Objective To evaluate the efficacy of liver grafts with warm ischemia and with different cold preservation time in liver transplantation.Methods The clinical data of 154 patients who received liver transplantation at the First Affiliated Hospital of Sun Yat-sen University from January 2006 to December 2007 were retrospectively analyzed.The warm ischemia time of the liver grafts obtained from the non-heart-beating donors was within 10 minutes.According to cold perservation time of the liver grafts,patients were divided into 3 groups:the cold preservation time of the liver grafts was within 8 hours,8-12 hours and above 12 hours in group I(n=58),group Ⅱ(n=62)and group Ⅲ(n=34),respectively.The peak level of alanine aminotransferase(ALT),primary graft dysfunction(PGD)after liver transplantation,acute rejection response,biliary complications,vessel complications,perioperative infections and the survival of liver grafts and recipients among the 3 groups were analyzed via chi-square test,t test and variance analysis.Results No PGD was detected in the 3 groups after liver transplantation.All patients were followed up for 8-32 months.The peak level of ALT,incidence of infection and biliary complication,survival of liver grafts and recipients were(482±357)U/L,12%(7/58),12%(7/58),86%(50/58)and 88%(51/58)in group Ⅰ,and were(1274±608)U/L,29%(10/34),26%(9/34),68%(23/34)and 71%(24/34)in group Ⅲ,with significant difference between the 2 groups(t=5.23,X~2=4.28,6.77,4.51,4.28,P<0.05).The peak level of ALT in group Ⅱ was(953±424)U/L,which was significant higher than(482±357)U/L in group Ⅰ(t=4.76,P＜0.05).Conclusions Liver grafts with a warm ischemia time shorter than 10 minutes could tolerate the injury caused by cold preservation with the maximum time of 12 hours.The incidences of biliary complications and postoperative infections are significantly increased and the survivals of liver grafts and recipients are decreased when the cold preservation time exceeds 12 hours.

Objective To investigate the features of familiar facial palsy,ophthalmoplegia and dysphagia characterized by autosomal dominant inheritance in a family and to discuss the classification and pathogenesis of the disease.Methods Clinical,electrophysiological,pathological examinations were performed and blood samples were obtained from 5 patients and 26 family members.PCR protocol was used to identify a certain gene. Results In the 5 patients receiving physical examination,all had ptosis,external ophthalmoplegia,facial paralysis,dyphagia,hoarseness,decreased pharyngeal reflex;4 had amyotrophy of muscle of tongue,temporal nuscle,masseter and muscles of distal lower limbs;3 had proximal limb asthenia and distal limbs amyotrophy.Compared to those of oculopharyngeal muscular dystrophy(OPMD)with similar symptoms and signs,both electrophysiological manifestation and pathological findings of the family members supported the diagnosis of muscular dystrophy,but the(GCG)6(GCA)3GCG in the first exon of PABPN1 mutated neither in normal family members nor in patients.Conclusions This family presents clinical manifestations somewhat resembling to those of OPMD and distinctive to other disorders,but has a totally different genetic background from OPMD.It may be a new subtype of muscular dystrophy.

BACKGROUND: Tumor recurrence in liver transplant recipients greatly affects prognosis of liver transplantation with hepatocellular carcinoma (HCC). How to prevent tumor recurrence has aroused increasing attention. Arsenious acid chemotherapy is considered effective on treating moderate or advanced liver cancer, but its utilization following liver transplantation remains few. OBJECTIVE: To explore the role of arsenious acid on tumor recurrence in liver transplant patients with primary HCC extending Milan criteria. RESULTS AND CONCLUSION: All patients were routinely followed up for 3-32 months. Thirty recipients were presented with tumor recurrence, 16 in the chemotherapy group and 14 in the non-chemotherapy group. Tumor recurred in lung, liver graft and bones in most cases. The total recurrence rate was similar in these two groups, but chemotherapy could delay recurrence after transplantation (P=0.026). There was no significance in 6-month, 1-year survival rate between two groups, but the 2-year survival in the chemotherapy group was higher (P=0.037); 6-month tumor-free survival rates in the two groups had no significance, 1-year and 2-year tumor-free in the chemotherapy group were significantly higher than those in the non-chemotherapy group (P=0.030, 0.023). Intravenous arsenious acid chemotherapy can delay tumor recurrence and prolong survival in liver transplant patients with HCC extending Milan criteria.

Objective To evaluate the effect of endoscopic retrograde cholangiopancreatography (ERCP) in treatment of biliary complications after liver transplantation. Methods Data of 39 patients who underwent ERCP between January 2005 and December 2007 because of biliary complications after liver trans-plantation were retrospectively evaluated. Endoscopic sphincterotomy, dilatation, nasal-biliary drainage (ENBD) and stent placement were performed in 25 patients with biliary strictures (14 at anastomosis site and 11 at non-anastomosis site). ENBD and stent placement were applied in 6 patients with biliary leakage, while endoscopic sphincterotomy, ENBD and stone extraction with baskets were performed in 16 patients with biliary stones. Procedures were repeated when necessary. Results ERCP was successfully performed at a rate of 95.9% (94/98) without any severe complications. Strictures at anastomosis site were resolved in all patients (100%, 14/14), while for strictures at non-anastomosis site, only 27.3% (3/11) were cured. Biliary leakage was resolved in 83.3% (5/6) patients. Complete bile duet clearance was achieved in 81.3% (13/16) of the patients with biliary stones. Conclusion ERCP proves to be safe and effective in the treatment of post liver transplantation biliary complications with low incidence of severe complications.

Objective To evaluate the diagnosis and treatment of early-stage hepatic artery thrombosis(HAT) after adult liver transplantation.Methods387 consecutive adult patients who underwent liver transplantation from June 2007 to October 2010 by the same surgery team in the Transplant Center,First Affiliated Hospital of Sun Yat-sen University were retrospectively studied.Hepatic arterial blood flow was monitored by color Doppler ultrasound (DUS) daily during the first week after transplantation.Ultrasonic contrast or hepatic artery angiography was performed on recipients with suspected HAT.Results10 patients developed HAT on 7(2-18)d after operation.The incidence of HAT was 2.6％ (10/387).Interventional therapy was performed in 2 patients with one patient who received a stent because of hepatic artery stricture.Three patients underwent emergent hepatic artery revascularization combined with intra-arterial urokinase thrombolysis treatment.One developed a rethrombosis and died.The remaining 2 patients received re-transplantation.Three patients died of liver failure and severe infection.The mortality rate was 40％ (10/387).ConclusionsIt is essential to diagnoses HAT by monitoring the artery flow by Doppler ultrasound screening in the early period after operation.Interventional therapy,emergent hepatic artery revascularization and re-transplantation are effective rescue treatments.Prevention of HAT is most important.

BACKGROUND:Nowadays,liver grafts for transplantation are clinically sourced from non-heart-beating donors.Moreover,there is still no uniform determination of safe time limit points for non-heart-beating donor liver in warm ischemia and cold preservation.OBJECTIVE:To evaluate the application safety and curative effects of warm ischemia liver graft affected by varied cold ischemia time (CIT) in liver transplantation.DESIGN,TIME AND SETTING:A randomized controlled observation was performed in the Organ Transplantation Center,First Affiliated Hospital of Sun Yat-sen University between January 2006 and December 2007.PARTICIPANTS:154 cases who underwent non-heart-beating liver transplantation were included in this study.All liver graftsinvolved had a warm ischemia time(WIT) less than 10 minutes.METHODS:All cases were assigned into 3 groups according to CIT:group Ⅰ (CIT＜8 hours,n=58),group Ⅱ (CIT 8-12hours,n=62),and group Ⅲ (CIT 13-16 hours,n=34).The liver grafts were randomly allocated for the patients.Following liver transplantation,the same immunosuppression protocol was employed for each group.MAIN OUTCOME MEASURES:Following surgery,peak level of alanine aminotransferase (ALT),primary graft dysfunction (PGD) after liver transplantation,acute rejection response,biliary complications,vessel complications,perioperative infections,graft and recipient survival rate were compared among 3 groups.RESULTS:Follow-up time was 8-32 months.No PGD was detected in all 154 cases.Group Ⅱ showed postoperative ALT peak levels significantly higher than group Ⅰ (P＜ 0.05).There was no significant difference between groups Ⅰ and Ⅱin terms of acute cellular rejection,perioperative infection,biliary complication,vessel complication,graft survival rate,and recipient survival rate (P＞0.05).Compared with group Ⅰ,the group Ⅲ exhibited significantly increased postoperative ALT peak level,biliary complications,and perioperative infections,and significantly decreased graft and recipient survival rate (P＜0.05).CONCLUSION:Non-heat-beating-liver grafts with less than 10 minutes of warm ischemia can tolerance 12 hours of cold ischemia.More than 12 hours,postoperative liver transplantation complications ascend and,contradictorily,graft and recipient survival rate descend.

Objective To investigate the efficacy and safety of sirolimus in treating calcineurin inhibitor-related renal insufficiency after liver transplantation. Methods Eleven patients with calci-neurin inhibitor-related renal insufficiency after liver transplantation received sirolimus conversion.Simultaneously, the dose of tacrolimus was decreased or tacrolimus was withdrawn. Blood creatinine,sirolimus level, tacrolimus level, liver function, rejection episodes and drug side-effect were moni-tored. Results All the 11 patients survived today with 6 to 20 months of follow-up. All patients showed improvement of renal function after conversion to sirolimus. Blood creatinine level was reduced from (163.8±47.9)μmol/L to(108.1±26.6)μmol/l. (P<0.05). One patient's liver function had an acute rejection episode that was successfully treated with increase of dose of tacrolimus. The side-effects of sirolimus included hyperlipidemia (4 patients), anaemia (1) and mouth ulcers (2).Conclusion Siolimus can be effectively and safely used in liver transplant recipients suffering from ta-crolimus-related renal insufficiency.

Objective To summarize the clinical characteristics and make genetic diagnosis in the patients with hereditary spinocerebellar ataxia type 7 (SCA7).Methods Pedigree analysis and clinical examination were performed in one family with SCA7 by clinical findings,of which retinal morphology and visual electrophysiology were available on part numbers.The polymorphic cytosine adenine guanine (CAG) repeats in the encode region of SCA7 gene were detected by combining polymerase chain reaction with deoxyribonucleic acide (DNA) sequencing on 19 familial numbers and 12 controls.Results 6 patients were identified,who manifesting cerebellar ataxia,decreased visual acuity and colour vision defect,as was pigmentary retinopathy on fundoscopy;The 6 patients had not only extinction of the electroretinogram (ERG) but also remarkably reduced amplitudes of oscillatory potentials and flash-visual evoked potentials. On normal alleles CAG repeat size ranges from 8 to 25 repeats,wherease on mutated alleles of the 6 numbers it ranges from 50 to 97 repeats.The 6 numbers were diagnosised as SCA7 patients.One asymptomatic individual of this family,who displayed a normal allele with 18 CAG repeats and another containing abnormal expantion of 56 repeats,was diagnosised as a asymptomatic carrier whose age maybe still below the age of onset.Conclusion The clinical manifestations of SCA7 are heterogeneous,and the detection of CAG repeats can provide an effective way for the gene diagnosis and the prediction of asymptomatic patients.

Objective To explore the causes and management of intra-abdominal hemorrhage after orthotopic liver transplantation (OLT). Methods Clinical data of 638 OLT patients were analyzed retrospectively from January 2004 to December 2008 in the First Affiliated Hospital of Sun Yat-Sen University. The diagnosis and treatment of postoperative intra-abdominal hemorrhage after OLT were reviewed. Results Among the 638 patients, 53 suffered from posttransplant intra-abdominal hemorrhage,the morbidity was 8. 3% (53/638). Thirty-one cases suffering from bleeding on raw surfaces or around the liver due to impairment of coagulation function were treated by non-surgery methods, 22 cases who suffered from active postoperative intra-abdominal hemorrhage due to surgical factors underwent laparotomy and bleeding control operation after failure of anti-shock treatments such as hemostatic drugs, blood reperfusion.Among the 53 patients who suffered from intra-abdominal hemorrhage, 12 patients died, and the main causes were serious infections and multiple organ dysfunction syndrome. The mortality associated with intraabdominal hemorrhage was 22. 6%. Conclusions Intra-abdominal hemorrhage at different locations were found after OLT, and the fatal rate is quite high. Timely and appropriate treatments especially laparotomy may improve the prosnosis of these patients.

ObjectiveTo investigate a reasonable perioperative nutrition support therapy for combined ‘en bloc’ liver/pancreas transplants (CLPT).MethodThe clinical data of 10 patients,5 with gastrointestinal malignancy and liver metastasis and the other 5 with end-stage liver cirrhosis complicated with insulin-dependent type 2 diabetes mellitus ( T2DM),who had undergone CLPT in our center from May 2004 to September 2010 were retrospectively analyzed.All these patients received preioperative nutrition support,including normal food combined with nutrient solution before surgery,and total parenteral nutrition (TPN) to parenteral nutrition (PN) +enteral nutrition ( EN ) and to EN after surgery.The intestinal absorption,nutritional status,laboratory test results,and complications were recorded.ResultsAll recipients experienced a smooth recovery from the procedure,with normal or almost normal liver,pancreas,and duodenum graft functions.Three patients suffered from intestinal fistula,and all of them received TPN therapy; two patients died of multiple organ failure and one recovered from the complication.The remaining seven patients had smooth transition from TPN to EN.Of the 5 patients with malignancies,three died of multiple organ failure and 2 died of cancer recurrence.Of the 5 patients with liver cirrhosis and T2DM,four patients survived and 1 patient died of graft-verse-host disease.ConclusionRational perioperative nutrition support is important for the successful recovery after CLPT.