Research advances in porto-sinusoidal vascular disease / 临床肝胆病杂志

Porto-sinusoidal vascular disease （PSVD） is a new disease nomenclature proposed in recent years， which is an important supplement to idiopathic non-cirrhotic portal hypertension. PSVD includes the patients with specific pathological conditions， but without portal hypertension symptoms， and the patients with portal vein thrombosis or viral hepatitis. This article elaborates on the naming， epidemiology， etiology， clinical manifestations， prognosis， and treatment of PSVD， in order to improve the understanding of this disease among clinicians.

Clinical features of myeloproliferative neoplasms with portal hypertension as the main manifestation / 临床肝胆病杂志

Objective To investigate the clinical features, liver histological features, and diagnostic and treatment methods for patients with myeloproliferative neoplasms (MPN) with portal hypertension as the main manifestation. Methods A retrospective analysis was performed for related data of the patients who attended the hospital due to portal hypertension and were finally diagnosed with MPN in Liver Research Center, Beijing Friendship Hospital, from January 2019 to February 2022, including clinical manifestation, liver pathological features, treatment, and follow-up results. Results Nine patients were included in this study, and all the patients had splenomegaly and esophageal and gastric varices, while portal vein thrombosis was observed in eight patients. All patients had normal or slightly abnormal liver function and routine blood test results. Six patients underwent liver biopsy, without the formation of fibrous septum and pseudolobule, and hepatic extramedullary hematopoiesis was observed in two patients. All nine patients underwent bone marrow biopsy and genetic testing, among whom six had essential thrombocythemia and three had primary myelofibrosis, and genetic testing revealed JAK - 2V617F gene mutation in seven patients and CALR gene mutation in two patients. Conclusion MPN is one of the rare causes of portal hypertension and has the clinical manifestations of esophageal and gastric varices, splenomegaly, and even megalosplenia, without the manifestations of hypersplenism such as leukopenia and thrombocytopenia. Detection of the JAK - 2V617F and CALR genes can improve the diagnostic rate of MPN.

Etiological and non-etiological therapies for cirrhotic portal hypertension / 临床肝胆病杂志

Portal hypertension is a serious complication of liver cirrhosis resulting from the increases in portal vascular resistance and portal blood inflow. Both etiological and non-etiological therapies can effectively reduce portal venous pressure to a certain degree, but with an unsatisfactory effect in improving prognosis. New therapeutic drugs targeting the reduction in intrahepatic vascular resistance may help to achieve the reversal of portal hypertension. Based on the pathogenesis of cirrhotic portal hypertension, this article summarizes the current pharmacotherapies from the aspects of etiological and non-etiological therapies, so as to provide a comprehensive theoretical and evidence-based basis for clinical treatment options.

Clinical diagnosis, staging, and therapeutic principles of liver cirrhosis / 临床肝胆病杂志

With in-depth studies on the pathogenesis, pathophysiology, treatment, and prognosis of liver cirrhosis in recent years, there have been great changes in staging and treatment concepts among scholars in China and globally. Besides the traditional staging system of compensated and decompensated liver cirrhosis, liver cirrhosis can be divided into five stages based on ascites, variceal bleeding, and severe infection, which highlights the features of this disease in different disease stages and this provides potential targets and basis for treatment. At present, the comprehensive management of liver cirrhosis, including etiological treatment, treatment targeting key pathogenesis and major complications, nutritional support, exercise guidance, and lifestyle adjustment (smoking cessation, alcohol withdrawal, and improvement of oral hygiene), is the key to delaying disease progression and improving prognosis, and liver transplantation remains the most effective approach for end-stage liver cirrhosis.

Direct intrahepatic portosystemic shunt in treatment of Budd-Chiari syndrome / 中华肝胆外科杂志

Objective:To study the efficacy of direct intrahepatic portosystemic shunt (DIPS) in treatment of Budd-Chiari syndrome (BCS).Methods:From January 1, 2015 to June 31, 2017, consecutive patients with BCS who were treated with DIPS at the Department of Interventional Therapy of Beijing Shijitan Hospital, the Liver Disease Research Center of Beijing Friendship Hospital and the General Surgery Department of Beijing Ditan Hospital were retrospectively analyzed. The symptoms, physical signs (including abdominal distension, ascites, pleural effusion, splenomegaly, hepatic encephalopathy) and perioperative laboratory results of these patients were collected and analyzed. Biochemical indicators including alanine aminotransferase (ALT), aspartate aminotransferase (AST), total bilirubin (TBil), direct bilirubin (DBil), and portal pressure gradient were compared before and 2 weeks after treatment. The patients were followed up for at least 3 years to assess their clinical symptoms, patency of shunt, oncological status and survival.Results:Of 67 patients with BCS who were included in the study, there were 45 males and 22 females, aged (38.12±23.22) years. The BCS classification of these patients were hepatic vein type ( n=65), including 62 patients with complete hepatic vein obstruction, 3 patients with hepatic vein occlusion due to thrombosis, and 2 patients with mixed hepatic vein and inferior vena cava occlusion. All 67 patients underwent DIPS with 93 stents being implanted. In addition, 43 patients underwent gastric coronary vein embolization, and 2 patients with mixed type of BCS underwent inferior vena cava stenting. The portal pressure gradient decreased from (22.17±9.16) mmHg (1 mmHg=0.133 kPa) to (9.87±4.75) mmHg, the difference was statistically significant ( P<0.05). Abdominal distension was relieved, at one month and ascites completely subsided in 3 months after operation. The liver congestion and swelling were obviously relieved. Comparison of patients 2 weeks after operation and before operation, ALT decreased from (65.28±27.75) U/L to (28.43±13.46)U/L, AST from (68.75±29.23) U/L to (26.92±13.33)U/L, TBil from (175.31±80.48)μmol/L to (45.08±26.54)μmol/L, DBil from (127.55±44.65)μmol/L to (35.12±10.77)μmol/L, and albumin increased from (31.56±7.22) g/L to (44.18±11.36)g/L, the difference was statistically significant (all P<0.05). All patients were followed up for at least 3 years. Shunt stenosis was detected in 5 patients (7.46%) with shunt expansion being performed, variceal bleeding in 2 patients (2.99%), ascites recurrence in 4 patients (5.97%) and hepatic encephalopathy in 2 patients (2.99%). No patients were diagnosed with hepatic cancer, and no patients died. Conclusion:DIPS was efficacious, safe and reliable to that BCS patients. It rapidly reduced portal venous pressure, relieved liver congestion, and restored liver morphology and liver function in these patients.

Value of hepatic venous pressure gradient in predicting clinical end events in liver cirrhosis / 临床肝胆病杂志

Liver cirrhosis is the end stage of liver disease, and decompensated liver cirrhosis has the significant feature of portal hypertension. At present, hepatic venous pressure gradient (HVPG) remains the “gold standard” for evaluating portal hypertension and thus has great significance in clinical practice. This article elaborates on the value of HVPG in predicting end events in compensated and decompensated liver cirrhosis and the application of HVPG in evaluating the therapeutic effect of drugs in the treatment of portal hypertension, so as to provide a basis for early prediction, early prevention, and early intervention of portal hypertension in clinical practice.

Clinical efficacy of transjugular intrahepatic portosystemic shunting for recurrent portal hypertension after liver transplantation / 中华消化外科杂志

Objective To investigate the clinical efficacy of transjugular intrahepatie portosystemie shunting (TIPS) for recurrent portal hypertension after liver transplantation.Methods The retrospective crosssectional study was conducted.The clinical data of 15 patients with recurrent portal hypertension after liver transplantation who underwent TIPS in the 9th School of Clinical Medicine between January 2008 to June 2016 were collected.Course of TIPS:the portal vein pressure was measured and varicose veins were embolized after puncture,cannulation and angiography.A balloon catheter with diameter of 7 mm or 8 mm was used to dilate the preshunt channel,and a covered stent or bare stent with a diameter of 7,8 or 10 mm was implanted to establish the shunt channel.Portal vein angiography was performed and the portal vein pressure was measured again.Observation indicators:(1) Surgical situations;(2) changes of portal vein pressure before and after TIPS;(3)follow-up and survival situations.Follow-up using outpatient examination was performed to record clinical symptoms at postoperative 1,3,6 and 12 months.Regular hepatic vascular ultrasonography was done at postoperative 1,3,6 and 12 months to detect patency of shunt.The follow-up period was up to June 2018.Measurement data with normal distribution were represented as (x) ±s and analyzed by the paired t test.Measurement data with skewed distribution were described as M (range).Count data were represented as percentage.Results (1) Surgical situations:all the 15 patients underwent successful TIPS,without any serious complications or death.Stent implantation situation:bare stent,covered stent and bare stent + covered stent were implanted in 4,8 and 3 patients,respectively.Among the 15 patients,7 mm,8 mm and 10 mm diameter shunt channel were established in 4,8 and 3 patients respectively.(2) Changes of portal vein pressure before and after TIPS:portal vein pressure of the 15 patients decreased from (34±8)mmHg (1 mmHg=0.133 kPa) to (21±7)mmHg before and after TIPS,with a statistically significant difference (t =7.07,P＜0.05).Portal vein pressure gradient decreased from (26± 9)mmHg to (12±5)mmHg before and after TIPS,with a statistically significant difference (t=6.43,P＜0.05).(3) Follow-up and survival situations:15 patients were followed up for 24.0-60.0 months,with a median follow-up time of 37.8 months.Main clinical symptoms:of 12 patients with gastrointestinal hemorrhage,3 had gastrointestinal rehemorrhage mainly due to portal vein pressure rising again caused by shunt restenosis or occlusion,9 had no gastrointestinal rehemorrhage.Of 5 patients with portal vein thrombosis,thrombus was disappeared basically in 3 patients and decreased obviously (no effect on blood flow) in 2 patients.Three patients with refractory ascites were effectively improved after TIPS,however,2 of them were recurred at postoperative 5 months.Postoperative restenosis or occlusion of shunt channel:among 15 patients,7 developed restenosis or occlusion of the shunt channel (including 4 with bare stents).Five of them underwent shunt recanalization and another 2 without special clinical symptoms had no treatment.Hepatic encephalopathy:6 of 15 patients including 1 with 7 nun shunt,3 with 8 mm shunt and 2 with 10 mm shunt developed hepatic encephalopathy,of which grade Ⅰ,Ⅱ,Ⅲ,and Ⅳ hepatic encephalopathy wee detected in 2,3,0 and 1 patients,respectively.Survival situations:of the 15 patients,1 died of hepatic failure at postoperative 6 months,3 were performed liver transplantation again at postoperative 3,8 and 14 months,respectively,11 survived more than 2 years with the longest survival time more than 6 years.Conclusion TIPS is safe and effective for recurrent portal hypertension after liver transplantation for patients who have not effective other treatment.