37例儿童噬血细胞综合征的临床分析

目的:分析儿童噬血细胞综合征(hemophagocytic syndrome,HPS)的病因、临床表现、实验室检查结果、治疗和预后特点。方法:回顾性分析我院收治的37例HPS患儿的临床资料。结果:37例HPS患儿(男24例、女13例),年龄2月~9岁,5例(13.5%)有明显家族史,获得性HPS32例(86.5%),包括EB病毒感染16例、巨细胞病毒感染7例,其他原因9例;所有患儿均表现为发热,肝脾肿大,外周血白细胞、血红蛋白、血小板、白蛋白、纤维蛋白原减低,TG、ALT、AST、LDH、铁蛋白升高;5例遗传性HPS患儿死亡4例,放弃治疗1例,剩余32例患儿中好转20例(62.5%),包括痊愈17例,完全缓解后继续治疗中3例,未好转12例(37.5%),其中死亡7例,病情危重放弃治疗3例,复发2例。12例未好转病例中,9例为EBV感染,1例为肾母细胞瘤,1例为幼年类风湿性关节炎合并CMV感染,1例原因不明。遗传性HPS的好转率较继发性HPS明显降低,差异有统计学意义(X2=5.30,P0.05),继发性HPS中EBV感染者的好转率较非EBV感染者低,差异有统计学意义(X2=4.80,P0.05)。结论:及时诊断儿童HPS并明确其病因,对该病的治疗及预后具有重要意义。     

噬血细胞综合征临床特征与影响预后因素分析

目的:探讨噬血细胞综合症的临床特征及影响预后因素,为临床治疗提供依据。方法:选择2006年1月~2011年12月我院收治的噬血细胞综合症患儿26例,回顾分析所有患儿的临床表现、实验室检查、病因及转归等资料。结果:死亡10例,好转或痊愈16例。原发与继发性HPS转归情况比较,X2=7.825,P<0.05,差异有统计学意义。两组年龄(t=2.807)、肝肿大(t=2.817)、脾肿大(t=2.651)、白细胞计数(t=2.580)、血小板(t=2.814)、纤维蛋白原(t=2.984)、乳酸脱氢酶(LDH)(t=2.667)和丙氨酸转氨酶(ALT)(t=2.514)比较,差异有统计学意义,P<0.05。结论:儿童噬血细胞综合征病因复杂,临床特征多样,应结合患儿个体特征进行临床治疗。     

儿童噬血细胞综合征38例临床分析

摘要目的：探讨噬血细胞综合征患儿的临床特征、治疗及预后。方法：对38例噬血细胞综合征患儿的Il盏床症状、体征、试验检查结果、治疗及预后,进行回顾性总结与分析。结果：38例患儿主要表现为发热、肝脾淋巴结肿大、外周血细胞减少、铁蛋白升高、凝血功能异常;经针对性治疗后,治愈3例,好转20例,疗效不佳自动出院9例,死亡6例。结论：噬血细胞综合征病因复杂,临床表现多种多样,病情凶险,预后较差,旱期诊断及治疗对预后十分关键。     

67例艾滋病合并新生隐球菌性脑膜脑炎临床分析

目的探讨艾滋病合并新生隐球菌性脑膜脑炎的临床特点及预后。方法回顾性收集2010年1月1日～2016年12月31日于北京佑安医院住院治疗的艾滋病合并新生隐球菌性脑膜脑炎患者,分析临床表现、实验室特点、治疗方案及转归。结果共67例患者确诊为艾滋病合并新生隐球菌性脑膜脑炎(男60例,女7例)。主要临床表现为头痛(74.6%),发热(47.8%)和恶心呕吐(40.3%),为最常见的3个症状。最常见的辅助检查异常是颅内压升高(78.9%),平均216.6 (±100.3 mmH_2O)。实验室检查CD4~+T细胞计数为53.7(±79.9)个/mm~3。本组患者病死率29.9%%(20/67)。结论新生隐球菌性脑膜脑炎是艾滋病患者的严重并发症,降低隐球菌性脑膜脑炎病死率的关键是早期诊断及充分、规范的治疗,以减少隐球菌对脑实质的损伤,挽救患者生命。     

铁蛋白和纤维蛋白原在噬血细胞综合征中的表达及意义

目的探讨铁蛋白(serum ferritin,SF)和纤维蛋白原(fibrinogen,FIB)联合检测在噬血细胞综合征(hemophagocytic syndrome,HPS)中的表达及意义。方法采用免疫比浊法和凝固法分别检测2008年1月~2014年10月在我院确诊的40例HPS患者(HPS组)和30例正常对照组的SF和FIB水平。结果 SF和FIB在HPS组中的表达均高于正常对照组,差异有统计学意义(均P0.01);将HPS组按治疗效果分为病情缓解组(n=18)与病情恶化组(n=22),比较治疗前的SF和FIB差异无统计学意义(均P0.05),而治疗后比较差异有统计学意义(均P0.01)。结论 SF和FIB在噬血细胞综合征中的联合表达对其诊断和治疗效果的评价有参考价值。     

嗜血细胞综合征患儿外周血CD_4~+CD_(25)~+调节性T细胞变化及临床意义

目的观察嗜血细胞综合征(hemophagocytic syndrome,HPS)患儿外周血中调节性T细胞(RegulatoryTcells,Treg细胞)的变化,探讨其在HPS发病中的作用及临床意义。方法应用流式细胞仪检测17例初诊HPS患者(初诊组)、11例经诱导治疗后临床缓解者(缓解组)及20例健康人群(对照组)外周血中CD4+CD25+调节性T细胞。结果与对照组相比较,HPS患者初诊组及缓解组外周血CD4+CD25+调节性T细胞均升高(P0.05)。与初诊组相比较,缓解组CD4+CD25+Treg细胞降低(P0.05),但仍高于正常对照组(P0.05)。结论CD4+CD25+Treg细胞增多可能是HPS患者免疫功能受抑的重要原因之一,其变化对于HPS的预后判断有一定的意义。     

原发性十二指肠恶性肿瘤的临床分析

目的:探讨原发性十二指肠恶性肿瘤的临床特点、诊断方法和预后影响因素。方法:回顾性分析随访资料完整的45例原发性十二指肠恶性肿瘤患者的临床病理资料。结果:腺癌33例(73.3%)为主要的病理类型。主要临床表现为腹痛、上腹部不适、黄疸、消化道出血等。胃十二指肠镜、内镜逆行胰胆管造影(Endoscopic Retrograde Cholangio-Pancreatography,ERCP)、十二指肠低张造影、超声内镜、CT及B超确诊率分别为91.1%(41/45),93.3%(42/45),82.2%(37/45),75.6%(34/45),68.9%(31/45)及26.7%(12/45)。本组45例均行开腹手术,包括根治性手术,胰十二指肠切除术36例;姑息性手术,胃肠吻合术2例、肿瘤局部切除术5例、短路手术2例。根治术和姑息术后5年生存率分别为46.7%和4.4%,两组生存率差异有统计学意义(P<0.05)。对全组45例患者的预后因素进行Cox回归分析的结果显示,手术方式、肿瘤浸润深度和淋巴节转移是影响预后的独立危险因素(均P<0.05)。结论:原发性十二指肠恶性肿瘤缺乏特异性临床表现;胃十二指肠镜、ERCP以及十二指肠低张造影等联合检查可提高诊断率;根治性手术远期疗效较好;淋巴结转移和局部侵犯是肿瘤预后不良的重要影响因素。     

伴血嗜酸性粒细胞增高的腹痛临床分析

目的探讨伴血嗜酸性粒细胞(Eosinophil,EOS)增高的腹痛病因、诊断、治疗方法。方法对我院31例伴血EOS增高的腹痛患者的临床表现、实验室检查、特殊检查结果进行分析,总结伴血EOS增高的腹痛病因及治疗方法。结果伴血EOS升高的腹痛的病因以寄生虫、变态反应性疾病为主,此外亦有可能为嗜酸性胃肠炎(Eosinophilic Gastroenteritis,EG)、恶性肿瘤、药物所致,少见病因为炎症性肠病、慢性胰腺炎、特发性嗜酸性细胞增多症(Idiopathic Hypereosinophilic Syndrome,HES)等。大部分EOS升高的腹痛以治疗原发病为主,EG及HES以激素治疗为主。结论伴血EOS升高的腹痛病因多样,大部分病因治疗原发病后EOS可自行下降,EG及HES以激素治疗效果好,作用迅速。     

高级别颅内动脉瘤超早期显微手术围手术期的临床护理体会

目的:总结超早期高级别颅内动脉瘤围手术期的护理经验及体会。方法:回顾性分析2010年4月至2013年6月就诊我科的32例高级别颅内动脉瘤患者的临床及护理资料。其中,Hunt-Hess分级IV级15例,V级17例。结果:32例患者经CTA或DSA检查动脉瘤完全夹闭。经过精心治疗和护理,预后良好7例(21.9%),重残9例(28.1%),极差16例(50%)。结论:超早期显微夹闭手术可有效降低颅内动脉瘤再出血的风险,减轻神经功能障碍。细致、严谨、专业的神经外科专科护理可以明显提高手术疗效,降低术后并发症,改善患者预后。     

肿瘤淋巴管入侵与无淋巴结转移膀胱癌复发及预后的相关性分析

目的:分析肿瘤淋巴管入侵与无淋巴结转移膀胱癌复发和预后之间的关系。方法:选取临床资料完整的膀胱癌病例72例,分为淋巴结转移组(32例)和无淋巴结转移组(40例)。采用Spearman相关分析探讨淋巴管入侵与膀胱癌复发和预后的相关性,应用Kaplan-Meier法描绘生存曲线,Cox比例危险度模型筛选影响膀胱癌患者预后的因素。结果:在72例膀胱癌组织中,淋巴管入侵的阳性率是48.6%(35/72),淋巴管入侵的阳性率随肿瘤分期和分级增加而显著升高(P0.05);淋巴结转移组的淋巴管入侵阳性率为68.8%(22/32),显著高于无淋巴结转移的32.5%(13/40)。淋巴管入侵与膀胱癌的临床分期、分级、淋巴结转移以及无淋巴结转移膀胱癌复发均显著相关(P0.05)。淋巴管入侵阴性的患者的五年总体生存率显著高于淋巴管入侵阳性者,淋巴管入侵是无淋巴结转移膀胱癌复发和预后不良的危险因素。结论:肿瘤淋巴管入侵与膀胱癌临床分期和淋巴结转移密切相关,并影响膀胱癌患者的总体生存率,可作为无淋巴结转移膀胱癌复发和预后的预测因素。     

乳酸和APACHEⅡ评分在评估老年脓毒症患者预后中的应用

目的：研究乳酸和急性生理学及慢性健康状况评分（APACHE Ⅱ评分）对老年脓毒症患者预后的评估作用。方法：老年脓毒症患者96例,按照入院时血乳酸值分成升高者60例,乳酸正常者36例,比较两组的病死率、休克、机械通气和MODS发生率、APACHE Ⅱ评分的区别;根据APACHE Ⅱ评分（〈15、15～24、≥25）分为3组,比较每组患者的病情和预后区别。结果：乳酸升高组老年脓毒症患者的机械通气、休克发生率、MODS发生率、APACHE Ⅱ评分明显大于乳酸正常组（P〈0.05）,病死率明显上升（28.3%vs 2.7%）,（P=0.005）;随着APACHE Ⅱ评分增高,患者病情逐渐加重,休克发生率和住院病死率明显升高,（P〈0.05）,患者乳酸水平也明显增高（P〈0.05）。结论：血乳酸和APACHE Ⅱ评分都可以评估老年脓毒症患者病情严重和预后,两者升高提示预后差。     

重型颅脑损伤后并发多器官功能障碍综合征治疗体会

目的:分析重型颅脑损伤后并发多器官功能障碍综合征的临床特点。方法:对42例重型颅脑损伤后并发多器官功能障碍病例进行回顾性分析总结。结果:42例共有106个器官功能障碍,其中肺32例,肾30例,胃肠17例。手术治疗30例,保守治疗12例;死亡28例,植物生存1例,病死率66．7%,所有死亡病例均合并肺及/或肾功能不全,30例合并肾功能不全患者仅2例存活。结论:重型颅脑损伤后并发多器官功能障碍是一个治疗困难、预后恶劣的综合征,及时预测并重点保护肺肾功能障碍器官和有效的预防是降低该病发生率和病死率的重要措施。     

限制性补液复合去甲肾上腺素对脓毒性休克患者血流动力学及氧代谢的影响

目的:探讨限制性补液复合去甲肾上腺素对脓毒性休克患者血流动力学及氧代谢的影响。方法:将62例脓毒性休克患者按照液体复苏策略随机分为限制性液体复苏(治疗组)和常规液体复苏(对照组),每组各31例。观察和比较复苏前后血流动力学指标、氧代谢指标的变化,记录两组低血压、弥散性血管内凝血(DIC)、多器官功能障碍综合征(MODS)、急性呼吸窘迫综合症(ARDS)的发生率及2周病死率。结果:治疗后1 h、3 h、6 h,两组患者CVP、MAP明显升高,HR明显下降(P0.05);治疗后3 h、6 h,治疗组MAP明显低于对照组(P0.05),而两组HR、CVP比较差异均无统计学意义(P0.05)。治疗后1 h、3 h、6 h,两组患者PaCO_2、PaO_2、SaO_2、PaO_2/Fi O_2均不同程度改善,治疗组治疗后3 h、6 h PaO_2、PaO_2/Fi O_2明显高于对照组(P0.05)。治疗后3 d,治疗组MODS的发生率较对照组显著降低(P0.05),而两组低血压、ARDS、DIC及2周病死率均无显著性差异(P0.05)。结论:限制性液体复合小剂量去甲肾上腺素对脓毒性休克患者有助于维持血流动力学稳定,改善全身氧代谢,减少并发症的发生,改善预后。     

Clinico‐molecular analysis of eleven patients with Hermansky–Pudlak type 5 syndrome,a mild form of HPS

Hermansky–Pudlak syndrome (HPS), first described in 1959, is a rare form of syndromic oculocutaneous albinism associated with bleeding diathesis and in some cases pulmonary fibrosis and granulomatous colitis. All 10 HPS types are caused by defects in vesicle trafficking of lysosome‐related organelles (LRO) proteins. The HPS5 protein associates with HPS3 and HPS6 to form the biogenesis of lysosome‐related organelles complex‐2 (BLOC‐2). Here, we report the clinical and genetic data of 11 patients with HPS‐5 analyzed in our laboratory. We report 11 new pathogenic variants. The 11 patients present with ocular features that are typical for albinism, with mild hypopigmentation, and with no other major complication, apart from a tendency to bleed. HPS‐5 therefore appears as a mild form of HPS, which is often clinically undistinguishable from mild oculocutaneous or ocular forms of albinism. Molecular analysis is therefore required to establish the diagnosis of this mild HPS form, which has consequences in terms of prognosis and of clinical management of the patients.     

Effects of alloxan-induced diabetes on hemorheology in rabbits.

Plasma viscosity (PV), apparent whole blood viscosity (WBV), relative blood viscosity (RV) and erythrocyte deformability (filterability) (EDF) were determined in 13 New Zealand White (NZW) rabbits with alloxan induced-diabetes (AID) and 8 normal NZW rabbits, matched for age, sex and weight. AID rabbits were divided into two groups depending on the duration of hyperglycemia (long-term, greater than 6.0 months (n = 7), and short-term, less than or equal to 3.0 months of hyperglycemia, n = 6). Comparing long-term AID rabbits to normal animals, we found significant increases in WBV (P less than 0.001, 0.005 for high and low rates of shear, respectively), and a marked reduction in EDF (P less than 0.001). There was no significant difference in PV between long-term AID and normal rabbits. Conversely, PV was significantly increased in rabbits with short-term diabetes (P less than 0.01) while there was a concurrent significant increase in WBV measured at high and low rates of shear (P less than 0.001, 0.001, respectively). No difference was detected in EDF between normal and short-term AID rabbits. Furthermore, in long-term AID rabbits there was a strongly positive correlation between RV and reduced erythrocyte deformability (r = 0.94, P = 0.006) while WBV strongly correlated with PV (r = 0.92, P = 0.004) in the short-term AID subgroup. We conclude from these data: (1) elevated blood viscosity in long term AID rabbits is associated with reduced erythrocyte filterability; and (2) elevated WBV in short-term AID rabbits is associated with increased PV.     

Genetic variants and mutational spectrum of Chinese Hermansky–Pudlak syndrome patients

Hermansky–Pudlak syndrome (HPS) is a rare recessive disorder characterized by oculocutaneous albinism or ocular albinism, bleeding diathesis, and other symptoms such as colitis and pulmonary fibrosis. Eleven causative genes have been identified for HPS‐1–HPS‐11 subtypes in humans. We have identified 16 newly reported patients including the first HPS‐2 case in the Chinese population. In a total of 40 HPS patients, hypopigmentation was milder in HPS‐3, HPS‐5, and HPS‐6 patients than in HPS‐1 and HPS‐4 patients. HPS‐1 accounted for 47.5% (19 of 40) of HPS cases which is the most common subtype. Exons 11 and 19 were the hotspots of the HPS1 gene mutations. In total, 55 allelic variants were identified in HPS1–HPS6 gene, of which 17 variants were previously unreported. These results will be useful for the evaluation of the relationship between HPS genotypes and phenotypes, and for the precise intervention of HPS patients in the Chinese population.     

Comparison of One or Two Weeks of Lansoprazole, Amoxicillin, and Clarithromycin in the Treatment of Helicobacter pylori

Background The simplest, most effective, and least expensive Helicobacter pylori therapy remains to be determined. Two weeks of 30 mg lansoprazole bid, 1 gm amoxicillin bid, and 500 mg clarithromycin bid (LAC2) had been shown to be an effective therapy for H. pylori. The aim of this study was to assess whether 1 week of this regimen (LAC1) would have a similar efficacy.
Materials and Methods. H. pylori -positive patients assessed histologically, by rapid urease test, microbiologically, and by a ¹³C-urea breath test (¹³C-UBT) were randomized to receive either LAC1 or LAC2 in a single-center open study. Patients were interviewed 1 to 3 days after completion of therapy to evaluate adverse events and compliance. Efficacy was determined by ¹³C-UBT at least 4 weeks after antibiotic therapy.
Results. Seventy evaluable patients were randomized to receive LAC1 (n = 33) and LAC2 (n = 37). Of the 33 LAC1 patients, 30 (91%) were treated successfully (95% confidence interval (CI) = 76–98%), compared with 32 of 37 (86%) in the LAC2 group (95% CI = 71–96%). There was no difference in efficacy between the two groups (Fisher's exact test p = 1.0; 95% CI =–10.3%–19.2%). Patients taking LAC1 experienced significantly fewer severe adverse events than those taking LAC2 (Mann-Whitney U test). One of 64 patients had primary resistance to clarithromycin, and treatment was unsuccessful in this case. Six of the 7 remaining treatment failures developed secondary resistance to clarithromycin.
Conclusions. LAC1 is as effective as LAC2 and is associated with less toxicity. Posttreatment clarithromycin resistance is common in patients who do not experience success with therapy.     

替罗非班联合尼可地尔对急诊PCI患者心功能和短期预后的影响

目的:探讨急诊PCI患者在联合使用替罗非班和尼可地尔后对心功能和短期预后的影响。方法:入选98例接受急诊PCI的AMI患者随机分为对照组(n=49)和联合治疗组(n=49)。对照组单纯给予替罗非班治疗,联合治疗组在对照组基础上给予尼可地尔5 mg tid口服治疗。观察记录两组患者治疗8周前后LVEF、NT-pro BNP水平,并随访MACE事件发生情况。结果:两组患者在治疗前LVEF及NT-pro BNP水平比较没有统计学差异(P0.05)。在治疗8周后联合治疗组和对照组LVEF及NT-pro BNP水平均较治疗前水平明显改善(P0.05)。治疗8周后联合治疗组患者LVEF及NT-pro BNP水平明显优于对照组患者(P0.05)。经过8周的治疗,联合治疗组再次发生心绞痛事件(6.12%VS 24.49%,P=0.025)、恶性心律失常(2.04%VS 16.33%,P=0.036)和心力衰竭事件(2.04%VS 18.37%,P=0.020)明显低于对照组。结论:替罗非班联合尼可地尔可进一步改善急诊PCI患者心功能及短期预后,值得临床推广。