手助腹腔镜及开腹脾切除巨脾的临床疗效比较

目的比较手助腹腔镜(HALS)和开腹切除(OP)巨脾的临床效果。方法回顾性分析2004年—2010年行脾切除术患者的临床资料,比较其中大于20 cm脾脏行HALS和OP的临床疗效。结果大于20 cm的巨脾切除术患者41例,21例为HALS手术,20例为开腹脾切除手术。HALS组的中转开腹率4.7%。HALS和OP组的平均出血量为227 mL和887 mL(P=0.01),平均输血为0和0.6单位(P<0.05)。HALS组的住院时间比OP显著为短(4.3 d vs.8.4 d)(P=0.001)。并发症发生率两者相比无统计学差异(P>0.05)。结论 HALS是一项安全和有效的巨脾(大于20 cm)切除技术。相比开腹脾切除其住院时间更短,出血及输血更少,是一种行之有效的替代方法。     

Systemic pathology in aged mouse models of Down's syndrome and Alzheimer's disease

Down's syndrome (DS) in humans is caused by trisomy of chromosome 21 (HSA 21). DS patients have a variety of pathologies, including mental retardation and an unusually high incidence of leukemia or lymphoma such as megakaryocytic leukemia. Individuals with DS develop the characteristic neuropathological hallmarks of Alzheimer's disease (AD) in early adulthood, generally by the fourth decade of life. There are several mouse models of DS that have a segmental trisomy of mouse chromosome 16 (MMU 16) with triplicated genes orthologous to HSA 21. These mice display neurodegeneration similar to DS. Although brain pathology in DS models is known, little information is available about other organs. We studied the extraneural pathology in aged DS mice (Ts65Dn, Ts2 and Ts1Cje aged 8 to 24 months) as well as other mouse models of neurodegeneration, including presenilin (PS), amyloid-β precursor protein (APP), and tau (hTau and JNPL) transgenic mice. An increased incidence of peripheral amyloidosis, positive for amyloid A (AA) but not amyloid-β peptide (Aβ), was found in APP over-expressing and tauopathic mice as compared to non-transgenic (ntg) littermates or to DS mouse models. A higher incidence of lymphoma was found in the DS models, including Ts1Cje that is trisomic for a small segment of MMU 16 not including the App gene, but not in the APP over-expressing mice, suggesting that high APP expression is not the cause of lymphoma in DS. The occurrence of lymphomas in mouse DS models is of interest in relation to the increased incidence of malignant conditions in human DS.     

尼曼匹克病C型诊疗新进展

尼曼匹克病C型(NPC)是一种常染色体隐性遗传的溶酶体脂质贮积病, 主要累及内脏器官和神经系统, 自婴幼儿至成人均可发病, 儿童期多见。新生儿期持续存在的胆汁淤积性黄疸、脾脏肿大、猝倒发作和垂直性核上性眼肌麻痹为该病的特征性表现, 因发病年龄不同, 首发的神经系统症状不一致, 临床有明显的异质性。NPC基因缺陷导致游离胆固醇转运障碍, 在细胞内大量沉积是疾病的始发因素, 细胞的自噬功能障碍、钙稳态失平衡、氧化应激等均参与疾病的病理生理过程。通过皮肤成纤维细胞培养发现异常沉积的游离胆固醇或行基因检查发现NPC的致病性突变可确诊该病。美格鲁特是唯一被批准上市的特效药物, 早期应用可以改善神经系统症状和延缓疾病的进展。     

Laparoscopic splenectomy for a littoral cell angioma of the spleen: Case report

A littoral cell angioma(LCA) is a primary vascular tumor of the spleen, that can have malignant potential and may present association with other malignancies. This is a case of LCA that was discovered incidentally in a 79-year-old woman who presented with a polycythemia at the time of consultation. The neoplasm was evaluated by ultrasound and computed tomography. The patient underwent a splenectomy that revealed LCA by pathological evaluation. The post-operative outcome was favorable with no complications or recurrent disease. This case presentation, clinical, radiographic, and pathological features of an uncommon splenic tumor can be studied in order to advance our knowledge in our understanding of LCA.     

Hand-assisted laparoscopic splenectomy is a useful surgical treatment method for patients with excessive splenomegaly: A metaanalysis

BACKGROUND Hand-assisted laparoscopic splenectomy(HALS) can help overcome the drawbacks of laparoscopic splenectomy(LS) while maintaining its advantages.AIM To evaluate the efficacy and advantages of HALS for splenomegaly.METHODS The relevant literature was reviewed using the PubMed, EMBASE, Cochrane,Ovid Medline, and Wanfang databases to compare the clinical outcomes of HALS and LS. Odds ratios or mean differences were calculated with 95% confidence intervals for fixed-effects and random-effects models. Overall, 754 patients from16 trials who met the inclusion criteria were selected.RESULTS In pure splenectomy, blood loss volume(P < 0.001) and conversion rate(P =0.008) were significantly lower in the HALS group than in the LS group.Conversely, for splenomegaly, the operative time(P = 0.04) was shorter and blood loss volume(P < 0.001) and conversion rate(P = 0.001) were significantly lower in the HALS group than in the LS group. However, no significant difference was observed in hospital stay length, blood transfusion, time to food intake, complications, or mortality rate between the two groups. Moreover, in splenectomy and devascularization of the upper stomach(DUS), the operative time(P = 0.04) was significantly shorter and blood loss volume(P < 0.001) andconversion rate(P = 0.05) were significantly lower in the HALS + DUS group than in the LS + DUS group. However, no significant difference was observed in hospital stay length, timing of diet, and complications between the two groups.CONCLUSION HALS is an ideal surgical treatment method for splenomegaly because it can maximize the benefits for patients while maintaining the advantages of LS.     

Asymptomatic and symptomatic embolic events in infective endocarditis: associated factors and clinical impact

Background

Embolic complications of infective endocarditis are common. The impact of asymptomatic embolism is uncertain.

脾大部切除术治疗肝硬化脾大脾亢的疗效分析

目的 探讨脾大部切除术治疗肝硬化脾大脾亢的疗效。方法 对２６例肝硬化脾大脾亢病人行脾大部分切除加断流术,术后１年内随访,观测门静脉压力和直径、外周血细胞计数、骨髓细胞学、血小板相关ＩｇＧ（ＰＡＩｇＧ）及残腺大小的变化。结果 脾大部切除加断流术后,门静脉压力下降,门静脉直径缩小,残脾显像良好,无明显增大,外周血红细胞、白细胞和血小板计数升高,骨髓像大致正常,ＰＡＩｇＧ下降,随访１年见脾亢明显改善或消     

原发性淀粉样变五例临床分析

目的：提高对原发性淀粉样样变的认识,方法：分析5例原发性淀粉样变的临床表现和实验室检查,结果：5例肾脏受累,4例心脏受累,3例肝肿大。结论：原发性淀粉样变临床表现多样化,大多为非特异性,易误诊,确诊靠病理活检,遇到肝脾肿大及肾功能不全等多系统损害的病人,应想到原发性淀粉样变。     

巨脾大部切除后残脾红髓笔毛微动脉超微结构的变化

目的 :观察门静脉高压巨脾大部切除后残脾红髓笔毛微动脉(PA)超微结构的变化,探讨高血流动力对脾小血管内皮细胞超微结构的影响。方法:选取门静脉高压脾肿大患者13例,术后切取脾组织为巨脾组,术后8年穿刺获取脾组织为残脾组,另设外伤性脾组织13例为正常组。采用透射电镜观察脾脏红髓PA内皮细胞的超微结构改变。结果:巨脾组PA内皮细胞细胞核结构完整,染色质分布正常,一部分线粒体肿胀,变大变圆,基质变浅,线粒体嵴变短变少,甚至消失;一部分线粒体扁平嵴变成烧瓶状,甚至空泡状,基质致密,出现髓样变。残脾组PA内皮细胞核结构完整,染色质分布正常,线粒体基质均匀,嵴断裂减少,未见空泡样变和髓样变。正常组PA内皮细胞细胞核结构完整,染色质分布正常,线粒体嵴片状排列,外膜连续,基质均匀,连续内膜中可见包裹。结论:巨脾大部切除术可减少残脾血管PA增生,逆转PA内皮细胞线粒体的变化。     

脾动脉部分栓塞与脾切除治疗脾功能亢进症的疗效比较

目的通过对脾动脉部分栓塞和脾切除治疗脾大伴脾功能亢进症的疗效及并发症分析,探讨两种方法治疗脾功能亢进的优越性。方法回顾分析35例行脾动脉部分栓塞和24例行脾切除治疗的脾功能亢进患者的临床资料,将两种方法治疗后的外周血液成份（血红蛋白,白细胞,血小板）改变及并发症等进行对比分析。结果两种方法近期均能有效治疗脾功能亢进,可明显改善血小板和白细胞计数（P〈0.01）。脾切除组对血小板和白细胞的改善优于部分脾动脉栓塞组（P〈0.01）。脾动脉部分栓塞组术后并发症较脾切除组多,而脾切除组手术适应症要求较高。结论两种方法均能有效治疗脾脏功能亢进,应根据患者情况选择适合的治疗方式。