BACKGROUND Autoimmune pancreatitis(AIP)is a chronic form of pancreatitis characterized by diffused enlargement of the pancreas and irregular stenosis of the main pancreatic duct.Some studies have reported that AIP can...BACKGROUND Autoimmune pancreatitis(AIP)is a chronic form of pancreatitis characterized by diffused enlargement of the pancreas and irregular stenosis of the main pancreatic duct.Some studies have reported that AIP can cause hemorrhage of gastric varices(GV)related to portal hypertension(PH).However,such cases are rare.In addition,the association of PH with AIP is unclear.At the same time,the efficacy and duration of glucocorticoid therapy is also controversial.CASE SUMMARY In this case,we reported a case of GV in pancreatic PH associated with AIP.Enhanced abdominal computed tomography(CT)suggested splenic vein(SV)and superior mesenteric vein(SMV)thromboses.The patient received a long-term glucocorticoid therapy,that the initial dose of 40 mg is reduced weekly by 5 mg,and then reduced to 5 mg for long-term maintenance.CT and gastroscopic examination after 8 mo of treatment indicated that SV and SMV were recanalized,pancreatic stiffness and swelling were ameliorated,and the GV almost completely disappeared.CONCLUSION Long-term glucocorticoid therapy can alleviate the development of GV in patients with AIP and has potential reversibility.展开更多
BACKGROUND: Non-cirrhotic portal hypertension is acommon cause of portal hypertension in developing coun-tries. To understand its etiopathogenesis we developed ananimal model by repeated portal endotoxemia inducedthro...BACKGROUND: Non-cirrhotic portal hypertension is acommon cause of portal hypertension in developing coun-tries. To understand its etiopathogenesis we developed ananimal model by repeated portal endotoxemia inducedthrough the gastrosplenic vein.METHODS: Twenty-nine rabbits (1.5-2.0 kg) were divid-ed into control (group n = 13) and experimental ( groupn = 16) groups. Heat killed E. coli were injected throughan indwelling cannula into the gastrosplenic vein in pre-sensitized animals. The animals were sacriflced at 1, 3 and6 months.RESULTS: The mean portal pressure in group animalswas significantly (P < 0. 05) higher than in group at 1(17.5 ±3.4 vs 10.4±2.2 mmHg), 3 (17.8±1.3 vs7.2 +3.6mmHg), and 6 (19.8±3.1 vs 10.3±4.8 mmHg) months.Similarly, the mean splenic weight in group was signifi-cantly greater than in group (P <0.05). Histopathologi-cally, the spleen showed medullary congestion, hemosid-rin-laden macrophages and mild fibrosis. Histologically,the liver had normal parenchyma with mild portal lympho-cytic infiltrates and kupffer cell hyperplasia. No significantanomalies were detected by liver function tests.CONCLUSIONS: The rabbit model showed significantsplenomegaly with a persistent increase in portal pressureand mild fibrosis without hepatic parenchymal injury, quiteakin to non-cirrhotic portal fibrosis as seen in humans. Re-current intra-abdominal infection may play an importantrole in the pathogenesis of non-cirrhotic portal fibrosis.展开更多
BACKGROUND Patients with liver cancer complicated by portal hypertension present complex challenges in treatment.AIM To evaluate the efficacy of radiofrequency ablation in combination with sorafenib for improving live...BACKGROUND Patients with liver cancer complicated by portal hypertension present complex challenges in treatment.AIM To evaluate the efficacy of radiofrequency ablation in combination with sorafenib for improving liver function and its impact on the prognosis of patients with this condition.METHODS Data from 100 patients with liver cancer complicated with portal hypertension from May 2014 to March 2019 were analyzed and divided into a study group(n=50)and a control group(n=50)according to the treatment regimen.The research group received radiofrequency ablation(RFA)in combination with sorafenib,and the control group only received RFA.The short-term efficacy of both the research and control groups was observed.Liver function and portal hypertension were compared before and after treatment.Alpha-fetoprotein(AFP),glypican-3(GPC-3),and AFP-L3 levels were compared between the two groups prior to and after treatment.The occurrence of adverse reactions in both groups was observed.The 3-year survival rate was compared between the two groups.Basic data were compared between the survival and non-surviving groups.To identify the independent risk factors for poor prognosis in patients with liver cancer complicated by portal hypertension,multivariate logistic regression analysis was employed.RESULTS When comparing the two groups,the research group's total effective rate(82.00%)was significantly greater than that of the control group(56.00%;P<0.05).Following treatment,alanine aminotransferase and aspartate aminotransferase levels increased,and portal vein pressure decreased in both groups.The degree of improvement for every index was substantially greater in the research group than in the control group(P<0.05).Following treatment,the AFP,GPC-3,and AFP-L3 levels in both groups decreased,with the research group having significantly lower levels than the control group(P<0.05).The incidence of diarrhea,rash,nausea and vomiting,and fatigue in the research group was significantly greater than that in the control group(P<0.05).The 1-,2-,and 3-year survival rates of the research group(94.00%,84.00%,and 72.00%,respectively)were significantly greater than those of the control group(80.00%,64.00%,and 40.00%,respectively;P<0.05).Significant differences were observed between the survival group and the non-surviving group in terms of Child-Pugh grade,history of hepatitis,number of tumors,tumor size,use of sorafenib,stage of liver cancer,histological differentiation,history of splenectomy and other basic data(P<0.05).Logistic regression analysis demonstrated that high Child-Pugh grade,tumor size(6–10 cm),history of hepatitis,no use of sorafenib,liver cancer stage IIIC,and previous splenectomy were independent risk factors for poor prognosis in patients with liver cancer complicated with portal hypertension(P<0.05).CONCLUSION Patients suffering from liver cancer complicated by portal hypertension benefit from the combination of RFA and sorafenib therapy because it effectively restores liver function and increases survival rates.The prognosis of patients suffering from liver cancer complicated by portal hypertension is strongly associated with factors such as high Child-Pugh grade,tumor size(6-10 cm),history of hepatitis,lack of sorafenib use,liver cancer at stage IIIC,and prior splenectomy.展开更多
BACKGROUND Acute upper gastrointestinal bleeding is a common medical emergency that has a 10%hospital mortality rate.According to the etiology,this disease can be divided into acute varicose veins and nonvaricose vein...BACKGROUND Acute upper gastrointestinal bleeding is a common medical emergency that has a 10%hospital mortality rate.According to the etiology,this disease can be divided into acute varicose veins and nonvaricose veins.Bleeding from esophageal varices is a life-threatening complication of portal hypertension.Portal hypertension is a clinical syndrome defined as a portal venous pressure that exceeds 10 mmHg.Cirrhosis is the most common cause of portal hypertension,and thrombosis of the portal system not associated with liver cirrhosis is the second most common cause of portal hypertension in the Western world.Primary myeloproliferative disorders are the main cause of portal venous thrombosis,and somatic mutations in the Janus kinase 2 gene(JAK2 V617F)can be found in approximately 90% of polycythemia vera,50% of essential thrombocyrosis and 50% of primary myelofibrosis.CASE SUMMARY We present a rare case of primary myelofibrosis with gastrointestinal bleeding as the primary manifestation that presented as portal-superior-splenic mesenteric vein thrombosis.Peripheral blood tests revealed the presence of the JAK2 V617F mutation.Bone marrow biopsy ultimately confirmed the diagnosis of myelofibrosis(MF-2 grade).CONCLUSION In patients with acute esophageal variceal bleeding due to portal hypertension and vein thrombosis without cirrhosis,the possibility of myeloproliferative neoplasms should be considered,and the JAK2 mutation test should be performed.展开更多
This editorial describes the milestones to optimize of transjugular intrahepatic portosystemic shunt(TIPS)technique,which have made it one of the main methods for the treatment of portal hypertension complications wor...This editorial describes the milestones to optimize of transjugular intrahepatic portosystemic shunt(TIPS)technique,which have made it one of the main methods for the treatment of portal hypertension complications worldwide.Innovative ideas,subsequent experimental studies and preliminary experience of use in cirrhotic patients contributed to the introduction of TIPS into clinical practice.At the moment,the main achievement in optimize of TIPS technique is progress in the qualitative characteristics of stents.The transition from bare metal stents to extended polytetrafluoroethylene–covered stent grafts made it possible to significantly prevent shunt dysfunction.However,the question of its preferred diameter,which contributes to an optimal reduction of portal pressure without the risk of developing post-TIPS hepatic encephalopathy,remains relevant.Currently,hepatic encephalopathy is one of the most common complications of TIPS,significantly affecting its effectiveness and prognosis.Careful selection of patients based on cognitive indicators,nutritional status,assessment of liver function,etc.,will reduce the incidence of post-TIPS hepatic encephalopathy and improve treatment results.Optimize of TIPS technique has significantly expanded the indications for its use and made it one of the main methods for the treatment of portal hypertension complications.At the same time,there are a number of limitations and unresolved issues that require further randomized controlled trials involving a large cohort of patients.展开更多
BACKGROUND: The abnormal metabolism caused by cir- rhosis always results in a complex problem about nutritional support, which will be more intricate while patients with portal hypertension are treated with pericardia...BACKGROUND: The abnormal metabolism caused by cir- rhosis always results in a complex problem about nutritional support, which will be more intricate while patients with portal hypertension are treated with pericardial devasculari- zation. Comparing the effects of early enteral and parente- ral nutritional support in patients with cirrhotic portal hy- pertension after pericardial devascularization, we try to rea lize the advantages and disadvantages of the two nutritional therapies and to guide our clinical practice. METHODS: After pericardial devascularization,40 patients with cirrhotic portal hypertension were divided randomly into 2 groups; enteral and parenteral nutritional support, respectively. The general nutritional condition, capability of producing protein, liver function, blood velocity of the portal vein, gut function, bowel bacterial translocation, mortality, complication rate, stay in ICU, duration of hos- pitalization and costs of treatment were determined in all the patients and compared between the 2 groups. RESULTS; Both enteral and parenteral nutritional supports could improve the general nutrition condition of the pa- tients; but patients receiving enteral nutritional support had fewer complications. Enteral nutrition was more ef- fective than parenteral nutrition in increasing the blood ve- locity of the portal vein, stimulating gut motion, preven- ting bowel bacterial translocation, shortening the stay in ICU and the duration of hospitalization, and saving costs of treatment. CONCLUSION: After pericardial devascularization, pa- tients with cirrhotic portal hypertension should be treated with enteral nutritional support as early as possible.展开更多
Mansonic schistosomiasis is the main cause of portal hypertension in Brazil. Hepatosplenic (HS) form is manifested by hepatomegaly mainly on the left hepatic lobe associated with large splenomegaly and bleeding due to...Mansonic schistosomiasis is the main cause of portal hypertension in Brazil. Hepatosplenic (HS) form is manifested by hepatomegaly mainly on the left hepatic lobe associated with large splenomegaly and bleeding due to esophageal varices with high mortality rates.展开更多
AIM: To explore portal hypertension and portosystemic shunts and to stage chronic liver disease (CLD) based on the pathophysiology of portal hemodynamics. METHODS: Per-rectal portal scintigraphy (PRPS) was perfo...AIM: To explore portal hypertension and portosystemic shunts and to stage chronic liver disease (CLD) based on the pathophysiology of portal hemodynamics. METHODS: Per-rectal portal scintigraphy (PRPS) was performed on 312 patients with CLD and liver angioscintigraphy (LAS) on 231 of them. The control group included 25 healthy subjects. We developed a new model of PRPS interpretation by introducing two new parameters, the liver transit time (LTT) and the circu-lation time between right heart and liver (RHLT). LTT for each lobe was used to evaluate the early portal hypertension. RHLT is useful in cirrhosis to detect liver areas missing portal inflow. We calculated the classical per-rectal portal shunt index (PRSI) at PRPS and the hepatic perfusion index (HPI) at LAS. RESULTS: The normal LTT value was 24 ± 1 s. Abnormal LTT had PPV = 100% for CLD. Twenty-seven noncirrhotic patients had LTT increased up to 35 s (median 27 s). RHLT (42 ± 1 s) was not related to liver disease. Cirrhosis could be excluded in all patients with PRSI 〈 5% (P 〈 0.01). PRSI 〉 30% had PPV = 100% for cirrhosis. Based on PRPS and LAS we propose the classification of CLD in 5 hemodynamic stages. Stage 0 is normal (LTT = 24 s, PRSI 〈 5%). In stage 1, LTT is increased, while PRSI remains normal. In stage 2, LTT is decreased between 16 s and 23 s, whereas PRSI is increased between 5% and 10%. In stage 3, PRSI is increased to 10%-30%, and LTT becomes undetectable by PRPS due to the portosystemic shunts. Stage 4 includes the patients with PRSI 〉 30%. RHLT and HPI were used to subtype stage 4. In our study stage 0 had NPV = 100% for CLD, stage 1 had PPV = 100% for non-cirrhotic CLD, stages 2 and 3 represented the transition from chronic hepatitis to cirrhosis, stage 4 had PPV = 100% for cirrhosis. CONCLUSION: LTT allows the detection of early portal hypertension and of opening of transhepatic shunts. PRSI is useful in CLD with extrahepatic portosystemic shunts. Our hemodynamic model stages the evolution of portal hypertension and portosystemic shunts. It may be of use in the selection of patients for interferon therapy.展开更多
AIM: To study the blood-brain barrier integrity in prehepatic portal hypertensive rats induced by partial portal vein ligation, at 14 and 40 dafer ligation when portal pressure is spontaneously normalized. METHODS: ...AIM: To study the blood-brain barrier integrity in prehepatic portal hypertensive rats induced by partial portal vein ligation, at 14 and 40 dafer ligation when portal pressure is spontaneously normalized. METHODS: Adult male Wistar rats were divided into four groups: Group Ⅰ: Sham14d, sham operated; Group Ⅱ: PHil, portal vein stenosis, (both groups were used 14 days after surgery); Group Ⅲ: Sham4od, Sham operated and Group Ⅳ: PH4od Portal vein stenosis (Groups Ⅱ and Ⅳ used 40 d afer surgery). Plasma ammonia, plasma and cerebrospinal fluid protein and liver enzymes concentrations were determined. Trypan and Evans blue dyes, systemically injected, were investigated in hippocampus to study blood-brain barrier integrity. Portal pressure was periodically recorded. RESULTS: Forty days afer stricture, portal pressure was normalized, plasma ammonia was moderately high, and both dyes were absent in central nervous system parenchyma. All other parameters were reestablished. When portal pressure was normalized and ammonia level was lowered, but not normal, the altered integrity of blood-brain barrier becomes reestablished. CONCLUSION: The impairment of blood-brain barrier and subsequent normalization could be a mechanism involved in hepatic encephalopathy reversibility. Hemodynamic changes and ammonia could trigger blood-brain barrier alterations and its reestablishment.展开更多
AIM: To develop a pharmacodynamic model of porta hypertension from chronic hepatitis. METHODS: Pathological changes and collagen depositions were analyzed using morphometry to confirm CCI4-induced chronic hepatitis....AIM: To develop a pharmacodynamic model of porta hypertension from chronic hepatitis. METHODS: Pathological changes and collagen depositions were analyzed using morphometry to confirm CCI4-induced chronic hepatitis. At do, d28, ds6 and d84 of the process, the portal perfused velocities (μL/min) in isolated rat livers were exactly controlled with a quanti-fied pump. The pressure (mmHg) was monitored with a Physiological System. The geometric concentrations of phenylephrine or acetylcholine were added to a fixed volume (300 mL) of the circulating perfusate. The equation, the median effective concentration and its 95% confidence intervals of phenylephrine or acetyl- choline were regressed with Prism-4 software in non-linear fit and various slopes. In the isolated perfused rat livers with chronic hepatitis, both median effective concentrations were defined as the pharmacodynamic model of portal hypertension.CONCLUSION: A pharmacodynamic model of portal hypertension in isolated perfused rat livers with chronic hepatitis was defined as the median effective concen- trations of phenylephrine and acetylcholine.展开更多
Regional portal hypertension is a rare cause of upper gastrointestinal bleeding.We reported an extremely rare case in which regional portal hypertension was associated with both the splenic arteriovenous fistula and c...Regional portal hypertension is a rare cause of upper gastrointestinal bleeding.We reported an extremely rare case in which regional portal hypertension was associated with both the splenic arteriovenous fistula and chronic pancreatitis.In June 2010,our patient,a 41-year-old man,was admitted to a local hospital due to a sudden melena and dizziness without haematemesis and jaundice.The splenic arteriovenous fistula in this patient was successfully occluded through transcatheter arterial embolization.At the 12-mo follow-up,our patient was in good condition.展开更多
Objective:To observe the protective effect of omeprazole on gastric mucosal of cirrhotic portal hypertension rats.Methods:All rats were randomly divided into normal control group,cirrhosis and treatment group.Thioacet...Objective:To observe the protective effect of omeprazole on gastric mucosal of cirrhotic portal hypertension rats.Methods:All rats were randomly divided into normal control group,cirrhosis and treatment group.Thioacetamide was used to establish rat model of cirrhotic portal hypertension.The necrotic tissue of gastric mucosa ulcer focus,degree of neutrophils infiltration at the ulcer margin,portal pressure,portal venous flow,abdominal aortic pressure,abdominal aortic blood flow at front end,gastric mucosal blood flow(GMBF),glycoprotein(GP)of gastric mucosa,basal acid secretion,H’ back-diffusion,gastric mucosal damage index,NO,prostaglandin E2(PGE2) and tumor necrosis factor-α(TNF-α) were determined respectively,and the pathological changes of gastric mucosa were also observed by microscope.Results:Compared with cirrhosis group and the control group,the ulcer bottom necrotic material,gastric neutrophil infiltration and UI of the treatment group were all decreased significantly(P【0.01),GMBF value,GP values,serum NO,PGE2,TNF- a were all significantly increased.Conclusions:Omeprazole has an important protective effect on gastric mucosal and it can increase gastric mucosal blood flow and related to many factors.展开更多
Non-cirrhotic portal hypertension is a poorly understood condition characterized by portal hypertension in the absence of conventional hepatic cirrhosis and described in association with blood coagulation disorders, m...Non-cirrhotic portal hypertension is a poorly understood condition characterized by portal hypertension in the absence of conventional hepatic cirrhosis and described in association with blood coagulation disorders, myeloproliferative and immunological diseases and with exposure to toxic drugs. Very recently, precise classification criteria have been proposed in order to define four distinct subcategories. The present case highlights how the clinical presentation, the confounding results from imaging studies, and the difficulties in the histological evaluation often render cases of non-cirrhotic portal hypertension a real diagnostic challenge. It also underscores the classification problems which can be faced once this diagnosis is performed. Indeed, the different subcategories proposed result from the prevalent subtypes in a spectrum of hepatic regenerative responses to a variety of injuries determining microcirculatory dis-turbances. More flexibility in classification should derive from this etiopathogenic background.展开更多
Purpose:To evaluate the feasibility and efficacy of a transmesenteric vein extrahepatic portosystemic shunt(TmEPS)for the treatment of cavernous transformation of the portal vein(CTPV).Materials and methods:The clinic...Purpose:To evaluate the feasibility and efficacy of a transmesenteric vein extrahepatic portosystemic shunt(TmEPS)for the treatment of cavernous transformation of the portal vein(CTPV).Materials and methods:The clinical data of 20 patients with CTPV who underwent TmEPS between December 2020and January 2022 at Henan Provincial People’s Hospital were retrospectively collected.The superior mesenteric vein(SMV)trunk was patent or partially occluded in these patients.An extrahepatic portosystemic shunt between the inferior vena cava and the SMV was established using a stent graft through an infraumbilical median longitudinal mini-laparotomy.The technical success,efficacy,and complication rates were evaluated,and the preand postoperative SMV pressures were compared.Patients’clinical outcomes and shunt patency were assessed.Results:TmEPS was successfully performed in 20 patients.The initial puncture success rate of the balloon-assisted puncture technique is 95%.The mean SMV pressure decreased from 29.1±2.9 mmHg to 15.6±3.3 mmHg(p<0.001).All symptoms of portal hypertension resolved.No fatal procedural complications occurred.During the follow-up period,hepatic encephalopathy occurred in two patients.The remaining patients remained asymptomatic.All shunts were patent.Conclusions:TmEPS is a feasible,safe,and effective treatment option for patients with CTPV.展开更多
BACKGROUND Sinusoidal obstruction syndrome has been reported after oxaliplatin-based chemotherapy,but liver fibrosis and non-cirrhotic portal hypertension(NCPH)are rarely reported.CASE SUMMARY Here,we describe the cas...BACKGROUND Sinusoidal obstruction syndrome has been reported after oxaliplatin-based chemotherapy,but liver fibrosis and non-cirrhotic portal hypertension(NCPH)are rarely reported.CASE SUMMARY Here,we describe the case of a 64-year-old woman who developed isolated gastric variceal bleeding 16 mo after completing eight cycles of oxaliplatin combined with capecitabine chemotherapy after colon cancer resection.Surprisingly,splenomegaly and thrombocytopenia were not accompanied by variceal bleeding,which has been reported to have predictive value for gastric variceal formation.However,a liver biopsy showed fibrosis in the portal area,suggesting NCPH.The patient underwent endoscopic treatment and experienced no further symptoms.CONCLUSION It is necessary to guard against long-term complications after oxaliplatin-based chemotherapy.Sometimes splenic size and platelet level may not always accurately predict the occurrence of portal hypertension.展开更多
BACKGROUND Sinistral portal hypertension associated with pancreatic pseudocysts is rare,often caused by extrinsic compression of splenic vein,the follow-up examinations by ultrasonography for early diagnosis are quiet...BACKGROUND Sinistral portal hypertension associated with pancreatic pseudocysts is rare,often caused by extrinsic compression of splenic vein,the follow-up examinations by ultrasonography for early diagnosis are quietly necessary since haematemesis,a life-threatening condition.Few studies have reported the ultrasonography findings of sinistral portal hypertension.CASE SUMMARY A 52-year-old man presented with acute abdominal pain after drinking,steatorrhea,weight loss and accidentally melena in the past 2 mo.He underwent ultrasound-guided fine needle aspiration in other hospital and diagnosed with pancreatic pseudocysts.Ultrasonography imaging,in our department,appeared as cystic heterogeneous hypoechoic area with the size of 4.7 cm×3.8 cm that located posterior to the body and tail of pancreas,adjacent to splenic vein associated with thrombosis resulted from compression.Spleen incrassated to approximately 7.3 cm,but no dilation of main portal vein was presented.Color Doppler Flow Imaging demonstrated the formation of splenic venous collateral,nevertheless no significantly flow signals was observed in splenic vein.Pulsed Doppler revealed that the peak velocity of splenic venous collateral was 18.4 cm/s with continuous waveform.Laparotomy confirmed sinistral portal hypertension associated with pancreatic pseudocysts,subsequently distal pancreatectomy combined with splenectomy and partial gastrectomy was performed.CONCLUSION It’s important clinically to know the ultrasound appearance of sinistral portal hypertension associated with pancreatic pseudocysts for sonographer and physician.展开更多
Expert consensus proposes an emergency treatment protocol for portal hypertension bleeding. Herein, the emergency treatment procedures, which include first aid, medical, interventional, and surgical treatments, are de...Expert consensus proposes an emergency treatment protocol for portal hypertension bleeding. Herein, the emergency treatment procedures, which include first aid, medical, interventional, and surgical treatments, are described. In addition, the indications, contraindications, operating norms, precautions, and prevention of complications of portal hypertension are described to optimize the first aid process.展开更多
BACKGROUND Abernethy malformation is a rare congenital vascular malformation with a portosystemic shunt that may clinically manifest as cholestasis,dyspnea,or hepatic encephalopathy,among other conditions.Early diagno...BACKGROUND Abernethy malformation is a rare congenital vascular malformation with a portosystemic shunt that may clinically manifest as cholestasis,dyspnea,or hepatic encephalopathy,among other conditions.Early diagnosis and classification are very important to further guide treatment.Typically,patients with congenital portosystemic shunts have no characteristics of portal hypertension.Herein,we report an 18-year-old female with prominent portal hypertension that manifested mainly as rupture and bleeding of esophageal varices.Imaging showed a thin main portal vein,no portal vein branches in the liver,and bleeding of the esophageal and gastric varices caused by the collateral circulation upwards from the proximal main portal vein.Patients with Abernethy malformation type I are usually treated with liver transplantation,and patients with type II are treated with shunt occlusion,surgery,or transcatheter coiling.Our patient was treated with endoscopic surgery combined with drug therapy and had no portal hypertension and good hepatic function for 24 mo of follow-up.CASE SUMMARY This case report describes our experience in the diagnosis and treatment of an 18-year-old female with Abernethy malformation type IIC and portal hypertension.This condition was initially diagnosed as cirrhosis combined with portal hypertension.The patient was ultimately diagnosed using liver histology and subsequent imaging,and the treatment was highly effective.To publish this case report,written informed consent was obtained from the patient,including the attached imaging data.CONCLUSION Abernethy malformation type IIC may develop portal hypertension,and traditional nonselective beta-blockers combined with endoscopic treatment can achieve high efficacy.展开更多
BACKGROUND Peripheral T-cell lymphoma(PTCL),an aggressive and rare disease that belongs to a heterogeneous group of mature T-cell lymphomas,develops rapidly and has a poor prognosis.Early detection and treatment are e...BACKGROUND Peripheral T-cell lymphoma(PTCL),an aggressive and rare disease that belongs to a heterogeneous group of mature T-cell lymphomas,develops rapidly and has a poor prognosis.Early detection and treatment are essential to improve patient cure and survival rates.Here,we report a rare case of PTCL with clinical presentation of noncirrhotic portal hypertension,which provides a basis for early vigilance of lymphomas in the future.CASE SUMMARY A 65-year-old Chinese woman was admitted to our hospital because of abdominal distension for 3 months and pitting oedema of both lower limbs for 2 months.Physical examinations and associated auxiliary examinations showed the presence of hepatosplenomegaly,and her hepatic venous pressure gradient was 10 mmHg.Immunohistochemical analysis of the liver biopsy confirmed the diagnosis of PTCL.The patient underwent combination therapy with dexamethasone,VP-16,and chidamide.Unfortunately,after 41 days of chemotherapy,the patient died of multiple organ failure.CONCLUSION PCTL accompanied by noncirrhotic portal hypertension is rarely reported.This case report discusses the diagnosis of a patient according to the literature.展开更多
BACKGROUND Hypersplenism associated with cirrhotic portal hypertension is a common condition often resulting from hepatitis B-related cirrhosis.However,the levels of immunoglobulin(Ig)and complement in patients with h...BACKGROUND Hypersplenism associated with cirrhotic portal hypertension is a common condition often resulting from hepatitis B-related cirrhosis.However,the levels of immunoglobulin(Ig)and complement in patients with hypersplenism associated with cirrhotic portal hypertension remain unclear.This study was undertaken to determine the levels of Ig and complement in these patients,the relationship between these levels and Child-Pugh class and their clinical significance.AIM To investigate the antibody(Ig)and complement levels in patients with hypersplenism associated with cirrhotic portal hypertension and their clinical significance.METHODS Clinical data of 119 patients with hypersplenism associated with cirrhotic portal hypertension were statistically analyzed and compared with those of 128 control patients.RESULTS IgA and IgG levels in patients with hypersplenism were significantly higher than controls(P<0.001).There was no significant difference in IgM between the two groups(P=0.109).C3 and C4 levels in patients with hypersplenism were significantly lower than controls(P<0.001).As liver function decreased,IgA and IgG levels increased(P<0.001),and C3 and C4 levels decreased(P<0.001).CONCLUSION Patients with hypersplenism associated with cirrhotic portal hypertension have significantly higher antibody(IgA and IgG)levels and significantly lower complement(C3 and C4)levels,which are both related to liver damage.Clinically,the administration of anti-hepatitis virus agents and protection of liver function should be strengthened.展开更多
基金Supported by Sichuan Science and Technology Program,China,No.MZGC20230031.
文摘BACKGROUND Autoimmune pancreatitis(AIP)is a chronic form of pancreatitis characterized by diffused enlargement of the pancreas and irregular stenosis of the main pancreatic duct.Some studies have reported that AIP can cause hemorrhage of gastric varices(GV)related to portal hypertension(PH).However,such cases are rare.In addition,the association of PH with AIP is unclear.At the same time,the efficacy and duration of glucocorticoid therapy is also controversial.CASE SUMMARY In this case,we reported a case of GV in pancreatic PH associated with AIP.Enhanced abdominal computed tomography(CT)suggested splenic vein(SV)and superior mesenteric vein(SMV)thromboses.The patient received a long-term glucocorticoid therapy,that the initial dose of 40 mg is reduced weekly by 5 mg,and then reduced to 5 mg for long-term maintenance.CT and gastroscopic examination after 8 mo of treatment indicated that SV and SMV were recanalized,pancreatic stiffness and swelling were ameliorated,and the GV almost completely disappeared.CONCLUSION Long-term glucocorticoid therapy can alleviate the development of GV in patients with AIP and has potential reversibility.
基金This study was financially supported by the Department of Science & Tech-nology, New Delhi, India.
文摘BACKGROUND: Non-cirrhotic portal hypertension is acommon cause of portal hypertension in developing coun-tries. To understand its etiopathogenesis we developed ananimal model by repeated portal endotoxemia inducedthrough the gastrosplenic vein.METHODS: Twenty-nine rabbits (1.5-2.0 kg) were divid-ed into control (group n = 13) and experimental ( groupn = 16) groups. Heat killed E. coli were injected throughan indwelling cannula into the gastrosplenic vein in pre-sensitized animals. The animals were sacriflced at 1, 3 and6 months.RESULTS: The mean portal pressure in group animalswas significantly (P < 0. 05) higher than in group at 1(17.5 ±3.4 vs 10.4±2.2 mmHg), 3 (17.8±1.3 vs7.2 +3.6mmHg), and 6 (19.8±3.1 vs 10.3±4.8 mmHg) months.Similarly, the mean splenic weight in group was signifi-cantly greater than in group (P <0.05). Histopathologi-cally, the spleen showed medullary congestion, hemosid-rin-laden macrophages and mild fibrosis. Histologically,the liver had normal parenchyma with mild portal lympho-cytic infiltrates and kupffer cell hyperplasia. No significantanomalies were detected by liver function tests.CONCLUSIONS: The rabbit model showed significantsplenomegaly with a persistent increase in portal pressureand mild fibrosis without hepatic parenchymal injury, quiteakin to non-cirrhotic portal fibrosis as seen in humans. Re-current intra-abdominal infection may play an importantrole in the pathogenesis of non-cirrhotic portal fibrosis.
文摘BACKGROUND Patients with liver cancer complicated by portal hypertension present complex challenges in treatment.AIM To evaluate the efficacy of radiofrequency ablation in combination with sorafenib for improving liver function and its impact on the prognosis of patients with this condition.METHODS Data from 100 patients with liver cancer complicated with portal hypertension from May 2014 to March 2019 were analyzed and divided into a study group(n=50)and a control group(n=50)according to the treatment regimen.The research group received radiofrequency ablation(RFA)in combination with sorafenib,and the control group only received RFA.The short-term efficacy of both the research and control groups was observed.Liver function and portal hypertension were compared before and after treatment.Alpha-fetoprotein(AFP),glypican-3(GPC-3),and AFP-L3 levels were compared between the two groups prior to and after treatment.The occurrence of adverse reactions in both groups was observed.The 3-year survival rate was compared between the two groups.Basic data were compared between the survival and non-surviving groups.To identify the independent risk factors for poor prognosis in patients with liver cancer complicated by portal hypertension,multivariate logistic regression analysis was employed.RESULTS When comparing the two groups,the research group's total effective rate(82.00%)was significantly greater than that of the control group(56.00%;P<0.05).Following treatment,alanine aminotransferase and aspartate aminotransferase levels increased,and portal vein pressure decreased in both groups.The degree of improvement for every index was substantially greater in the research group than in the control group(P<0.05).Following treatment,the AFP,GPC-3,and AFP-L3 levels in both groups decreased,with the research group having significantly lower levels than the control group(P<0.05).The incidence of diarrhea,rash,nausea and vomiting,and fatigue in the research group was significantly greater than that in the control group(P<0.05).The 1-,2-,and 3-year survival rates of the research group(94.00%,84.00%,and 72.00%,respectively)were significantly greater than those of the control group(80.00%,64.00%,and 40.00%,respectively;P<0.05).Significant differences were observed between the survival group and the non-surviving group in terms of Child-Pugh grade,history of hepatitis,number of tumors,tumor size,use of sorafenib,stage of liver cancer,histological differentiation,history of splenectomy and other basic data(P<0.05).Logistic regression analysis demonstrated that high Child-Pugh grade,tumor size(6–10 cm),history of hepatitis,no use of sorafenib,liver cancer stage IIIC,and previous splenectomy were independent risk factors for poor prognosis in patients with liver cancer complicated with portal hypertension(P<0.05).CONCLUSION Patients suffering from liver cancer complicated by portal hypertension benefit from the combination of RFA and sorafenib therapy because it effectively restores liver function and increases survival rates.The prognosis of patients suffering from liver cancer complicated by portal hypertension is strongly associated with factors such as high Child-Pugh grade,tumor size(6-10 cm),history of hepatitis,lack of sorafenib use,liver cancer at stage IIIC,and prior splenectomy.
文摘BACKGROUND Acute upper gastrointestinal bleeding is a common medical emergency that has a 10%hospital mortality rate.According to the etiology,this disease can be divided into acute varicose veins and nonvaricose veins.Bleeding from esophageal varices is a life-threatening complication of portal hypertension.Portal hypertension is a clinical syndrome defined as a portal venous pressure that exceeds 10 mmHg.Cirrhosis is the most common cause of portal hypertension,and thrombosis of the portal system not associated with liver cirrhosis is the second most common cause of portal hypertension in the Western world.Primary myeloproliferative disorders are the main cause of portal venous thrombosis,and somatic mutations in the Janus kinase 2 gene(JAK2 V617F)can be found in approximately 90% of polycythemia vera,50% of essential thrombocyrosis and 50% of primary myelofibrosis.CASE SUMMARY We present a rare case of primary myelofibrosis with gastrointestinal bleeding as the primary manifestation that presented as portal-superior-splenic mesenteric vein thrombosis.Peripheral blood tests revealed the presence of the JAK2 V617F mutation.Bone marrow biopsy ultimately confirmed the diagnosis of myelofibrosis(MF-2 grade).CONCLUSION In patients with acute esophageal variceal bleeding due to portal hypertension and vein thrombosis without cirrhosis,the possibility of myeloproliferative neoplasms should be considered,and the JAK2 mutation test should be performed.
文摘This editorial describes the milestones to optimize of transjugular intrahepatic portosystemic shunt(TIPS)technique,which have made it one of the main methods for the treatment of portal hypertension complications worldwide.Innovative ideas,subsequent experimental studies and preliminary experience of use in cirrhotic patients contributed to the introduction of TIPS into clinical practice.At the moment,the main achievement in optimize of TIPS technique is progress in the qualitative characteristics of stents.The transition from bare metal stents to extended polytetrafluoroethylene–covered stent grafts made it possible to significantly prevent shunt dysfunction.However,the question of its preferred diameter,which contributes to an optimal reduction of portal pressure without the risk of developing post-TIPS hepatic encephalopathy,remains relevant.Currently,hepatic encephalopathy is one of the most common complications of TIPS,significantly affecting its effectiveness and prognosis.Careful selection of patients based on cognitive indicators,nutritional status,assessment of liver function,etc.,will reduce the incidence of post-TIPS hepatic encephalopathy and improve treatment results.Optimize of TIPS technique has significantly expanded the indications for its use and made it one of the main methods for the treatment of portal hypertension complications.At the same time,there are a number of limitations and unresolved issues that require further randomized controlled trials involving a large cohort of patients.
文摘BACKGROUND: The abnormal metabolism caused by cir- rhosis always results in a complex problem about nutritional support, which will be more intricate while patients with portal hypertension are treated with pericardial devasculari- zation. Comparing the effects of early enteral and parente- ral nutritional support in patients with cirrhotic portal hy- pertension after pericardial devascularization, we try to rea lize the advantages and disadvantages of the two nutritional therapies and to guide our clinical practice. METHODS: After pericardial devascularization,40 patients with cirrhotic portal hypertension were divided randomly into 2 groups; enteral and parenteral nutritional support, respectively. The general nutritional condition, capability of producing protein, liver function, blood velocity of the portal vein, gut function, bowel bacterial translocation, mortality, complication rate, stay in ICU, duration of hos- pitalization and costs of treatment were determined in all the patients and compared between the 2 groups. RESULTS; Both enteral and parenteral nutritional supports could improve the general nutrition condition of the pa- tients; but patients receiving enteral nutritional support had fewer complications. Enteral nutrition was more ef- fective than parenteral nutrition in increasing the blood ve- locity of the portal vein, stimulating gut motion, preven- ting bowel bacterial translocation, shortening the stay in ICU and the duration of hospitalization, and saving costs of treatment. CONCLUSION: After pericardial devascularization, pa- tients with cirrhotic portal hypertension should be treated with enteral nutritional support as early as possible.
文摘Mansonic schistosomiasis is the main cause of portal hypertension in Brazil. Hepatosplenic (HS) form is manifested by hepatomegaly mainly on the left hepatic lobe associated with large splenomegaly and bleeding due to esophageal varices with high mortality rates.
文摘AIM: To explore portal hypertension and portosystemic shunts and to stage chronic liver disease (CLD) based on the pathophysiology of portal hemodynamics. METHODS: Per-rectal portal scintigraphy (PRPS) was performed on 312 patients with CLD and liver angioscintigraphy (LAS) on 231 of them. The control group included 25 healthy subjects. We developed a new model of PRPS interpretation by introducing two new parameters, the liver transit time (LTT) and the circu-lation time between right heart and liver (RHLT). LTT for each lobe was used to evaluate the early portal hypertension. RHLT is useful in cirrhosis to detect liver areas missing portal inflow. We calculated the classical per-rectal portal shunt index (PRSI) at PRPS and the hepatic perfusion index (HPI) at LAS. RESULTS: The normal LTT value was 24 ± 1 s. Abnormal LTT had PPV = 100% for CLD. Twenty-seven noncirrhotic patients had LTT increased up to 35 s (median 27 s). RHLT (42 ± 1 s) was not related to liver disease. Cirrhosis could be excluded in all patients with PRSI 〈 5% (P 〈 0.01). PRSI 〉 30% had PPV = 100% for cirrhosis. Based on PRPS and LAS we propose the classification of CLD in 5 hemodynamic stages. Stage 0 is normal (LTT = 24 s, PRSI 〈 5%). In stage 1, LTT is increased, while PRSI remains normal. In stage 2, LTT is decreased between 16 s and 23 s, whereas PRSI is increased between 5% and 10%. In stage 3, PRSI is increased to 10%-30%, and LTT becomes undetectable by PRPS due to the portosystemic shunts. Stage 4 includes the patients with PRSI 〉 30%. RHLT and HPI were used to subtype stage 4. In our study stage 0 had NPV = 100% for CLD, stage 1 had PPV = 100% for non-cirrhotic CLD, stages 2 and 3 represented the transition from chronic hepatitis to cirrhosis, stage 4 had PPV = 100% for cirrhosis. CONCLUSION: LTT allows the detection of early portal hypertension and of opening of transhepatic shunts. PRSI is useful in CLD with extrahepatic portosystemic shunts. Our hemodynamic model stages the evolution of portal hypertension and portosystemic shunts. It may be of use in the selection of patients for interferon therapy.
基金Supported by Grant TB 56 from the University of Buenos Aires, Buenos Aires, Argentina
文摘AIM: To study the blood-brain barrier integrity in prehepatic portal hypertensive rats induced by partial portal vein ligation, at 14 and 40 dafer ligation when portal pressure is spontaneously normalized. METHODS: Adult male Wistar rats were divided into four groups: Group Ⅰ: Sham14d, sham operated; Group Ⅱ: PHil, portal vein stenosis, (both groups were used 14 days after surgery); Group Ⅲ: Sham4od, Sham operated and Group Ⅳ: PH4od Portal vein stenosis (Groups Ⅱ and Ⅳ used 40 d afer surgery). Plasma ammonia, plasma and cerebrospinal fluid protein and liver enzymes concentrations were determined. Trypan and Evans blue dyes, systemically injected, were investigated in hippocampus to study blood-brain barrier integrity. Portal pressure was periodically recorded. RESULTS: Forty days afer stricture, portal pressure was normalized, plasma ammonia was moderately high, and both dyes were absent in central nervous system parenchyma. All other parameters were reestablished. When portal pressure was normalized and ammonia level was lowered, but not normal, the altered integrity of blood-brain barrier becomes reestablished. CONCLUSION: The impairment of blood-brain barrier and subsequent normalization could be a mechanism involved in hepatic encephalopathy reversibility. Hemodynamic changes and ammonia could trigger blood-brain barrier alterations and its reestablishment.
基金Supported by The Major State Creative New Drug Project,No.2009ZX09502-017Education Ministry Science Foundation ofChina,No. 108019
文摘AIM: To develop a pharmacodynamic model of porta hypertension from chronic hepatitis. METHODS: Pathological changes and collagen depositions were analyzed using morphometry to confirm CCI4-induced chronic hepatitis. At do, d28, ds6 and d84 of the process, the portal perfused velocities (μL/min) in isolated rat livers were exactly controlled with a quanti-fied pump. The pressure (mmHg) was monitored with a Physiological System. The geometric concentrations of phenylephrine or acetylcholine were added to a fixed volume (300 mL) of the circulating perfusate. The equation, the median effective concentration and its 95% confidence intervals of phenylephrine or acetyl- choline were regressed with Prism-4 software in non-linear fit and various slopes. In the isolated perfused rat livers with chronic hepatitis, both median effective concentrations were defined as the pharmacodynamic model of portal hypertension.CONCLUSION: A pharmacodynamic model of portal hypertension in isolated perfused rat livers with chronic hepatitis was defined as the median effective concen- trations of phenylephrine and acetylcholine.
基金Supported by The National Natural Science Foundation of China,No.81171444
文摘Regional portal hypertension is a rare cause of upper gastrointestinal bleeding.We reported an extremely rare case in which regional portal hypertension was associated with both the splenic arteriovenous fistula and chronic pancreatitis.In June 2010,our patient,a 41-year-old man,was admitted to a local hospital due to a sudden melena and dizziness without haematemesis and jaundice.The splenic arteriovenous fistula in this patient was successfully occluded through transcatheter arterial embolization.At the 12-mo follow-up,our patient was in good condition.
基金supported by Medical Scientific Research of Health Department of Hebei Province(No 02199)
文摘Objective:To observe the protective effect of omeprazole on gastric mucosal of cirrhotic portal hypertension rats.Methods:All rats were randomly divided into normal control group,cirrhosis and treatment group.Thioacetamide was used to establish rat model of cirrhotic portal hypertension.The necrotic tissue of gastric mucosa ulcer focus,degree of neutrophils infiltration at the ulcer margin,portal pressure,portal venous flow,abdominal aortic pressure,abdominal aortic blood flow at front end,gastric mucosal blood flow(GMBF),glycoprotein(GP)of gastric mucosa,basal acid secretion,H’ back-diffusion,gastric mucosal damage index,NO,prostaglandin E2(PGE2) and tumor necrosis factor-α(TNF-α) were determined respectively,and the pathological changes of gastric mucosa were also observed by microscope.Results:Compared with cirrhosis group and the control group,the ulcer bottom necrotic material,gastric neutrophil infiltration and UI of the treatment group were all decreased significantly(P【0.01),GMBF value,GP values,serum NO,PGE2,TNF- a were all significantly increased.Conclusions:Omeprazole has an important protective effect on gastric mucosal and it can increase gastric mucosal blood flow and related to many factors.
文摘Non-cirrhotic portal hypertension is a poorly understood condition characterized by portal hypertension in the absence of conventional hepatic cirrhosis and described in association with blood coagulation disorders, myeloproliferative and immunological diseases and with exposure to toxic drugs. Very recently, precise classification criteria have been proposed in order to define four distinct subcategories. The present case highlights how the clinical presentation, the confounding results from imaging studies, and the difficulties in the histological evaluation often render cases of non-cirrhotic portal hypertension a real diagnostic challenge. It also underscores the classification problems which can be faced once this diagnosis is performed. Indeed, the different subcategories proposed result from the prevalent subtypes in a spectrum of hepatic regenerative responses to a variety of injuries determining microcirculatory dis-turbances. More flexibility in classification should derive from this etiopathogenic background.
基金supported by the Henan medical science and technology research projects(222102310014)
文摘Purpose:To evaluate the feasibility and efficacy of a transmesenteric vein extrahepatic portosystemic shunt(TmEPS)for the treatment of cavernous transformation of the portal vein(CTPV).Materials and methods:The clinical data of 20 patients with CTPV who underwent TmEPS between December 2020and January 2022 at Henan Provincial People’s Hospital were retrospectively collected.The superior mesenteric vein(SMV)trunk was patent or partially occluded in these patients.An extrahepatic portosystemic shunt between the inferior vena cava and the SMV was established using a stent graft through an infraumbilical median longitudinal mini-laparotomy.The technical success,efficacy,and complication rates were evaluated,and the preand postoperative SMV pressures were compared.Patients’clinical outcomes and shunt patency were assessed.Results:TmEPS was successfully performed in 20 patients.The initial puncture success rate of the balloon-assisted puncture technique is 95%.The mean SMV pressure decreased from 29.1±2.9 mmHg to 15.6±3.3 mmHg(p<0.001).All symptoms of portal hypertension resolved.No fatal procedural complications occurred.During the follow-up period,hepatic encephalopathy occurred in two patients.The remaining patients remained asymptomatic.All shunts were patent.Conclusions:TmEPS is a feasible,safe,and effective treatment option for patients with CTPV.
基金Supported by Tianjin Science and Technology Plan Project,No.19ZXDBSY00030。
文摘BACKGROUND Sinusoidal obstruction syndrome has been reported after oxaliplatin-based chemotherapy,but liver fibrosis and non-cirrhotic portal hypertension(NCPH)are rarely reported.CASE SUMMARY Here,we describe the case of a 64-year-old woman who developed isolated gastric variceal bleeding 16 mo after completing eight cycles of oxaliplatin combined with capecitabine chemotherapy after colon cancer resection.Surprisingly,splenomegaly and thrombocytopenia were not accompanied by variceal bleeding,which has been reported to have predictive value for gastric variceal formation.However,a liver biopsy showed fibrosis in the portal area,suggesting NCPH.The patient underwent endoscopic treatment and experienced no further symptoms.CONCLUSION It is necessary to guard against long-term complications after oxaliplatin-based chemotherapy.Sometimes splenic size and platelet level may not always accurately predict the occurrence of portal hypertension.
文摘BACKGROUND Sinistral portal hypertension associated with pancreatic pseudocysts is rare,often caused by extrinsic compression of splenic vein,the follow-up examinations by ultrasonography for early diagnosis are quietly necessary since haematemesis,a life-threatening condition.Few studies have reported the ultrasonography findings of sinistral portal hypertension.CASE SUMMARY A 52-year-old man presented with acute abdominal pain after drinking,steatorrhea,weight loss and accidentally melena in the past 2 mo.He underwent ultrasound-guided fine needle aspiration in other hospital and diagnosed with pancreatic pseudocysts.Ultrasonography imaging,in our department,appeared as cystic heterogeneous hypoechoic area with the size of 4.7 cm×3.8 cm that located posterior to the body and tail of pancreas,adjacent to splenic vein associated with thrombosis resulted from compression.Spleen incrassated to approximately 7.3 cm,but no dilation of main portal vein was presented.Color Doppler Flow Imaging demonstrated the formation of splenic venous collateral,nevertheless no significantly flow signals was observed in splenic vein.Pulsed Doppler revealed that the peak velocity of splenic venous collateral was 18.4 cm/s with continuous waveform.Laparotomy confirmed sinistral portal hypertension associated with pancreatic pseudocysts,subsequently distal pancreatectomy combined with splenectomy and partial gastrectomy was performed.CONCLUSION It’s important clinically to know the ultrasound appearance of sinistral portal hypertension associated with pancreatic pseudocysts for sonographer and physician.
基金supported by the Key Science and Technology Project of Hunan Province:Key Technology Collaborative Innovation Project of Improving Emergency and Critical Care Ability and Public Health Emergency Treatment in Hunan Province (2020SK1010)。
文摘Expert consensus proposes an emergency treatment protocol for portal hypertension bleeding. Herein, the emergency treatment procedures, which include first aid, medical, interventional, and surgical treatments, are described. In addition, the indications, contraindications, operating norms, precautions, and prevention of complications of portal hypertension are described to optimize the first aid process.
文摘BACKGROUND Abernethy malformation is a rare congenital vascular malformation with a portosystemic shunt that may clinically manifest as cholestasis,dyspnea,or hepatic encephalopathy,among other conditions.Early diagnosis and classification are very important to further guide treatment.Typically,patients with congenital portosystemic shunts have no characteristics of portal hypertension.Herein,we report an 18-year-old female with prominent portal hypertension that manifested mainly as rupture and bleeding of esophageal varices.Imaging showed a thin main portal vein,no portal vein branches in the liver,and bleeding of the esophageal and gastric varices caused by the collateral circulation upwards from the proximal main portal vein.Patients with Abernethy malformation type I are usually treated with liver transplantation,and patients with type II are treated with shunt occlusion,surgery,or transcatheter coiling.Our patient was treated with endoscopic surgery combined with drug therapy and had no portal hypertension and good hepatic function for 24 mo of follow-up.CASE SUMMARY This case report describes our experience in the diagnosis and treatment of an 18-year-old female with Abernethy malformation type IIC and portal hypertension.This condition was initially diagnosed as cirrhosis combined with portal hypertension.The patient was ultimately diagnosed using liver histology and subsequent imaging,and the treatment was highly effective.To publish this case report,written informed consent was obtained from the patient,including the attached imaging data.CONCLUSION Abernethy malformation type IIC may develop portal hypertension,and traditional nonselective beta-blockers combined with endoscopic treatment can achieve high efficacy.
文摘BACKGROUND Peripheral T-cell lymphoma(PTCL),an aggressive and rare disease that belongs to a heterogeneous group of mature T-cell lymphomas,develops rapidly and has a poor prognosis.Early detection and treatment are essential to improve patient cure and survival rates.Here,we report a rare case of PTCL with clinical presentation of noncirrhotic portal hypertension,which provides a basis for early vigilance of lymphomas in the future.CASE SUMMARY A 65-year-old Chinese woman was admitted to our hospital because of abdominal distension for 3 months and pitting oedema of both lower limbs for 2 months.Physical examinations and associated auxiliary examinations showed the presence of hepatosplenomegaly,and her hepatic venous pressure gradient was 10 mmHg.Immunohistochemical analysis of the liver biopsy confirmed the diagnosis of PTCL.The patient underwent combination therapy with dexamethasone,VP-16,and chidamide.Unfortunately,after 41 days of chemotherapy,the patient died of multiple organ failure.CONCLUSION PCTL accompanied by noncirrhotic portal hypertension is rarely reported.This case report discusses the diagnosis of a patient according to the literature.
基金Supported by Hainan Provincial Department of Science and Technology,Qiongke[2020]256。
文摘BACKGROUND Hypersplenism associated with cirrhotic portal hypertension is a common condition often resulting from hepatitis B-related cirrhosis.However,the levels of immunoglobulin(Ig)and complement in patients with hypersplenism associated with cirrhotic portal hypertension remain unclear.This study was undertaken to determine the levels of Ig and complement in these patients,the relationship between these levels and Child-Pugh class and their clinical significance.AIM To investigate the antibody(Ig)and complement levels in patients with hypersplenism associated with cirrhotic portal hypertension and their clinical significance.METHODS Clinical data of 119 patients with hypersplenism associated with cirrhotic portal hypertension were statistically analyzed and compared with those of 128 control patients.RESULTS IgA and IgG levels in patients with hypersplenism were significantly higher than controls(P<0.001).There was no significant difference in IgM between the two groups(P=0.109).C3 and C4 levels in patients with hypersplenism were significantly lower than controls(P<0.001).As liver function decreased,IgA and IgG levels increased(P<0.001),and C3 and C4 levels decreased(P<0.001).CONCLUSION Patients with hypersplenism associated with cirrhotic portal hypertension have significantly higher antibody(IgA and IgG)levels and significantly lower complement(C3 and C4)levels,which are both related to liver damage.Clinically,the administration of anti-hepatitis virus agents and protection of liver function should be strengthened.