胆管消失综合征1例报告

<正>胆管消失综合征(VBDS)最早见Sherlock报道,是以肝内胆管减少为病理学特征,以胆汁淤积为主要临床表现的综合征[1]。病因中包括发育、代谢和免疫学异常、血管病变、感染、淋巴病、药物等因素,真正病因及发病机制尚不明确,临床较为少见[2]。1病历资料患者,女,56岁,无业,因"间断乏力1年"于2011年4月8日入院。既往19年前行剖宫产手术;口服避孕药17年(为短     

Hodgkin's lymphoma-related vanishing bile duct syndrome: A case report and literature review

We report the case of a 38-year-old man who developed vanishing bile duct syndrome in association with Hodgkin's lymphoma. He was noted to have cervical lymphadenopathy and marked elevation of total serum bilirubin at diagnosis. He achieved complete remission with normalization of serum bilirubin after eight courses of Adriamycin, bleomycin, vinblastine, and dacarbazine chemotherapy followed with autologous hematopoietic cell transplantation. Consecutive liver biopsies performed at diagnosis and at the stage of complete remission revealed the disappearance and regeneration of interlobular bile ducts, respectively. Our case provides pathological evidence that Hodgkin's lymphoma-related vanishing bile duct syndrome is a reversible bile duct injury disease. Bilirubin is a reliable serum marker to monitor the treatment response of these cases. The mechanism to develop hyperbilirubinemia with vanishing bile duct in such a case of Hodgkin's lymphoma remains to be studied. A literature review was carried out.     

Resection of intrahepatic bile duct carcinoma with hilar bile duct and portal vein invasion--a case report

A case of resected intrahepatic bile duct cancer with hilar bile duct and portal vein invasion is presented. Percutaneous transhepatic biliary drainage was performed to alleviate jaundice and evaluate the biliary system. Intraductal tumor extension was determined, and an accurate histological diagnosis was made in biopsy material obtained under percutaneous transhepatic cholangioscopy. Preoperative surgical planning was carried out on the basis of an evaluation of the findings of ultrasonography, computed tomography, arteriography, portography and percutaneous transhepatic cholangioscopy. Curative surgery, which included right hepatic lobectomy with total caudate lobectomy and combined resection and reconstruction of the portal vein, was performed. Bilioenteric continuity was re-established by a Roux-en-Y jejunal loop. The histological diagnosis was moderately differentiated tubular adenocarcinoma originated in the right posterior branch of the intrahepatic bile duct. Postoperative recovery was very good, and the patient has now been enjoying a good active social life for the past three years with no signs of tumor recurrence. This case report discusses the accurate diagnosis and rational surgical treatment for intrahepatic bile duct carcinoma with hilar invasion.     

An autopsy case of granulocyte-colony-stimulating-factor-producing extrahepatic bile duct carcinoma

A 79-year-old man was referred to this department due to the presence of extrahepatic bile duct carcinoma with a tumor at the left chest wall. The lesion was suspected to be a metastasis of bile duct carcinoma to the left wall, however, computed tomography （CT） revealed no regional lymph node or liver metastases. In addition, cytological and pathological examinations did not show malignancy. At the time of admission, the white blood cell count was 21 460 cells/μL （neutrophils, 18 240 cells/μL） and this elevated to 106 040 before death. In addition, serum granulocyte colony-stimulating factor （G-CSF） was elevated. At 28 d after admission, the patient died. An autopsy showed a poorly differentiated adenocarcinoma with sarcomatous change, which had slightly invaded into the pancreas around the bile duct, and was found in the distal bile duct with multiple metastases to the chest wall, lung, kidney, adrenal body, liver, mesentery, vertebra and mediastinal and para-aortic lymph nodes, without locoregional lymph node and liver metastasis. The cancer cells showed positive immunohistochemical staining for anti-G-CSF antibody. This is believed to be the first report of an extrahepatic bile duct carcinoma that produces G-CSF. Since G-CSF-producing carcinoma and sarcomatous change of the biliary tract leads to poor prognosis, early diagnosis and treatment are needed. When infection is ruled out, the G-CSF in serum should be examined. In addition, examinations such as bonescintigraphy and chest CT should also be considered for distant metastasis.     

An autopsy case of pseudosarcoma of the common bile duct

An autopsy case of pseudosarcoma in the common bile duct is reported. An 82-year-old Japanese male complaining of jaundice was admitted to our hospital; he was examined by abdominal ultrasonography (US), revealing biliary calculus, dilatation of the common bile duct, and choledocholithiasis, considered to be the possible cause of the obstructive jaundice. Endoscopic retrograde biliary drainage (ERBD) and cholangioscopy were performed concurrently, revealing a vaguely whitish tumor near the papilla of Vater. Two months later, the patient died from complications of the liver, infection, and disseminated intravascular coagulation (DIC). An autopsy study revealed tumor cells with extreme pleomorphic changes, growing diffusely, very like sarcoma. Further examination revealed epithelioid arrangements in the metastatic lymph node. Twelve kinds of immunohistochemical examination showed a positive reaction, reflecting the presence of an epithelioid cytoskeleton. Of 28 cases of true and pseudosarcoma of the biliary system reported in the Japanese literature, only 1 case was reported, in 1990, to involve the common bile duct. We therefore report the present case of pseudosarcoma of the common bile duct.     

Hamartoma of the bile duct. Report of a case

BACKGROUND: Gallstones and neoplasms generally cause obstructions in the biliary duct. There are some infrequent problems that may lead to obstructions, such as hamartomas of the biliary duct. Case report: A 34-year-old diabetic female patient begins with abdominal pain and jaundice. The diagnostic approach initiated with hepatobiliary ultrasound that reported cholelithiasis and enlargement of the biliary ducts. Cholangiopancreatography was subsequently performed and a biliary tree lesion was discovered; the patient underwent cholecystectomy with exploration of the biliary ducts. Clinical evolution was satisfactory. Pathologic findings indicated hamartoma of the biliary duct.     

肝结节病出现重度黄疸致肝硬化1例

一、临床资料1．病历摘要：患者女性,68岁,主诉间断乏力、腹胀、尿黄2年,加重半月于2006年5月第2次住院。患者2年前开始有乏力、腹胀、尿黄等不适症状,肝功能轻度异常,以“慢性肝炎”第1次住院,经保肝对症治疗20 d,肝功能好转,病原学检查阴性。其间出现咳嗽、气短症状,无发热,胸片检查提示肺门淋巴结肿大,双肺野可见点片状阴影,[第一段]     

Cytomegalovirus infection persists in the liver graft in the vanishing bile duct syndrome.

Cytomegalovirus infection is one factor implicated in the cause of the vanishing bile duct syndrome complicating liver transplantation. To further investigate the role of cytomegalovirus in this syndrome, we studied serial liver biopsy material by in situ hybridization for cytomegalovirus DNA using a highly sensitive technique that allows the localization of viral replication. Cytomegalovirus DNA was identified in hepatocytes in 10 of 12 patients with the vanishing bile duct syndrome, 1 of whom had no serological evidence of cytomegalovirus infection. It was also present in all 18 patients with uncomplicated cytomegalovirus infection but was not identified in any of 10 subjects with transplants who had neither complication. Nine of the patients in this series underwent a diagnostic liver biopsy at 1 wk and subsequently had cytomegalovirus infection develop; cytomegalovirus DNA was identified in liver tissue of all nine patients, indicating that cytomegalovirus replication commences at an early stage. In those with uncomplicated cytomegalovirus, infection occurred earlier (p less than 0.05) but was eliminated more quickly (p less than 0.0005), and the number of infected hepatocytes was greater (p less than 0.05) when compared with those with the vanishing bile duct syndrome; in these, cytomegalovirus DNA was detectable until death or retransplantation. Cytomegalovirus DNA was never identified in either biliary or endothelial tissue. These data indicate that the vanishing bile duct syndrome is associated with persistent cytomegalovirus replication within hepatocytes.     

Ulcerative colitis, primary sclerosing cholangitis, bile duct carcinoma, and generalized sarcoidosis. Report of a unique association

We describe a young man with a hitherto unreported association of chronic ulcerative colitis, primary sclerosing cholangitis (PSC), bile duct carcinoma, and generalized sarcoidosis with features of high-intensity alveolitis. This finding suggests that common immunological mechanisms may be involved in the pathogenesis of these diseases.     

Lack of association between cytomegalovirus infection, HLA matching and the vanishing bile duct syndrome after liver transplantation.

In this study we evaluated the association between cytomegalovirus infection alone or in relation to human leukocyte antigen matching and the development of vanishing bile duct syndrome, a form of chronic hepatic allograft rejection. A total of 81 consecutive liver transplant recipients were studied. Cytomegalovirus infection developed in 46 recipients (57%), and vanishing bile duct syndrome occurred in 9 recipients (11%). Cytomegalovirus infection developed in only five of the nine patients with vanishing bile duct syndrome. Univariate analysis of pretransplant recipient/donor cytomegalovirus serological tests and human leukocyte antigen typing showed they were not significant risk factors for the development of vanishing bile duct syndrome. Time-dependent analysis of cytomegalovirus infection after transplantation as a risk factor for vanishing bile duct syndrome, in a multivariate analysis with human leukocyte antigen match, showed no statistical significance. In our study, no association was found between cytomegalovirus infection alone or in relation to class I or II human leukocyte antigen match and the subsequent development of vanishing bile duct syndrome.