AIM: To examine the vitamin D status in patients with alcoholic cirrhosis compared to those with primary biliary cirrhosis. METHODS: Our retrospective case series comprised 89 patients with alcoholic cirrhosis and 34 ...AIM: To examine the vitamin D status in patients with alcoholic cirrhosis compared to those with primary biliary cirrhosis. METHODS: Our retrospective case series comprised 89 patients with alcoholic cirrhosis and 34 patients with primary biliary cirrhosis who visited our outpatient clinic in 2005 and underwent a serum vitamin D status assessment. RESULTS: Among the patients with alcoholic cirrhosis, 85% had serum vitamin D levels below 50 nmol/L and 55% had levels below 25 nmol/L, as compared to 60% and 16% of the patients with primary biliary cirrhosis, respectively (P < 0.001). In both groups, serum vitamin D levels decreased with increasing liver disease severity, as determined by the Child-Pugh score. CONCLUSION: Vitamin D deficiency in cirrhosis relates to liver dysfunction rather than aetiology, with lower levels of vitamin D in alcoholic cirrhosis than in primary biliary cirrhosis.展开更多
BACKGROUND Progressive familial intrahepatic cholestasis(PFIC)encompasses a group of autosomal recessive disorders with high morbidity and mortality.Variants in the gene encoding tight junction protein-2(TJP2)have bee...BACKGROUND Progressive familial intrahepatic cholestasis(PFIC)encompasses a group of autosomal recessive disorders with high morbidity and mortality.Variants in the gene encoding tight junction protein-2(TJP2)have been linked to PFIC type 4(PFIC4),which predominantly presents in childhood.However,there are only limited data from adults with TJP2-related PFIC4.We report a family with an autosomal recessive disorder with a novel variant in the TJP2 gene in adults with very variable expression of PFIC4.CASE SUMMARY The index patient presented at 19 years old with liver cirrhosis and variceal bleeding and was treated with endoscopic banding and beta-blockers.In 2018,he developed primary liver cancer that was treated with radiofrequency ablation followed by liver transplantation in 2019.Genetic testing revealed a novel homozygous TJP2 variant causing PFIC4(TJP2([NM_004817.3]:c.[3334C>T];[3334C>T])).The consanguineous family consists of the father and mother(both heterozygous)and their 12 children,of which five carry the variant in a homozygous state;however,these five siblings have highly variable expression of PFIC4.Two homozygous brothers had cirrhosis and portal hypertension at diagnosis at the ages of 19 and 36.Two other homozygous brothers,age 23 and 19,and the homozygous sister,age 21,have elevated liver enzymes but presently no cirrhosis,which may suggest an age-dependent penetrance.In addition,five sisters had severe and mild intrahepatic cholestasis of pregnancy and carry the TJP2 variant in a homozygous and heterozygous state,respectively.CONCLUSION This novel TJP2 variant is associated with PFIC4 causing severe liver disease with cirrhosis and primary liver cancer in adolescents/adults.展开更多
Rotationally symmetric triangulation(RST)sensor has more flexibility and less uncertainty limits because of the abaxial rotationally symmetric optical system.But if the incident laser is eccentric,the symmetry of the ...Rotationally symmetric triangulation(RST)sensor has more flexibility and less uncertainty limits because of the abaxial rotationally symmetric optical system.But if the incident laser is eccentric,the symmetry of the image will descend,and it will result in the eccentric error especially when some part of the imaged ring is blocked.The model of rotationally symmetric triangulation that meets the Schimpflug condition is presented in this paper.The error from eccentric incident laser is analysed.It is pointed out that the eccentric error is composed of two parts,one is a cosine in circumference and proportional to the eccentric departure factor,and the other is a much smaller quadric factor of the departure.When the ring is complete,the first error factor is zero because it is integrated in whole ring, but if some part of the ring is blocked,the first factor will be the main error.Simulation verifies the result of the a- nalysis.At last,a compensation method to the error when some part of the ring is lost is presented based on neural network.The results of experiment show that the compensation will make the absolute maximum error descend to half,and the standard deviation of error descends to 1/3.展开更多
AIM： To investigate if sildenafil increases splanchnic blood flow and changes the hepatic venous pressure gradient （HVPG） in patients with cirrhosis. Phosphodiesterase type-5 inhibitors are valuable in the treatmen...AIM： To investigate if sildenafil increases splanchnic blood flow and changes the hepatic venous pressure gradient （HVPG） in patients with cirrhosis. Phosphodiesterase type-5 inhibitors are valuable in the treatment of erectile dysfunction and pulmonary hypertension in patients with end-stage liver disease. However, the effect of phosphodiesterase type-5 inhibitors on splanchnic blood flow and portal hypertension remains essentially unknown. METHODS： Ten patients with biopsy proven cirrhosis （five females/five males, mean age 54：1：8 years） and an HVPG above 12 mmHg were studied after informed consent. Measurement of splanchnic blood flow and the HVPG during liver vein catheterization were done before and 80 min after oral administration of 50 mg sildenafil. Blood flow was estimated by use of indocyanine green clearance technique and Fick＇s principle, with correction for non-steady state. RESULTS： The plasma concentration of sildenafil was 222 ± 136 ng/mL 80 min after administration. Mean arterial blood pressure decreased from 77 ±7 mmHg to 66 ± 12 mmHg, P = 0.003, while the splanchnicblood flow and oxygen consumption remained unchanged at 1.14 ± 0.71 L/min and 2.3 ± 0.6 mmol/ min, respectively. Also the HVPG remained unchanged （18 ± 2 mmHg vs 16 ± 2 mmHg） with individual changes ranging from -8 mmHg to ±2 mmHg. In seven patients, HVPG decreased and in three it increased. CONCLUSION： In spite of arterial blood pressure decreases 80 min after administration of the phosphodiesterase type-5 inhibitor sildenafil, the present study could not demonstrate any clinical relevant influence on splanichnic blood flow, oxygen consumption or the HVPG.展开更多
Bile acid diarrhea results from excessive amounts of bile acids entering the colon due to hepatic overexcretion of bile acids or bile acid malabsorption in the terminal ileum. The main therapies include bile acid sequ...Bile acid diarrhea results from excessive amounts of bile acids entering the colon due to hepatic overexcretion of bile acids or bile acid malabsorption in the terminal ileum. The main therapies include bile acid sequestrants, such as colestyramine and colesevelam, which may be given in combination with the opioid receptor agonist loperamide. Some patients are refractory to conventional treatments. We report the use of the farnesoid X receptor agonist obeticholic acid in a patient with refractory bile acid diarrhea and subsequent intestinal failure. A 32-year-old woman with quiescent colonic Crohn's disease and a normal terminal ileum had been diagnosed with severe bile acid malabsorption and complained of watery diarrhea and fatigue. The diarrhea resulted in hypokalemia and sodium depletion that made her dependent on twice weekly intravenous fluid and electrolyte infusions. Conventional therapies with colestyramine, colesevelam, and loperamide had no effect. Second-line antisecretory therapies with pantoprazole, liraglutide, and octreotide also failed. Third-line treatment with obeticholic acid reduced the number of stools from an average of 13 to an average of 7 per 24h and improved the patient's quality of life. The fluid and electrolyte balances normalized. The effect was sustained during follow-up for 6 mo with treatment at a daily dosage of 25 mg. The diarrhea worsened shortly after cessation of obeticholic acid. This case report supports the initial report that obeticholic acid may reduce bile acid production and improve symptoms in patients with bile acid diarrhea.展开更多
This investigation examines experimentally the behavior of swirled jets produced by axial flow fans blowing into a crossflow at low velocity ratios. The main difference with non-swirl cases is an asymmetry of the domi...This investigation examines experimentally the behavior of swirled jets produced by axial flow fans blowing into a crossflow at low velocity ratios. The main difference with non-swirl cases is an asymmetry of the dominant kidney vortex and a slight distortion of the jet trace downstream of the injection hole. The effect of jet rotation at relatively low swirl numbers and similar velocity ratios is also investigated by a validated computational analysis tool. The numerical results are analyzed by means of various post-processing procedures, aiming to clarify, quantify and analyze the impact of swirl on the characteristics and the flow domain of a jet in crossflow. In general, swirl introduces an asymmetry in all examined quantities and prevents the penetration of the jet into the crossflow, causing the jet to remain closer to the wall surface. The rotation of the injected fluid results in an imparity of the two parts of the Counter Rotating Vortex Pair （CVP） which is no longer symmetric to the axial centerline plane. High swirl numbers result in the destruction of the CVP and the dominant kidney shape vortex is transformed into a comma shape vortex, rotating close to the wall.展开更多
This study examines experimentally the effect of stators on the performance and heat transfer characteristics of small axial cooling fans. A single fan impeller, followed by nine stator blades in the case of a complet...This study examines experimentally the effect of stators on the performance and heat transfer characteristics of small axial cooling fans. A single fan impeller, followed by nine stator blades in the case of a complete stage, was used for all the experimental configurations. Performance measurements were carried out in a constant speed stage performance test rig while the transient liquid crystal technique was used for the heat transfer measurements. Full surface heat transfer coefficient distributions were obtained by recording the temperature history of liquid crystals on a target plate. The experimental data indicated that the results are highly affected by the flow conditions at the fan outlet. Stators can be beneficial in terms of pressure drop and efficiency, and thus more economical operation, as well as, in the local heat transfer distribution at the wake of the stator blades if the fan is installed very close to the cooling object. However, as the separation distance increases, enhanced heat transfer rate in the order of 25% is observed in the case of the fan impeller.展开更多
In many engineering applications,heat transfer enhancement techniques are of vital importance in order to ensure reliable thermal designs of convective heat transfer applications.This study examines experimentally the...In many engineering applications,heat transfer enhancement techniques are of vital importance in order to ensure reliable thermal designs of convective heat transfer applications.This study examines experimentally the heat transfer characteristics on the base plate around various surface mounted obstacles.Local convection coefficients are evaluated in the vicinity of each individual protruding body with great spatial resolution using the transient liquid crystal technique.Five different obstacles of constant height-to-hydraulic diameter ratio(~1.3) are considered.These include:a cylinder,a square,a triangle,a diamond and a vortex generator of delta wing shape design.The experiments were carried out over a range of freestream Reynolds numbers,based on the hydraulic diameter of each obstacle,varying from 4,000 to 13,000.The results indicate a negligible effect of the flow speed on the heat transfer topological structure and a considerable effect of the obstacle geometry on the level and distribution of heat transfer enhancement.展开更多
文摘AIM: To examine the vitamin D status in patients with alcoholic cirrhosis compared to those with primary biliary cirrhosis. METHODS: Our retrospective case series comprised 89 patients with alcoholic cirrhosis and 34 patients with primary biliary cirrhosis who visited our outpatient clinic in 2005 and underwent a serum vitamin D status assessment. RESULTS: Among the patients with alcoholic cirrhosis, 85% had serum vitamin D levels below 50 nmol/L and 55% had levels below 25 nmol/L, as compared to 60% and 16% of the patients with primary biliary cirrhosis, respectively (P < 0.001). In both groups, serum vitamin D levels decreased with increasing liver disease severity, as determined by the Child-Pugh score. CONCLUSION: Vitamin D deficiency in cirrhosis relates to liver dysfunction rather than aetiology, with lower levels of vitamin D in alcoholic cirrhosis than in primary biliary cirrhosis.
基金Supported by Sanming Project of Medicine in Shenzhen of China,No.SZSM201612074
文摘BACKGROUND Progressive familial intrahepatic cholestasis(PFIC)encompasses a group of autosomal recessive disorders with high morbidity and mortality.Variants in the gene encoding tight junction protein-2(TJP2)have been linked to PFIC type 4(PFIC4),which predominantly presents in childhood.However,there are only limited data from adults with TJP2-related PFIC4.We report a family with an autosomal recessive disorder with a novel variant in the TJP2 gene in adults with very variable expression of PFIC4.CASE SUMMARY The index patient presented at 19 years old with liver cirrhosis and variceal bleeding and was treated with endoscopic banding and beta-blockers.In 2018,he developed primary liver cancer that was treated with radiofrequency ablation followed by liver transplantation in 2019.Genetic testing revealed a novel homozygous TJP2 variant causing PFIC4(TJP2([NM_004817.3]:c.[3334C>T];[3334C>T])).The consanguineous family consists of the father and mother(both heterozygous)and their 12 children,of which five carry the variant in a homozygous state;however,these five siblings have highly variable expression of PFIC4.Two homozygous brothers had cirrhosis and portal hypertension at diagnosis at the ages of 19 and 36.Two other homozygous brothers,age 23 and 19,and the homozygous sister,age 21,have elevated liver enzymes but presently no cirrhosis,which may suggest an age-dependent penetrance.In addition,five sisters had severe and mild intrahepatic cholestasis of pregnancy and carry the TJP2 variant in a homozygous and heterozygous state,respectively.CONCLUSION This novel TJP2 variant is associated with PFIC4 causing severe liver disease with cirrhosis and primary liver cancer in adolescents/adults.
基金Supported by China Scholarship Council(CSC),Deutscher Akademischer Austausch Dienst(DAAD),National Natural Science Foundation of China(60375011,60575028)Natural Science Foundation of Anhui Province(04042044)Supported by Program for New Century Excellent Talents in University(NCET-04-0560)
文摘Rotationally symmetric triangulation(RST)sensor has more flexibility and less uncertainty limits because of the abaxial rotationally symmetric optical system.But if the incident laser is eccentric,the symmetry of the image will descend,and it will result in the eccentric error especially when some part of the imaged ring is blocked.The model of rotationally symmetric triangulation that meets the Schimpflug condition is presented in this paper.The error from eccentric incident laser is analysed.It is pointed out that the eccentric error is composed of two parts,one is a cosine in circumference and proportional to the eccentric departure factor,and the other is a much smaller quadric factor of the departure.When the ring is complete,the first error factor is zero because it is integrated in whole ring, but if some part of the ring is blocked,the first factor will be the main error.Simulation verifies the result of the a- nalysis.At last,a compensation method to the error when some part of the ring is lost is presented based on neural network.The results of experiment show that the compensation will make the absolute maximum error descend to half,and the standard deviation of error descends to 1/3.
基金Rigshospitalet,University of Copenhagen,The Laerdal Foundation for Acute MedicineSavvaerksejer Jeppe Juhl and wife Ovita Juhls Foundation+2 种基金The Novo Nordisk FoundationThe AP-Mфller Foundationan unrestricted grant from Pfizer,Denmark
文摘AIM： To investigate if sildenafil increases splanchnic blood flow and changes the hepatic venous pressure gradient （HVPG） in patients with cirrhosis. Phosphodiesterase type-5 inhibitors are valuable in the treatment of erectile dysfunction and pulmonary hypertension in patients with end-stage liver disease. However, the effect of phosphodiesterase type-5 inhibitors on splanchnic blood flow and portal hypertension remains essentially unknown. METHODS： Ten patients with biopsy proven cirrhosis （five females/five males, mean age 54：1：8 years） and an HVPG above 12 mmHg were studied after informed consent. Measurement of splanchnic blood flow and the HVPG during liver vein catheterization were done before and 80 min after oral administration of 50 mg sildenafil. Blood flow was estimated by use of indocyanine green clearance technique and Fick＇s principle, with correction for non-steady state. RESULTS： The plasma concentration of sildenafil was 222 ± 136 ng/mL 80 min after administration. Mean arterial blood pressure decreased from 77 ±7 mmHg to 66 ± 12 mmHg, P = 0.003, while the splanchnicblood flow and oxygen consumption remained unchanged at 1.14 ± 0.71 L/min and 2.3 ± 0.6 mmol/ min, respectively. Also the HVPG remained unchanged （18 ± 2 mmHg vs 16 ± 2 mmHg） with individual changes ranging from -8 mmHg to ±2 mmHg. In seven patients, HVPG decreased and in three it increased. CONCLUSION： In spite of arterial blood pressure decreases 80 min after administration of the phosphodiesterase type-5 inhibitor sildenafil, the present study could not demonstrate any clinical relevant influence on splanichnic blood flow, oxygen consumption or the HVPG.
文摘Bile acid diarrhea results from excessive amounts of bile acids entering the colon due to hepatic overexcretion of bile acids or bile acid malabsorption in the terminal ileum. The main therapies include bile acid sequestrants, such as colestyramine and colesevelam, which may be given in combination with the opioid receptor agonist loperamide. Some patients are refractory to conventional treatments. We report the use of the farnesoid X receptor agonist obeticholic acid in a patient with refractory bile acid diarrhea and subsequent intestinal failure. A 32-year-old woman with quiescent colonic Crohn's disease and a normal terminal ileum had been diagnosed with severe bile acid malabsorption and complained of watery diarrhea and fatigue. The diarrhea resulted in hypokalemia and sodium depletion that made her dependent on twice weekly intravenous fluid and electrolyte infusions. Conventional therapies with colestyramine, colesevelam, and loperamide had no effect. Second-line antisecretory therapies with pantoprazole, liraglutide, and octreotide also failed. Third-line treatment with obeticholic acid reduced the number of stools from an average of 13 to an average of 7 per 24h and improved the patient's quality of life. The fluid and electrolyte balances normalized. The effect was sustained during follow-up for 6 mo with treatment at a daily dosage of 25 mg. The diarrhea worsened shortly after cessation of obeticholic acid. This case report supports the initial report that obeticholic acid may reduce bile acid production and improve symptoms in patients with bile acid diarrhea.
文摘This investigation examines experimentally the behavior of swirled jets produced by axial flow fans blowing into a crossflow at low velocity ratios. The main difference with non-swirl cases is an asymmetry of the dominant kidney vortex and a slight distortion of the jet trace downstream of the injection hole. The effect of jet rotation at relatively low swirl numbers and similar velocity ratios is also investigated by a validated computational analysis tool. The numerical results are analyzed by means of various post-processing procedures, aiming to clarify, quantify and analyze the impact of swirl on the characteristics and the flow domain of a jet in crossflow. In general, swirl introduces an asymmetry in all examined quantities and prevents the penetration of the jet into the crossflow, causing the jet to remain closer to the wall surface. The rotation of the injected fluid results in an imparity of the two parts of the Counter Rotating Vortex Pair （CVP） which is no longer symmetric to the axial centerline plane. High swirl numbers result in the destruction of the CVP and the dominant kidney shape vortex is transformed into a comma shape vortex, rotating close to the wall.
文摘This study examines experimentally the effect of stators on the performance and heat transfer characteristics of small axial cooling fans. A single fan impeller, followed by nine stator blades in the case of a complete stage, was used for all the experimental configurations. Performance measurements were carried out in a constant speed stage performance test rig while the transient liquid crystal technique was used for the heat transfer measurements. Full surface heat transfer coefficient distributions were obtained by recording the temperature history of liquid crystals on a target plate. The experimental data indicated that the results are highly affected by the flow conditions at the fan outlet. Stators can be beneficial in terms of pressure drop and efficiency, and thus more economical operation, as well as, in the local heat transfer distribution at the wake of the stator blades if the fan is installed very close to the cooling object. However, as the separation distance increases, enhanced heat transfer rate in the order of 25% is observed in the case of the fan impeller.
文摘In many engineering applications,heat transfer enhancement techniques are of vital importance in order to ensure reliable thermal designs of convective heat transfer applications.This study examines experimentally the heat transfer characteristics on the base plate around various surface mounted obstacles.Local convection coefficients are evaluated in the vicinity of each individual protruding body with great spatial resolution using the transient liquid crystal technique.Five different obstacles of constant height-to-hydraulic diameter ratio(~1.3) are considered.These include:a cylinder,a square,a triangle,a diamond and a vortex generator of delta wing shape design.The experiments were carried out over a range of freestream Reynolds numbers,based on the hydraulic diameter of each obstacle,varying from 4,000 to 13,000.The results indicate a negligible effect of the flow speed on the heat transfer topological structure and a considerable effect of the obstacle geometry on the level and distribution of heat transfer enhancement.
