成人肝母细胞瘤的诊治:近20年国内文献分析

自1969年Carter[1]首次报道肝母细胞瘤(hepatoblastoma)以来,截止2006年11月英文文献共报道肝母细胞瘤约1000余例.因肝母细胞瘤多发生于儿童,故在文献中亦被称为婴儿型肝癌、肝混合瘤或肝畸胎瘤,而成人肝母细胞瘤则非常罕见.为提高成人肝母细胞瘤诊疗水平,作者复习近20年国内文献报道,对成人肝母细胞瘤的诊断与治疗进行分析.     

成人先天性巨输尿管症的诊断治疗

目的探讨成人先天性巨输尿管症（CM）的诊断治疗.方法回顾性分析19例成人CM的临床资料.男6例,女13例,平均年龄36岁.左侧11例,右侧5例,双侧3例.B超、IVU、RGP及MRU等检查,输尿管全段扩张伴肾盂积水16例,患肾无功能4例,仅输尿管下段扩张3例,扩张输尿管最大管径3.3 cm,无输尿管狭窄.尿道膀胱造影（MCUG）及尿动力学检查,无神经性膀胱及膀胱输尿管返流.结果 19例患者中行肾输尿管切除2例,肾造瘘2例,1例1年后行输尿管膀胱再植术,术后5年无异常,另1例4个月后行肾输尿管切除.保守观察3例,随访11～19年,病变无进行性加重.间断性双J管置入3例,随访5～9年,肾功能正常.输尿管裁剪修整,膀胱再植9例,6例随访3～17年无异常;吻合口狭窄及膀胱输尿管返流各1例,再次术后随访6年无异常;1例术后3年行患肾及输尿管切除.结论成人CM诊断主要依据影像学和尿动力学检查,治疗原则为及早手术、去除病因、解除梗阻、保留和恢复肾功能,输尿管裁剪修整、膀胱再植术为有效手术方法.     

Hemophagocytic lymphohistiocytosis in adults: Diagnosis and treatment

Hemophagocytic lymphohistiocytosis occurring as a primary or acquired disorder is a condition of chaotic and uncontrolled immune system stimulation. Cytotoxic cells and macrophages cause multiorgan damage, hemophagocytosis, and severe systemic inflammation. Clinical manifestations include a fever, organ enlargement, and weight loss. Laboratory tests show bicytopenia or pancytopenia, cytolysis and cholestasis, serum ferritin elevation, and clotting disorders. The reference standard for the diagnosis remains the presence in histological specimens of hemophagocytic macrophages, which may be lacking early in the disease, leading to diagnostic challenges. Inherited forms produce symptoms in early childhood and are fatal in the absence of specific treatment. In adults, the clinical spectrum ranges from mild and self-limited hemophagocytic lymphohistiocytosis to rapidly fatal multiorgan failure. Many questions remain unresolved regarding the diagnosis and treatment in adults. This update is an attempt at providing answers.     

成人乙状结肠冗长症的诊断与治疗八例分析

目的探讨成人乙状结肠冗长症的临床特点和诊治方法。方法总结4年来收治的8例成人乙状结肠冗长症患者的临床资料，并结合文献进行分析。结果经1年随访择期行冗长肠襻切除术的病例均获满意疗效；仅行肠减压术不能缓解临床症状。结论成人乙状结肠冗长症主要表现是顽固性便秘和反复发作的急性肠梗阻；钡剂灌肠检查可明确诊断；治疗方法首选择期冗长肠襻切除术。     

成人先天性胆管扩张症的诊断与治疗

目的探讨成人先天性胆管扩张症的诊断与治疗。方法回顾分析我院1985年至2004年30例成人先天性胆管扩张症的临床资料。结果28例行B超检查,确诊率为92.9%(26/28)。5例CT、4例ERCP、3例PTC、6例MRCP检查均全部确诊。本组30例确诊率100%。手术27例,其中囊肿内引流术5例,术后疗效欠佳,囊肿切除肝管空肠Roux-Y吻合术20例,疗效满意。全组无手术死亡。结论上腹痛是成人先天性胆管扩张症最常见的临床表现,结合影像学检查可确诊,囊肿切除肝管空肠Roux-Y吻合术为首选术式。     

成人先天性胆管囊肿的诊断与治疗

作者总结了近３０年来成人先天性胆管囊肿９７例的治疗经验。患者平均就诊年龄为３７岁。多数患者症状不典型,常致诊断延误。７４例（７６％）合并肝胆胰系统疾病,１６例合并癌变,癌变率１７％。发现胰胆管合流异常２８例。９４例患者共行胆道手术１６９例次,囊肿切除、肝管空肠Ｒｏｕｘ－Ｙ吻合术及囊肿内引流术为主要术式。术后长期随访表明内引流术后常因胆管炎复发和囊肿瘤变而需再次手术,相反囊肿切除术明显降低了胆管炎的     

成人脊髓栓系综合征的诊治

目的研究成人脊髓栓系综合征（ＴＣＳ）的临床特征以指导诊断和治疗。方法报告１８例成人ＴＣＳ的诊治情况。患者均经ＭＲＩ证实并行手术松解;根据手术所见和影像学分析其致病机理及手术要点。结果栓系原因分别为椎管内肿瘤（６例）、脊髓脊膜膨出（６例）、终丝增粗（３例）、脊髓双裂畸形（２例）及术后粘连（１例）。经８个月至５年随访,其中１４例获得满意疗效。结论成人ＴＣＳ在临床表现及预后等方面有别于幼儿ＴＣＳ;术中应避免损伤圆锥和马尾神经;病程过长及再栓系是疗效不佳的主要原因     

成人先天性胆管囊肿的诊断与治疗(附97例报告)

作者总结了近３０年来成人先天性胆管囊肿９７例的治疗经验。患者平均就诊年龄为３７岁。多数患者症状不典型，常致诊断延误。７４例（７６％）合并肝胆胰系统疾病，１６例合并癌变，癌变率１７％。发现胰胆管合流异常２８例。９４例患者共行胆道手术１６９例次，囊肿切除、肝管空肠Ｒｏｕｘ－Ｙ吻合术及囊肿内引流术为主要术式。术后长期随访表明内引流术后常因胆管炎复发和囊肿癌变而需再次手术，相反囊肿切除术明显降低了胆管炎的复发率及癌变的发生。作者认为囊肿切除、肝管空肠Ｒｏｕｘ－Ｙ吻合术应作为成人胆管囊肿的首选术式。     

成人肠套叠28例诊治体会

目的探讨成人肠套叠的诊断和治疗方法。方法对我院10年来收治的28例成人肠套叠的诊断及治疗进行回顾性分析。结果术前确诊9例,术中确诊19例。引起套叠的主要原因有良性肿瘤(10例)、恶性肿瘤(7例)及炎性损伤(5例)等。28例均经手术治疗康复出院。结论成人肠套叠多由器质性病变引起,诊断主要依据临床表现和辅助检查,必须手术治疗。     

成人睾丸良性占位病变的诊断与治疗选择(附16例报告)

目的 探讨成人睾丸良性占位病变的诊断与治疗选择.方法 对2003～2010年诊治的16例睾丸良性肿瘤的临床资料及随访情况进行回顾性分析.结果 16例睾丸肿瘤病例术前经超声、CT或MRI、AFP和β- HCG检查,诊断13例考虑为良性病变,3例可疑恶性.术中行冰冻切片检查10例,其中7例行睾丸部分切除术,3例性质不定行睾丸根治性切除术.6例未行术中病理检查直接行根治性睾丸切除.术后经病理诊断睾丸间质细胞瘤2例,成熟畸胎瘤4例,腺瘤样瘤3例,平滑肌瘤3例,皮样囊肿1例,混合肿瘤3例.术后随访2个月～12年均未出现复发及转移.结论 睾丸良性占位的术前诊断对手术处理方式选择非常重要,B超、CT或MRI、肿瘤标记物以及术中冰冻切片活检均具有临床诊断价值.睾丸良性肿瘤的治疗应首选保留睾丸的肿瘤切除.     

Diagnosis and treatment of isolated tuberculous mediastinal lymphadenopathy in adults.

OBJECTIVE: To evaluate the diagnostic yield of bronchoscopy and mediastinoscopy in adults with isolated mediastinal tuberculous lymphadenitis and to assess the effect of antituberculous treatment. DESIGN: Prospective longitudinal cohort study of 34 patients with mediastinal tuberculous lymphadenitis followed for 6 to 19 months after completion of treatment. SETTING: Tertiary care hospital, Kuwait. PATIENTS: 34 consecutive patients who presented with isolated mediastinal lymphadenopathy from 1996 to 1998. INTERVENTIONS: Bronchoscopy and cervical mediastinoscopy for all patients. MAIN OUTCOME MEASURES: Diagnostic yield of bronchoscopy and mediastinoscopy, and the outcome of treatment in patients with tuberculous lymphadenopathy. RESULTS: The mean age was 35 years (range 15-58). The most common symptoms were cough, fever, and weight loss. The chest radiographs and computed tomograms showed abnormal mediastinal shadows with no evidence of parenchymal disease. All patients had right sided paratracheal lymphadenopathy. Tuberculin skin test gave a weal of >15 mm in 17 patients (50%). Sputum smears and cultures failed to grow acid-fast bacilli in any patient. Seven patients had an endobronchial abnormality and samples taken at bronchoscopy gave a definite diagnosis in 3 (9%). Paratracheal lymph node biopsy and culture by mediastinoscopy diagnosed tuberculosis in all cases. All patients were treated by a six month course of rifampicin and isoniazid supplemented initially by pyrazinamide for two months. Twenty-eight patients had a good response and the remaining patients were treated for a further 3 months. CONCLUSIONS: Bronchoscopy has a low diagnostic yield in mediastinal tuberculous lymphadenopathy in the absence of a parenchymal lesion. Mediastinoscopy is a safe but invasive procedure and provides a tissue diagnosis in most cases. Six months treatment with rifampicin and isoniazid supplemented initially by pyrazinamide is adequate treatment for most adults with tuberculous mediastinal lymphadenopathy.     

成人重复肾畸形并肾积水的诊疗分析

目的分析总结成人重复肾畸形并肾积水的诊疗方案。方法回顾性分析40例成人重复肾畸形并肾积水患者的临床资料,其中男性11例,女性29例,年龄18～77岁。并发重度肾积水29例,轻度肾积水11例。结果 29例重度肾积水患者均行肾部分切除术,其中开放手术23例,腹腔镜手术6例;11例轻度肾积水患者中5例行输尿管膀胱再植术,5例行输尿管囊肿电切术,1例行肾盂成形术。40例患者中3例出现术后并发症。结论成人重复肾畸形并重度肾积水者应行肾部分切除术,腹腔镜较之开放手术具有创伤小、出血少、恢复快等优点;轻度肾积水者可通过解除膀胱输尿管返流、输尿管口囊肿、肾盂输尿管连接部梗阻等改善肾功能。     

成人门静脉海绵样变性的诊断和治疗

目的 探讨成人门静脉海绵状变性（ＰＶＣＴ）的诊断和治疗。方法 对１５例成人ＰＶＣＴ的临床资料进行回顾性分析。结果 所有病人术前均经Ｂ超或多普勒超声检查提示肝脏正常及ＰＶＣＴ,并经经皮脾穿刺门静脉造影或选择性肠系膜上动脉造确诊。９例行脾动脉结扎、冠状静脉结扎、肠系膜上民下腔静脉Ｃ型架桥术（肠腔Ｃ型架桥）;１例行脾动脉结扎、脾肾静脉Ｃ型架桥术;５例已行脾切除加断流术后再出血者４例行肠腔Ｃ型架桥术,１例     

109例成人型胆管囊肿的诊治经验

Objective To discuss the diagnosis and treatment of congenital choledochal cyst (CCC).Methods The clinical data of 109 adult patients with congenital choledochal cyst admitted in Peking Union Medical College Hospita between 1984 and 2007 were retrospectively analyzed.The male: female ratio was 1 : 3.8.The mean age was 32.8 years.Seventy-eight of the patients belonged to Todani type Ⅰ, while twenty-six to type Ⅳ and five to type Ⅴ.There was no case of type Ⅱ or Ⅲ.Results Right upper abdominal pain or discomfort and recurrent cholangitis were the predominant presentations.Thirty patients had undergone previous surgical procedures while 26 procedures were done before 2000.B-US and MRCP were the primary methods for diagnosis after 2000.Ninety-four patients underwent complete excision of the extrahepatic cyst and hepaticojejunostomy.Four patients required the left lateral hepateetomy in addition.Four patients were testified malignant change by pathological examination.There were four pancreatic fistulas and two Subphrenic abscesses.There was no postoperative death.Forty-six patients were available for follow-up.The late complications in-eluded recurrent cholangitis, hepaticojejunostomy anastomosis stricture and intrahepatic biliary calcu-la.Conclusions Right upper abdominal pain or discomfort and recurrent cholangitis were the most common presentations in adult congenital choledochal cyst patients.B-US and MRCP should be the prefered imaging methods for diagnosis.Complete excision of the extrahepatic cyst and hepaticojeju-nostomy were ideal treatment.     

109例成人型胆管囊肿的诊治经验

Objective To discuss the diagnosis and treatment of congenital choledochal cyst (CCC).Methods The clinical data of 109 adult patients with congenital choledochal cyst admitted in Peking Union Medical College Hospita between 1984 and 2007 were retrospectively analyzed.The male: female ratio was 1 : 3.8.The mean age was 32.8 years.Seventy-eight of the patients belonged to Todani type Ⅰ, while twenty-six to type Ⅳ and five to type Ⅴ.There was no case of type Ⅱ or Ⅲ.Results Right upper abdominal pain or discomfort and recurrent cholangitis were the predominant presentations.Thirty patients had undergone previous surgical procedures while 26 procedures were done before 2000.B-US and MRCP were the primary methods for diagnosis after 2000.Ninety-four patients underwent complete excision of the extrahepatic cyst and hepaticojejunostomy.Four patients required the left lateral hepateetomy in addition.Four patients were testified malignant change by pathological examination.There were four pancreatic fistulas and two Subphrenic abscesses.There was no postoperative death.Forty-six patients were available for follow-up.The late complications in-eluded recurrent cholangitis, hepaticojejunostomy anastomosis stricture and intrahepatic biliary calcu-la.Conclusions Right upper abdominal pain or discomfort and recurrent cholangitis were the most common presentations in adult congenital choledochal cyst patients.B-US and MRCP should be the prefered imaging methods for diagnosis.Complete excision of the extrahepatic cyst and hepaticojeju-nostomy were ideal treatment.     

109例成人型胆管囊肿的诊治经验

Objective To discuss the diagnosis and treatment of congenital choledochal cyst (CCC).Methods The clinical data of 109 adult patients with congenital choledochal cyst admitted in Peking Union Medical College Hospita between 1984 and 2007 were retrospectively analyzed.The male: female ratio was 1 : 3.8.The mean age was 32.8 years.Seventy-eight of the patients belonged to Todani type Ⅰ, while twenty-six to type Ⅳ and five to type Ⅴ.There was no case of type Ⅱ or Ⅲ.Results Right upper abdominal pain or discomfort and recurrent cholangitis were the predominant presentations.Thirty patients had undergone previous surgical procedures while 26 procedures were done before 2000.B-US and MRCP were the primary methods for diagnosis after 2000.Ninety-four patients underwent complete excision of the extrahepatic cyst and hepaticojejunostomy.Four patients required the left lateral hepateetomy in addition.Four patients were testified malignant change by pathological examination.There were four pancreatic fistulas and two Subphrenic abscesses.There was no postoperative death.Forty-six patients were available for follow-up.The late complications in-eluded recurrent cholangitis, hepaticojejunostomy anastomosis stricture and intrahepatic biliary calcu-la.Conclusions Right upper abdominal pain or discomfort and recurrent cholangitis were the most common presentations in adult congenital choledochal cyst patients.B-US and MRCP should be the prefered imaging methods for diagnosis.Complete excision of the extrahepatic cyst and hepaticojeju-nostomy were ideal treatment.     

109例成人型胆管囊肿的诊治经验

Objective To discuss the diagnosis and treatment of congenital choledochal cyst (CCC).Methods The clinical data of 109 adult patients with congenital choledochal cyst admitted in Peking Union Medical College Hospita between 1984 and 2007 were retrospectively analyzed.The male: female ratio was 1 : 3.8.The mean age was 32.8 years.Seventy-eight of the patients belonged to Todani type Ⅰ, while twenty-six to type Ⅳ and five to type Ⅴ.There was no case of type Ⅱ or Ⅲ.Results Right upper abdominal pain or discomfort and recurrent cholangitis were the predominant presentations.Thirty patients had undergone previous surgical procedures while 26 procedures were done before 2000.B-US and MRCP were the primary methods for diagnosis after 2000.Ninety-four patients underwent complete excision of the extrahepatic cyst and hepaticojejunostomy.Four patients required the left lateral hepateetomy in addition.Four patients were testified malignant change by pathological examination.There were four pancreatic fistulas and two Subphrenic abscesses.There was no postoperative death.Forty-six patients were available for follow-up.The late complications in-eluded recurrent cholangitis, hepaticojejunostomy anastomosis stricture and intrahepatic biliary calcu-la.Conclusions Right upper abdominal pain or discomfort and recurrent cholangitis were the most common presentations in adult congenital choledochal cyst patients.B-US and MRCP should be the prefered imaging methods for diagnosis.Complete excision of the extrahepatic cyst and hepaticojeju-nostomy were ideal treatment.     

109例成人型胆管囊肿的诊治经验

Objective To discuss the diagnosis and treatment of congenital choledochal cyst (CCC).Methods The clinical data of 109 adult patients with congenital choledochal cyst admitted in Peking Union Medical College Hospita between 1984 and 2007 were retrospectively analyzed.The male: female ratio was 1 : 3.8.The mean age was 32.8 years.Seventy-eight of the patients belonged to Todani type Ⅰ, while twenty-six to type Ⅳ and five to type Ⅴ.There was no case of type Ⅱ or Ⅲ.Results Right upper abdominal pain or discomfort and recurrent cholangitis were the predominant presentations.Thirty patients had undergone previous surgical procedures while 26 procedures were done before 2000.B-US and MRCP were the primary methods for diagnosis after 2000.Ninety-four patients underwent complete excision of the extrahepatic cyst and hepaticojejunostomy.Four patients required the left lateral hepateetomy in addition.Four patients were testified malignant change by pathological examination.There were four pancreatic fistulas and two Subphrenic abscesses.There was no postoperative death.Forty-six patients were available for follow-up.The late complications in-eluded recurrent cholangitis, hepaticojejunostomy anastomosis stricture and intrahepatic biliary calcu-la.Conclusions Right upper abdominal pain or discomfort and recurrent cholangitis were the most common presentations in adult congenital choledochal cyst patients.B-US and MRCP should be the prefered imaging methods for diagnosis.Complete excision of the extrahepatic cyst and hepaticojeju-nostomy were ideal treatment.