Aim: To explore the interaction between bladder compliance (BC) and bladder outflow obstruction (BOO) in men with benign prostatic hyperplasia (BPH) using cross-sectional and longitudinal studies. Methods: A t...Aim: To explore the interaction between bladder compliance (BC) and bladder outflow obstruction (BOO) in men with benign prostatic hyperplasia (BPH) using cross-sectional and longitudinal studies. Methods: A total of 181 men with BPH were recruited, and 100 of them were followed for one year. Cystometry was performed in a standing or a sitting position with 30 mL/min infusion. BC was manually corrected and defined. Obstruction coefficient (OCO), linear passive urethral resistance relation and international continence society (ICS) nomogram were used to diagnose BOO. The obstructed parameters were compared between the reduced BC group and the non-reduced group. BC was compared between the first investigation at the beginning of study and the second investigation at the end of study during the one-year follow-up period. Results: The group with reduced BC had increased OCO and linear passive urethral resistance relation. BC was significantly lower in the obstructed group (55.7 mL/cm water) than that in unobstructed and equivocal one (74.9 mL/cm water, P 〈 0.01). BC gradually reduced with the increased obstructed grade. There was a significantly weak negative correlation between BC and OCO (r = - 0.132, P 〈 0.01). Over the one-year follow-up period in the longitudinal study, BC for all men changed from 54.4 to 48.8 mL/cm water (P 〉 0.05), and BC for the group with BOO fell from 58.4 ± 70.1 to 46.5 ± 38.7 mL/cm water (P 〉 0.05). Conclusion: In men with BPH, a significant systematic decrease occurred in BC in the obstructed group and a significant systematic increase with urethral resistance occurred in the low BC group. A longitudinal study of the tendency of BC reduction in a group with BOO is necessary in the future.展开更多
AIM To assess the efficacy and safety of balloon dilatation for the treatment of hepatic venous outflow obstruction(HVOO) following pediatric liver transplantation.METHODS A total of 246 pediatric patients underwent l...AIM To assess the efficacy and safety of balloon dilatation for the treatment of hepatic venous outflow obstruction(HVOO) following pediatric liver transplantation.METHODS A total of 246 pediatric patients underwent liver transplantation at our hospital between June 2013 and September 2016. Among these patients, five were ultimately diagnosed with HVOO. Seven procedures(two patients underwent two balloon dilatation procedures) were included in this analysis. The demographic data,types of donor and liver transplant, interventional examination and therapeutic outcomes of these five children were analyzed. The median interval time between pediatric liver transplantation and balloon dilatation procedures was 9.8 mo(range: 1-32).RESULTS Five children with HVOO were successfully treated by balloon angioplasty without stent placement, with seven procedures performed for six stenotic lesions. All children underwent successful percutaneous intervention. Among these five patients, four were treated by single balloon angioplasty, and these patients did not develop recurrent stenosis. In seven episodes of balloon angioplasty across the stenosis, the pressure gradient was 12.0 ± 8.8 mm Hg before balloon dilatation and 1.1 ± 1.5 mm Hg after the procedures, which revealed a statistically significant reduction(P < 0.05). The overall technical success rate among these seven procedures was 100%(7/7), and clinical success was achieved in all five patients(100%). The patients were followed for 4-33 mo(median: 15 mo). No significant procedural complications or procedurerelated deaths occurred.CONCLUSION Balloon dilatation is an effective and safe therapeutic option for HVOO in children undergoing pediatric liver transplantation. Venous angioplasty is also recommended in cases with recurrent HVOO.展开更多
Hepatic venous outflow obstruction after piggyback liver transplantation is a very rare complication. An unusual mechanism aggravating it is reported. A 33-year-old man with end-stage hepatitis B liver cirrhosis under...Hepatic venous outflow obstruction after piggyback liver transplantation is a very rare complication. An unusual mechanism aggravating it is reported. A 33-year-old man with end-stage hepatitis B liver cirrhosis underwent a piggyback orthotopic liver transplantation using a full-size cadaveric graft. Two months after transplantation, he developed gross ascites refractory to maximal diuretic therapy. Doppler ultrasound showed patent portal and hepatic veins. Serial computed tomography scans revealed a hypoperfused right posterior segment of the liver which subsequently underwent atrophy. Hepatic venography demonstrated a high-grade stenosis with an element of torsion of venous drainage at the anastomosis. The stenosis was successfully treated with repeated percutaneous balloon angioplasty. The patient remained asymptomatic six months afterwards with complete resolution of ascites and peripheral edema. We postulate that liver allograft segmental hypoperfusion and atrophy may aggravate or result in a hepatic venous outflow problem by the mechanism of torsion effect. Percutaneous balloon angioplasty is a safe and effective treatment modality for anastomotic stenosis.展开更多
BACKGROUND The critical diagnostic criteria for esophagogastric junction outflow obstruction(EGJOO)were published in the latest Chicago Classification version 4.0(CCv4.0).In addition to the previous criterion[elevated...BACKGROUND The critical diagnostic criteria for esophagogastric junction outflow obstruction(EGJOO)were published in the latest Chicago Classification version 4.0(CCv4.0).In addition to the previous criterion[elevated integrated relaxation pressure(IRP)in supine position],manometric diagnosis of EGJOO requires meeting the criteria of elevated median-IRP during upright wet swallows and elevated intrabolus pressure.However,with the diagnostic criteria modification,the change in manometric features of EGJOO remained unclear.AIM To evaluate the esophageal motility characteristics of patients with EGJOO and select valuable parameters for confirming the diagnosis of EGJOO.METHODS We performed a retrospective analysis of 370 patients who underwent highresolution manometry with 5 mL water swallows×10 in supine,×5 in upright position and the rapid drink challenge(RDC)with 200 mL water from November 2016 to November 2021 at Peking University First Hospital.Fifty-one patients with elevated integrated supine IRP and evidence of peristalsis were enrolled,with 24 patients meeting the updated manometric EGJOO diagnosis(CCv4.0)as the EGJOO group and 27 patients not meeting the updated EGJOO criteria as the isolated supine IRP elevated group(either normal median IRP in upright position or less than 20%of supine swallows with elevated IBP).Forty-six patients with normal manometric features were collected as the normal high-resolution manometry(HRM)group.Upper esophageal sphincter(UES),esophageal body,and lower esophageal sphincter(LES)parameters were compared between groups.RESULTS Compared with the normal HRM group,patients with EGJOO(CCv4.0)had significantly lower proximal esophageal contractile integral(PECI)and proximal esophageal length(PEL),with elevated IRP on RDC(P<0.05 for each comparison),while isolated supine IRP elevated patients had no such feature.Patients with EGJOO also had more significant abnormalities in the esophagogastric junction than isolated supine IRP elevated patients,including higher LES resting pressure(LESP),intrabolus pressure,median supine IRP,median upright IRP,and IRP on RDC(P<0.05 for each comparison).Patients with dysphagia had significantly lower PECI and PEL than patients without dysphagia among the fifty-one with elevated supine IRP.Further multivariate analysis revealed that PEL,LESP,and IRP on RDC are factors associated with EGJOO.The receiver-operating characteristic analysis showed UES nadir pressure,PEL,PECI,LESP,and IRP on RDC are parameters supportive for confirming the diagnosis of EGJOO.CONCLUSION Based on CCv4.0,patients with EGJOO have more severe esophagogastric junction dysfunction and are implicated in the proximal esophagus.Additionally,several parameters are supportive for confirming the diagnosis of EGJOO.展开更多
Objective To compare the clinical features and high-resolution esophageal motility-impedance characteristics among esophagogastric junction outflow obstruction(Eoo)patients,typeⅡachalasia(Ach)patients and healthy con...Objective To compare the clinical features and high-resolution esophageal motility-impedance characteristics among esophagogastric junction outflow obstruction(Eoo)patients,typeⅡachalasia(Ach)patients and healthy controls(Con),in order to explore the values of esophageal high-resolution manometry(HRM)in diagnosis and treatment of Eoo patients.Methods Patients展开更多
Hepatic vena cava syndrome(HVCS) also known as membranous obstruction of inferior vena cava reported mainly from Asia and Africa is an important cause of hepatic venous outflow obstruction(HVOO) that is complicated by...Hepatic vena cava syndrome(HVCS) also known as membranous obstruction of inferior vena cava reported mainly from Asia and Africa is an important cause of hepatic venous outflow obstruction(HVOO) that is complicated by high incidence of liver cirrhosis(LC) and moderate to high incidence of hepatocellular carcinoma(HCC). In the past the disease was considered congenital and was included under Budd-Chiari syndrome(BCS). HVCS is a chronic disease common in developing countries, the onset of which is related to poor hygienic living condition. The initial lesion in the disease is a bacterial infection induced localized thrombophlebitis in hepatic portion of inferior vena cava at the site where hepatic veins open which on resolution transforms into stenosis, membrane or thick obstruction,and is followed by development of cavo-caval collateral anastomosis. The disease is characterized by long asymptomatic period and recurrent acute exacerbations(AE) precipitated by clinical or subclinical bacterial infection. AE is managed with prolonged oral antibiotic. Development of LC and HCC in HVCS is related to the severity and frequency of AEs and not to the duration of the disease or the type or severity of the caval obstruction. HVOO that develops during severe acute stage or AE is a pre-cirrhotic condition. Primary BCS on the other hand is a rare disease related to prothrombotic disorders reported mainly among Caucasians that clinically manifest as acute, subacute disease or as fulminant hepatic failure; and is managed with life-long anticoagulation, portosystemic shunt/endovascular angioplasty and stent or liver transplantation. As epidemiology, etiology and natural history of HVCS are different from classical BCS, it is here, recognized as a separate disease entity, a third primary cause of HVOO after sinusoidal obstruction syndrome and BCS. Understanding of the natural history has made early diagnosis of HVCS possible. This paper describes epidemiology, natural history and diagnosis of HVCS and discusses the pathogenesis of LC in the disease and mentions distinctive clinical features of HVCS related LC.展开更多
BACKGROUND There is scant literature on hepatocellular carcinoma(HCC)in patients with Budd-Chiari syndrome(BCS).AIM To assess the magnitude,clinical characteristics,feasibility,and outcomes of treatment in BCS-HCC.MET...BACKGROUND There is scant literature on hepatocellular carcinoma(HCC)in patients with Budd-Chiari syndrome(BCS).AIM To assess the magnitude,clinical characteristics,feasibility,and outcomes of treatment in BCS-HCC.METHODS A total of 904 BCS patients from New Delhi,India and 1140 from Mumbai,India were included.The prevalence and incidence of HCC were determined,and among patients with BCS-HCC,the viability and outcomes of interventional therapy were evaluated.RESULTS In the New Delhi cohort of 35 BCS-HCC patients,18 had HCC at index presentation(prevalence 1.99%),and 17 developed HCC over a follow-up of 4601 person-years,[incidence 0.36(0.22-0.57)per 100 person-years].BCS-HCC patients were older when compared to patients with BCS alone(P=0.001)and had a higher proportion of inferior vena cava block,cirrhosis,and long-segment vascular obstruction.The median alpha-fetoprotein level was higher in patients with BCS-HCC at first presentation than those who developed HCC at follow-up(13029 ng/mL vs 500 ng/mL,P=0.01).Of the 35 BCS-HCC,26(74.3%)underwent radiological interventions for BCS,and 22(62.8%)patients underwent treatment for HCC[transarterial chemoembolization in 18(81.8%),oral tyrosine kinase inhibitor in 3(13.6%),and transarterial radioembolization in 1(4.5%)].The median survival among patients who underwent interventions for HCC compared with those who did not was 3.5 years vs 3.1 mo(P=0.0001).In contrast to the New Delhi cohort,the Mumbai cohort of BCS-HCC patients were predominantly males,presented with a more advanced HCC[Barcelona Clinic Liver Cancer C and D],and 2 patients underwent liver transplantation.CONCLUSION HCC is not uncommon in patients with BCS.Radiological interventions and liver transplantation are feasible in select primary BCS-HCC patients and may improve outcomes.展开更多
The recent International Consultation on Urological Disease(ICUD)panel 2010 confirmed that a urethral stricture is defined as a narrowing of the urethra consequent upon ischaemic spongiofibrosis,as distinct from sphin...The recent International Consultation on Urological Disease(ICUD)panel 2010 confirmed that a urethral stricture is defined as a narrowing of the urethra consequent upon ischaemic spongiofibrosis,as distinct from sphincter stenoses and a urethral disruption injury.Whenever possible,an anastomotic urethroplasty should be performed because of the higher success rate as compared to augmentation urethroplasty.There is some debate currently regarding the critical stricture length at which an anastomotic procedure can be used,but clearly the extent of the spongiofibrosis and individual anatomical factors(the length of the penis and urethra)are important,the limitation for this being extension of dissection beyond the peno-scrotal junction and the subsequent production of chordee.More recently,there has been interest in whether to excise and anastomose or to carry out a stricturotomy and reanastomosis using a Heineke-Miculicz technique.Augmentation urethroplasty has evolved towards the more extensive use of oral mucosa grafts as compared to penile skin flaps,as both flaps and grafts have similar efficacy and certainly the use of either dorsal or ventral positioning seems to provide comparable results.It is important that the reconstructive surgeon is well versed in the full range of available repair techniques,as no single method is suitable for all cases and will enable the management of any unexpected anatomical findings discovered intra-operatively.展开更多
Persistent ascites(PA)after liver transplantation(LT),commonly defined as ascites lasting more than 4 wk after LT,can be expected in up to 7%of patients.Despite being relatively rare,it is associated with worse clinic...Persistent ascites(PA)after liver transplantation(LT),commonly defined as ascites lasting more than 4 wk after LT,can be expected in up to 7%of patients.Despite being relatively rare,it is associated with worse clinical outcomes,including higher 1-year mortality.The cause of PA can be divided into vascular,hepatic,or extrahepatic.Vascular causes of PA include hepatic outflow and inflow obstructions,which are usually successfully treated.Regarding modifiable hepatic causes,recurrent hepatitis C and acute cellular rejection are the leading ones.Considering predictors for PA,the presence of ascites,refractory ascites,hepatorenal syndrome type 1,spontaneous bacterial peritonitis,hepatic encephalopathy,and prolonged ischemic time significantly influence the development of PA after LT.The initial approach to patients with PA should be to diagnose the treatable cause of PA.The stepwise approach in evaluating PA includes diagnostic paracentesis,ultrasound with Doppler,and an echocardiogram when a cardiac cause is suspected.Finally,a percutaneous or transjugular liver biopsy should be performed in cases where the diagnosis is unclear.PA of unknown cause should be treated with diuretics and paracentesis,while transjugular intrahepatic portosystemic shunt and splenic artery embolization are treatment methods in patients with refractory ascites after LT.展开更多
AIM:To re-examine whether hepatic vein thrombosis(HVT)(classical Budd-Chiari syndrome)and hepatic vena cava-Budd Chiari syndrome(HVC-BCS)are the same disorder.METHODS:A systematic review of observational studies condu...AIM:To re-examine whether hepatic vein thrombosis(HVT)(classical Budd-Chiari syndrome)and hepatic vena cava-Budd Chiari syndrome(HVC-BCS)are the same disorder.METHODS:A systematic review of observational studies conducted in adult subjects with primary BCS,hepatic vein outflow tract obstruction,membranous obstruction of the inferior vena cava(IVC),obliterative hepatocavopathy,or HVT during the period of January2000 until February 2015 was conducted using the following databases:Cochrane Library,CINAHL,MEDLINE,Pub Med and Scopus.RESULTS:Of 1299 articles identified,26 were included in this study.Classical BCS is more common in women with a pure hepatic vein obstruction(49%-74%).HVCBCS is more common in men with the obstruction often located in both the inferior vena cava and hepatic veins(14%-84%).Classical BCS presents with acute abdominal pain,ascites,and hepatomegaly.HVC-BCS presents with chronic abdominal pain and abdominalwall varices.Myeloproliferative neoplasms(MPN)are the most common etiology of classical BCS(16%-62%)with the JAK2V617-F mutation found in 26%-52%.In HVCBCS,MPN are found in 4%-5%,and the JAK2V617-F mutation in 2%-5%.Classical BCS responds well to medical management alone and 1st line management of HVC-BCS involves percutaneous recanalization,with few managed with medical management alone.CONCLUSION:Systematic review of recent data suggests that classical BCS and HVC-BCS may be two clinically different disorders that involve the disruption of hepatic venous outflow.展开更多
Hypertrophic cardiomyopathy (HCM), the most common single-gene cardiovascular disease, is associated with increased risk for arrhythmias, heart failure, and sudden cardiac death. The hemodynamic changes known to occur...Hypertrophic cardiomyopathy (HCM), the most common single-gene cardiovascular disease, is associated with increased risk for arrhythmias, heart failure, and sudden cardiac death. The hemodynamic changes known to occur during pregnancy can exacerbate heart failure and arrhythmias in women with HCM. We present a 30-year-old woman with HCM to illustrate the benefits of multidisciplinary team management of severe left ventricular outflow tract obstruction (peak gradient >100 mmHg) for optimal maternal and fetal outcomes.展开更多
Budd-Chiari syndrome(BCS)is a rare disease consisting of obstruction of the hepatic venous outflow tract,which can occur at any level ranging from small hepatic veins to the inferior vena cava(1-4).BCS can result from...Budd-Chiari syndrome(BCS)is a rare disease consisting of obstruction of the hepatic venous outflow tract,which can occur at any level ranging from small hepatic veins to the inferior vena cava(1-4).BCS can result from intravascular thrombosis or membranous obstruction of the hepatic venous outflow tract,or,less commonly,due to external compression from tumors,nodules,abscesses,cysts,or other intrahepatic lesions(1-3).展开更多
Baekground Although a lot of studies have been performed on the long term outcome in adults with repaired tetralogy of Fallot (TOF) in developed countries, but rare information for primary correction of adult TOF is...Baekground Although a lot of studies have been performed on the long term outcome in adults with repaired tetralogy of Fallot (TOF) in developed countries, but rare information for primary correction of adult TOF is available. The research focusing on the effect of transanular patch (TAP) for primary correction of TOF in adulthood is still absent. Via retrograde analysis of 7-year follow-up, this study was designed to explore the effect of the transanular patch for primary correction in adult TOF on the surgical outcome, postoperative cardiac function and morbidity, as well as to address the management of the complication. Methods A total of 151 consecutive adult patients (age ≥ 18) who underwent primary radical correction of TOF form 2007-2014 were selected and divided into TAP demographic statistic characteristics, and non-TAP groups based on the EACTS database. Results of echocardiography, color-Doppler echocardiography, cardiovascular enhanced contrast computed tomography (CT), and/or cardiac catheterization; intraoperative information, postoperative results and outcomes were reported. During follow-up, short term was defined within 3 months after discharge, and midterm was defined as 6-12 months after discharge. Results Total postoperative mortality was 5.96% in all the cases, 6.96% in TAP group, and 2.78% (1/36) in non-TAP group. There was no significant difference between two groups. Follow-up period ranged from 3 months to 62 months. Readmission occurred and was followed by medical treatment without re-do surgery in 6 cases (3.97%). The short term echocardiography demonstrated that pulmonary regurgitation and short term tricuspid regurgitation after discharge in TAP group were more severe (P 〈 0.001). The short term residual pulmonary stenosis (RVOTO) severity after discharge in TAP group was less severe (P = 0.018). Midterm echocardiography after discharge demonstrated pulmonary regurgitation and tricuspid regurgitation in TAP group were still more severe (P = 0.003). The severity of residual pulmonary stenosis in TAP group was less severe (P = 0.044). Multivariate unconditional logistic regression analysis showed that risk factors for mortality of adult TOF primary correction included: the acquirement of repeated cardiopulmonary bypass, OR = 126.28 (5.17 - 3082.23), P = 0.003; the application of DHCA, OR = 61.08(2.26 - 1652.51), P = 0.015; postoperative pulmonary regurgitation, OR = 33.84(2.53 - 452.53), P = 0.008, long intensive care time, OR = 1.00 (1.00 - 1.01), P = 0.012. The first three variables were high risk factors. Conclusions Primary radical correction of adult TOF has a good outcome, acceptable morbidity and mortality rates with mid-term surgical outcome in terms of effort tolerance. The acquirement of repeated cardiopulmonary bypass, the application of DHCA and postoperative pulmonary regurgitation are high risk factors of mortality. IS Chin J Cardiol 2015; 16 (2): 72 - 79]展开更多
文摘Aim: To explore the interaction between bladder compliance (BC) and bladder outflow obstruction (BOO) in men with benign prostatic hyperplasia (BPH) using cross-sectional and longitudinal studies. Methods: A total of 181 men with BPH were recruited, and 100 of them were followed for one year. Cystometry was performed in a standing or a sitting position with 30 mL/min infusion. BC was manually corrected and defined. Obstruction coefficient (OCO), linear passive urethral resistance relation and international continence society (ICS) nomogram were used to diagnose BOO. The obstructed parameters were compared between the reduced BC group and the non-reduced group. BC was compared between the first investigation at the beginning of study and the second investigation at the end of study during the one-year follow-up period. Results: The group with reduced BC had increased OCO and linear passive urethral resistance relation. BC was significantly lower in the obstructed group (55.7 mL/cm water) than that in unobstructed and equivocal one (74.9 mL/cm water, P 〈 0.01). BC gradually reduced with the increased obstructed grade. There was a significantly weak negative correlation between BC and OCO (r = - 0.132, P 〈 0.01). Over the one-year follow-up period in the longitudinal study, BC for all men changed from 54.4 to 48.8 mL/cm water (P 〉 0.05), and BC for the group with BOO fell from 58.4 ± 70.1 to 46.5 ± 38.7 mL/cm water (P 〉 0.05). Conclusion: In men with BPH, a significant systematic decrease occurred in BC in the obstructed group and a significant systematic increase with urethral resistance occurred in the low BC group. A longitudinal study of the tendency of BC reduction in a group with BOO is necessary in the future.
文摘AIM To assess the efficacy and safety of balloon dilatation for the treatment of hepatic venous outflow obstruction(HVOO) following pediatric liver transplantation.METHODS A total of 246 pediatric patients underwent liver transplantation at our hospital between June 2013 and September 2016. Among these patients, five were ultimately diagnosed with HVOO. Seven procedures(two patients underwent two balloon dilatation procedures) were included in this analysis. The demographic data,types of donor and liver transplant, interventional examination and therapeutic outcomes of these five children were analyzed. The median interval time between pediatric liver transplantation and balloon dilatation procedures was 9.8 mo(range: 1-32).RESULTS Five children with HVOO were successfully treated by balloon angioplasty without stent placement, with seven procedures performed for six stenotic lesions. All children underwent successful percutaneous intervention. Among these five patients, four were treated by single balloon angioplasty, and these patients did not develop recurrent stenosis. In seven episodes of balloon angioplasty across the stenosis, the pressure gradient was 12.0 ± 8.8 mm Hg before balloon dilatation and 1.1 ± 1.5 mm Hg after the procedures, which revealed a statistically significant reduction(P < 0.05). The overall technical success rate among these seven procedures was 100%(7/7), and clinical success was achieved in all five patients(100%). The patients were followed for 4-33 mo(median: 15 mo). No significant procedural complications or procedurerelated deaths occurred.CONCLUSION Balloon dilatation is an effective and safe therapeutic option for HVOO in children undergoing pediatric liver transplantation. Venous angioplasty is also recommended in cases with recurrent HVOO.
文摘Hepatic venous outflow obstruction after piggyback liver transplantation is a very rare complication. An unusual mechanism aggravating it is reported. A 33-year-old man with end-stage hepatitis B liver cirrhosis underwent a piggyback orthotopic liver transplantation using a full-size cadaveric graft. Two months after transplantation, he developed gross ascites refractory to maximal diuretic therapy. Doppler ultrasound showed patent portal and hepatic veins. Serial computed tomography scans revealed a hypoperfused right posterior segment of the liver which subsequently underwent atrophy. Hepatic venography demonstrated a high-grade stenosis with an element of torsion of venous drainage at the anastomosis. The stenosis was successfully treated with repeated percutaneous balloon angioplasty. The patient remained asymptomatic six months afterwards with complete resolution of ascites and peripheral edema. We postulate that liver allograft segmental hypoperfusion and atrophy may aggravate or result in a hepatic venous outflow problem by the mechanism of torsion effect. Percutaneous balloon angioplasty is a safe and effective treatment modality for anastomotic stenosis.
基金Supported by the China Central Health Research Fund,No.W2013BJ29the Interdisciplinary Clinical Research Project of Peking University First Hospital,No.2019CR40.
文摘BACKGROUND The critical diagnostic criteria for esophagogastric junction outflow obstruction(EGJOO)were published in the latest Chicago Classification version 4.0(CCv4.0).In addition to the previous criterion[elevated integrated relaxation pressure(IRP)in supine position],manometric diagnosis of EGJOO requires meeting the criteria of elevated median-IRP during upright wet swallows and elevated intrabolus pressure.However,with the diagnostic criteria modification,the change in manometric features of EGJOO remained unclear.AIM To evaluate the esophageal motility characteristics of patients with EGJOO and select valuable parameters for confirming the diagnosis of EGJOO.METHODS We performed a retrospective analysis of 370 patients who underwent highresolution manometry with 5 mL water swallows×10 in supine,×5 in upright position and the rapid drink challenge(RDC)with 200 mL water from November 2016 to November 2021 at Peking University First Hospital.Fifty-one patients with elevated integrated supine IRP and evidence of peristalsis were enrolled,with 24 patients meeting the updated manometric EGJOO diagnosis(CCv4.0)as the EGJOO group and 27 patients not meeting the updated EGJOO criteria as the isolated supine IRP elevated group(either normal median IRP in upright position or less than 20%of supine swallows with elevated IBP).Forty-six patients with normal manometric features were collected as the normal high-resolution manometry(HRM)group.Upper esophageal sphincter(UES),esophageal body,and lower esophageal sphincter(LES)parameters were compared between groups.RESULTS Compared with the normal HRM group,patients with EGJOO(CCv4.0)had significantly lower proximal esophageal contractile integral(PECI)and proximal esophageal length(PEL),with elevated IRP on RDC(P<0.05 for each comparison),while isolated supine IRP elevated patients had no such feature.Patients with EGJOO also had more significant abnormalities in the esophagogastric junction than isolated supine IRP elevated patients,including higher LES resting pressure(LESP),intrabolus pressure,median supine IRP,median upright IRP,and IRP on RDC(P<0.05 for each comparison).Patients with dysphagia had significantly lower PECI and PEL than patients without dysphagia among the fifty-one with elevated supine IRP.Further multivariate analysis revealed that PEL,LESP,and IRP on RDC are factors associated with EGJOO.The receiver-operating characteristic analysis showed UES nadir pressure,PEL,PECI,LESP,and IRP on RDC are parameters supportive for confirming the diagnosis of EGJOO.CONCLUSION Based on CCv4.0,patients with EGJOO have more severe esophagogastric junction dysfunction and are implicated in the proximal esophagus.Additionally,several parameters are supportive for confirming the diagnosis of EGJOO.
文摘Objective To compare the clinical features and high-resolution esophageal motility-impedance characteristics among esophagogastric junction outflow obstruction(Eoo)patients,typeⅡachalasia(Ach)patients and healthy controls(Con),in order to explore the values of esophageal high-resolution manometry(HRM)in diagnosis and treatment of Eoo patients.Methods Patients
文摘Hepatic vena cava syndrome(HVCS) also known as membranous obstruction of inferior vena cava reported mainly from Asia and Africa is an important cause of hepatic venous outflow obstruction(HVOO) that is complicated by high incidence of liver cirrhosis(LC) and moderate to high incidence of hepatocellular carcinoma(HCC). In the past the disease was considered congenital and was included under Budd-Chiari syndrome(BCS). HVCS is a chronic disease common in developing countries, the onset of which is related to poor hygienic living condition. The initial lesion in the disease is a bacterial infection induced localized thrombophlebitis in hepatic portion of inferior vena cava at the site where hepatic veins open which on resolution transforms into stenosis, membrane or thick obstruction,and is followed by development of cavo-caval collateral anastomosis. The disease is characterized by long asymptomatic period and recurrent acute exacerbations(AE) precipitated by clinical or subclinical bacterial infection. AE is managed with prolonged oral antibiotic. Development of LC and HCC in HVCS is related to the severity and frequency of AEs and not to the duration of the disease or the type or severity of the caval obstruction. HVOO that develops during severe acute stage or AE is a pre-cirrhotic condition. Primary BCS on the other hand is a rare disease related to prothrombotic disorders reported mainly among Caucasians that clinically manifest as acute, subacute disease or as fulminant hepatic failure; and is managed with life-long anticoagulation, portosystemic shunt/endovascular angioplasty and stent or liver transplantation. As epidemiology, etiology and natural history of HVCS are different from classical BCS, it is here, recognized as a separate disease entity, a third primary cause of HVOO after sinusoidal obstruction syndrome and BCS. Understanding of the natural history has made early diagnosis of HVCS possible. This paper describes epidemiology, natural history and diagnosis of HVCS and discusses the pathogenesis of LC in the disease and mentions distinctive clinical features of HVCS related LC.
基金This study was reviewed and approved by the Ethics Committee of the All India Institute of Medical Sciences,New Delhi(Approval No.IEC/NP-458/12.12.2014,RP 22-2015).
文摘BACKGROUND There is scant literature on hepatocellular carcinoma(HCC)in patients with Budd-Chiari syndrome(BCS).AIM To assess the magnitude,clinical characteristics,feasibility,and outcomes of treatment in BCS-HCC.METHODS A total of 904 BCS patients from New Delhi,India and 1140 from Mumbai,India were included.The prevalence and incidence of HCC were determined,and among patients with BCS-HCC,the viability and outcomes of interventional therapy were evaluated.RESULTS In the New Delhi cohort of 35 BCS-HCC patients,18 had HCC at index presentation(prevalence 1.99%),and 17 developed HCC over a follow-up of 4601 person-years,[incidence 0.36(0.22-0.57)per 100 person-years].BCS-HCC patients were older when compared to patients with BCS alone(P=0.001)and had a higher proportion of inferior vena cava block,cirrhosis,and long-segment vascular obstruction.The median alpha-fetoprotein level was higher in patients with BCS-HCC at first presentation than those who developed HCC at follow-up(13029 ng/mL vs 500 ng/mL,P=0.01).Of the 35 BCS-HCC,26(74.3%)underwent radiological interventions for BCS,and 22(62.8%)patients underwent treatment for HCC[transarterial chemoembolization in 18(81.8%),oral tyrosine kinase inhibitor in 3(13.6%),and transarterial radioembolization in 1(4.5%)].The median survival among patients who underwent interventions for HCC compared with those who did not was 3.5 years vs 3.1 mo(P=0.0001).In contrast to the New Delhi cohort,the Mumbai cohort of BCS-HCC patients were predominantly males,presented with a more advanced HCC[Barcelona Clinic Liver Cancer C and D],and 2 patients underwent liver transplantation.CONCLUSION HCC is not uncommon in patients with BCS.Radiological interventions and liver transplantation are feasible in select primary BCS-HCC patients and may improve outcomes.
文摘The recent International Consultation on Urological Disease(ICUD)panel 2010 confirmed that a urethral stricture is defined as a narrowing of the urethra consequent upon ischaemic spongiofibrosis,as distinct from sphincter stenoses and a urethral disruption injury.Whenever possible,an anastomotic urethroplasty should be performed because of the higher success rate as compared to augmentation urethroplasty.There is some debate currently regarding the critical stricture length at which an anastomotic procedure can be used,but clearly the extent of the spongiofibrosis and individual anatomical factors(the length of the penis and urethra)are important,the limitation for this being extension of dissection beyond the peno-scrotal junction and the subsequent production of chordee.More recently,there has been interest in whether to excise and anastomose or to carry out a stricturotomy and reanastomosis using a Heineke-Miculicz technique.Augmentation urethroplasty has evolved towards the more extensive use of oral mucosa grafts as compared to penile skin flaps,as both flaps and grafts have similar efficacy and certainly the use of either dorsal or ventral positioning seems to provide comparable results.It is important that the reconstructive surgeon is well versed in the full range of available repair techniques,as no single method is suitable for all cases and will enable the management of any unexpected anatomical findings discovered intra-operatively.
基金Supported by the Croatian Science Foundation,Emerging and Neglected Hepatotropic Viruses after Solid Organ and Hematopoietic Stem Cell Transplantation(to Mrzljak A),No.IP-2020-02-7407.
文摘Persistent ascites(PA)after liver transplantation(LT),commonly defined as ascites lasting more than 4 wk after LT,can be expected in up to 7%of patients.Despite being relatively rare,it is associated with worse clinical outcomes,including higher 1-year mortality.The cause of PA can be divided into vascular,hepatic,or extrahepatic.Vascular causes of PA include hepatic outflow and inflow obstructions,which are usually successfully treated.Regarding modifiable hepatic causes,recurrent hepatitis C and acute cellular rejection are the leading ones.Considering predictors for PA,the presence of ascites,refractory ascites,hepatorenal syndrome type 1,spontaneous bacterial peritonitis,hepatic encephalopathy,and prolonged ischemic time significantly influence the development of PA after LT.The initial approach to patients with PA should be to diagnose the treatable cause of PA.The stepwise approach in evaluating PA includes diagnostic paracentesis,ultrasound with Doppler,and an echocardiogram when a cardiac cause is suspected.Finally,a percutaneous or transjugular liver biopsy should be performed in cases where the diagnosis is unclear.PA of unknown cause should be treated with diuretics and paracentesis,while transjugular intrahepatic portosystemic shunt and splenic artery embolization are treatment methods in patients with refractory ascites after LT.
文摘AIM:To re-examine whether hepatic vein thrombosis(HVT)(classical Budd-Chiari syndrome)and hepatic vena cava-Budd Chiari syndrome(HVC-BCS)are the same disorder.METHODS:A systematic review of observational studies conducted in adult subjects with primary BCS,hepatic vein outflow tract obstruction,membranous obstruction of the inferior vena cava(IVC),obliterative hepatocavopathy,or HVT during the period of January2000 until February 2015 was conducted using the following databases:Cochrane Library,CINAHL,MEDLINE,Pub Med and Scopus.RESULTS:Of 1299 articles identified,26 were included in this study.Classical BCS is more common in women with a pure hepatic vein obstruction(49%-74%).HVCBCS is more common in men with the obstruction often located in both the inferior vena cava and hepatic veins(14%-84%).Classical BCS presents with acute abdominal pain,ascites,and hepatomegaly.HVC-BCS presents with chronic abdominal pain and abdominalwall varices.Myeloproliferative neoplasms(MPN)are the most common etiology of classical BCS(16%-62%)with the JAK2V617-F mutation found in 26%-52%.In HVCBCS,MPN are found in 4%-5%,and the JAK2V617-F mutation in 2%-5%.Classical BCS responds well to medical management alone and 1st line management of HVC-BCS involves percutaneous recanalization,with few managed with medical management alone.CONCLUSION:Systematic review of recent data suggests that classical BCS and HVC-BCS may be two clinically different disorders that involve the disruption of hepatic venous outflow.
文摘Hypertrophic cardiomyopathy (HCM), the most common single-gene cardiovascular disease, is associated with increased risk for arrhythmias, heart failure, and sudden cardiac death. The hemodynamic changes known to occur during pregnancy can exacerbate heart failure and arrhythmias in women with HCM. We present a 30-year-old woman with HCM to illustrate the benefits of multidisciplinary team management of severe left ventricular outflow tract obstruction (peak gradient >100 mmHg) for optimal maternal and fetal outcomes.
文摘Budd-Chiari syndrome(BCS)is a rare disease consisting of obstruction of the hepatic venous outflow tract,which can occur at any level ranging from small hepatic veins to the inferior vena cava(1-4).BCS can result from intravascular thrombosis or membranous obstruction of the hepatic venous outflow tract,or,less commonly,due to external compression from tumors,nodules,abscesses,cysts,or other intrahepatic lesions(1-3).
基金supported by The Twelfth National Five-Year Plan(No.2011BAI11B22)
文摘Baekground Although a lot of studies have been performed on the long term outcome in adults with repaired tetralogy of Fallot (TOF) in developed countries, but rare information for primary correction of adult TOF is available. The research focusing on the effect of transanular patch (TAP) for primary correction of TOF in adulthood is still absent. Via retrograde analysis of 7-year follow-up, this study was designed to explore the effect of the transanular patch for primary correction in adult TOF on the surgical outcome, postoperative cardiac function and morbidity, as well as to address the management of the complication. Methods A total of 151 consecutive adult patients (age ≥ 18) who underwent primary radical correction of TOF form 2007-2014 were selected and divided into TAP demographic statistic characteristics, and non-TAP groups based on the EACTS database. Results of echocardiography, color-Doppler echocardiography, cardiovascular enhanced contrast computed tomography (CT), and/or cardiac catheterization; intraoperative information, postoperative results and outcomes were reported. During follow-up, short term was defined within 3 months after discharge, and midterm was defined as 6-12 months after discharge. Results Total postoperative mortality was 5.96% in all the cases, 6.96% in TAP group, and 2.78% (1/36) in non-TAP group. There was no significant difference between two groups. Follow-up period ranged from 3 months to 62 months. Readmission occurred and was followed by medical treatment without re-do surgery in 6 cases (3.97%). The short term echocardiography demonstrated that pulmonary regurgitation and short term tricuspid regurgitation after discharge in TAP group were more severe (P 〈 0.001). The short term residual pulmonary stenosis (RVOTO) severity after discharge in TAP group was less severe (P = 0.018). Midterm echocardiography after discharge demonstrated pulmonary regurgitation and tricuspid regurgitation in TAP group were still more severe (P = 0.003). The severity of residual pulmonary stenosis in TAP group was less severe (P = 0.044). Multivariate unconditional logistic regression analysis showed that risk factors for mortality of adult TOF primary correction included: the acquirement of repeated cardiopulmonary bypass, OR = 126.28 (5.17 - 3082.23), P = 0.003; the application of DHCA, OR = 61.08(2.26 - 1652.51), P = 0.015; postoperative pulmonary regurgitation, OR = 33.84(2.53 - 452.53), P = 0.008, long intensive care time, OR = 1.00 (1.00 - 1.01), P = 0.012. The first three variables were high risk factors. Conclusions Primary radical correction of adult TOF has a good outcome, acceptable morbidity and mortality rates with mid-term surgical outcome in terms of effort tolerance. The acquirement of repeated cardiopulmonary bypass, the application of DHCA and postoperative pulmonary regurgitation are high risk factors of mortality. IS Chin J Cardiol 2015; 16 (2): 72 - 79]