Clinical features of idiopathic non-cirrhotic portal hypertension: An analysis of 74 patients / 临床肝胆病杂志

Objective Idiopathic non-cirrhotic portal hypertension (INCPH) is a rare cause of portal hypertension, and this study aims to analyze the clinical features of patients with INCPH, and to assist in diagnosis and differential diagnosis. Methods A total of 74 patients who were hospitalized in Beijing YouAn Hospital from January 2019 to July 2022 and were diagnosed with INCPH were enrolled, and 332 patients with liver cirrhosis who were hospitalized during the same period of time were enrolled as control group. Demographic data, laboratory markers, gastroscopy, liver elasticity, pathological examination, and complications were recorded and compared between the two groups. The receiver operating characteristic (ROC) curve was used to investigate the ability of liver stiffness measurement (LSM), aspartate aminotransferase-to-platelet ratio index (APRI), and fibrosis-4 (FIB-4) in the differential diagnosis of INCPH, and the DeLong test was used to compare the area under the ROC curve (AUC). The independent-samples t test was used for comparison of normally distributed continuous data between two groups, and the Mann-Whitney U test was used for comparison of non-normally distributed continuous data between two groups; the chi-square test was used for comparison of categorical data between two groups. Results Among the patients with INCPH, 46.55% had no obvious symptoms at disease onset and 43.24% were misdiagnosed with liver cirrhosis. Compared with the patients with liver cirrhosis, the patients with INCPH had a significantly higher proportion of patients with gastrointestinal bleeding (62.16% vs 41.27%, χ 2 =10.67, P < 0.01) and a significantly lower proportion of patients with moderate-to-severe ascites (16.21% vs 29.82%, χ 2 =34.98, P < 0.01), and there were few patients with hepatic encephalopathy. As for pathology, 89.19% (66/74) of the INCPH patients manifested as typical occlusive portal vein disease. The statistical analysis showed that compared with the patients with liver cirrhosis, the patients with INCPH had significantly better liver function parameters, MELD score, and Child-Pugh score and significantly lower LSM [9.05(7.18-12.33) vs 25.32(16.21-47.23), Z =-8.41, P < 0.01], APRI score [0.70(0.41-1.28) vs 1.35(0.80-2.39), Z =-6.21, P < 0.01], and FIB-4 index [2.99(1.62-4.81) vs 6.68(4.06-10.42), Z =-8.39, P < 0.01]. LSM, FIB-4, and APRI had a good ability in differentiating INCPH from liver cirrhosis, and in particular, LSM had an AUC of up to 0.92 (95% confidence interval: 0.87-0.96), with a sensitivity of 92.68% and a specificity of 81.60%. Conclusion INCPH patients tend to have an insidious onset, a relatively high incidence rate of portal hypertension-related complications, and relatively good liver function, especially the patients with LSM < 14.5 kPa. The possibility of INCPH should be considered for such patients in clinical practice.

Clinical efficacy of transjugular intrahepatic portosystemic shunt for gastrointestinal hemorrhage in patients with idiopathic noncirrhotic portal hypertension / 中华内科杂志

Objective:To explore the medium-long term efficacy of transjugular intrahepatic portosystemic shunt (TIPS) for gastrointestinal hemorrhage in patients with idiopathic non-cirrhotic portal hypertension (INCPH).Methods:From March 2013 to July 2018, clinical data of 13 INCPH patients, including 5 males, 8 females,with gastrointestinal hemorrhage were retrospectively analyzed, who were diagnosed at the First Affiliated Hospital of Zhengzhou University, Anyang Fifth People′ s Hospital and Yuncheng Central Hospital. All patients received TIPS treatment. The general information, postoperative survival rate, the incidence of rebleeding, shunt dysfunction rate, and incidence of hepatic encephalopathy were analyzed.Results:All 13 patients with INCPH completed TIPS successfully with an average age of 45±8 (33 to 59) years. The hepatic venous pressure gradient (HVPG) decreased from 20.0-26.0 (22.6±1.9) mmHg before procedure to 8.0-14.0 (9.4±3.2) mmHg after. The median follow-up time was 44±7 (31 to 53) months. One patient died of liver failure 27 months after TIPS. Hepatic encephalopathy occurred cumulatively in 1 case (1/13), 1 case (1/13) and 1 case (1/13) in 12, 24 and 36 months after TIPS. Stent restenosis occurred cumulatively in 2 cases (2/13), 3 cases (3/13) and 3 cases (3/13) in 12, 24 and 36 months after TIPS. Portal vein thrombosis occurred cumulatively in 2 cases (2/13), and no primary liver cancer developed.Conclusions:TIPS is safe and effective in the treatment of INCPH with gastrointestinal bleeding with favorable medium-long term outcome.

Surgical Management of Portal Hypertension: Experience at a Medical College Hospital in South India

Introduction: Portal hypertension in the presence of cirrhosisof liver carries poor prognosis. The medical managementalong with endoscopic therapy helps to reduce bleeding.Surgery is reserved for patients who fail medical therapy.Patients with portal hypertension with good functioning liverbenefit from surgery. Study aimed to evaluate the resultsof surgical treatment for portal hypertension at our centerKarnataka Institute of Medical Sciences Hubli. Karnataka.Material and methods. This was a prospective observationalstudy. There were 34 patients undergoing surgical treatmentfor various presentations of portal hypertension during theperiod of 2015 to 2019.They were analyzed for demographics,etiology, presentation, various surgeries and outcome. Thedata was entered intoMicrosoft excel sheet and analyzed.Results: Of the 34 patients males were most common.Variceal bleeding was most common presentation followedby painful splenomegaly and anemia. ‘Extrahepatic portalvein obstruction’ was the leading cause of non-cirrhoticportal hypertension followed by ‘non cirrhotic portal fibrosis’and ‘left sided or sinistral portal hypertension’. Proximallinorenal shunt was the most common procedure followedby splenectomy with esophagogastric devascularization. Themorbidity and mortality were very low and yielded durablesatisfactory outcome.Conclusion: The surgery for non-cirrhotic portalhypertension has durable and satisfactory results and canbe done with minimal morbidity and mortality at trainedhands. For few selected cirrhotic patients surgery in the formof devascularization or shunt offers immediate relief frombleeding and gives time for future transplant if any.

Analysis of influencing factors on hepatic encephalopathy in patients with non-cirrhotic portal hypertension / 中华肝脏病杂志

Objective@#To investigate the incidence of hepatic encephalopathy (HE) in patients with non-cirrhotic portal hypertension (NCPH) and to explore its risk factors.@*Methods@#The incidence rate of HE in 150 cases with NCPH was evaluated in two hospitals, and 188 cases of compensated cirrhosis patients were taken as control. Logistic regression was used to screen for independent risk factors for HE in patients with NCPH.@*Results@#The incidence of overt hepatic encephalopathy (OHE) in patients with NCPH was not statistically significantly different from that in patients with cirrhosis (4.7% vs. 6.9%, P = 0.682). The incidence of mild hepatic encephalopathy (MHE) was significantly lower than that of cirrhosis patients (32.7% vs. 46.3%, P < 0.05). The presence of upper gastrointestinal bleeding, infection and portosystemic venous shunt were the main independent factors for HE in NCPH patients (OR > 1, P < 0.05).@*Conclusion@#HE is one of the important complications of NCP, and may be influenced by factors such as upper gastrointestinal bleeding, infection and portosystemic venous shunt.

Pathogeny and treatment of cirrhotic portal hypertension complicated by peripheral blood cytope-nia / 临床外科杂志

There are three causes of cirrhotic portal hypertension(CPH)complicated by peripher-al blood cytopenia:splenic factors(about 80%),non-splenic factors(about 4%)and comprehensive fac-tors(about 16%).The treatment includes non-surgical treatment and surgical treatment.Mild to moderate peripheral blood cell reduction is suitable for non-surgical treatment.Severe peripheral blood cytopenia is feasible to surgical treatment.Splenic factors are the main cause of peripheral cytopenia in CPH,but not all;The treatment method should be based on the degree of peripheral blood cytopenia.

Research advances in non-cirrhotic portal hypertension / 临床肝胆病杂志

Although liver cirrhosis is the most common cause of portal hypertension (PH), about 20% of PH cases are caused by non-cirrhotic reasons, which are referred to as non-cirrhotic portal hypertension (NCPH), with a high incidence rate in developing countries. NCPH is a group of heterogeneous hepatic vascular diseases, including idiopathic portal hypertension (IPH) and extrahepatic portal vein obstruction (EHPVO), as well as the rare diseases in clinical practice such as Budd-Chiari syndrome, congenital hepatic fibrosis, and nodular regenerative hyperplasia. The patients with NCPH usually have the symptoms of portal hypertension, such as recurrent variceal bleeding and splenomegaly, but liver function is well preserved in these patients. At present, the diagnosis of NCPH lacks a universally accepted standard and remains a challenge. In clinical practice, the method of exclusion is usually applied for the diagnosis of HCPH, and liver biopsy is performed when necessary to make a confirmed diagnosis. This paper introduces the pathogenesis and pathological manifestations of IPH and EHPVO, as well as the selection of diagnostic methods and therapeutic strategies. If upper gastrointestinal bleeding can be effectively controlled, NCPH is considered to have a relatively good prognosis.

Esclerosis hepatoportal como causa de hipertensión portal no cirrótica en un paciente colombiano infectado con VIH / Hepatoportal Sclerosis as a Cause of Portal Hypertension in a Colombian HIV patient without Cirrhosis

Antecedentes: la esclerosis hepatoportal se manifiesta como hipertensión portal no cirrótica. Su etiología parece estar relacionada con alteraciones idiopáticas en la microvasculatura hepática. Las manifestaciones de la esclerosis hepatoportal incluyen sangrado de vías digestivas altas, pancitopenia, esplenomegalia e hipertensión portal no cirrótica. Presentamos el primer caso reportado en Colombia de esclerosis hepatoportal en un paciente con serología positiva para el virus de la inmunodeficiencia humana (VIH). Métodos: paciente masculino de 60 años de edad, VIH-positivo, quien ingresa a nuestra institución por hemorragia de vías digestivas alta (várices esofágicas y fúndicas) y ascitis, cuyo manejo requirió la toma de biopsia hepática. Resultados: se realizó biopsia Trucut de hígado que evidenció la presencia de 6 a 8 espacios porta con parénquima arquitectónico conservado que demostró fibrosis perivenular y dilatación sinusoidal pericentral severa. Conclusión: la esclerosis hepatoportal es una causa de morbilidad en pacientes VIH-positivos. Debe considerarse en cada paciente que manifiesta hipertensión portal no cirrótica asociada con hemorragia de la vía digestiva alta. Sin embargo, una investigación adicional es imprescindible con el fin de describir la relación entre el desarrollo de alteraciones intrahepáticas (microtrombosis), la patogénesis del VIH y el uso de terapia antirretroviral, particularmente el uso de didanosina.

Background: Hepatoportal sclerosis manifests as non-cirrhotic portal hypertension. Its etiology appears to be related to alterations in the idiopathic micro-vasculature of the liver. Manifestations of hepatoportal sclerosis include upper gastrointestinal bleeding, pancytopenia, splenomegaly and non-cirrhotic portal hypertension. We present the first reported case of hepatoportal sclerosis in Colombia which occurred in an HIV positive patient. Methods: A 60-year-old male HIV patient positive was admitted to our institution because of ascites and upper digestive tract bleeding due to esophageal and fundal varices. Management required taking a liver biopsy. Results: A Tru-Cut biopsy needle was used to take a liver biopsy sample percutaneously. The biopsy revealed six to eight portal tracts with preserved architectural parenchyma, perivenular fibrosis and severe pericentral sinusoidal dilatation. Conclusions: Hepatoportal sclerosis is a cause of morbidity in HIV-positive patients and should be considered in each patient manifesting non-cirrhotic portal hypertension associated with upper gastrointestinal bleeding. However, further research is necessary to describe the relationship between the development of intrahepatic alterations (microthrombosis), HIV, and the use of anti-retroviral therapy, particularly the use of didanosine.

Thrombogenesis and its treatment in portal system after devascularization / 中国血吸虫病防治杂志

Objective To discuss the occurrence of thrombosis in portal system of patients with cirrhotic portal hypertension after devascularization and the methods for treatment and prevention. Methods The clinical data of 113 patients with cirrhotic portal hypertension after the devascularization were collected and analyzed retrospectively and the occurrence time parts as well as the treatment and prevention methods were discussed. Results Among the 113 patients 33 of them were found with thrombosis in their portal system and the occurrence rate was 29.2%. The occurrence time of thrombosis was 2?15 days post?de?vascularization and the median time was 6 days post?operation. Among the 33 cases with thrombosis there were 19 cases of splenic vein thrombosis 10 cases of portal thrombosis and 4 cases of both of them. After the thrombolytic therapy the thrombo?sises in 30 cases disappeared. Conclusions Most of the thrombosises in portal system happen in splenic vein post?devascular?ization. Avoiding clamping the trunk of splenic vein in the operation and taking thrombolytic therapy at the early stage after the operation can effectively prevent the occurrence of thrombosis.

Protective effect of omeprazole on gastric mucosal of cirrhotic portal hypertension rats

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-α 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.

Prognostic risk factors for surgery in patients with cirrhotic portal hypertension / 中华肝胆外科杂志

Objective To investigate the prognostic risk factors for surgery in patients with cirrhotic portal hypertension.Methods One hundred and sixty one patients with cirrhotic portal hypertension who received surgery in our hospital in the past 10 years were studied.The data were entered into a pre-designed form.24 predictors including patients′ age,sex,degree of liver atrophy,ChildPugh classification,coagulation profile,splenic size,renal function,blood pH,base excess (BE),operative time,volume of ascites,and intraoperative and postoperative hemorrhage were recorded and analyzed.For each of the predictors,2-3 subgroups were compared.Results Seven predictors were clearly related to surgical prognosis:postoperative bleeding within 30h (B0.356,P＜0.001) and a bleeding volume ＞2 L were awarded 3 points; liver volume (B-0.160,P＜0.001) and severe liver atrophy (antero-posterior diameter of the left liver lobe ≤55 mm,oblique diameter of the right lobe ≤110 mm) were awarded three points; blood pH (B0.141,P＜0.001),pH＜7.35 was awarded 2 points; BE (B-0.123,P＜0.001),BE＜-3 (mmol/L) was awarded 2 points; decrease in PLT (B0.065,P =0.015),PLT＜ 3 (T/L) was awarded 2 points; intraoperative bleeding (B0.062,P=0.014),bleeding volume ＞2 L was awarded 2 points; decrease in RBC (B0.053,P=0.024),＜3(G/L) was awarded 1 point.Of the 147 patients who recovered from surgery,all had ≤3 points,except one who had 4 points.Of the 14 patients who died,all had ≥5 points except one who had 4 points.Conclusions Postoperative bleeding (＞2 L),severe liver atrophy (antero-posterior diameter of the left live lobe ≤55 mm,oblique diameter of the right lobe ≤110 mm),blood PH＜7.35,BE ＜-3 (mmol/L),PLT＜30 000(T/L),intraoperative major bleeding (＞2 L) and RBC＜3 (G/L) were significant prognostic risk factors for surgery.For patients who had a score of 5-6 points; death was likely following surgery.A score ≥8 points should be considered as a contraindication to surgery.To reduce operative mortality,active treatment should be given before surgery to keep the score to be 4 points or less.

Diagnosis and treatment of on-cirrhotic portal hypertension / 中国实用内科杂志

Non-cirrhotic portal hypertension(NCPH)is a group of diseases that show evidences of portal hypertension but no cirrhosis is present.Common causes of NCPH include pre-sinusoidal portal lesions such as portal vein thrombosis,congenital liver fibrosis and idiopathic portal hypertension,and post-sinusoidal portal lesions.The major feature of this group of diseases is well preserved liver function in spite of prominent portal hypertensive manifestations such as esophageal varices/gastrointestinal bleeding and splenomegaly/hypersplenism.Careful differentiation from cirrhosis requires thorough clinical,radiological and pathological investigation.Preventing and control of variceal bleeding and hypersplenism through medical,endoscopic and interventional procedures yield good prognosis in most of the patients with NCPH.

Non-cirrhotic Portal Hypertension in Idiopathic Myelofibrosis: A case report

We report a case of non-cirrhotic portal hypertension in a 73 year-old woman, who had 19-year history of idiopathic myelofibrosis. There were esophageal varix, splenomegaly, and ascites. The biopsied liver showed irregular sinusoidal/ perisinusoidal fibrosis and occasional central-to-central fibrous connection. In areas with extensive fibrosis, coarse collagen fibers filled the sinusoidal spaces and compressed hepatocytes. However, nodular regeneration was absent. Double immunohistochemical stain for smooth muscle actin and proliferation cell nuclear antigen (PCNA) revealed diffusely activated stellate cells, some of which showed nuclear PCNA staining. There was also extramedullary hematopoiesis with bizarre megakaryocytes. The portal vein and its branches were patent. Idiopathic myelofibrosis is a rare cause of non-cirrhotic portal hypertension: the portal hypertension was considered to be the result of sinusoidal/perisinusoidal fibrosis in this case.