BACKGROUND Percutaneous transhepatic cholangiodrainage(PTCD)and endoscopic retrograde cholangiopancreatography/endoscopic nasobiliary drainage are the most common clinical procedures for jaundice control in patients w...BACKGROUND Percutaneous transhepatic cholangiodrainage(PTCD)and endoscopic retrograde cholangiopancreatography/endoscopic nasobiliary drainage are the most common clinical procedures for jaundice control in patients with unresectable malignant obstructive jaundice,yet the safety and effect of endobiliary radiofrequency ablation(EB-RFA)combined PTCD is rarely reported,in this article,we report our experience of EB-RFA combined PTCD in such patients.AIM To retrospectively study the efficacy and safety of EB-RFA combined PTCD in patients with unresectable malignant obstructive jaundice.METHODS Patients with unresectable malignant obstructive jaundice treated with EB-RFA under PTCD were selected,the bile ducts of the right posterior lobe was selected as the target bile ducts in all cases.The general conditions of all patients,preoperative tumour markers,total bilirubin(TBIL),direct bilirubin(DBIL),albumin(ALB),alkaline phosphatase(ALP),and glutamyl transferase(GGT)before and on the 7th day after the procedure,as well as perioperative complications,stent patency time and patient survival were recorded.RESULTS All patients successfully completed the operation,TBIL and DBIL decreased significantly in all patients at the 7th postoperative day(P=0.009 and 0.006,respectively);the values of ALB,ALP and GGT also decreased compared with the preoperative period,but the difference was not statistically significant.Perioperative biliary bleeding occurred in 2 patients,which was improved after transfusion of blood and other conservative treatments,pancreatitis appeared in 1 patient after the operation,no serious complication and death happened after operation.Except for 3 patients with loss of visits,the stent patency rate of the remaining 14 patients was 100%71%and 29%at the 1^(st),3^(rd),and 6^(th)postoperative months respectively,with a median survival of 4 months.CONCLUSION EB-RFA under PTCD in patients with unresectable malignant obstructive jaundice has a satisfactory therapeutic effect and high safety,which is worthy of further clinical practice.展开更多
Introduction: Neonatal jaundice (NNJ) is a common disorder in neonates that can impact negatively on the brain and cause death. The peculiarities in aetiology and solutions for different settings are a knowledge gap. ...Introduction: Neonatal jaundice (NNJ) is a common disorder in neonates that can impact negatively on the brain and cause death. The peculiarities in aetiology and solutions for different settings are a knowledge gap. This informed the desire to determine local aetiology and solutions for neonatal jaundice in a missionary hospital in Abeokuta, Southwestern Nigeria. Methods: Consecutive consenting babies diagnosed with significant neonatal jaundice were studied between July 2016 and June 2019. Institutional ethical consent was obtained. A proforma was used to obtain socio-demographic data and other relevant information such as age, sex, birthweight, gestational age and history regarding the jaundice. All the information obtained was inputted into a computer and analysed with SPSS version 16. Results: A total of 179 babies with neonatal jaundice comprising of 120 (67.0%) boys and 59 (33.0%) girls with ages ranging between 1 and 12 days (mean 2.7 ± 2.9) were studied. Prematurity, ABO incompatibility, neonatal sepsis and glucose-6-phosphate enzyme deficiency accounted for over 80% of the causes of significant NNJ. Sixty (33.5%) of the 179 babies studied developed acute bilirubin encephalopathy and 11 (6.1%) mortalities were recorded. Higher proportions of babies that were out-born with spontaneous vaginal delivery modes had acute bilirubin encephalopathy (p < 0.05). Mothers with formal education had better outcome compared to mothers without, in terms of mortalities (p < 0.05). Conclusion: Neonatal jaundice is still a significant cause of morbidity and mortality in the neonatal age group. Maternal education is key to good outcome in neonatal jaundice.展开更多
Background: To examine the differences in prevalence of respiratory distress syndrome, early-onset sepsis and jaundice, between late preterm infants versus term infants in Ecuadorian newborns. Methods: Study design: E...Background: To examine the differences in prevalence of respiratory distress syndrome, early-onset sepsis and jaundice, between late preterm infants versus term infants in Ecuadorian newborns. Methods: Study design: Epidemiological, observational, and cross-sectional, with two cohorts of patients. Settings: IESS Quito Sur Hospital at Quito, Ecuador, from February to April of 2020. Participants: This study included 204 newborns, 102 preterm infants, 102 term infants. Results: There are significant differences between late preterm infants and term infants, with a p-value of 0.000 in the prevalence of early sepsis, 70.59% vs. 35.29%. In respiratory distress syndrome between late and term premature infants, significant differences were observed with a p-value of 0.000, the proportion being 55.58% vs. 24.51% respectively. The prevalence of jaundice is higher in term infants with a p value of 0.002, 72.55%, versus 51.96% in late preterm infants, and the mean value of bilirubins in mg/dL was higher in term infants 14.32 versus 12.33 in late preterm infants;this difference is statistically significant with a p value of 0.004. Admission to the NICU is more frequent in late preterm infants with a p-value of 0.000, being 42.16% for late preterm infants vs. 7.84% in term infants;the mean of the hospital days with p-value 0.005, was higher in late preterm infants 4.97 days vs. 3.55 days for term newborns. Conclusion: Due to the conditions of their immaturity, late preterm infants are 2.86 times more likely to present early sepsis than full-term newborns. It is shown that late preterm infants are 2.69 times more likely to have respiratory distress syndrome compared to term infants, therefore, late preterm infants have a longer hospital stay of 4.97 days versus 3.55 days in term infants. Jaundice and mean bilirubin levels are higher in term infants due to blood group incompatibility and insufficient breastfeeding.展开更多
BACKGROUND Light chain(AL)amyloidosis is a plasma cell dyscrasia characterized by the pathologic production and extracellular tissue deposition of fibrillar proteins derived from immunoglobulin AL fragments secreted b...BACKGROUND Light chain(AL)amyloidosis is a plasma cell dyscrasia characterized by the pathologic production and extracellular tissue deposition of fibrillar proteins derived from immunoglobulin AL fragments secreted by a clone of plasma cells,which leads to progressive dysfunction of the affected organs.The two most commonly affected organs are the heart and kidneys,and liver is rarely the dominant affected organ with only 3.9%of cases,making them prone to misdia-gnosis and missed diagnosis.CASE SUMMARY A 65-year-old woman was admitted with a 3-mo history of progressive jaundice and marked hepatomegaly.Initially,based on enhanced computed tomography scan and angiography,Budd-Chiari syndrome was considered and balloon dilatation of significant hepatic vein stenoses was performed.However,addi-tional diagnostic procedures,including liver biopsy and bone marrow-exami-nation,revealed immunoglobulin kapa AL amyloidosis with extensive liver involvement and hepatic vascular compression.The disease course was progre-ssive and fatal,and the patient eventually died 5 mo after initial presentation of symptoms.CONCLUSION AL amyloidosis with isolated liver involvement is very rare,and can be easily misdiagnosed as a vascular disease.展开更多
This editorial discusses an article by Peng et al.This study reviewed the efficacy and safety of a new approach for treating obstructive jaundice.Although the pathophysiology of obstructive jaundice has not yet been f...This editorial discusses an article by Peng et al.This study reviewed the efficacy and safety of a new approach for treating obstructive jaundice.Although the pathophysiology of obstructive jaundice has not yet been fully elucidated,pro-gress has been made in its management.There are two aspects of obstructive jaundice:Cholestatic status and absence of bile in the intestinal lumen.Internal biliary drainage resolved both the conditions.Clinically,endoscopic retrograde biliary drainage(ERBD)has replaced percutaneous transhepatic biliary drainage,and ERBD is transitioning to endoscopic ultrasound guided biliary drainage.This editorial briefly explains the mechanism and treatment of obstructive jaundice and the prospects of this new internal biliary drainage technique.展开更多
BACKGROUND Malignant obstructive jaundice(MOJ)is a condition characterized by varying degrees of bile duct stenosis and obstruction,accompanied by the progressive development of malignant tumors,leading to high morbid...BACKGROUND Malignant obstructive jaundice(MOJ)is a condition characterized by varying degrees of bile duct stenosis and obstruction,accompanied by the progressive development of malignant tumors,leading to high morbidity and mortality rates.Currently,the two most commonly employed methods for its management are percutaneous transhepatic bile duct drainage(PTBD)and endoscopic ultrasound-guided biliary drainage(EUS-BD).While both methods have demonstrated favorable outcomes,additional research needs to be performed to determine their relative efficacy.To compare the therapeutic effectiveness of EUS-BD and PTBD in treating MOJ.METHODS This retrospective analysis,conducted between September 2015 and April 2023 at The Third Affiliated Hospital of Soochow University(The First People’s Hospital of Changzhou),involved 68 patients with MOJ.The patients were divided into two groups on the basis of surgical procedure received:EUS-BD subgroup(n=33)and PTBD subgroup(n=35).Variables such as general data,preoperative and postoperative indices,blood routine,liver function indices,myocardial function indices,operative success rate,clinical effectiveness,and complication rate were analyzed and compared between the subgroups.RESULTS In the EUS-BD subgroup,hospital stay duration,bile drainage volume,effective catheter time,and clinical effect-iveness rate were superior to those in the PTBD subgroup,although the differences were not statistically significant(P>0.05).The puncture time for the EUS-BD subgroup was shorter than that for the PTBD subgroup(P<0.05).Postoperative blood routine,liver function index,and myocardial function index in the EUS-BD subgroup were significantly lower than those in the PTBD subgroup(P<0.05).Additionally,the complication rate in the EUS-BD subgroup was lower than in the PTBD subgroup(P<0.05).CONCLUSION EUS-BD may reduce the number of punctures,improve liver and myocardial functions,alleviate traumatic stress,and decrease complication rates in MOJ treatment.展开更多
Obstructive jaundice(OJ)is a common problem in daily clinical practice.However,completely understanding the pathophysiological changes in OJ remains a challenge for planning current and future management.The effects o...Obstructive jaundice(OJ)is a common problem in daily clinical practice.However,completely understanding the pathophysiological changes in OJ remains a challenge for planning current and future management.The effects of OJ are widespread,affecting the biliary tree,hepatic cells,liver function,and causing systemic complications.The lack of bile in the intestine,destruction of the intestinal mucosal barrier,and increased absorption of endotoxins can lead to endotoxemia,production of proinflammatory cytokines,and induce systemic inflammatory response syndrome,ultimately leading to multiple organ dysfunction syndrome.Proper management of OJ includes adequate water supply and electrolyte replacement,nutritional support,preventive antibiotics,pain relief,and itching relief.The surgical treatment of OJ depends on the cause,location,and severity of the obstruction.Biliary drainage,surgery,and endoscopic intervention are potential treatment options depending on the patient's condition.In addition to modern medical treatments,Traditional Chinese medicine may offer therapeutic benefits for OJ.A comprehensive search was conducted on PubMed for relevant articles published up to August 1970.This review discusses in detail the pathophysiological changes associated with OJ and presents effective strategies for managing the condition.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
BACKGROUND The aim of the present study was to enhance understanding of the diagnosis and treatment of atypical hereditary spherocytosis(HS),and to broaden the diagnostic thoughts of physicians for patients with jaund...BACKGROUND The aim of the present study was to enhance understanding of the diagnosis and treatment of atypical hereditary spherocytosis(HS),and to broaden the diagnostic thoughts of physicians for patients with jaundice.CASE SUMMARY A 28-year-old male presented with jaundice,bile duct stone,and splenomegaly,but without anemia.Other causes of jaundice were excluded,and gene se-quencing revealed a novel heterozygous variant of c.1801C>T(p.Q601X)in exon 14 of the SPTB(NM_01355436)gene on chromosome 14(chr14:65260580)in the patient’s blood;the biological parents and child of the patient did not have similar variants.A splenectomy was performed on the patient and his bilirubin levels returned to normal after surgery.Thus,a novel gene variant causing HS was identified.This variant may result in the truncation ofβ-hemoglobin in the erythrocyte membrane,leading to loss of normal function,jaundice,and hemolytic anemia.The clinical manifestations of the patient were hyperjaundice and an absence of typical hemolysis during the course of the disease,which caused challenges for diagnosis by the clinicians.CONCLUSION Following a definitive diagnosis,genetic testing and response to treatment identified a gene variant site for a novel hemolytic anemia.展开更多
In this case, a young female presented with non-specific features such as fever, sore throat, headache and fatigue. She went on to develop epigastric pain, darkening of urine and jaundice, with no resolution of prior ...In this case, a young female presented with non-specific features such as fever, sore throat, headache and fatigue. She went on to develop epigastric pain, darkening of urine and jaundice, with no resolution of prior symptoms. Physical and Laboratory tests confirmed the primary diagnosis of infectious mononucleosis, however, prior history of treatment with multiple drugs led to a diagnosis of DILI as a complication. Appropriate treatment with I.V. antibiotics, hepatoprotective agents, steroids as well as discontinuation of all potential hepatotoxic agents showed significant improvement in patients’ symptoms and overall condition.展开更多
BACKGROUND We aimed to investigate the effectiveness of endoscopic retrograde cholangiopan-creatography(ERCP)for treating obstructive jaundice(OJ)post hepatoblastoma(HB)surgery(post-HB OJ)by analyzing the data of a ca...BACKGROUND We aimed to investigate the effectiveness of endoscopic retrograde cholangiopan-creatography(ERCP)for treating obstructive jaundice(OJ)post hepatoblastoma(HB)surgery(post-HB OJ)by analyzing the data of a case and performing a literature review.CASE SUMMARY Clinical data of one patient with post-HB OJ treated by ERCP were retrospectively analyzed.Furthermore,clinical characteristics and insights into the diagnosis and treatment of post-HB OJ in children were summarized via searching various databases and platforms,such as China National Knowledge Infrastructure,Wanfang database,CQVIP database,PubMed,Ringer Link,and Google Scholar.The patient reported herein underwent five chemotherapy sessions after the diagnosis of HB and right hemihepatectomy after tumor size reduction;these were followed by two postoperative chemotherapy sessions.Three months postoperatively,the patient developed icteric sclera,strong tea-colored urine,and clay-like stools,and showed signs of skin itchiness;blood analysis showed significantly an increased conjugated bilirubin(CB)level(200.3μmol/L).Following the poor efficacy of anti-jaundice and hepatoprotective treatments,the patient underwent ERCP.Intraoperative imaging showed a dilated bile duct in the porta hepatis with significant distal stenosis.A 5 Fr nasopancreatic tube was placed in the dilated area through the stricture for external drainage,and the patient was extubated on postoperative day 6.Postoperatively,the patient’s stool turned yellow,and the CB level decreased to 78.2μmol/L.Fifteen days later,ERCP was repeated due to unrelieved jaundice symptoms,wherein a 7 Fr naso-biliary drainage tube was successfully placed.Three months post-ERCP,the jaundice symptoms resolved,and the CB level was reduced to 33.2μmol/L.A follow-up examination one year postoperatively revealed no jaundice symptoms and normal CB level.CONCLUSION Post-HB OJ is rare.Compared to biliary tract reconstruction,ERCP is less invasive and has a better therapeutic effect.展开更多
BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare but life-threatening condition.It is an immune-mediated disease that has a wide range of causes,elicits a hyperinflammatory response,and results in multiple ...BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare but life-threatening condition.It is an immune-mediated disease that has a wide range of causes,elicits a hyperinflammatory response,and results in multiple organ damage.Clinical presentations vary,and in some cases,jaundice occurs as the first symptom.CASE SUMMARY We report the case of a 71-year-old female patient who presented with jaundice.She was admitted to our hospital because of the occurrence of“jaundice for half a month”,and upon examination,obstructive jaundice with choledocholithiasis and gallstones was suggested.Cholecystectomy and choledocholithotomy were performed.However,the jaundice did not improve after surgery.We found splenomegaly,cytopenia,hypertriglyceridemia,hypofibrinogenemia,and elevated ferritin.Bone marrow biopsy revealed hemophagocytosis.Later,cardiac arrest occurred when she returned 3 wk after the surgery.We considered that HLH was triggered by septic shock.The patient’s condition deteriorated rapidly,with multiple organ dysfunction and severe gastrointestinal bleeding.Corticosteroid therapy and symptomatic treatment failed to save her life.CONCLUSION Jaundice rarely presents as the first symptom in HLH patients.The HLH in this case was triggered by septic shock with jaundice as the first symptom.Clinicians should try hard to reduce missed diagnoses and misdiagnoses.展开更多
针对注水井分层注水量诊断技术难题,提出基于分布式光纤温度传感(Distributed Temperature Sensing,DTS)的注水井吸水剖面解释方法。建立考虑微量热效应的注水井温度剖面预测模型,模拟分析注水量、注水时间、储层导热系数等7个因素对温...针对注水井分层注水量诊断技术难题,提出基于分布式光纤温度传感(Distributed Temperature Sensing,DTS)的注水井吸水剖面解释方法。建立考虑微量热效应的注水井温度剖面预测模型,模拟分析注水量、注水时间、储层导热系数等7个因素对温度剖面的影响规律。通过正交试验模拟分析,确定不同因素对注水井温度剖面的影响程度从强到弱分别为注入水温度、注水时间、注水量、井筒半径、储层导热系数、井筒倾斜角度、注水层渗透率,明确影响注水井温度剖面的主控因素为注入水温度、注水时间和注入量。采用模拟退火(Simulated Annealing,SA)算法建立注水井DTS数据反演模型,对一口注水井现场实测DTS数据进行反演,获得较为准确的吸水剖面,单层最大吸水量误差百分比14.25%,平均误差11.09%,验证该反演方法的可靠性。通过DTS数据反演可以实现注水井吸水剖面定量解释,为注水效果评价提供直接依据。展开更多
文摘BACKGROUND Percutaneous transhepatic cholangiodrainage(PTCD)and endoscopic retrograde cholangiopancreatography/endoscopic nasobiliary drainage are the most common clinical procedures for jaundice control in patients with unresectable malignant obstructive jaundice,yet the safety and effect of endobiliary radiofrequency ablation(EB-RFA)combined PTCD is rarely reported,in this article,we report our experience of EB-RFA combined PTCD in such patients.AIM To retrospectively study the efficacy and safety of EB-RFA combined PTCD in patients with unresectable malignant obstructive jaundice.METHODS Patients with unresectable malignant obstructive jaundice treated with EB-RFA under PTCD were selected,the bile ducts of the right posterior lobe was selected as the target bile ducts in all cases.The general conditions of all patients,preoperative tumour markers,total bilirubin(TBIL),direct bilirubin(DBIL),albumin(ALB),alkaline phosphatase(ALP),and glutamyl transferase(GGT)before and on the 7th day after the procedure,as well as perioperative complications,stent patency time and patient survival were recorded.RESULTS All patients successfully completed the operation,TBIL and DBIL decreased significantly in all patients at the 7th postoperative day(P=0.009 and 0.006,respectively);the values of ALB,ALP and GGT also decreased compared with the preoperative period,but the difference was not statistically significant.Perioperative biliary bleeding occurred in 2 patients,which was improved after transfusion of blood and other conservative treatments,pancreatitis appeared in 1 patient after the operation,no serious complication and death happened after operation.Except for 3 patients with loss of visits,the stent patency rate of the remaining 14 patients was 100%71%and 29%at the 1^(st),3^(rd),and 6^(th)postoperative months respectively,with a median survival of 4 months.CONCLUSION EB-RFA under PTCD in patients with unresectable malignant obstructive jaundice has a satisfactory therapeutic effect and high safety,which is worthy of further clinical practice.
文摘Introduction: Neonatal jaundice (NNJ) is a common disorder in neonates that can impact negatively on the brain and cause death. The peculiarities in aetiology and solutions for different settings are a knowledge gap. This informed the desire to determine local aetiology and solutions for neonatal jaundice in a missionary hospital in Abeokuta, Southwestern Nigeria. Methods: Consecutive consenting babies diagnosed with significant neonatal jaundice were studied between July 2016 and June 2019. Institutional ethical consent was obtained. A proforma was used to obtain socio-demographic data and other relevant information such as age, sex, birthweight, gestational age and history regarding the jaundice. All the information obtained was inputted into a computer and analysed with SPSS version 16. Results: A total of 179 babies with neonatal jaundice comprising of 120 (67.0%) boys and 59 (33.0%) girls with ages ranging between 1 and 12 days (mean 2.7 ± 2.9) were studied. Prematurity, ABO incompatibility, neonatal sepsis and glucose-6-phosphate enzyme deficiency accounted for over 80% of the causes of significant NNJ. Sixty (33.5%) of the 179 babies studied developed acute bilirubin encephalopathy and 11 (6.1%) mortalities were recorded. Higher proportions of babies that were out-born with spontaneous vaginal delivery modes had acute bilirubin encephalopathy (p < 0.05). Mothers with formal education had better outcome compared to mothers without, in terms of mortalities (p < 0.05). Conclusion: Neonatal jaundice is still a significant cause of morbidity and mortality in the neonatal age group. Maternal education is key to good outcome in neonatal jaundice.
文摘Background: To examine the differences in prevalence of respiratory distress syndrome, early-onset sepsis and jaundice, between late preterm infants versus term infants in Ecuadorian newborns. Methods: Study design: Epidemiological, observational, and cross-sectional, with two cohorts of patients. Settings: IESS Quito Sur Hospital at Quito, Ecuador, from February to April of 2020. Participants: This study included 204 newborns, 102 preterm infants, 102 term infants. Results: There are significant differences between late preterm infants and term infants, with a p-value of 0.000 in the prevalence of early sepsis, 70.59% vs. 35.29%. In respiratory distress syndrome between late and term premature infants, significant differences were observed with a p-value of 0.000, the proportion being 55.58% vs. 24.51% respectively. The prevalence of jaundice is higher in term infants with a p value of 0.002, 72.55%, versus 51.96% in late preterm infants, and the mean value of bilirubins in mg/dL was higher in term infants 14.32 versus 12.33 in late preterm infants;this difference is statistically significant with a p value of 0.004. Admission to the NICU is more frequent in late preterm infants with a p-value of 0.000, being 42.16% for late preterm infants vs. 7.84% in term infants;the mean of the hospital days with p-value 0.005, was higher in late preterm infants 4.97 days vs. 3.55 days for term newborns. Conclusion: Due to the conditions of their immaturity, late preterm infants are 2.86 times more likely to present early sepsis than full-term newborns. It is shown that late preterm infants are 2.69 times more likely to have respiratory distress syndrome compared to term infants, therefore, late preterm infants have a longer hospital stay of 4.97 days versus 3.55 days in term infants. Jaundice and mean bilirubin levels are higher in term infants due to blood group incompatibility and insufficient breastfeeding.
基金Tianjin Key Medical Discipline(Specialty)Construction Project,No.TJYXZDXK-034A.
文摘BACKGROUND Light chain(AL)amyloidosis is a plasma cell dyscrasia characterized by the pathologic production and extracellular tissue deposition of fibrillar proteins derived from immunoglobulin AL fragments secreted by a clone of plasma cells,which leads to progressive dysfunction of the affected organs.The two most commonly affected organs are the heart and kidneys,and liver is rarely the dominant affected organ with only 3.9%of cases,making them prone to misdia-gnosis and missed diagnosis.CASE SUMMARY A 65-year-old woman was admitted with a 3-mo history of progressive jaundice and marked hepatomegaly.Initially,based on enhanced computed tomography scan and angiography,Budd-Chiari syndrome was considered and balloon dilatation of significant hepatic vein stenoses was performed.However,addi-tional diagnostic procedures,including liver biopsy and bone marrow-exami-nation,revealed immunoglobulin kapa AL amyloidosis with extensive liver involvement and hepatic vascular compression.The disease course was progre-ssive and fatal,and the patient eventually died 5 mo after initial presentation of symptoms.CONCLUSION AL amyloidosis with isolated liver involvement is very rare,and can be easily misdiagnosed as a vascular disease.
文摘This editorial discusses an article by Peng et al.This study reviewed the efficacy and safety of a new approach for treating obstructive jaundice.Although the pathophysiology of obstructive jaundice has not yet been fully elucidated,pro-gress has been made in its management.There are two aspects of obstructive jaundice:Cholestatic status and absence of bile in the intestinal lumen.Internal biliary drainage resolved both the conditions.Clinically,endoscopic retrograde biliary drainage(ERBD)has replaced percutaneous transhepatic biliary drainage,and ERBD is transitioning to endoscopic ultrasound guided biliary drainage.This editorial briefly explains the mechanism and treatment of obstructive jaundice and the prospects of this new internal biliary drainage technique.
文摘BACKGROUND Malignant obstructive jaundice(MOJ)is a condition characterized by varying degrees of bile duct stenosis and obstruction,accompanied by the progressive development of malignant tumors,leading to high morbidity and mortality rates.Currently,the two most commonly employed methods for its management are percutaneous transhepatic bile duct drainage(PTBD)and endoscopic ultrasound-guided biliary drainage(EUS-BD).While both methods have demonstrated favorable outcomes,additional research needs to be performed to determine their relative efficacy.To compare the therapeutic effectiveness of EUS-BD and PTBD in treating MOJ.METHODS This retrospective analysis,conducted between September 2015 and April 2023 at The Third Affiliated Hospital of Soochow University(The First People’s Hospital of Changzhou),involved 68 patients with MOJ.The patients were divided into two groups on the basis of surgical procedure received:EUS-BD subgroup(n=33)and PTBD subgroup(n=35).Variables such as general data,preoperative and postoperative indices,blood routine,liver function indices,myocardial function indices,operative success rate,clinical effectiveness,and complication rate were analyzed and compared between the subgroups.RESULTS In the EUS-BD subgroup,hospital stay duration,bile drainage volume,effective catheter time,and clinical effect-iveness rate were superior to those in the PTBD subgroup,although the differences were not statistically significant(P>0.05).The puncture time for the EUS-BD subgroup was shorter than that for the PTBD subgroup(P<0.05).Postoperative blood routine,liver function index,and myocardial function index in the EUS-BD subgroup were significantly lower than those in the PTBD subgroup(P<0.05).Additionally,the complication rate in the EUS-BD subgroup was lower than in the PTBD subgroup(P<0.05).CONCLUSION EUS-BD may reduce the number of punctures,improve liver and myocardial functions,alleviate traumatic stress,and decrease complication rates in MOJ treatment.
基金Tianjin Municipal Education Commission Scientific Research Program,China,No.2022KJ271。
文摘Obstructive jaundice(OJ)is a common problem in daily clinical practice.However,completely understanding the pathophysiological changes in OJ remains a challenge for planning current and future management.The effects of OJ are widespread,affecting the biliary tree,hepatic cells,liver function,and causing systemic complications.The lack of bile in the intestine,destruction of the intestinal mucosal barrier,and increased absorption of endotoxins can lead to endotoxemia,production of proinflammatory cytokines,and induce systemic inflammatory response syndrome,ultimately leading to multiple organ dysfunction syndrome.Proper management of OJ includes adequate water supply and electrolyte replacement,nutritional support,preventive antibiotics,pain relief,and itching relief.The surgical treatment of OJ depends on the cause,location,and severity of the obstruction.Biliary drainage,surgery,and endoscopic intervention are potential treatment options depending on the patient's condition.In addition to modern medical treatments,Traditional Chinese medicine may offer therapeutic benefits for OJ.A comprehensive search was conducted on PubMed for relevant articles published up to August 1970.This review discusses in detail the pathophysiological changes associated with OJ and presents effective strategies for managing the condition.
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.
文摘针对Oligo(d T)亲和层析介质的吸附性能,以poly(A)为模型分子,考察了4种Oligo(d T)亲和层析介质的静态吸附平衡、吸附动力学和动态结合载量(DBC),探讨了载量影响相关机制。结果表明,4种介质的合适吸附条件均为0.6 mol·L-1Na Cl、p H=6~7;Monomix d T20静态吸附容量最大,且poly(A)能扩散至介质微球深层孔内,而Poros Oligo(d T)25、Praesto Jetted (d T)25和Nano Gel d T20等3种介质中poly(A)均主要为表层吸附、静态吸附容量稍低;对于DBC,Nano Gel d T20和Monomix d T20的10%穿透的DBC较高,而Poros Oligo (d T)25和Praesto Jetted (d T)25相对略低。经分析,影响载量的主要因素包含基质种类、微球孔径、配基密度、间隔臂和配基长度等。对于基质种类,聚苯乙烯基质可能孔道结构较为特别。对于微球孔径,应针对不同大小的m RNA分子定制不同孔径的微球,以平衡传质阻力与可及吸附表面积之间的矛盾,从而增大DBC。
基金Supported by Natural Science Foundation of Gansu Province,No. 21JR1RA070Construction of Clinical Medical Research Center,No. 21JR7RA392
文摘BACKGROUND The aim of the present study was to enhance understanding of the diagnosis and treatment of atypical hereditary spherocytosis(HS),and to broaden the diagnostic thoughts of physicians for patients with jaundice.CASE SUMMARY A 28-year-old male presented with jaundice,bile duct stone,and splenomegaly,but without anemia.Other causes of jaundice were excluded,and gene se-quencing revealed a novel heterozygous variant of c.1801C>T(p.Q601X)in exon 14 of the SPTB(NM_01355436)gene on chromosome 14(chr14:65260580)in the patient’s blood;the biological parents and child of the patient did not have similar variants.A splenectomy was performed on the patient and his bilirubin levels returned to normal after surgery.Thus,a novel gene variant causing HS was identified.This variant may result in the truncation ofβ-hemoglobin in the erythrocyte membrane,leading to loss of normal function,jaundice,and hemolytic anemia.The clinical manifestations of the patient were hyperjaundice and an absence of typical hemolysis during the course of the disease,which caused challenges for diagnosis by the clinicians.CONCLUSION Following a definitive diagnosis,genetic testing and response to treatment identified a gene variant site for a novel hemolytic anemia.
文摘In this case, a young female presented with non-specific features such as fever, sore throat, headache and fatigue. She went on to develop epigastric pain, darkening of urine and jaundice, with no resolution of prior symptoms. Physical and Laboratory tests confirmed the primary diagnosis of infectious mononucleosis, however, prior history of treatment with multiple drugs led to a diagnosis of DILI as a complication. Appropriate treatment with I.V. antibiotics, hepatoprotective agents, steroids as well as discontinuation of all potential hepatotoxic agents showed significant improvement in patients’ symptoms and overall condition.
文摘BACKGROUND We aimed to investigate the effectiveness of endoscopic retrograde cholangiopan-creatography(ERCP)for treating obstructive jaundice(OJ)post hepatoblastoma(HB)surgery(post-HB OJ)by analyzing the data of a case and performing a literature review.CASE SUMMARY Clinical data of one patient with post-HB OJ treated by ERCP were retrospectively analyzed.Furthermore,clinical characteristics and insights into the diagnosis and treatment of post-HB OJ in children were summarized via searching various databases and platforms,such as China National Knowledge Infrastructure,Wanfang database,CQVIP database,PubMed,Ringer Link,and Google Scholar.The patient reported herein underwent five chemotherapy sessions after the diagnosis of HB and right hemihepatectomy after tumor size reduction;these were followed by two postoperative chemotherapy sessions.Three months postoperatively,the patient developed icteric sclera,strong tea-colored urine,and clay-like stools,and showed signs of skin itchiness;blood analysis showed significantly an increased conjugated bilirubin(CB)level(200.3μmol/L).Following the poor efficacy of anti-jaundice and hepatoprotective treatments,the patient underwent ERCP.Intraoperative imaging showed a dilated bile duct in the porta hepatis with significant distal stenosis.A 5 Fr nasopancreatic tube was placed in the dilated area through the stricture for external drainage,and the patient was extubated on postoperative day 6.Postoperatively,the patient’s stool turned yellow,and the CB level decreased to 78.2μmol/L.Fifteen days later,ERCP was repeated due to unrelieved jaundice symptoms,wherein a 7 Fr naso-biliary drainage tube was successfully placed.Three months post-ERCP,the jaundice symptoms resolved,and the CB level was reduced to 33.2μmol/L.A follow-up examination one year postoperatively revealed no jaundice symptoms and normal CB level.CONCLUSION Post-HB OJ is rare.Compared to biliary tract reconstruction,ERCP is less invasive and has a better therapeutic effect.
文摘BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare but life-threatening condition.It is an immune-mediated disease that has a wide range of causes,elicits a hyperinflammatory response,and results in multiple organ damage.Clinical presentations vary,and in some cases,jaundice occurs as the first symptom.CASE SUMMARY We report the case of a 71-year-old female patient who presented with jaundice.She was admitted to our hospital because of the occurrence of“jaundice for half a month”,and upon examination,obstructive jaundice with choledocholithiasis and gallstones was suggested.Cholecystectomy and choledocholithotomy were performed.However,the jaundice did not improve after surgery.We found splenomegaly,cytopenia,hypertriglyceridemia,hypofibrinogenemia,and elevated ferritin.Bone marrow biopsy revealed hemophagocytosis.Later,cardiac arrest occurred when she returned 3 wk after the surgery.We considered that HLH was triggered by septic shock.The patient’s condition deteriorated rapidly,with multiple organ dysfunction and severe gastrointestinal bleeding.Corticosteroid therapy and symptomatic treatment failed to save her life.CONCLUSION Jaundice rarely presents as the first symptom in HLH patients.The HLH in this case was triggered by septic shock with jaundice as the first symptom.Clinicians should try hard to reduce missed diagnoses and misdiagnoses.
文摘针对注水井分层注水量诊断技术难题,提出基于分布式光纤温度传感(Distributed Temperature Sensing,DTS)的注水井吸水剖面解释方法。建立考虑微量热效应的注水井温度剖面预测模型,模拟分析注水量、注水时间、储层导热系数等7个因素对温度剖面的影响规律。通过正交试验模拟分析,确定不同因素对注水井温度剖面的影响程度从强到弱分别为注入水温度、注水时间、注水量、井筒半径、储层导热系数、井筒倾斜角度、注水层渗透率,明确影响注水井温度剖面的主控因素为注入水温度、注水时间和注入量。采用模拟退火(Simulated Annealing,SA)算法建立注水井DTS数据反演模型,对一口注水井现场实测DTS数据进行反演,获得较为准确的吸水剖面,单层最大吸水量误差百分比14.25%,平均误差11.09%,验证该反演方法的可靠性。通过DTS数据反演可以实现注水井吸水剖面定量解释,为注水效果评价提供直接依据。