同种异体原位肝脏移植术的临床应用研究(附9例报告)

目的总结原位全肝移植术的经验教训，以进一步提高肝移植治疗终末期肝病的效果。方法2001年3月～2006年8月，为9例终末期肝病患者实施了原位全肝移植术，其中6例为良性终末期肝病，3例为肝癌患者。前5例实施背驮式肝移植，后4例实施经典式非转流肝移植。结果1例病人存活3年以上，2例病人存活2年以上，1例病人存活1年以上，2例术后3个月，均恢复正常的工作和生活。手术后死亡病例中，1例术后1年9个月死于应用大剂量激素抗排斥反应所致的应激性溃疡大出血。2例巨大肝癌病人分别于术后27d死于肾功能衰竭、高血钾和术后56d死于脑出血。1例小肝癌病人术后出现神经并发症，术后4个月死于脑出血。结论肝移植是治疗终末期肝病的唯一有效方法。     

Wilson's disease in childhood. A plea for increased awareness

Wilson's disease, a hepatic-based metabolic disease, is treatable with a relatively good prognosis if diagnosed before severe complications occur. It has been diagnosed in eight children (five boys, three girls) in 11 years at our institution. The presenting symptoms were hepatic in four children, neurological in one and non-specific in one, whereas two children were asymptomatic siblings of index patients. The mean age at diagnosis was 8.9 years (range, 4.7-11.7 years). Two boys died soon after diagnosis: one had fulminating hepatic failure and the other, who had neurological disease, died of aspiration pneumonia. Six children are well, with regression of clinical disease, two to 10 years after the initiation of chelation therapy by mouth. The diagnosis was delayed for all symptomatic patients because of the disease's rarity, its nonspecific early manifestations and a low index of suspicion for the disease on the part of physicians.     

Bone marrow transplantation for childhood acute lymphoblastic leukaemia after marrow relapse

Children with acute lymphoblastic leukaemia in whom relapse in bone marrow occurs have a poor outlook when treated with chemotherapy alone. Twenty-seven patients with childhood acute lymphoblastic leukaemia were treated for marrow relapse with high-dose chemotherapy with or without total body irradiation followed by bone marrow transplantation (BMT). Twenty patients received allogeneic marrow from partially or completely matched histocompatible donors. In this group, nine patients (45%) were free of disease with a median follow-up of 57 months (range, 22 to 126 months) after transplantation, four (20%) died from interstitial pneumonitis and seven (35%) died after a further relapse. Seven patients received autologous marrow collected while they were in remission. In this group, one patient died from infection and six died after a further relapse. We conclude that allogeneic BMT is more effective than autologous transplantation and results in long-term disease-free survival in a significant number of patients. New methods are needed to eradicate residual disease in the patient and to purge marrow ex vivo.     

冠心病介入诊治的临床应用及随访观察

目的总结经皮冠状动脉介入在冠心病诊断和治疗中的初步经验及随访观察。方法选择2003年9月至2005年2月在我院接受经皮冠状动脉介入手术患者24例,采用Judkins法冠状动脉造影,管腔狭窄≥50%为冠状动脉病变。狭窄≥75%者施行PTCA和支架术。术后随访心血管事件的发生和生活质量改善。结果24例中管腔狭窄≥50%病变者18例,其中1支病变者8例,2支病变者7例,3支病变者3例。9例施行PTCA和支架术,2例于术后1个月、14个月猝死,1例支架置入后3个月再发胸痛,为支架内再狭窄行切割球囊术后症状好转,6例无心血管事件发生及生活质量明显改善。9例未行PCI者中6例再发胸痛住院,其中1例再发心肌梗死,1例猝死。结论选择性冠脉造影术是诊断冠心病可靠的方法,PTCA和支架术后患者胸痛复发减少,生活质量明显提高。     

阴囊Paget''''s病的诊断与治疗(附29例报告)

目的 探讨阴囊Paget's病的临床表现特点、治疗及影响预后的因素.方法 回顾性分析本院1987-2006年收治的29例阴囊Paget's病的临床表现、病理、治疗及其预后.患者平均年龄69.4岁,病程平均4.9年.28例行病灶皮肤广泛切除术,1例接受放疗.结果 28例病理可见Paget's病的典型特征.27例获得随访,随访时间5个月-16年.3例淋巴结转移者,2例术后2年死于广泛转移,1例未手术接受放疗者5个月后死于多脏器功能衰竭;3例死于心血管疾病;2例分别于术后1年、3年局部复发,再行手术切除;余19例无复发.结论 阴囊Paget's病应尽早行活检确诊,局部广泛切除术是首选治疗方法,手术效果好,对局部复发可行二次手术.     

慢性肾功能不全心脏病人行体循环手术的围术期处理

目的 探讨合并慢性肾功能不全心脏病人行体外循环手术的安全性。方法 对慢性肾功能不全的病人15例，施行了体外循环手术，其中氮质血症8例，肾功能不全代偿期5例，尿毒症期2例，在心脏手术围术期均给予积极治疗。结果 9例病人术后早期肾功能指标均较术前差；6例病人术后早期肾功能较术前无明显变化。围术期腹透4例，血透2例，其中一例行术中血透，肾功能指标均有所改善；全组无术中死亡，1例术后第3天死于多器官功能衰竭。结论 术前肾功能不全的病人，经过积极的术中及围术期处理，大多可以安全渡过肾功能衰竭关，接受体外循环手术，达到改善症状延长生命的目的。     

17例结肠癌再手术治疗体会

目的：探讨结肠癌术后治疗的指征和注意事项。方法：对1990年1月至2001年12月间17例结肠癌术后再手术治疗进行总结。结果：全组17例病人均行手术治疗，无再手术死亡，局限性病灶切除席子经为52.3%(9/17)。病灶未切除者（短路吻合，肠造瘘和剖腹探查）8例，再手术后生存时间：Duke'sA2例。存活10年健在：Duke'sB2例，6年健在：Duke'sC2例，1例4年死于本病，1例3年因心肌梗塞死亡，A，B，C期病人均获得良好的治疗效果，Duke'sD11例中，2例1年健在，1例1年死亡，余8例延长了中位生存期为8个月。结论：对结肠癌术后具备再手术指征者应积极行再手术治疗，有利于挽救一部分患者，使病人能获得治愈或长期生存，并改善了生存质量，即便不可治愈，可采用综合治疗，仍可减轻症状，延长生存期。     

30例伴糖尿病的冠心病患者行冠脉搭桥术的临床分析

目的探讨伴糖尿病的冠心病冠脉搭桥术(coronary artery bypass grafting,CABG)及围手术期处理.方法2000年1月～2003年6月,CABG治疗30例伴糖尿病的冠心病.结果术后发生低心排需要主动脉内气囊反搏(intra-aortic balloon pumping,IABP)2例.出现肾功能不全2例.脑血管意外长期昏迷1例,半年后死于肺部感染;术后多器官功能衰竭而死亡1例,全组死亡率为6.7%.胸部伤口感染3例,下肢伤口感染5例,占28.6%.术后出现心率失常9例,占32.1%.28例病人冠心病症状得到缓解或消失.结论伴糖尿病的冠心病的冠脉搭桥术,尽管手术死亡率及并发症稍偏高,但术前明确诊断及制订良好的手术方案,完善的围手术期处理,伴糖尿病的冠心病患者行冠脉搭桥术的治疗可取得良好效果.     

老年胆道疾病138例分析

1983～1988年共收治老年胆道疾病138例。其中手术治疗81例,痊愈68例、好转4例、退院4例、死亡5例;非手术治疗57例,痊愈32例、好转20例、退院1例、死亡4例。体会:老年胆道疾病病情危重、进展快、死亡率高,应早期诊断,并行以手术为主的综合治疗。     

原发性脑干出血32例临床分析

目的：探讨原发性脑干出血患的临床特点。方法：回顾性分析32例脑干出血患的临床资料。结果：本组32例中，有17例死亡，占53％，入院1d死亡8例，其余9例均在入院4d内死亡，15例好转出院，平均住院天数34．2d。结论：高血压动脉硬化为本病的主要病因。脑干出血起病急骤，临床表现多种多样，发病初期就有意识障碍的患预后差。     

成人斯蒂尔病的肝损害

目的；探讨成人斯蒂尔病（adult-onset Stil's disease,AOSD))合并肝损害的发生、表现、治疗和预后。方法；对27例成人斯蒂尔病患者中出现肝损害的13例，根据病情轻重采用中药水剂茵陈蒿汤中味或益肝灵片加服联苯双酯滴丸、强力宁注射液进行治疗。结果：11例（84.6%）肝功能恢复正常，1例好转，1例因急性肝功能衰竭死亡。结论：成人斯蒂尔病患者合并肝损害甚为常见。经中药和西药联合应用合理治疗后多数患者可恢复或好转。应重视和警惕发生危及生命和急性肝功能衰竭。     

Audit of mortality in upper gastrointestinal bleeding

The outcome in 1017 patients with haematemesis and malaena referred to two major hospitals in Nottingham within a 2-year period has been prospectively evaluated. Ninety one (9%) patients died during the time period under consideration and all but four were found to have been over 60 years of age. There were 13 (14%) deaths following rebleeding, of whom 5 (5%) could have been potentially avoided by alterations in management. Some improvement of mortality might result from intensive-care facilities with better management of transfusions and earlier detection of rebleeding allowing earlier endoscopy or surgery. The majority of patients (81%), however, died from concomitant disease which was exacerbated by gastrointestinal haemorrhage or bleeding developed in patients with an already existing end-stage disease. These results show that a reduction of mortality in patients with gastrointestinal bleeding is hampered by the high number of poor-risk patients. The rise in the proportion of elderly patients with this disorder seems to continue.     

Place of Death of Pediatric Cancer Patients in a Single Institute during 7 Years

Place of death is an important issue at the end-of-life. It is poorly understood in pediatric cancer patients in Japan. This study aimed to clarify place of death of children with cancer as well as variables associated with place of death. Study population was pediatric cancer patients who died in the Department of Pediatrics at Kobe University Hospital during the last 7 years. The medical records were retrospectively reviewed regardless of cause of death to derive data relating to patients' characteristics and disease. 18 patients were included. Median age at death was 12.2 years old. 6 patients including 5 children in complete remission had hematological disease and 12 patients suffered from solid tumors. 4 patients (22.2%) died at home, whereas 14 patients (77.8%) died in the hospital including 6 ICU deaths. No one died in hospices. Preference of patients was unavailable due to the lack of inquiry. Factors influencing place of death (home, ICU, non-ICU) were disease (hematological disease vs. solid tumor, p=0.010, brain tumor vs. non-brain tumor, p=0.023), disease status (complete remission vs. non-complete remission, p=0.0014) and preference of families (p=0.029). Among 6 families who expressed preference, no disparity was observed between actual and preferred place of death. This is the first English publication of place of death of pediatric cancer patients in Japan. The low percentage of home death, factors influencing place of death and the lack of disparity between actual and preferred place of death were indicated. Further studies are required to better understand place of death.     

18例白塞氏病临床分析及治疗体会

回顾分析白塞氏病１８例的临床资料，对本病的病因、诊断、治疗及预后进行了探讨。结果显示：口腔溃疡１８例，外生殖器溃疡１１例，眼部损害８例，皮肤损害１３例，眼、口、生殖器均受累７例；感染可能参与本病，尤其是结核感染；皮质类固醇激素与免疫抑制剂联合使用，可提高本病治愈率；本组痊愈和好转１５例，病情无变化２例，死亡１例，死亡原因为合并阴茎癌并转移     

Haemophagocytic lymphohistiocytosis in Hong Kong children

Haemophagocytic lymphohistiocytosis is a rare but potentially fatal disease. Diagnosing this disease may be difficult and is often delayed because the clinical presentation mimics other conditions like severe sepsis, hepatic failure and malignancies. We reviewed the clinical presentations, response to treatment, and outcomes of children diagnosed with haemophagocytic lymphohistiocytosis from 1991 to 2006 in a Hong Kong tertiary paediatric haematology centre. All patients had typical presentations with prolonged fever, organomegaly, and pancytopaenia. Six children had hepatic dysfunction and two had neurological symptoms. The median time from disease onset to diagnosis was 21 days. Elevated serum ferritin levels and evidence of haemophagocytosis in bone marrow examinations aided diagnosis. The overall mortality was 57%. Three patients who presented in the first few years studied had relatively long lag times between disease onset and definitive treatment; all died of active disease. Three patients diagnosed more recently were given timely treatment using the haemophagocytic lymphohistiocytosis-94 protocol of etoposide and dexamethasone, with or without cyclosporin. All three achieved remission, but two had a recurrence and one died during the recurrence.     

胃周血管离断及胃底环扎术治疗门静脉高压症并发消化道大出血32例临床观察

目的:探讨胃周血管离断及胃底环扎术在治疗门静脉高压症并发消化道大出血中的效果。方法:对门静脉高压症并发消化道大出血32例患者行胃周血管离断及胃底环扎手术,其中ChildA级者4例,B级12例C级16例。结果:经随访1～7年,31例中26例无并发症发生,生存质量较高;1例术后近期死于肾功能衰竭;1例手术后1年死于肝衰;1例术后3年死于消化道出血;2例在随访中出现腹水。本组出血控制31例,出血控制率为96.88%,3～5年生存率为90.63%。结论:胃周血管离断及胃底环扎术对治疗门静脉高压症并发大出血有较好疗效,值得临床推广应用。     

二尖瓣病变伴左房血栓的外科治疗

本文报道了18例风湿性心脏病伴有左房血栓病人的手术治疗,其中瓣膜置换者5例,二尖瓣直视成形者13例。死亡4例中1例死于脑栓塞。血栓形成和房颤有直接关系。左房血栓术前诊断主要依靠心脏B超,血栓治疗以手术清除为主。为预防术后再发,应纠正房颤及适当应用抗凝血药物。     

先天性心脏病合并重度肺动脉高压的诊断性治疗

目的探讨先天性心脏病(简称先心病)合并重度肺动脉高压患者手术指征的判断及围手术期治疗策略。方法总结2008年1月至2011年12月海军总医院收治的先心病合并重度肺动脉高压患者83例,其中室间隔缺损65例,房间隔缺损8例,动脉导管未闭5例,右室双出口3例,主动脉缩窄及主动脉弓离断各1例,给予强心、利尿、选择性扩张肺动脉的药物治疗;选择同期16例患者为对照组,仅给予强心、利尿治疗。监测末梢血氧饱和度变化,以氧饱和度稳定在95%以上为标准,判断手术指征。结果治疗组手术64例(77.1%),围手术期死亡2例,无远期死亡患者。对照组手术5例(31.3%),远期死亡1例。结论通过药物对重度肺动脉高压患者进行诊断性治疗,可以对肺动脉高压性质做出准确的判断,判断手术指征,降低手术死亡率,获得良好的疗效。     

Sarcoidosis with renal involvement.

Nine of ninety patients with sarcoidosis were found to have significant renal impairment. Epithelioid granulomata were present in five of eight patients who had renal biopsies and glomerular lesions were present in six. There was close correlation between hypercalcaemia, hyperuricaemia, nephrocalcinosis and creatinine clearance. In one patient, renal sarcoidosis complicated membrano-proliferative glomerulonephritis and one patient died in end-stage renal disease.     

三尖瓣病变合并心脏恶病质的手术治疗

目的：探讨合并心脏恶病质三尖瓣病变的手术治疗。方法：对7例符合心脏恶病质诊断标准的重度三尖瓣病变患者采用三尖瓣置换术，围手术期予以积极纠正心力衰竭并改善营养状况。结果：7例手术均获成功，早期死亡1例，死于严重心律失常和心力衰竭。平均随访32个月，1例存在一定程度的右心功能不全表现，余患者恢复良好。结论：三尖瓣置换是合并心脏恶病质三尖瓣病变的有效治疗方法，但应严格掌握手术适应征，术中应注意避免损伤传导束。围手术期改善心功能及加强营养支持对提高手术疗效有重要的意义。