Objective: To evaluate the possible protective effect of Citrus aurantium peel extract(CAE) against apoptosis in cholestatic liver fibrosis induced by bile duct ligation in mice. Methods: Male ICR mice were divided to...Objective: To evaluate the possible protective effect of Citrus aurantium peel extract(CAE) against apoptosis in cholestatic liver fibrosis induced by bile duct ligation in mice. Methods: Male ICR mice were divided to 5 groups: 1) Control group(Sham-operated mice), 2) Cholestatic liver injury group induced by bile duct ligation(BDL), 3) BDL mice treated with silymarin(200 mg/kg) for 4 weeks, 4) BDL mice treated with 50 mg/kg CAE for 4 weeks, 5) BDL mice treated with 200 mg/kg CAE for 4 weeks. Mice were sacrificed and liver fibrosis was evaluated by serum and hepatic tissue biochemistry tests and liver histopathological examination. Effects of CAE on inflammation and apoptosis gene regulation were investigated through real-time PCR. CAE effect on lipid metabolism related signaling was determined by western blot analysis. Results: In BDL mice, administration of CAE for 4 weeks markedly attenuated liver fibrosis based on histopathological alteration. Serum and hepatic tissue biochemistry results revealed that CAE(50 and 200 mg/kg) decreased the levels of alanine transaminase, aspartate transaminase, gamma glutamyl transferase, total bilirubin, nitric oxide, and thiobarbituric acid reactive substances. Real-time PCR and western blot analysis showed that CAE regulated inflammation, apoptosis, and lipid metabolism factors increased by BDL. Interleukin family, tumor necrosis factor α, and related apoptosis factors m RNA levels were increased by BDL treatment. However, these increases were suppressed by CAE administration. In addition, CAE effectively increased phosphorylation of AMPactivated protein kinase, nuclear factor E2-related factor 2, and related cytoprotective proteins. Conclusions: CAE can efficiently regulate BDL-induced liver injury with antioxidant, antiinflammatory, and antiapoptotic activities.展开更多
An 80-year-old woman presenting with chest pain was found to have a large,lobulated soft tissue mass in the liver and nearby tissues on abdominal computed tomography(CT).The tumor had invaded the common hepatic artery...An 80-year-old woman presenting with chest pain was found to have a large,lobulated soft tissue mass in the liver and nearby tissues on abdominal computed tomography(CT).The tumor had invaded the common hepatic artery and main portal vein.Jaundice developed 4 wk later,at which point,a pancreas and biliary CT scan revealed a large mass in the right lobe of the liver and a hilar duct obstruction,which was found to be a small cell carcinoma.Despite its rarity,liver and bile duct small cell carcinoma should be considered in the differential diagnosis of atypical chest pain without jaundice.展开更多
Progressive familial intrahepatic cholestasis(PFIC) is a constellation of inherited disorders that result in the impairment of bile flow through the liver that predominantly affects children. The accumulation of bile ...Progressive familial intrahepatic cholestasis(PFIC) is a constellation of inherited disorders that result in the impairment of bile flow through the liver that predominantly affects children. The accumulation of bile results in progressive liver damage, and if left untreated leads to end stage liver disease and death. Patients often present with worsening jaundice and pruritis within the first few years of life. Many of these patients will progress to end stage liver disease and require liver transplantation. The role and timing of liver transplantation still remains debated especially in the management of PFIC1. In those patients who are appropriately selected, liver transplantation offers an excellent survival benefit. Appropriate timing and selection of patients for liver transplantation will be discussed, and the short and long term management of patients post liver transplantation will also be described.展开更多
AIM： To investigate the effects of heme oxygenase-1 （HO-1） against oxidant-induced injury caused by bile duct ligation （BDL）.METHODS： Either cobalt protoporphyrin （CoPP）, a HO-1 inducer, or saline were injecte...AIM： To investigate the effects of heme oxygenase-1 （HO-1） against oxidant-induced injury caused by bile duct ligation （BDL）.METHODS： Either cobalt protoporphyrin （CoPP）, a HO-1 inducer, or saline were injected intraperitoneally in male SD-rats. Three days later, BDL or sham-operations were performed. Rats were sacrificed 3 wk after BDL and livers were harvested for histology. Fibrosis was evaluated by sirius red staining and image analysis. Alpha-smooth muscular actin, which indicates activation of stellate cells, was detected by immunohistochemical staining, and q/tokine and collagen- Iα （Col- I α） mRNA expression was detected using RNase protection assays.RESULTS： Serum alanine transaminase increased 8-fold above normal levels one day after BDL. Surprisingly, enzyme release was not reduced in rats receiving CoPP. Liver fibrosis was evaluated 3 wk after BDL and the sirius red-positive area was found to be increased to about 7.8%. However, in CoPP pretreated rats sirius redpositive areas were increased to about 11.7% after BDL. Collagen-1 α and TGF-β mRNA increased significantly by BDL. Again, this effect was increased by HO-1 overexpression.CONCLUSION： Hepatic fibrosis due to BDL is not reduced by the HO-1 inducer CoPP. In contrast, HO-1 overexpression increases liver injury in rats under conditions of experimental chronic cholestasis.展开更多
AIM： To assess the effects of obstructive cholestasis on a wider range of gene expression using microarray technology. METHODS： Male C57BI_/6J mice underwent common bile duct ligation （BDL） and were matched with p...AIM： To assess the effects of obstructive cholestasis on a wider range of gene expression using microarray technology. METHODS： Male C57BI_/6J mice underwent common bile duct ligation （BDL） and were matched with pairfed sham-operated controls. After 7 d, the animals were sacrificed and total RNA was isolated from livers and kidneys. Equal amounts of RNA from each tissue were pooled for each group and hybridized to Affymetrix GeneChip^MG-U74Av2 containing a total of 12488 probe sets. Data analysis was performed using GeneSpring 6.0 software. Northern analysis and immunofluorescence were used for validation. RESULTS： In sham-operated and BDL mice, 44 and 50% of 12488 genes were expressed in livers, whereas 49 and 51% were expressed in kidneys, respectively. Seven days after BDL, 265 liver and 112 kidney genes with GeneOntology annotation were up-regulated and 113 liver and 36 kidney genes were down-regulated in comparison with sham-operated controls. Many genes were commonly regulated in both tissues and metabolism-related genes represented the largest functional group. CONCLUSION： Following BDL, microarray analysis reveals a broad range of gene alterations in both liver and kidney.展开更多
BACKGROUND Endoscopic management is the first-line therapy for post-liver-transplant anas-tomotic strictures.Although the optimal duration of treatment with plastic stents has been reported to be 8-12 months,data on s...BACKGROUND Endoscopic management is the first-line therapy for post-liver-transplant anas-tomotic strictures.Although the optimal duration of treatment with plastic stents has been reported to be 8-12 months,data on safety and duration for metal stents in this setting is scarce.Due to limited access to endoscopic retrograde cholan-giopancreatography(ERCP)during the coronavirus disease 2019 pandemic in our centre,there was a change in practice towards increased usage and length-of-stay of the Kaffes biliary intraductal self-expanding stent in patients with suitable anatomy.This was mainly due to the theoretical benefit of Kaffes stents allowing for longer indwelling periods compared to the traditional plastic stents.METHODS Adult liver transplant recipients aged 18 years and above who underwent ERCP were retrospectively identified during a 10-year period through a database query.Unplanned admissions post-Kaffes stent insertion were identified manually through electronic and scanned medical records.The main outcome was the incidence of complications when stents were left indwelling for 3 months vs 6 months.Stent efficacy was calculated via rates of stricture recurrence between patients that had stenting courses for≤120 d or>120 d.RESULTS During the study period,a total of 66 ERCPs with Kaffes insertion were performed in 54 patients throughout their stenting course.In 33 ERCPs,the stent was removed or exchanged on a 3-month interval.No pancreatitis,perfor-ations or deaths occurred.Minor post-ERCP complications were similar between the 3-month(abdominal pain and intraductal migration)and 6-month(abdominal pain,septic shower and embedded stent)groups-6.1%vs 9.1%respectively,P=0.40.All strictures resolved at the end of the stenting course,but the stenting course was variable from 3 to 22 months.The recurrence rate for stenting courses lasting for up to 120 d was 71.4%and 21.4%for stenting courses of 121 d or over(P=0.03).There were 28 patients that were treated with a single ERCP with Kaffes,21 with removal after 120 d and 7 within 120 d.There was a significant improvement in stricture recurrence when the Kaffes was removed after 120 d when a single ERCP was used for the entire stenting course(71.0%vs 10.0%,P=0.01).CONCLUSION Utilising a single Kaffes intraductal fully-covered metal stent for at least 4 months is safe and efficacious for the management of post-transplant anastomotic strictures.展开更多
Vanishing bile duct syndrome (VBDS) refers to a group of disorders characterized by prolonged cholestasis as a result of destruction and disappearance ofintrahepatic bile ducts. Multiple etiologies have been indentifi...Vanishing bile duct syndrome (VBDS) refers to a group of disorders characterized by prolonged cholestasis as a result of destruction and disappearance ofintrahepatic bile ducts. Multiple etiologies have been indentifi ed including infections, neoplastic disorders, autoimmune conditions and drugs. The natural history of this condition is variable and may involve resolution of cholestasis or progression with irreversible damage. VBDS is extremely rare in human immunodeficiency virus (HIV)-infected patients and anti-retroviral therapy has never been implicated as a cause. We encountered a young pregnant female with HIV and VBDS secondary to anti-retroviral therapy. Here, we report her clinical course and outcome.展开更多
Vanishing bile duct syndrome (VBDS) has been described in different pathologic conditions including infection, ischemia, adverse drug reactions, autoimmune diseases, allograft rejection, and humoral factors associated...Vanishing bile duct syndrome (VBDS) has been described in different pathologic conditions including infection, ischemia, adverse drug reactions, autoimmune diseases, allograft rejection, and humoral factors associated with malignancy. It is an acquired condition characterized by progressive destruction and loss of the intra-hepatic bile ducts leading to cholestasis. Prognosis is variable and partially dependent upon the etiology of bile duct injury. Irreversible bile duct loss leads to significant ductopenia, biliary cirrhosis, liver failure, and death. If biliary epithelial regeneration occurs, clinical recovery may occur over a period of months to years. VBDS has been described in a number of cases of patients with Hodgkin’s lymphoma (HL) where it is thought to be a paraneoplastic phenomenon. This case describes a 25-year-old man found on liver biopsy to have VBDS. Given poor response to medical treatment, the patient underwent transplant evaluation at that time and was found to have classical stage IIB HL. Early recognition of this underlying cause or association of VBDS, including laboratory screening, and physical exam for lymphadenopathy are paramount to identifying potential underlying VBDS-associated malignancy. Here we review the literature of HL-associated VBDS and report a case of diagnosed HL with biopsy proven VBDS.展开更多
BACKGROUND Patients with obstructive jaundice caused by intrahepatic bile duct stones can be effectively managed by surgery.However,some patients may develop postope-rative complications,liver failure,and other life-t...BACKGROUND Patients with obstructive jaundice caused by intrahepatic bile duct stones can be effectively managed by surgery.However,some patients may develop postope-rative complications,liver failure,and other life-threatening situations.Here,we report a patient with mutations in the uridine 5’-diphospho-glucuronosyltrans-ferase 1A1(UGT1A1)and bile salt export pump(adenosine triphosphate-binding cassette subfamily B member 11,ABCB11)genes who presented multiple intrahe-patic bile duct stones and cholestasis,and the jaundice of the patient increased after partial hepatectomy.CASE SUMMARY A 52-year-old male patient admitted to the hospital on October 23,2021,with a progressive exacerbation of jaundice,was found to have multiple intrahepatic bile duct stones with the diagnoses of obstructive jaundice and acute cholecystitis.Subsequently,the patient underwent left hepatectomy with biliary exploration,stone extraction,T-tube drainage,and cholecystectomy without developing any intraoperative complications.The patient had a dark urine color with worsening jaundice postoperatively and did not respond well to plasma exchange and other symptomatic and supportive treatments.Since the progressive increase in postoperative bilirubin could not be clinically explained with any potential reason,including,if not at all,viral infection,cholangitis,autoimmune liver disease,and other causes,the patient underwent whole-exon screening for any genetic diseases,which surprisingly identified UGT1A1 and ABCB11 gene mutations related to glucuronidation of indirect bilirubin as well as bile acid transport in hepatocytes,respectively.Thus,we hypothesized that postoperative refractory cholestasis might result from UGT1A1 and ABCB11 gene mutations and further recommended liver transplantation to the patient,who eventually declined it and died from liver failure six months later.CONCLUSION Surgery may aggravate cholestasis in patients with multiple intrahepatic bile duct stones and cholestasis associated with UGT1A1 and ABCB11 gene mutations.A liver transplant may be the best option if active medical treatment fails.展开更多
目的对比观察经内镜逆行胰胆管造影(ERCP)置入金属支架与经皮经肝胆管穿刺引流术(PTCD)治疗肝外胆管恶性肿瘤致梗阻性黄疸的临床疗效。方法采用前瞻性研究方法,选取2016年1月至2018年1月首都医科大学附属北京友谊医院收治的90例肝外胆...目的对比观察经内镜逆行胰胆管造影(ERCP)置入金属支架与经皮经肝胆管穿刺引流术(PTCD)治疗肝外胆管恶性肿瘤致梗阻性黄疸的临床疗效。方法采用前瞻性研究方法,选取2016年1月至2018年1月首都医科大学附属北京友谊医院收治的90例肝外胆管恶性肿瘤致梗阻性黄疸患者,采用简单随机分组方法,分为对照组和观察组,每组各45例。对照组患者采用PTCD治疗,观察组患者采用ERCP置入金属支架治疗。比较两组患者的住院天数、术后黄疸缓解率、术后腹痛、术后发热、支架通畅时间、术后并发症发生情况及总生存期等差异。结果观察组患者的住院天数[(12.53±3.98)d]较对照组[(18.77±4.26)d]明显缩短,差异具有统计学意义(P<0.01)。观察组和对照组患者的术后黄疸缓解率(93.33%vs.84.44%)和发热发生率(24.44%vs.33.33%)比较,差异均无统计学意义(P>0.05);观察组患者的术后腹痛发生率(8.89%)较对照组(31.11%)明显下降,差异具有统计学意义(P<0.05);观察组和对照组患者的腹痛消失时间(6.95±1.35 d vs.7.38±1.46 d)和体温恢复正常时间(2.48±0.69 d vs.2.74±0.83 d)比较,差异均无统计学意义(P>0.05)。观察组患者的支架通畅时间[(224.85±48.95)d]和总生存期[(331.14±46.84)d]较对照组[(157.89±42.16)d、(223.16±39.80)d]明显延长,差异具有统计学意义(P<0.01)。观察组患者的术后并发症总发生率(6.67%)较对照组(26.67%)明显降低,差异具有统计学意义(P<0.05)。结论对肝外胆管恶性肿瘤致梗阻性黄疸患者而言,ERCP置入金属支架与PTCD的效果相当,均可有效缓解胆道梗阻性黄疸。但相比于PTCD,ERCP置入金属支架治疗后胆道通畅时间延长,住院时间缩短,并发症发生率低,可促进患者肝功能恢复,延长生存时间,因此,ERCP置入金属支架治疗可作为临床治疗此类患者的一种安全、有效的方法。展开更多
基金supported by‘Cooperative Research Program for Agriculture Science&Technology Development’(No.PJ01132001)funded by Rural Development Administration,Republic of Korea
文摘Objective: To evaluate the possible protective effect of Citrus aurantium peel extract(CAE) against apoptosis in cholestatic liver fibrosis induced by bile duct ligation in mice. Methods: Male ICR mice were divided to 5 groups: 1) Control group(Sham-operated mice), 2) Cholestatic liver injury group induced by bile duct ligation(BDL), 3) BDL mice treated with silymarin(200 mg/kg) for 4 weeks, 4) BDL mice treated with 50 mg/kg CAE for 4 weeks, 5) BDL mice treated with 200 mg/kg CAE for 4 weeks. Mice were sacrificed and liver fibrosis was evaluated by serum and hepatic tissue biochemistry tests and liver histopathological examination. Effects of CAE on inflammation and apoptosis gene regulation were investigated through real-time PCR. CAE effect on lipid metabolism related signaling was determined by western blot analysis. Results: In BDL mice, administration of CAE for 4 weeks markedly attenuated liver fibrosis based on histopathological alteration. Serum and hepatic tissue biochemistry results revealed that CAE(50 and 200 mg/kg) decreased the levels of alanine transaminase, aspartate transaminase, gamma glutamyl transferase, total bilirubin, nitric oxide, and thiobarbituric acid reactive substances. Real-time PCR and western blot analysis showed that CAE regulated inflammation, apoptosis, and lipid metabolism factors increased by BDL. Interleukin family, tumor necrosis factor α, and related apoptosis factors m RNA levels were increased by BDL treatment. However, these increases were suppressed by CAE administration. In addition, CAE effectively increased phosphorylation of AMPactivated protein kinase, nuclear factor E2-related factor 2, and related cytoprotective proteins. Conclusions: CAE can efficiently regulate BDL-induced liver injury with antioxidant, antiinflammatory, and antiapoptotic activities.
文摘An 80-year-old woman presenting with chest pain was found to have a large,lobulated soft tissue mass in the liver and nearby tissues on abdominal computed tomography(CT).The tumor had invaded the common hepatic artery and main portal vein.Jaundice developed 4 wk later,at which point,a pancreas and biliary CT scan revealed a large mass in the right lobe of the liver and a hilar duct obstruction,which was found to be a small cell carcinoma.Despite its rarity,liver and bile duct small cell carcinoma should be considered in the differential diagnosis of atypical chest pain without jaundice.
文摘Progressive familial intrahepatic cholestasis(PFIC) is a constellation of inherited disorders that result in the impairment of bile flow through the liver that predominantly affects children. The accumulation of bile results in progressive liver damage, and if left untreated leads to end stage liver disease and death. Patients often present with worsening jaundice and pruritis within the first few years of life. Many of these patients will progress to end stage liver disease and require liver transplantation. The role and timing of liver transplantation still remains debated especially in the management of PFIC1. In those patients who are appropriately selected, liver transplantation offers an excellent survival benefit. Appropriate timing and selection of patients for liver transplantation will be discussed, and the short and long term management of patients post liver transplantation will also be described.
基金grants from the National Institute of Health and by a grant from the Deutsche Forschungsgemeinschaft, No. FR 1644/4-1
文摘AIM： To investigate the effects of heme oxygenase-1 （HO-1） against oxidant-induced injury caused by bile duct ligation （BDL）.METHODS： Either cobalt protoporphyrin （CoPP）, a HO-1 inducer, or saline were injected intraperitoneally in male SD-rats. Three days later, BDL or sham-operations were performed. Rats were sacrificed 3 wk after BDL and livers were harvested for histology. Fibrosis was evaluated by sirius red staining and image analysis. Alpha-smooth muscular actin, which indicates activation of stellate cells, was detected by immunohistochemical staining, and q/tokine and collagen- Iα （Col- I α） mRNA expression was detected using RNase protection assays.RESULTS： Serum alanine transaminase increased 8-fold above normal levels one day after BDL. Surprisingly, enzyme release was not reduced in rats receiving CoPP. Liver fibrosis was evaluated 3 wk after BDL and the sirius red-positive area was found to be increased to about 7.8%. However, in CoPP pretreated rats sirius redpositive areas were increased to about 11.7% after BDL. Collagen-1 α and TGF-β mRNA increased significantly by BDL. Again, this effect was increased by HO-1 overexpression.CONCLUSION： Hepatic fibrosis due to BDL is not reduced by the HO-1 inducer CoPP. In contrast, HO-1 overexpression increases liver injury in rats under conditions of experimental chronic cholestasis.
基金Supported by the USPHS grants DK 25636 (J. L. B.)the Yale Liver Center Cellular and Molecular Physiology and Morphology Cores (P30-34989)the Deutsche Forschungsgemeinschaft Grant DE 872/1-1 (G. U. D.)
文摘AIM： To assess the effects of obstructive cholestasis on a wider range of gene expression using microarray technology. METHODS： Male C57BI_/6J mice underwent common bile duct ligation （BDL） and were matched with pairfed sham-operated controls. After 7 d, the animals were sacrificed and total RNA was isolated from livers and kidneys. Equal amounts of RNA from each tissue were pooled for each group and hybridized to Affymetrix GeneChip^MG-U74Av2 containing a total of 12488 probe sets. Data analysis was performed using GeneSpring 6.0 software. Northern analysis and immunofluorescence were used for validation. RESULTS： In sham-operated and BDL mice, 44 and 50% of 12488 genes were expressed in livers, whereas 49 and 51% were expressed in kidneys, respectively. Seven days after BDL, 265 liver and 112 kidney genes with GeneOntology annotation were up-regulated and 113 liver and 36 kidney genes were down-regulated in comparison with sham-operated controls. Many genes were commonly regulated in both tissues and metabolism-related genes represented the largest functional group. CONCLUSION： Following BDL, microarray analysis reveals a broad range of gene alterations in both liver and kidney.
文摘BACKGROUND Endoscopic management is the first-line therapy for post-liver-transplant anas-tomotic strictures.Although the optimal duration of treatment with plastic stents has been reported to be 8-12 months,data on safety and duration for metal stents in this setting is scarce.Due to limited access to endoscopic retrograde cholan-giopancreatography(ERCP)during the coronavirus disease 2019 pandemic in our centre,there was a change in practice towards increased usage and length-of-stay of the Kaffes biliary intraductal self-expanding stent in patients with suitable anatomy.This was mainly due to the theoretical benefit of Kaffes stents allowing for longer indwelling periods compared to the traditional plastic stents.METHODS Adult liver transplant recipients aged 18 years and above who underwent ERCP were retrospectively identified during a 10-year period through a database query.Unplanned admissions post-Kaffes stent insertion were identified manually through electronic and scanned medical records.The main outcome was the incidence of complications when stents were left indwelling for 3 months vs 6 months.Stent efficacy was calculated via rates of stricture recurrence between patients that had stenting courses for≤120 d or>120 d.RESULTS During the study period,a total of 66 ERCPs with Kaffes insertion were performed in 54 patients throughout their stenting course.In 33 ERCPs,the stent was removed or exchanged on a 3-month interval.No pancreatitis,perfor-ations or deaths occurred.Minor post-ERCP complications were similar between the 3-month(abdominal pain and intraductal migration)and 6-month(abdominal pain,septic shower and embedded stent)groups-6.1%vs 9.1%respectively,P=0.40.All strictures resolved at the end of the stenting course,but the stenting course was variable from 3 to 22 months.The recurrence rate for stenting courses lasting for up to 120 d was 71.4%and 21.4%for stenting courses of 121 d or over(P=0.03).There were 28 patients that were treated with a single ERCP with Kaffes,21 with removal after 120 d and 7 within 120 d.There was a significant improvement in stricture recurrence when the Kaffes was removed after 120 d when a single ERCP was used for the entire stenting course(71.0%vs 10.0%,P=0.01).CONCLUSION Utilising a single Kaffes intraductal fully-covered metal stent for at least 4 months is safe and efficacious for the management of post-transplant anastomotic strictures.
文摘Vanishing bile duct syndrome (VBDS) refers to a group of disorders characterized by prolonged cholestasis as a result of destruction and disappearance ofintrahepatic bile ducts. Multiple etiologies have been indentifi ed including infections, neoplastic disorders, autoimmune conditions and drugs. The natural history of this condition is variable and may involve resolution of cholestasis or progression with irreversible damage. VBDS is extremely rare in human immunodeficiency virus (HIV)-infected patients and anti-retroviral therapy has never been implicated as a cause. We encountered a young pregnant female with HIV and VBDS secondary to anti-retroviral therapy. Here, we report her clinical course and outcome.
基金Supported by National Institute of Health,NIH 5 T32 DK 7356-37(BN)
文摘Vanishing bile duct syndrome (VBDS) has been described in different pathologic conditions including infection, ischemia, adverse drug reactions, autoimmune diseases, allograft rejection, and humoral factors associated with malignancy. It is an acquired condition characterized by progressive destruction and loss of the intra-hepatic bile ducts leading to cholestasis. Prognosis is variable and partially dependent upon the etiology of bile duct injury. Irreversible bile duct loss leads to significant ductopenia, biliary cirrhosis, liver failure, and death. If biliary epithelial regeneration occurs, clinical recovery may occur over a period of months to years. VBDS has been described in a number of cases of patients with Hodgkin’s lymphoma (HL) where it is thought to be a paraneoplastic phenomenon. This case describes a 25-year-old man found on liver biopsy to have VBDS. Given poor response to medical treatment, the patient underwent transplant evaluation at that time and was found to have classical stage IIB HL. Early recognition of this underlying cause or association of VBDS, including laboratory screening, and physical exam for lymphadenopathy are paramount to identifying potential underlying VBDS-associated malignancy. Here we review the literature of HL-associated VBDS and report a case of diagnosed HL with biopsy proven VBDS.
基金Supported by The Science and Technology Planning Projects of Guizhou Province and Zunyi City,No.QKHJCZK[2022]YB642,No.ZSKH·HZ(2022)344,No.gzwjkj2021-071,ZMC·YZ[2018]38,No.ZSKH·HZ[2021]58,and No.ZSKH·HZ[2021]60The General Project of Hubei Province and Jingmen City,No.2021YFYB074.
文摘BACKGROUND Patients with obstructive jaundice caused by intrahepatic bile duct stones can be effectively managed by surgery.However,some patients may develop postope-rative complications,liver failure,and other life-threatening situations.Here,we report a patient with mutations in the uridine 5’-diphospho-glucuronosyltrans-ferase 1A1(UGT1A1)and bile salt export pump(adenosine triphosphate-binding cassette subfamily B member 11,ABCB11)genes who presented multiple intrahe-patic bile duct stones and cholestasis,and the jaundice of the patient increased after partial hepatectomy.CASE SUMMARY A 52-year-old male patient admitted to the hospital on October 23,2021,with a progressive exacerbation of jaundice,was found to have multiple intrahepatic bile duct stones with the diagnoses of obstructive jaundice and acute cholecystitis.Subsequently,the patient underwent left hepatectomy with biliary exploration,stone extraction,T-tube drainage,and cholecystectomy without developing any intraoperative complications.The patient had a dark urine color with worsening jaundice postoperatively and did not respond well to plasma exchange and other symptomatic and supportive treatments.Since the progressive increase in postoperative bilirubin could not be clinically explained with any potential reason,including,if not at all,viral infection,cholangitis,autoimmune liver disease,and other causes,the patient underwent whole-exon screening for any genetic diseases,which surprisingly identified UGT1A1 and ABCB11 gene mutations related to glucuronidation of indirect bilirubin as well as bile acid transport in hepatocytes,respectively.Thus,we hypothesized that postoperative refractory cholestasis might result from UGT1A1 and ABCB11 gene mutations and further recommended liver transplantation to the patient,who eventually declined it and died from liver failure six months later.CONCLUSION Surgery may aggravate cholestasis in patients with multiple intrahepatic bile duct stones and cholestasis associated with UGT1A1 and ABCB11 gene mutations.A liver transplant may be the best option if active medical treatment fails.
文摘目的对比观察经内镜逆行胰胆管造影(ERCP)置入金属支架与经皮经肝胆管穿刺引流术(PTCD)治疗肝外胆管恶性肿瘤致梗阻性黄疸的临床疗效。方法采用前瞻性研究方法,选取2016年1月至2018年1月首都医科大学附属北京友谊医院收治的90例肝外胆管恶性肿瘤致梗阻性黄疸患者,采用简单随机分组方法,分为对照组和观察组,每组各45例。对照组患者采用PTCD治疗,观察组患者采用ERCP置入金属支架治疗。比较两组患者的住院天数、术后黄疸缓解率、术后腹痛、术后发热、支架通畅时间、术后并发症发生情况及总生存期等差异。结果观察组患者的住院天数[(12.53±3.98)d]较对照组[(18.77±4.26)d]明显缩短,差异具有统计学意义(P<0.01)。观察组和对照组患者的术后黄疸缓解率(93.33%vs.84.44%)和发热发生率(24.44%vs.33.33%)比较,差异均无统计学意义(P>0.05);观察组患者的术后腹痛发生率(8.89%)较对照组(31.11%)明显下降,差异具有统计学意义(P<0.05);观察组和对照组患者的腹痛消失时间(6.95±1.35 d vs.7.38±1.46 d)和体温恢复正常时间(2.48±0.69 d vs.2.74±0.83 d)比较,差异均无统计学意义(P>0.05)。观察组患者的支架通畅时间[(224.85±48.95)d]和总生存期[(331.14±46.84)d]较对照组[(157.89±42.16)d、(223.16±39.80)d]明显延长,差异具有统计学意义(P<0.01)。观察组患者的术后并发症总发生率(6.67%)较对照组(26.67%)明显降低,差异具有统计学意义(P<0.05)。结论对肝外胆管恶性肿瘤致梗阻性黄疸患者而言,ERCP置入金属支架与PTCD的效果相当,均可有效缓解胆道梗阻性黄疸。但相比于PTCD,ERCP置入金属支架治疗后胆道通畅时间延长,住院时间缩短,并发症发生率低,可促进患者肝功能恢复,延长生存时间,因此,ERCP置入金属支架治疗可作为临床治疗此类患者的一种安全、有效的方法。
