经药物等治疗均有不同程度改善.发病后4年无1例死亡,5年3例死于睡眠呼吸暂停综合征,2例死于肺部感染所致呼吸衰竭.结论:SDS临床以自主神经功能障碍为主要表现,早期诊断和针对性的治疗能够降低患者死亡率、提高生存时间和生活质量.     

Shy-Drager综合征26例的临床分析

目的:探讨Shy-Drager综合征(SDS)的临床和辅助检查的特点,为临床诊断提供依据。方法:回顾性分析26例临床诊断为SDS患者的临床资料、头颅磁共振(MRI)、肛门括约肌肌电图(EAS-EMG)、校正QT间期和尿流动力学检查。结果:14例患者行头颅MRI检查,6例患者出现壳核T2低信号。19例患者行EAS-EMS检查,均有自发电位。13例患者行尿流动力学检查,9例有尿道流出梗阻现象。20例患者行心电图检查,平均校正QT间期达0.409 ms。结论:临床表现结合头颅MRI和EAS-EMG有助于提高SDS的诊断。     

Gilbert综合征9例

目的:总结Gilbert综合征的临床特点及肝组织改变情况,提高临床医师对该疾病的认识,掌握该病的诊断及治疗.方法:2007-2011年我科住院的9例患者,排除其他慢性肝病导致肝功异常,经饥饿实验、苯巴比妥试验及肝组织病理检查明确诊断为Gilbert综合征,对其年龄、性别、症状、体征、实验室检查结果(血尿常规、肝功、肝炎病毒标志物)、肝胆脾彩超及肝组织病理结果等进行整理分析.结果:Gilbert综合征患者多于青少年期发病(本研究中占77.8%),男性多于女性.患者多无临床症状及异常体征,血清胆红素水平大多波动于50?mol/L以下,且肝组织病理改变轻微,无慢性肝炎及肝纤维化改变.结论:Gilbert综合征症状及肝组织病理改变轻微,不导致慢性肝炎及肝纤维化,无需治疗,不影响患者寿命.加强对该病的认识,给予正确的诊断,可以减少患者不必要的检查及治疗,减轻患者的心理及经济负担.     

伴6号染色体异常的治疗相关性骨髓增生异常综合征1例并文献复习

目的:探讨治疗相关性骨髓增生异常综合征的临床特征及诊断、治疗方法。方法:分析1例胃癌治疗相关性骨髓增生异常综合征患者的诊疗经过,并进行相关文献复习。结果:单核苷酸多态性微阵列技术检查提示:6号染色体短臂获得性杂合性缺失,患者诊断为胃癌治疗相关性骨髓增生异常综合征并进行积极治疗,病情无明显缓解。结论:治疗相关性骨髓增生异常综合征临床少见,目前无特异性疗法,可尝试进行个体化治疗。     

延髓背外侧综合征15例

延髓背外侧综合征,又称Wallenberg综合征,是神经内科常见病、多发病;但由于其临床表现有时不典型,尤其发病早期,常常以普通头晕或少见临床表现起病,故极易误诊,延误治疗,甚至因出现呼吸衰竭等而危及患者生命.因此,临床医生有必要对此引起足够重视,尽可能早诊断、早治疗以改善患者预后.本文对15例Wallenberg综合征患者的临床资料进行回顾性分析.     

Sweet综合征5例临床分析

目的分析Sweet综合征的临床特点及诊断.方法对5例Sweet综合征患者的病史、临床表现、治疗作回顾性分析.结果 Sweet综合征以浸润性红色斑块,发热,血沉增快等为特点,诊断应综合评定,注意与多形红斑等疾病鉴别.结论边界清楚、皮温升高的浸润性疼痛性斑块或结节对确定诊断意义较大,病理检查可明确诊断.     

Mirizzi综合征诊治分析53例

目的:探讨Mirizzi综合征的诊断和治疗方法.方法:回顾性分析本院1997-10/2006-09共收治的Mirizzi综合征53例患者的临床资料,男15例,女38例,年龄28-82(平均62)岁.术前主要以B超、计算机体层摄影术(CT)、内窥镜逆行胰胆管造影术(ERCP)和磁共振胰胆管造影术(MRCP)等检查手段进行筛查,以术中结果为最终诊断,针对不同的病例选用适合不同个体的手术治疗方式.结果:Mirizzi综合征患者53例按Csendes分型法分为Ⅰ型31例,Ⅱ型12例,Ⅲ型7例,Ⅳ型3例.均行手术治疗,患者住院时间最短5 d,最长2 mo,平均15 d.术后随访6 mo-10年,5例失访,1例因其他疾病死亡,2例胆道再发结石手术治愈,全组无手术死亡,近期发生胆瘘1例再次手术治愈.结论:Mirizzi综合征术前诊断困难,需借助多种影像学技术,术前明确诊断可减少Mirizzi综合征术中胆道损伤的发生率,要针对不同的病例选用适合不同个体的诊断和治疗手段.     

以脊髓压迫症状为表现的Richter综合征1例并文献复习

目的:探讨少见部位侵犯的Richter综合征的临床特点、诊治方法与预后.方法:报道1例具脊髓硬膜外占位病变的Richter综合征,通过病理组织学、骨髓细胞学、免疫组织化学方法的实验室检查,结合文献复习,探讨其发病机制、诊断、治疗与预后特点.结果:患者诊断慢性淋巴细胞白血病(CLL)6年,接受氟达拉滨治疗后第3日出现双下肢瘫痪和排尿困难.经磁共振检查发现患者胸椎段硬脊膜外占位病变,经外科手术切除,病理检查证实为弥漫大B细胞淋巴瘤.结论:此例慢性淋巴细胞白血病进展为恶性度较高的淋巴瘤,即Richter综合征,而此例发生的硬脊膜外的侵犯尤为少见,应加深对其的认识以避免漏诊和误诊.该疾病预后不良.     

布-加综合征的诊断治疗59例

目的:分析布-加综合征(Budd-Chiari syndrome,BCS)的临床特点,提供该综合征的诊断治疗经验.方法:对59例BCS患者进行临床症状、体征、实验室检查、影像学检查及治疗情况进行回顾性分析.结果:59例患者主要临床表现为双下肢水肿17例,占28.8%,腹胀15例,占25.4%,腹胀伴双下肢水肿7例,占11.9%,彩色多普勒血管超声和血管造影的诊断率可达100%.55例患者行介入治疗,出院转归治愈23例,好转30例,未愈2例.结论:BCS的临床表现繁杂,极易误诊误治,对高度怀疑本病者,应行彩超多普勒血管检查和静脉造影进行确诊,早期诊断及治疗对预后有重要的影响.介入手术是有效的治疗手段.     

Adult onset of acute myeloid leukaemia (M6) in patients with Shwachman-Diamond syndrome

Three male patients (two of whom were brothers) with Shwachman-Diamond (SDS) syndrome presented with acute myeloid leukaemia in adulthood. In all three cases there was trilineage myelodysplasia and the morphology was consistent with FAB subtype M6. SDS is an inherited bone marrow failure syndrome with a high propensity to leukaemic transformation. Since this may not occur until adulthood, SDS should be considered in the differential diagnosis of adults presenting with acute myeloid leukaemia, particularly where features of myelodysplasia are prominent.     

The Obstructive Lobe Syndrome

Ten cases of obstructive lobe syndrome as a complication of asthma and 81 cases of atelectasis which were observed at the Mayo Clinic from 1940 through 1965 are reviewed and compared. It is postulated that the obstructive lobe syndrome is part of a spectrum of disease in which it is a mild or early manifestation and atelectasis is the more severe manifestation.     

The Obstructive Lobe Syndrome

Ten cases of obstructive lobe syndrome as a complication of asthma and 81 cases of atelectasis which were observed at the Mayo Clinic from 1940 through 1965 are reviewed and compared. It is postulated that the obstructive lobe syndrome is part of a spectrum of disease in which it is a mild or early manifestation and atelectasis is the more severe manifestation.     

脾胃湿热证与Th1/Th2细胞平衡的相关研究

目的研究脾胃湿热证中Th1/Th2细胞免疫平衡改变的情况。方法病例为经胃镜检查诊断为慢性浅表性胃炎及消化性溃疡病人,共57例,分为脾胃湿热证(33例)及脾气虚证(24例)两组,正常对照组15例。采用ELISA法测定血清细胞因子IL-4和IFN-γ水平,并比较IFN-γ/IL-4值。结果脾胃湿热组IL-4值均较脾气虚组和对照组显著降低(P<0.05);而脾胃湿热组IFN-γ值和IFN-γ/IL-4比值升高,差异有统计学意义(P<0.05)。结论提示慢性浅表性胃炎和消化性溃疡中脾胃湿热证患者机体Th1/Th2细胞免疫失衡,细胞因子网络调节紊乱,以Th1细胞反应占优势。     

Experience in using split-day studies for suspected obstructive sleep apnea syndrome

BACKGROUND: Because in many locations the demand for sleep studies exceeds resources, we evaluated the utility of split-day in-laboratory studies (SDS) in highly selected patients. METHODS: We studied 100 eligible cases: 68 males (age 48.6+/-1.3 [standard error, se] years, body mass index (BMI): 32.6+/-0.8 kg/m(2)) and 32 females (age 50.9+/-2.4 years, BMI: 36.3+/-1.3 kg/m(2)) with severe subjective sleepiness (Epworth sleepiness scale: ESS 16) and suspected obstructive sleep apnea syndrome (OSAS). RESULTS: There were 86 conclusive studies that yielded both a diagnosis and sufficient information for management (86.0%) and 14 inconclusive studies that did not yield sufficient information for management (14.0%). In six cases (6.0%) with an inconclusive study a diagnosis was made, however, no titration data was obtained. Thus a definitive diagnosis was obtained in 92.0% of all cases. Those with inconclusive studies had additional assessment, and eight of them ultimately had a final diagnosis of a sleep breathing disorder (SBD) and six had another sleep disorder: four had narcolepsy, one had a movement disorder, one had sleep deprivation. Thus there were six patients (6.0%) in whom SDS yielded only an SBD diagnosis but there was insufficient data for titration; two patients (2.0%) who ultimately had severe OSAS who were not diagnosed on SDS. CONCLUSIONS: SDS was found to be useful in the evaluation and treatment of highly selected patients with severe daytime sleepiness (ESS 16) and suspected OSAS.     

Osteomalacia revealing Sjögren's syndrome: a case report

INTRODUCTION: The most common renal disease in Sj?gren's syndrome is tubulo-interstitial nephritis, responsible for tubular acidosis in around 20 % of patients. Osteomalacia exceptionally occurs as the first manifestation of a renal tubule disorder due to a Sj?gren's syndrome. EXEGESIS: We report a case of a 20-year-old woman with tubular acidosis induced osteomalacia secondary to primary Sj?gren's syndrome. Improvement was obtained with bicarbonates, vitamin D, calcium and high-dose steroid therapy. CONCLUSION: During Sj?gren's syndrome, osteomalacia can complicate the distal renal tubular acidosis. In spite of the rare cases of osteomalacia revealing Sj?gren's syndrome, this auto-immune disease must appear in the list of the aetiologies of osteomalacia.     

综合心理干预对急性冠脉综合征患者负性情绪和生活质量的影响

目的：探讨综合心理干预对急性冠脉综合征（ACS）患者负性情绪及生活质量的影响。方法：116例ACS患者被随机分为干预组和常规治疗组,各58例。常规治疗组采取常规护理;干预组在常规护理的基础上,进行综合心理干预。采用焦虑自评量表（SAS）、抑郁自评量表（SDS）和西雅图心绞痛量表（SAQ）以及90项症状自评量表（SCL-90）分别于入院时和入院1个月后对两组患者进行评分。结果：两组患者入院时SDS、SAS评分比较差异无显著性（P〉0.05）;1个月后,与常规治疗组比较,干预组SDS[（57.41±9.10）分∶（51.34±9.02）分]、SAS评分[（53.33±8.64）分∶（45.59±8.22）分]显著降低,P均〈0.01,SAQ量表中各项分值均有显著增加,SCL-90量表中各项分值均显著降低（P〈0.05或P〈0.01）。结论：综合性心理干预能够改善急性冠脉综合征患者的焦虑、抑郁情绪,还能明显提高其生活质量。     

以大量蛋白尿、水肿为主要表现的乙型肝炎病毒相关性肾炎临床特征分析

[目的]探讨以大量蛋白尿、水肿为主要表现的乙型肝炎病毒相关性肾炎(HBV-GN)的临床特征,以提高对该病的认识。[方法]对我院近5年来收治的5例以大量蛋白尿、水肿为主要表现的HBV-GN患者的临床资料进行回顾分析,并结合文献进行复习。[结果]全部患者均有明显水肿和蛋白尿,3例有腹水,尿蛋白定量为3.58~7.50g/d,(平均5.42g/d),血浆白蛋白为20.4~28.2g/L(平均24.5g/L);4例表现为肾病综合征,1例为肾炎综合征;病理类型4例为膜性肾病,1例为局灶性节段性肾小球硬化。阿德福韦酯与泼尼松联合治疗取得了很好的近期疗效。[结论]以大量蛋白尿、水肿为主要表现的HBV-GN患者大多以肾病综合征、膜性肾病为主要表现,应早期给予抗病毒与糖皮质激素联合治疗。消化内科医师应不断提高对该病的认识,早诊早治,减少误诊。     

A disturbance of serum lipids in three cases of Werner's syndrome

3 cases of Werner's syndrome were studied on serum lipid metabolism in comparison with clinical manifestation. These 3 cases showed hyperlipemia all with hypertriglycemia and increased level of very low density lipoprotein. The lipoprotein lipase activity was shown after intravenous injection of heparin. These results indicate a possible pathogenic facet of Werner's syndrome from the lipid metabolic point of view.