检索结果-南通市图书馆

Long-term clinical outcomes and health-related quality of life in patients with isolated methylmalonic acidemia after liver transplantation:experience from the largest cohort study in China

引用

World Journal of Pediatrics 2024年第8期20卷 809-821页

作者： Yi-Zhou Jiang Guang-Peng Zhou Lin Wei Wei Qu zhi-Gui Zeng Ying Liu Yu-Le Tan jun Wang zhi-jun zhu Li-Ying Sun Department of Critical Liver Diseases Liver Research CenterBeijing Friendship HospitalCapital Medical UniversityNo.101 Lu Yuan Dong RoadTong-Zhou DistrictBeijing110112China Liver Transplantation Center National Clinical Research Center for Digestive DiseasesBeijing Friendship HospitalCapital Medical UniversityBeijingChina Clinical Center for Pediatric Liver Transplantation No.101 Lu Yuan Dong RoadTong-Zhou DistrictCapital Medical UniversityBeijing110112China

Background Liver transplantation(LT)has been proposed as a viable treatment option for selected methylmalonic acidemia(MMA)***,there are still controversies regarding the therapeutic value of LT for *** systematic assessment of health-related quality of life(HRQoL)-targeted MMA children before and after LT is also *** study aimed to comprehensively assess the long-term impact of LT on MMA,including multiorgan sequelae and HRQoL in children and *** We retrospectively evaluated 15 isolated MMA patients undergoing LT at our institution between june 2013 and March ***-and post-transplant data were compared,including metabolic profiles,neurologic consequences,growth parameters,and *** further assess the characteristics of the HRQoL outcomes in MMA,we compared the results with those of children with biliary atresia(BA).Results All patients had early onset MMA,and underwent LT at a mean age of 4.3 *** 1.3–8.2 years of follow-up,the patient and graft survival rates were 100%.Metabolic stability was achieved in all patients with liberalized dietary protein *** was a significant overall improvement in height Z scores(P=0.0047),and some preexisting neurological complications remained stable or even improved after *** the Pediatric Quality of Life Inventory(PedsQL™)generic core scales,the mean total,physical health,and psychosocial health scores improved significantly posttransplant(P<0.05).In the family impact module,higher mean scores were noted for all subscales post-LT,especially family function and daily activities(P<0.01).However,the total scores on the generic core scales and transplant module were significantly lower(Cohen’s d=0.57–1.17)when compared with BA *** particular,social and school functioning(Cohen’s d=0.86–1.76),treatment anxiety,and communication(Cohen’s d=0.99–1.81)were far behind,with a large effect *** This large single-center study of the mainland of China sh

关键词： Health-related quality of life.Inborn error of metabolism Isolated methylmalonic acidemia Liver transplantation:Outcomes

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Editorial on“Is there any correlation between liver graft regeneration and recipient’s pretransplant skeletal muscle mass?”

引用

Hepatobiliary Surgery and Nutrition 2021年第6期10卷 881-883页

作者： zhi-jun zhu Liver Transplantation Center Clinical Center for Pediatric Liver TransplantationNational Clinical Research Center for Digestive DiseasesBeijing Friendship HospitalCapital Medical UniversityBeijingChina

Liver regeneration is necessary for recovery following liver *** liver’s regenerative capacity can,to a certain extent,alter the critical point between failure and eventual recovery of the transplanted liver(1).Poor liver function and failure of liver regeneration are most likely to be the direct result of liver injury ***,in living donor liver transplantation(LDLT),the impact of these factors on liver regeneration is diminished by standardized surgical procedures and detailed donor evaluation and *** the context of severe liver disease,the recipient’s state is more important for liver regeneration(2).

关键词： donor Liver graft

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

The role of innate immune cells in post-hepatectomy liver regeneration

引用

Hepatobiliary Surgery and Nutrition 2023年第2期12卷 267-268页

作者： Yong-Fa Huang zhi-jun zhu Liver Transplantation Center National Clinical Research Center for Digestive DiseasesBeijing Friendship HospitalCapital Medical UniversityBeijingChina Clinical Center for Pediatric Liver Transplantation Capital Medical UniversityBeijingChina

The fame of regeneration has been attributed to the liver ever since the era of Greek mythology,in which Prometheus’liver could grow back overnight against the daily predation of Zeus’*** is the very basis of liver surgery including liver transplantation,and post-hepatectomy liver regeneration remains the center of interest for both clinicians and *** tools for structural and functional assessment of liver regeneration have been constantly developed and validated,laying the groundwork for clinical management and scientific analysis(1).

关键词： Post-hepatectomy liver regeneration innate immune cell cytokine paracrine signaling

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Laparoscopic left lateral sectionectomy in pediatric living donor liver transplantation by single-port approach:A case report

引用

World Journal of Clinical Cases 2020年第23期8卷 6103-6109页

作者： Hong-Yu Li Lin Wei zhi-Gui Zeng Wei Qu zhi-jun zhu Liver Transplantation Center Beijing Friendship HospitalCapital Medical UniversityBeijing 100050China

BACKGROUND Single-port laparoscopy has been used in a variety of abdominal *** report the first case of single-port laparoscopic left lateral sectionectomy in pediatric laparoscopic living donor liver *** SUMMARY A 28-year-old man volunteered for living liver donation to his daughter who was diagnosed with liver cirrhosis and portal hypertension after the Kasai procedure for biliary *** body mass index was 20.5 kg/*** dynamic computed tomography showed:(1)Left lateral graft volume of 232.76 cm3 with a graft-torecipient weight ratio of 2.59%;and(2)Right hepatic artery derived from the superior mesenteric artery.A single-port access system was placed through a transumbilical incision,including four trocars:two 12-mm ports for a camera and endoscopic stapler and two 5-mm working *** parenchyma was dissected by a Harmonic and Cavitron Ultrasonic Surgical Aspirator,while bipolar was used for *** bile duct was transected above the bifurcation by indocyanine green fluorescence *** specimen was retrieved from the umbilical *** total operation time was 4 h without blood *** final graft weight was 233.6 g with graft-torecipient weight ratio of 2.60%.The donor was discharged uneventfully on postoperative day *** Single-port laparoscopic left lateral sectionectomy is feasible in pediatric laparoscopic living donor liver transplantation in an experienced transplant center.

关键词： Pediatric living donor liver transplantation Laparoscopy Single-port approach Intraoperative indocyanine green fluorescence cholangiography Treatment Case report

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Glypican-3 expression and its relationship with recurrence of HCC after liver transplantation

引用

World Journal of Gastroenterology 2012年第19期18卷 2408-2414页

作者： Yu-Liang Wang zhi-jun zhu Da-Hong Teng zhi Yao Wei Gao Zhong-Yang Shen Department of Transplantation Surgery Tianjin First Central Hospital Key Laboratory for Critical CareMedicine of the Ministry of Health Tianjin 300192 China Department of Immunology TianjinMedical University Tianiin 300070 China

AIM:To investigate the diagnostic value of glypican-3(GPC3) and its relationship with hepatocellular carcinoma(HCC) recurrence after liver ***:HCC tissue samples(n = 31) obtained from patients who had undergone liver transplantation were ***3 mRNA and protein expression were analyzed by TaqMan real-time reverse transcription-polymerase chain reaction and *** between the GPC3 expression and clinicopathological features was *** potential prognostic value of GPC3 was investigated by comparing recurrence-free survival between HCC patients with and without GPC3 ***:Using a cutoff value of 3.5 × 10-2,20 of 31 cancerous tissues had expression values of > 3.5 × 10-2,whereas 3 of 31 adjacent non-neoplastic parenchyma and 0 of 20 control liver tissues had expression values of > 3.5 × 10-2(P 3.5 × 10-2) and those without vascular invasion(P < 0.05 for both).CONCLUSION:GPC3 expression may serve as a valuable diagnostic marker for ***3 mRNA overexpression may be an adverse indicator for HCC patients after liver transplantation.

关键词： Liver transplantation Hepatocellular carci-noma Glypican-3 mRNA Recurrence

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Novel approach for the diagnosis of occult cytomegalovirus cholangitis after pediatric liver transplantation:A case report

引用

World Journal of Clinical Cases 2020年第12期8卷 2597-2602页

作者： Ying Liu Li-Ying Sun zhi-jun zhu Wei Qu Liver Transplantation Center National Clinical Research Center for Digestive DiseasesBeijing Friendship HospitalCapital Medical UniversityBeijing 100050China Intensive Care Unit Beijing Friendship HospitalCapital Medical UniversityBeijing 100050China

BACKGROUND Cytomegalovirus(CMV)infection is a common infection in liver transplant recipients,which is related to chronic rejection and biliary *** is often diagnosed based on serum CMV-DNA or CMV *** our knowledge,this is the first report of the successful treatment of occult CMV cholangitis in a pediatric liver transplantation(LT)*** SUMMARY A 7-mo-old baby girl received LT due to biliary atresia and cholestasis *** 1 mo following LT,the patient suffered from aggravated jaundice with no apparent *** imaging results showed intrahepatic and extrahepatic bile duct dilatation,the patient was diagnosed with biliary complications and percutaneous cholangiography and biliary drainage was ***,there was little biliary drainage and her liver function ***-DNA was isolated from the bile with the surprising outcome that 3×106 copies/mL were present,whereas the CMV-DNA in serum was *** antiviral therapy with ganciclovir,she gradually recovered and bilirubin decreased to normal *** the 4-year follow-up period,her liver function remained *** Bile CMV sampling can be used for the diagnosis of occult CMV infection,especially in patients with negative serum CMV-DNA and CMV *** for CMV in the biliary tract may serve as a novel approach for the diagnosis of cholestasis *** diagnosis and treatment will decrease the risk of graft loss.

关键词： Occult cytomegalovirus cholangitis Biliary complications Pediatric Liver transplantation Bile cytomegalovirus-DNA detection Case report

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Is sinusoidal obstructive syndrome a recurrent disease after liver transplantation? A case report

引用

World Journal of Clinical Cases 2021年第2期9卷 489-495页

作者： Ying Liu Li-Ying Sun zhi-jun zhu Lin Wei Wei Qu zhi-Gui Zeng Liver Transplantation Center Beijing Friendship HospitalCapital Medical UniversityBeijing 100050China Intensive Care Unit Beijing Friendship HospitalCapital Medical UniversityBeijing 100050China

BACKGROUND Sinusoidal obstructive syndrome(SOS)is a disease that damages hepatic sinusoidal endothelial cells,resulting in progressive occlusion and fibrosis of the lobular central vein and the occurrence of intrahepatic sinusoidal portal ***,SOS after liver transplantation(LT)is uncommon and potentially ***,we report a rare case of second-time recurrence of SOS after liver retransplantation(rLT).CASE SUMMARY A 22-year-old woman received a living donor LT due to *** years later,she developed abdominal distention and ascites with no apparent *** was diagnosed with recurrence of SOS and underwent *** 2 mo post rLT,the patient suffered from aggravated jaundice and ascites *** was diagnosed with second-time recurrence of SOS post-rLT according to computed tomography and liver *** treatment with warfarin anticoagulation and immunosuppressant conversion,she gradually recovered with improvement of liver function and liver *** the 17-mo follow-up period,she was in good condition with normal liver function and no *** SOS can be a recurrent disease after LT,and autoimmune antibody and genetic sequencing should be screened before *** susceptible patients,anticoagulant drugs should be used for an extended period,and tacrolimus or other pathogenic agents should be *** diagnosis and treatment can improve the prognosis of patients and avoid graft failure or death.

关键词： Sinusoidal obstructive syndrome Liver transplantation Recurrence Sinusoidal dilatation and congestion Patchy enhancement Case report

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Status epilepticus as an initial manifestation of hepatic encephalopathy:A case report

引用

World Journal of Clinical Cases 2020年第24期8卷 6480-6486页

作者： Bin Cui Lin Wei Wei Qu zhi-Gui Zeng Ying Liu zhi-jun zhu Li-Ying Sun Liver Transplantation Center National Clinical Research Center for Digestive DiseasesBeijing Friendship HospitalCapital Medical UniversityBeijing 100050China Liver Transplantation Center National Clinical Research Center for Digestive DiseasesIntensive Care UnitBeijing Friendship HospitalCapital Medical UniversityBeijing 100050China

BACKGROUND Status epilepticus in patients with hepatic encephalopathy (HE) is a rare butserious condition that is refractory to antiepileptic drugs, and current treatmentplans are vague. Diagnosis may be difficult without a clear history of *** transplantation (LT) is effective to alleviate symptoms, however, there arefew reports about LT in the treatment of status epilepticus with HE. To ourknowledge, this is the first report of status epilepticus present as initialmanifestation of *** SUMMARY A 59-year-old woman with a 20-year history of heavy drinking was hospitalizedfor generalized tonic-clonic seizures. She reported no history of episodes of HE,stroke, spontaneous bacterial peritonitis, ascites or gastrointestinal *** examination revealed a comatose patient, without *** examination suggested liver cirrhosis. Plasma ammonia levels uponadmission were five times normal. Brain computed tomography (CT) was normal,while abdominal CT and ultrasound revealed mild ascites, liver cirrhosis andsplenomegaly. Electroencephalography (EEG)showed diffuse slow waves rhythm,consistent with HE, and sharp waves during ictal EEG corresponding to clinicalsemiology of focal tonic seizures. The symptoms were reversed by continuousantiepileptic treatment and lactulose. She was given oral levetiracetam, and focalaware seizures occasionally affected her 10 mo after *** Status epilepticus could be an initial manifestation of HE. Antiepileptic drugs combined with lactulose are essential for treatment of status epilepticus with HE,and LT is effective to prevent the relapse.

关键词： Status epilepticus Hepatic encephalopathy Decompensated alcoholic liver cirrhosis Antiepileptic drugs Liver transplantation Case report

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Compromised therapeutic value of pediatric liver transplantation in ethylmalonic encephalopathy: A case report

引用

World Journal of Gastroenterology 2020年第40期26卷 6295-6303页

作者： Guang-Peng Zhou Wei Qu zhi-jun zhu Li-Ying Sun Lin Wei zhi-Gui Zeng Ying Liu Liver Transplantation Center National Clinical Research Center for Digestive DiseasesBeijing Friendship HospitalCapital Medical UniversityBeijing 100050China Liver Transplantation Center Intensive Care UnitBeijing Friendship HospitalCapital Medical UniversityBeijing 100050China

BACKGROUND Ethylmalonic encephalopathy(EE) is a rare autosomal recessive metabolic disorder caused by impaired mitochondrial sulfur dioxygenase. Due to poor therapeutic effect of current conventional treatments, progressive psychomotor regression and neurological impairment usually contribute to early death in the first decade of life. Liver transplantation(LT) is emerging as a novel therapeutic option for EE;however, worldwide experience is still *** SUMMARY An 18-mo-old patient with the diagnosis of EE received a living donor liver transplant in our institution, which, to our knowledge, is the first case in AsianPacific countries. During 20 mo of follow-up, the longitudinal metabolic evaluations revealed a wild fluctuation in urinary EMA levels, still far beyond the normal range. Urinary 2-methylsuccinic acid levels gradually restored to normal, whereas the concentrations of urinary isobutyrylglycine and plasma C4-and C5-acylcarnitines fluctuated markedly and still remained above the reference limits. Only mild amelioration of petechiae and ecchymosis was observed, and no dramatic reversion of chronic mucoid diarrhea and orthostatic acrocyanosis occurred. Although brain magnetic resonance imaging suggested a certain improvement in basal ganglia lesions, the patient still presented developmental delay and neurologic *** LT may bring about a partial but not complete cure to EE. Given its definite role in defending against the devastating natural progression of EE, LT should still be considered for patients with EE in the absence of other superior therapeutic options. Early establishment of diagnosis and initiation of conventional treatment pre-transplant, timely LT, and continuous administration of metabolismcorrecting medications post-transplant may be helpful in minimizing the neurologic impairment and maximizing the therapeutic value of LT in EE.

关键词： Ethylmalonic encephalopathy Liver transplantation Mitochondrial disorder ETHE1 Pediatric Case report

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Liver transplantation for severe portopulmonary hypertension: A case report and literature review

引用

World Journal of Clinical Cases 2019年第21期7卷 3569-3574页

作者： Xiao-Jie Chen zhi-jun zhu Li-Ying Sun Lin Wei zhi-Gui Zeng Ying Liu Wei Qu Liang Zhang Liver Transplantation Center National Clinical Research Center for Digestive DiseasesBeijing Friendship HospitalCapital Medical UniversityBeijing 100050China Clinical Center for Pediatric Liver Transplantation Capital Medical UniversityBeijing 100050China Beijing Key Laboratory of Tolerance Induction and Organ Protection in Transplantation Beijing 100050China Department of Intensive Care Unit Beijing Friendship HospitalCapital Medical UniversityBeijing 100050China

BACKGROUND Portopulmonary hypertension(PoPH) is not uncommon in patients waiting for liver transplantation(LT).Severe PoPH has a very high perioperative mortality rate and is still considered a contraindication for *** patients with liver disease require but cannot receive LT due to severe PoPH and eventually *** report a patient with severe PoPH who underwent successful LT and had near normal pulmonary pressure without drug *** SUMMARY A 39-year-old woman was hospitalized with the chief complaint of jaundice and exertional dyspnea and *** disease and liver cirrhosis was diagnosed 6 years *** liver disease met the criteria for ***, right heart catheterization showed that her mean pulmonary artery pressure was increased at 50 mmHg, pulmonary vascular resistance was 460 dyn?s/cm^5 and pulmonary artery wedge pressure was 20 mmHg, which may have been the reasons for her chief *** patient was diagnosed with severe PoPH and was not listed for LT *** 5 mo of pharmacotherapy, her severe PoPH was moderate, and she underwent successful *** artery pressure steadily decreased according to post-operative echocardiographic monitoring and drugs have been discontinued for a *** The safety of LT can be greatly improved by reducing mean pulmonary artery pressure to a low level, and LT may cure PoPH.

关键词： Portopulmonary hypertension Pharmacotherapy Liver transplantation Pulmonary hypertension crisis Treatment Case report

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

欢迎您,

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

请选择保存的检索档案：

请选择收藏分类：

通借通还

欢迎您,

建议与咨询 留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

请选择保存的检索档案： 新增检索档案 确定 取消

请选择收藏分类： 新增自定义分类 确定 取消

通借通还

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

请选择保存的检索档案：

请选择收藏分类：