Objective To investigate the surgical measures for relieving biliary malignancies that occur in bile duct cysts (BDCs). Methods Twenty four cases of biliary carcinoma occurring in BDCs treated in our Department from October 1965 to June 1999 were reviewed. Results Of the patients, 5 were male and 19 female. The average age at the time of being diagnosed was 38. Nine patients had undergone cystoenterostomy before. The mean duration between operation of drainage and detection of carcinoma was 10 years. Operations included excision of the extrahepatic bile duct plus pancreatoduodenectomy in 5, excision of extrahepatic bile duct in 6, and various palliative procedures because of extensive spread of tumor in 13. Follow up data were obtained in all but 2 patients who died in hospital. The mean survival time for the 16 death cases after discharge was 11 months. Four patients have been alive and free from disease for 12, 7, 6.5 and 0.5 years, respectively. Conclusions 1) Early diagnosis and curative resection for the biliary carcinoma in BDCs are the key points to achieve better outcomes. 2) Prophylactic excision of cyst in adults with BDCs, especially those previously treated with cystoenterostomy, can effectively reduce the risk of malignant changes.

Objective To characterize the spectrum of adult choledochal cyst and to determine suitable surgical procedures for this disorder.Methods The medical records of 169 patients who were treated for choledochal cyst from July 1977 to October 2008 in our Department were retrospectively reviewed.The cysts were classified using Todani's classification as type Ⅰ in 110 patients (65.1％),type Ⅱ in one patient (0.6％),and type Ⅳ in 56 patients (33.1％).The choledochal cysts in two patients could not be classified because of the lack of clinical data.163 patients received surgical treatment,which included cystectomy (n=119),cystenterosotmy (n=33),T-tube drainage following bile duct exploration (n=5),surgical exploration/biopsy (n =5),and endoscopic sphincterotomy (n =1).Results There was an increase in the number of patients in the 1980s which became stable at round 30 patients per every four years since the late 1990s.The proportion of patients presenting with a history of biliary surgery decreased and accounted for about 25％ of patients in the past decades.The rate of cyst malignancy dropped from 16.5％ in 1977=1995 to 9.7％ in 1996=2008.Of the 163 patients who received surgical treatment,long-term complications included anastomotic strictures and intrahepatic bile ductal stones presenting with repeated cholangitis.Conclusions The incidence of adult congenital choledochal cysts had become stable following years of rapid increase.The rate of cancer progression had gradually decreased.The use of different surgical treatments based on the condition of the proximal bile duct helped to prevent postoperative stenosis of biliary anastomosis.

Objective To assess the long-term survival and prognosfie factors in a series of patients with distal bile duet carcinoma. Methods A retrospective clinical analysis was made on 76 cases of distal bile duct cancer who were admitted into our hospital from January 1996 to December 2006. Clinicopathologic factors with possible prognostic significanees were selected and analyzed. Survival was calculated with the Kaplan-Meier method. A multivariate analysis of these individuals was performed using the Cox proportional Hazards Model. Results There were 46 males and 30 females. The age ranged from 21 to 88 years with a mean of 65.21 patients received palliative surgery including, bypass procedure, intraoperative biliary stenting, or percutaneous transhepatie biliary drainage. Radical resection was performed on 42 cases and the 1-, 3- and 5-yeur survival rates were 88.0%, 41.3% and 29.2% respectively. 38 cases died of liver metastasis or recurrence. In multivariate analysis, surgical procedure (P = 0.006) and liver metastasis (P = 0.008), but not sex, age, invasion of pancreas, invasion of duodenum, diameter or differentiation of tumor, were significant independent prognostic factors. Conclusions Radical resection is only curative treatment modality. Prevention on postoperative liver metastasis is essential for improving survival.

Objective To study prognostic factors after surgical resection for distal bile duct cancer. Methods A retrospective muhicenter clinical analysis,including Beijing Shijitan Hospital,Peking University People's Hospital and Peking University First Hospital,was made for 103 patients of distal bile duct cancer receiving surgical resection from 1995 to 2009.Potential clinicopathological prognostic factors were examined bv univariate and multivariate survival analysis. Results The 1.3 and 5 years overall survival rate was 72%,41%and 25% respectively (median survival time,24.13 months).Univariate analysis revealed operative modality,lymph node status,surgical margin and TNM stage as significant factors influencing postoperative survival.Positive surgical margin,lymph node metastasis and TNM stage were significant independent predictors of poor prognosis by a Cox proportional hazards regression model. Condusions Surgical margin margin,lymph node metastasis and TNM stage were the most important prognostic factors for bile duct carcinoma after resection.Radical pancreaticoduodenectomy was the choice of therapy for distal bile duct carcinoma.

Objective To assess the safety and efficacy of extended liver resection using preoperative PTCD (percutaneous transhepatic cholangial drainage) and PVE (portal vein embolization) to treat patients with locally advanced hilar cholangiocarcinoma.Methods We collected and analyzed the clinical data of 27 patients with Bismuth types Ⅲ and Ⅳ hilar cholangiocarcinoma who underwent extended hepatecomy using preoperative PTCD and PVE in our hospital.Results There were 21 patients with R0 resection and 6 patients with R1 resection.The mortality rate was 0％.Eight patients died of cancer recurrence.Conclusion Preoperative PTCD and PVE combined with extended hepatectomy were safe and efficacious in treating patients with locally advanced hilar cholangiocarcinoma,which resulted in potential cure.

Objective To study the role of Ⅲb isoform of human fibroblast growth factor receptor 1 (FGFR1-Ⅲb) in PANC-1 pancreatic cancer cells. Methods The plasmid of human full-length FGFR1-Ⅲb isoform，pSVK4/FGFR1-Ⅲb, was stable transfected into cultured PNAC-1 pancreatic cancer cell lines facilitated by lipofectamine. The function of FGFR1-Ⅲb in transfected pancreatic cancer cells were examined by MTT assay, soft agar assay, cell migration assay, single cell movement assay, In vivo tumorigenicity assay. Results The basal anchorage-dependent and -independent cell growth was significantly inhibited. Additionally, FGFR1-Ⅲb expression inhibited single cell movement and in vitro invasion as determined by time-lapse microscopy and boyden chamber assay as well as in vivo tumor formation and growth in nude mice. Microscopic analysis of the xenograft tumors revealed a reduced Ki-67 labelling, lower amount of tumor necrosis and higher grade of differentiation in FGFR1-Ⅲb expressing tumors. Conclusion We identified a functional human FGFR mRNA splice variant that inhibits the transforming potential of pancreatic cancer cells.

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a type of newly recognized autoimmune encephalitis which is commonly seen in children, but its precise etiology is still uncertain. To reveal the etiology of anti-NMDAR encephalitis is very necessary for understanding its pathology, and for starting immune-related therapy as early as possible to improve its prognosis. In the initial literature, tumor, especially teratoma is more related with the anti-NMDAR encephalitis. In recent research, its etiology is related to infection and heredity. This article reviews the recognition and variation of the etiology of anti-NMDAR encephalitis.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis ; etiology ; genetics ; Humans ; Infection ; complications

OBJECTIVETo study the correlation of insulin resistance (IR) with TCM syndrome type and activity of fibrinolytic system in patients with coronary arterial disease (CAD).

METHODSOne hundred and twelve CAD patients were classified according to TCM Syndrome into 4 types, the Xin-blood stasis (XBS) type, the phelgm blocking Xin-channel (PBXC) type, the Qi-insufficiency with blood stasis (QIBS) type and the both Qi-Yin deficiency (QYD) type. Patients' fasting blood glucose (FBG), fasting blood insulin (FIns) were measured, the insulin sensitive index (ISI) calculated. Data were compared between various types, also with those obtained from 30 healthy persons in the control group respectively. Moreover, activity of tissue plasminogen activator (t-PA) and content of plasminogen activator inhibitor-1 (PAI-1) were determined in 90 patients selected from the 112 to conduct linear correlation analysis of IR with t-PA activity and PAI-1 content.

RESULTSFBG and FIns levels in the CAD patients were higher than those in the healthy control significantly (P < 0.01); ISI in the 4 syndrome type of CAD patients were all lower than that in the control (P < 0.01). IR existed in all the 4 types, but the level in the XBS type and the PBXC type was more severe than in the other two types. Correlation analysis showed that IR was correlated with t-PA activity and PAI-1 content (P < 0.01).

CONCLUSIONIR often exists in CAD patients, the severity of IR varies in patients of different TCM syndrome types, and IR is correlated with the abnormality of fibrinolytic system activity.

Adult ; Aged ; Aged, 80 and over ; Angina Pectoris ; blood ; diagnosis ; Diagnosis, Differential ; Female ; Fibrinolysis ; Humans ; Insulin Resistance ; Male ; Medicine, Chinese Traditional ; Middle Aged ; Myocardial Infarction ; blood ; diagnosis ; Plasminogen Activator Inhibitor 1 ; metabolism ; Tissue Plasminogen Activator ; metabolism

BACKGROUNDFamilial cerebral cavernous malformations (CCMs), characterized by hemorrhagic stroke, recurrent headache and epilepsy, are congenital vascular anomalies of the central nervous system. Familial CCMs is an autosomal dominant inherited disorder and three CCM genes have been identified. We report a Chinese family with CCMs and intend to explore clinical, pathological, magnetic resonance imaging (MRI) features and pathogenic gene mutation of this family.

METHODSTotally 25 family members underwent brain MRI examination and clinical check. Two patients with surgical indications had surgical treatment and the specimens were subjected to histopathological and microstructural examination. In addition, polymerase chain reaction (PCR) and direct sequencing were performed with genomic DNA extracted from 25 family members' blood samples for mutation detection.

RESULTSBrain MRI identified abnormal results in seven family members. All of them had multiple intracranial lesions and four cases had skin cavernous hemangioma. T2-weighted sequence showed that the lesions were typically characterized by an area of mixed signal intensity. Gradient-echo (GRE) sequence was more sensitive to find micro-cavernous hemangiomas. There was a wide range in the clinical manifestations as well as the age of onset in the family. The youngest patient was an 8-year-old boy with least intracranial lesions. Histopathological and microstructural examination showed that CCMs were typically discrete multi-sublobes of berry-like lesions, with hemorrhage in various stages of illness evolution. They were formed by abnormally enlarged sinusoids and the thin basement membranes. A novel T deletion mutation in exon 14 of CCM1 gene was identified by mutation detection in the seven patients. But unaffected members and healthy controls did not carry this mutation.

CONCLUSIONSThe clinical manifestations were heterogenic within this family. We identified a novel mutation (c.1396delT) was the disease-causing mutation for this family and extended the mutational spectrum of CCMs.

Adult ; Animals ; Female ; Hemangioma, Cavernous, Central Nervous System ; diagnosis ; genetics ; Humans ; KRIT1 Protein ; Magnetic Resonance Imaging ; Male ; Microtubule-Associated Proteins ; genetics ; Middle Aged ; Mutation ; Pedigree ; Proto-Oncogene Proteins ; genetics

OBJECTIVETo investigate the mechanism of Panax notoginseng saponins (PNS), an effective component extracted from Panax notoginseng, on atherosclerotic plaque angiogenesis in atherosclerosis-prone apolipoprotein E-knockout (ApoE-KO) mice fed with high-fat, high-cholesterol diet.

METHODSTwenty ApoE-KO mice were divided into two groups, the model group and the PNS group. Ten normal C57BL/6J mice were used as a control group. PNS (60 mg/kg) was orally administered daily for 12 weeks in the PNS group. The ratio of plaque area to vessel area was examined by histological staining. The tissue sample of aortic root was used to detect the CD34 and vascular endothelial growth factor (VEGF) expression areas by immunohistochemistry. The expression of VEGF and nicotinamide adenine dinucleotide phosphate oxidase subunit 4 (NOX4) were measured by reverse transcription polymerase chain reaction and Western blotting respectively.

RESULTSAfter treatment with PNS, the plaque areas were decreased (P<0.05). CD34 expressing areas and VEGF expression areas in plaques were significantly decreased (P<0.05). Meanwhile, VEGF and NOX4 mRNA expression were decreased after treatment with PNS. VEGF and NOX4 protein expression were also decreased by about 72% and 63%, respectively (P<0.01).

CONCLUSIONPNS, which decreases VEGF and NOX4 expression, could alleviate plaque angiogenesis and attenuate atherosclerosis.

Animals ; Down-Regulation ; drug effects ; genetics ; Drugs, Chinese Herbal ; pharmacology ; Male ; Mice ; Mice, Inbred C57BL ; Mice, Knockout ; NADPH Oxidase 4 ; NADPH Oxidases ; genetics ; metabolism ; Neovascularization, Pathologic ; pathology ; prevention & control ; Panax notoginseng ; chemistry ; Plaque, Atherosclerotic ; pathology ; prevention & control ; Saponins ; pharmacology ; Vascular Endothelial Growth Factor A ; genetics ; metabolism