Clinical characteristics and management of patient with portal hypertension at tertial level hospital in Tanzania.

BACKGROUND: Portal hypertension is a common diagnosis in Sub-Saharan African countries, with the majority of patients presenting late. This study aimed to understand Clinical characteristics, aetiology, the treatment offered in our setting, and factors associated with portal hypertension at a tertiary-level hospital, in Tanzania. METHODOLOGY: A prospective cross-sectional observational single hospital-based study was conducted at MNH, from May 2021 to April 2022. A minimum of 152 subjects were required with an error of less than 5% and a study power of 80% at a 95% confidence interval. A structured questionnaire was used to collect data. Ethical clearance was obtained from the MUHAS/MNH IRB. RESULTS: A total of 154 eligible participants consented and participated in this study. The mean age of participants was 42 ± 15.8 years (range 2-87). Most of the study participants were males 64.9% with a male-to-female (M: F) ratio of 1.8:1. Vomiting blood was the common symptom among the study participants 51.3%. Schistosomiasis 53.9% and viral infection 26.6% were the common etiologies followed by alcohol abuse 7.8%. Most were medically treated at 89.61% followed by radiological treatment at 8.44% while only 1.95% of patients received surgical treatment. There was a significant association between the grade of oesophagal varices and bleeding consequences (p-value < 0.01). CONCLUSION: The majority of patients were medically treated while patients who require surgical care are unable to assess it. We recommend the establishment of a transplant services program to counteract the unmet need and more retrospective research toward policy establishment.

Histopathological features of idiopathic portal hypertension: A systematic review and meta-analysis.

BACKGROUND: Portal hypertension (PH) is a clinically significant entity that could present with life-threatening gastrointestinal bleeding. Cirrhosis is the most common cause of PH, with well-documented histopathology and etiology. However, in idiopathic portal hypertension (IPH), no single histopathologic finding is associated with PH. Our systematic review aims to identify and summarize the prevalence of the common histological findings of IPH. METHODS: We systematically searched PubMed, Cochrane CENTRAL, Web of Science, and Scopus till 1ST March 2022 for studies describing the histopathological features of IPH. Data were extracted from eligible studies and pooled as events rate and 95% confidence interval (CI) using binary random-effects model by open meta-analyst software. RESULTS: We included 23 retrospective studies with a total sample size of 813 patients. The overall incidence of nodular regenerative hyperplasia was 38.6%, 59.8% for portal fibrosis, 51.3% for periportal fibrosis, 39.3% for perisinusoidal fibrosis, 89.8% for portal vein sclerosis, 42.2% for portal inflammation, 53.3% for mega-sinusoids, 39.5% for thickening of portal vein branches, 93.8% for narrowing of portal veins, 53.3% for hepatic veins/venous outflow obstruction, 51.4% for aberrant portal/periportal vessels, 42.4% for shunt vessel, 50.9% for ductular proliferation, and 16.3% for steatosis. CONCLUSION: Due to the relatively non-pathognomonic and non-specific nature of IPH, a combination of different histological features such as the portal and periportal fibrosis, portal vein sclerosis, mega-sinusoids, narrowing of portal veins, hepatic venous outflow obstruction, aberrant portal or periportal vessels, and ductular proliferation may be of value in diagnosing IPH as the incidence rate of these features was at approximately 50%.

Porto-sinusoidal Vascular Disease and Portal Hypertension.

Porto-sinusoidal vascular disease (PSVD) is the medical diagnosis for a patient who has portal hypertension in the absence of cirrhosis on liver biopsy. There are several specific histologic findings for PSVD, including obliterative portal venopathy, nodular regenerative hyperplasia, and incomplete septal fibrosis. Epidemiologic reports vary widely among regions; PSVD comprises less than 10% of causes of portal hypertension in Western countries but incidence has been found to be as high as 48% in India. There is an expansive list of etiologies that have been reported to cause PSVD.

Evaluating clinical outcomes and prognosis in patients with cirrhosis and portal hypertension: a retrospective observational cohort study.

OBJECTIVE: Cirrhosis describes the end-stage of chronic liver disease. Irreversible changes in the liver cause portal hypertension, which can progress to serious complications and death. Only a few studies with small sample sizes have investigated the prognosis of cirrhosis with portal hypertension. We used electronic healthcare records to examine liver-related outcomes in patients with diagnosed/suspected portal hypertension. DESIGN: This retrospective observational cohort study used secondary health data between 1 January 2017 and 3 December 2020 from the TriNetX Network, a federated electronic healthcare records platform. Three patient groups with cirrhosis and diagnosed/suspected portal hypertension were identified ('most severe', 'moderate severity' and 'least severe'). Outcomes studied individually and as a composite were variceal haemorrhage, hepatic encephalopathy, complications of ascites and recorded mortality up to 24 months. RESULTS: There were 13 444, 23 299, and 23 836 patients in the most severe, moderate severity and least severe groups, respectively. Mean age was similar across groups; most participants were white. The most common individual outcomes at 24 months were variceal haemorrhage in the most severe group, recorded mortality and hepatic encephalopathy in the moderate severity group, and recorded mortality in the least severe group. Recorded mortality rate was similar across groups. For the composite outcome, cumulative incidence was 59% in the most severe group at 6 months. Alcohol-associated liver disease and metabolic-associated steatohepatitis were significantly associated with the composite outcome across groups. CONCLUSION: Our analysis of a large dataset from electronic healthcare records illustrates the poor prognosis of patients with diagnosed/suspected portal hypertension.

Determinants and outcomes of acute pancreatitis in patients hospitalized for COVID-19: Early pandemic experience.

OBJECTIVES: To examine the predictors and outcomes associated with the development of acute pancreatitis (AP) in patients hospitalized with Coronavirus Disease 2019 (COVID-19). METHODS: This is an observational analysis of the 2020 National Inpatient Sample Database. The study includes adult patients who were admitted with a confirmed diagnosis of COVID-19 and stratifies them based on the presence or absence of AP during their hospitalization. Predictors of AP development between the two groups and differences in outcomes are examined. Multivariate logistic regression analysis using Stata/BE 17.0 is conducted, with adjustments made for age, sex, race, and Charlson Comorbidity Index (CCI). Statistical significance is determined at a p-value of <0.05. RESULTS: Significant factors associated with an increased risk of AP in COVID-19 patients include Hispanic ethnicity, higher Charlson Comorbidity Index (CCI) score, residence in states located in the southern region, history of chronic kidney disease, chronic liver disease, malnutrition, portal hypertension, and alcohol use. COVID-19 patients who developed AP were also found to be at higher risk of adverse outcomes, including mortality, acute coronary syndrome, acute kidney injury, sepsis, septic shock, in-hospital cardiac arrest, invasive mechanical ventilation, upper gastrointestinal bleeding, prolonged length of stay, and increased healthcare cost. CONCLUSIONS: In hospitalized patients with COVID-19, the presence of AP is associated with increased mortality and morbidity. Risk factors for developing AP in this population include Hispanic ethnicity, residence in the southern region, higher Charlson Comorbidity Index (CCI) score, history of chronic kidney disease, chronic liver disease, malnutrition, portal hypertension, and alcohol use.

The natural history of cystic fibrosis liver disease a prospective cohort study.

BACKGROUND: Our understanding of the natural history of cystic fibrosis liver disease (CFLD) is limited, leading to uncertainty for patients their families and clinicians when liver abnormalities are identified. AIM: to determine the incidence of CFLD, identify risk factors and document the natural history of liver abnormalities in cystic fibrosis (CF). METHODS: The Irish longitudinal study of CFLD (ILSCFLD) prospectively enrolled 95% of children with CF in 2007. Their liver disease status was classified as (i) advanced liver disease with portal hypertension (CFLD). (ii) nonspecific cystic fibrosis liver disease (NSCFLD) (iii) no liver disease (NoLD) RESULTS: 480/522 (91.9%) children were followed for a median 8.53 years IQR 1.28, of whom 35 (7.29%) had CFLD, 110 (22.9%) NSCFLD and 335 (69.79%) had NoLD. At follow-up 28/445 (6.29%) participants without CFLD at baseline, progressed to CFLD (Incidence 7.51/1000 person years (Pyrs) (95%CI 4.99-10.86). Of these 25/28(89.28%) were <10 years. No participant >10 years of age without clinical or radiological evidence of liver disease at baseline progressed to CFLD. During follow-up 18/35(51.43%) participants with CFLD died or received a transplant, MTx rate 7.75/100 Pyrs (95%CI 4.59-12.25) compared to NSCFLD 2.33/100 Pyrs (95%CI 1.44-3.56) and NoLD 1.13/100 Pyrs (95%CI 0.77-1.59). CFLD was an independent risk factor for mortality in CF. Children with CFLD also had a shorter life expectancy. CONCLUSION: The incidence of CFLD was highest in children under10 years. Children over10 years, with normal hepatic function did not develop CFLD. Research to identify the cause and improve outcome should focus on young children.

Nodular Regenerative Hyperplasia Is Not a Rare Condition After Liver Transplantation: Incidence, Predictive Factors, and Impact on Survival.

BACKGROUND: The objectives of this study were to evaluate incidence and to identify the risk factors of occurrence and the predictive factors of symptomatic forms of nodular regenerative hyperplasia (NRH) after liver transplantation (LT). METHODS: To identify risk factors of NRH following LT, we included 1648 patients transplanted from 2004 to 2018 and compared the patients developing NRH after LT to those who did not. To identify predictive factors of symptomatic NRH, we selected 115 biopsies displaying NRH and compared symptomatic to asymptomatic forms. Symptomatic NRH was defined as the presence of ascites, esophageal varices, hepatic encephalopathy, portal thrombosis, retransplantation, or death related to NRH. RESULTS: The incidence of NRH following LT was 5.1%. In multivariate analysis, the independent factor of developing NRH after LT was the donor's age (odds ratio [OR] = 1.02; confidence interval, 1.01-1.03; P = 0.02). Symptomatic forms occurred in 29 (25.2%) patients: 19 (16.5%) patients presented with ascites, 13 (11.3%) with esophageal varices, 4 (3.5%) with hepatic encephalopathy, and 8 (7%) with portal thrombosis. The median period before the onset of symptoms was 8.4 (1.5-11.3) y after LT. The spleen size at diagnosis/before LT ratio (OR = 12.5; 114.17-1.37; P = 0.0252) and thrombectomy during transplantation (OR = 11.17; 1.48-84.11; P = 0.0192) were associated with symptomatic NRH in multivariate analysis. CONCLUSIONS: NRH following LT is frequent (5.1%) and leads to symptomatic portal hypertension in 25.2% of patients. Using older grafts increases the risk of developing NRH after LT. Clinicians should screen for signs of portal hypertension, particularly in measuring spleen size.

Portal Hypertension among Patients with Chronic Liver Disease Admitted to the Department of Internal Medicine of a Tertiary Care Centre.

Introduction: Portal hypertension is increased pressure within the portal vein. A portal pressure gradient of more than 10 mmHg is defined as "clinically significant portal hypertension" due to manifestations such as splenomegaly, gastroesophageal varices, ascites, hepatorenal syndrome, hepatopulmonary syndrome, hepatic encephalopathy, and spontaneous bacterial peritonitis. The aim of this study was to find out the prevalence of portal hypertension among patients with chronic liver disease admitted to the Department of Internal Medicine of a tertiary care centre. Methods: A descriptive cross-sectional study was conducted among patients with chronic liver disease in the Department of Internal Medicine of a tertiary care centre from 1 January 2021 to 31 December 2022 after obtaining ethical approval from the Institutional Review Committee. Convenience sampling method was used. The point estimate was calculated at a 95% Confidence Interval. Results: Among 247 patients with chronic liver disease, the prevalence of portal hypertension was 38 (15.38%) (10.88-19.88, 95% Confidence Interval). A total of 16 (42.11%) patients were in the age group of 51-60 years and males were 36 (94.74%). Ascites as a complication were found in 4 (10.53%). Conclusions: The prevalence of portal hypertension among chronic liver disease inpatients in a tertiary care centre was found to be lower than other studies done in international settings. Keywords: inpatients; liver disease; portal hypertension; prevalence.

Is There an Association between Variceal Bleed and Helicobacter pylori Infection in Cirrhotic Patients with Portal Hypertension?: A prospective cohort study.

Objectives: This study aimed to determine the association between Helicobacter pylori infection and variceal bleeding as well as rebleeding in cases of cirrhosis with portal hypertension. Methods: This prospective cohort study included patients with bleeding oesophageal varices and was conducted at the Department of Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India, from August 2016 to July 2018. Results: A total of 190 patients were assessed for eligibility, of which 159 patients were included in this study. Among the 159 patients, 124 (78.0%) had alcohol-related liver disease and eight had hepatitis B virus-related liver disease. Seven patients with varices had bled neither at presentation nor during the follow-up period. A total of 78 (49.1%) patients were H. pylori infected. The primary outcome, which was correlation between prevalence of H. pylori and the incidence of bleeding/rebleeding from varices as well as encephalopathy found that patients with oesophageal varices (adjusted risk [AR] = 0.714) and H. pylori infection (AR = 0.682) had a lower risk of variceal rebleeding. Among H. pylori-negative patients, pepsinogen I was higher in patients with rebleeding (30.7 versus 14.4 ng/mL; P <0.001). The secondary outcome, which was correlation of the site of bleeding with H. pylori infection and the association of the ratio of pepsinogen I/II with bleeding showed that among H. pylori-positive patients, the ratio of pepsinogen I/II was higher in patients with rebleeding (2.9 versus 1.3 ng/mL; P = 0.023). Conclusion: H. pylori infection was associated with a lower risk of rebleeding in cases of cirrhosis with portal hypertension. Irrespective of the status of the H. pylori infection, rebleeding was associated with increased levels of gastric acid output, as demonstrated by the level of pepsinogen.

[Pre-cirrhotic portal hypertension syndrome: a certain stage of the progression of cirrhosis].

In this article, the author proposes the concept of pre-cirrhotic portal hypertension syndrome (PcPH), aiming to optimize the clinical management of patients with cirrhosis. PcPH is a certain stage of the progression of cirrhosis: starting from the presence of clinically significant portal hypertension, and ending with the occurrence of high-risk varices or any decompensated events (including ascites, variceal hemorrhage, hepatic encephalopathy). Defining PcPH is conducive to the early screening, early diagnosis and early treatment of cirrhosis and homogeneous management, conducive to patients with chronic liver disease to pay more attention to the hazards of cirrhosis and portal hypertension and popularization of science and education, and conducive to the sinking of high-quality hepatic medical resources and the construction of a hierarchical diagnosis and treatment system.

Accuracy of International Classification of Diseases-10 Codes for Cirrhosis and Portal Hypertensive Complications.

BACKGROUND AND AIMS: International Classification of Diseases (ICD)-10 codes may correspond to cirrhosis diagnosis. However, these codes have not been as well studied as ICD-9 codes. We aimed to evaluate the positive predictive value (PPV) and specificity of ICD-10 codes for cirrhosis. METHODS: We conducted a single-center retrospective study of patients in Michigan Medicine (Ann Arbor, MI, USA). We evaluated patients with at least one of 28 ICD-10 codes for cirrhosis and randomly selected controls for the presence of cirrhosis and/or portal hypertensive complications. RESULTS: Among 1317 patients with at least one ICD-10 code consistent with cirrhosis and/or portal hypertension, 796 had confirmed cirrhosis. After excluding ICD-10 codes found in < 10 patients, we evaluated the PPV of the 19 remaining codes. Of these, 15 had a high PPV for cirrhosis (> 80%), including codes for cirrhosis itself, gastroesophageal varices, hepatic encephalopathy, and other portal hypertensive complications. Specificity of ICD codes for cirrhosis for these 15 codes was high (> 94% for all). PPV and specificity were high across liver disease etiologies. Among patients without portal hypertension, PPVs of ICD-10 codes for cirrhosis were lower but still > 80% for the most common codes. PPVs of most codes for portal hypertensive complications were > 70%. Defining cirrhosis based on the presence of any of the 15 codes resulted in a PPV of 86% and by two different codes, a PPV 94%. CONCLUSIONS: ICD-10 codes for cirrhosis can accurately identify patients with cirrhosis with or without portal hypertensive complications.

Ursodeoxycholic acid and liver disease associated with cystic fibrosis: A multicenter cohort study.

BACKGROUND: The efficacy and safety of ursodeoxycholic acid (UDCA) for the treatment of liver disease associated with cystic fibrosis (CF) are under discussion, and clinical practice varies among centers. The study aimed at evaluating if the incidence of severe liver disease differs between CF centers routinely prescribing or not prescribing UDCA. METHODS: We carried out a retrospective multicenter cohort study including 1591 CF patients (1192 patients from UDCA-prescribing centers and 399 from non-prescribing centers) born between 1990 and 2007 and followed from birth up to 31 December 2016. We computed the crude cumulative incidence (CCI) of portal hypertension (PH) at the age of 20 years in the two groups and estimated the subdistribution hazard ratio (HR) through a Fine and Gray model. RESULTS: Over the observation period, 114 patients developed PH: 90 (7.6%) patients followed-up in UDCA prescribing centers and 24 (6.0%) in non-prescribing centers. The CCI of PH at 20 years was 10.1% (95% CI: 7.9-12.3) in UDCA-prescribing and 7.7% (95% CI: 4.6-10.7) in non-prescribing centers. The HR among patients followed in prescribing centers indicated no significant difference in the rate of PH either in the unadjusted model (HR: 1.21, 95% CI: 0.69-2.11) or in the model adjusted for pancreatic insufficiency (HR: 1.28, 95% CI: 0.77-2.12). CONCLUSIONS: CF patients followed-up in UDCA prescribing centers did not show a lower incidence of PH as compared to those followed in centers not prescribing UDCA. These results question the utility of UDCA in reducing the occurrence of severe liver disease in CF.

Endoscopic findings unrelated to portal hypertension in patients with liver cirrhosis undergoing a varicose vein screening programme. / Hallazgos endoscópicos no relacionados con hipertensión portal en pacientes con cirrosis hepática sometidos a un programa de cribado de varices.

AIM: To determine the prevalence of endoscopic lesions unrelated with portal hypertension in patients with cirrhosis. PATIENTS AND METHODS: Cross-sectional study including a consecutive cohort of patients with liver cirrhosis enrolled in a screening program of oesophageal varices who underwent an upper gastrointestinal endoscopy from November, 2013, to November, 2018. Clinical predictors of endoscopic lesions unrelated to portal hypertension were analyzed by univariate and multivariate logistic regression. RESULTS: A total of 379 patients were included. The most frequent aetiology of liver disease was alcohol consumption (60.4%). The prevalence of endoscopic lesions unrelated with portal hypertension was 39.6% (n=150). Among 96 patients with peptic lesions, urease was obtained in 56.2% of patients (positive in 44.4% of them). The prevalence of endoscopic lesions unrelated to portal hypertension was not associated with age, gender, liver function or ultrasound findings of portal hypertension. The prevalence of endoscopic lesions unrelated to portal hypertension was not associated with age, gender, liver function or ultrasound findings of portal hypertension. Smokers had a trend to increased prevalence of endoscopic lesions unrelated to portal hypertension (43.2% vs. 34.6%; p=0.09), particularly peptic ulcer (6.4% vs. 0.6%; p=0.05) and peptic duodenitis (17.3% vs. 6.3%; p=0.002). Active smoking was the only independent predictor of peptic ulcer or duodenitis (OR=2.56; p=0.017). CONCLUSION: Active smoking is a risk factor for endoscopic lesions unrelated to portal hypertension. This finding should be further investigated to reassess endoscopic screening programs in cirrhotic smokers.

Prevalence and clinical significance of clinically evident portal hypertension in patients with hepatocellular carcinoma undergoing transarterial chemoembolization.

BACKGROUND: Clinically evident portal hypertension (CEPH) was previously identified as a prognostic factor for patients with hepatocellular carcinoma (HCC). However, little is known about the prognostic influence of CEPH on the long-term outcome of patients with HCC undergoing transarterial chemoembolization (TACE), particularly in Western populations. OBJECTIVES: This study investigated the prevalence and prognostic influence of CEPH in a Western population of patients with HCC undergoing TACE. METHODS: This retrospective study included 349 treatment-naïve patients that received initial TACE treatment at our tertiary care center between January 2010 and November 2020. CEPH was defined as a combination of ascites, esophageal/gastric varices, splenomegaly and a low platelet count. We assessed the influence of CEPH and its defining factors on median overall survival (OS) in HCC patients. We compared the effects of CEPH to those of well-known prognostic factors. RESULTS: Of the 349 patients included, 304 (87.1%) patients had liver cirrhosis. CEPH was present in 241 (69.1%) patients. The median OS times were 10.6 months for patients with CEPH and 17.1 months for patients without CEPH (log rank p = 0.036). Median OS without a present surrogate was 17.1 months, while patients with one respectively more than two present CEPH surrogates had a median OS of 10.8 and 9.4 months (log rank p = 0.053). In multivariate analysis, CEPH was no significant risk factor for OS (p = 0.190). Of the CEPH-defining factors, only ascites reached significance in a univariate analysis. CONCLUSION: CEPH was present in more than two thirds of the patients with HCC undergoing TACE in our cohort of Western patients. Patients with CEPH had a significantly impaired survival in univariate analysis. However, no significance was reached in multivariate analysis. Thus, when TACE treatment is deemed oncologically reasonable, patients should not be excluded from TACE treatment due to the presence of surrogates of portal hypertension alone.

HISTOPATHOLOGICAL, CLINICAL AND EPIDEMIOLOGICAL FEATURES OF HEPATOPORTAL SCLEROSIS IN A REFERRAL CENTER FOR LIVER DISEASE IN NORTHEASTERN BRAZIL.

BACKGROUND: Hepatoportal sclerosis HPS or obliterative portal venopathy (OPV), one of the differential diagnoses for non-cirrohtic portal hypertension, is characterized by the disappearance of the portal branches, portal and septal fibrosis, perisinusoidal fibrosis and regenerative nodular hyperplasia (RNH). It is a spectral disease that may progress to severe portal hypertension. Its etiopathogenesis is still little understood, especially in Brazil, it has been probably misdiagnosed due to its histopatological similarities with the hepatosplenic form of schistosomiasis. OBJECTIVE: To analyze the profile of patients with HPS in Northeastern Brazil and to demonstrate the pathological characteristics of HPS. METHODS: We retrospectively analyzed cases of OPV in liver biopsies and explants from a referral center for liver in Bahia - Brazil. The qualitative and quantitative analysis of the portal tracts and liver parenchyma was made so that comparisons could be done among the HPS findings of our population and the findings described by other authors. RESULTS: From the 62 patients identified with HPS, 42% were male, while 58% were female. The average age at diagnosis was 48.3 years. From this group, we analyzed the liver biopsy of 10 patients whose diagnosis of schistosomiasis could be ruled out. From these 100% (10/10) presented dense portal fibrosis and portal venous obliteration. Liver parenchymal atrophy was present in 60% (6/10) of the patients, sinusoidal dilation was present in 30% (3/10), the presence of portal septa occurred in 50% (5/10) and dense portal fibrosis in all patients analyzed. Nodular regenerative hyperplasia was found in 30% (3/10) of the patients. CONCLUSION: HPS seems to be neglected and misdiagnosed in Brazil, due to its similarities with schistossomiasis. In our study dense portal fibrosis, obliteration of the portal vein branches, parenchymal atrophy, sinusoidal dilatation and parenchymal nodular hyperplasia were the main histopathological findings and were similar to that described in other countries.

Características histopatológicas, clínicas e epidemiológicas da esclerose hepatoportal em um centro de referência para doenças do fígado no Nordeste do Brasil / Histopathological, clinical and epidemiological features of hepatoportal sclerosis in a referral center for liver disease in northeastern brazil

ABSTRACT BACKGROUND: Hepatoportal sclerosis HPS or obliterative portal venopathy (OPV), one of the differential diagnoses for non-cirrohtic portal hypertension, is characterized by the disappearance of the portal branches, portal and septal fibrosis, perisinusoidal fibrosis and regenerative nodular hyperplasia (RNH). It is a spectral disease that may progress to severe portal hypertension. Its etiopathogenesis is still little understood, especially in Brazil, it has been probably misdiagnosed due to its histopatological similarities with the hepatosplenic form of schistosomiasis. OBJECTIVE: To analyze the profile of patients with HPS in Northeastern Brazil and to demonstrate the pathological characteristics of HPS. METHODS: We retrospectively analyzed cases of OPV in liver biopsies and explants from a referral center for liver in Bahia - Brazil. The qualitative and quantitative analysis of the portal tracts and liver parenchyma was made so that comparisons could be done among the HPS findings of our population and the findings described by other authors. RESULTS: From the 62 patients identified with HPS, 42% were male, while 58% were female. The average age at diagnosis was 48.3 years. From this group, we analyzed the liver biopsy of 10 patients whose diagnosis of schistosomiasis could be ruled out. From these 100% (10/10) presented dense portal fibrosis and portal venous obliteration. Liver parenchymal atrophy was present in 60% (6/10) of the patients, sinusoidal dilation was present in 30% (3/10), the presence of portal septa occurred in 50% (5/10) and dense portal fibrosis in all patients analyzed. Nodular regenerative hyperplasia was found in 30% (3/10) of the patients. CONCLUSION: HPS seems to be neglected and misdiagnosed in Brazil, due to its similarities with schistossomiasis. In our study dense portal fibrosis, obliteration of the portal vein branches, parenchymal atrophy, sinusoidal dilatation and parenchymal nodular hyperplasia were the main histopathological findings and were similar to that described in other countries.

RESUMO CONTEXTO: Esclerose hepatoportal EHP ou venopatia portal obliterativa VPO, um dos diagnósticos diferenciais para a hipertensão portal não cirrótica, é caracterizada pelo desaparecimento dos ramos portais, fibrose portal e septal, fibrose sinusoidal e hiperplasia nodular regenerativa HNR. A EHP é um doença espectral, que pode progredir para hipertensão portal severa. Sua etiopatologia é ainda pouco compreendida, especialmente no Brasil, onde ela é provavelmente subdiagnoticada devido as suas similaridades com a forma hepatoesplênica da esquistossomose. OBJETIVO: Analizar o perfil dos pacientes com EHP no Nordeste do Brasil, e demontrar as características patológicas da EHP. MÉTODOS: Analisamos restrospectivamente os casos de VPO em biópsias hepáticas e explantes de um centro de referência em fígado na Bahia, Brasil. A análise qualiquantitativa dos tratos portais e parênquima hepático foi realizada, permitindo a comparação entre os nossos paciente e os achados descritos por outros autores. RESULTADOS: Entre os 62 paciente identificados com EHP, 42% era do sexo masculino, 58% era do sexo feminino. A média de idade no diagnótico foi 48,3 anos. Desse grupo, analizamos a biópsia hepática de 10 pacientes nos quais o diagnóstico de esquistossomose pode ser excluído. Desses pacientes, 100% 10/10 se apresentou com fibrose portal densa e obliteração venosa portal. Atrofia do perênquima hepático estava presente em 60% 6/10 dos pacientes, dilatação sinusiodal em 30% 3/10 a presença de septos portais ocorreu em 50% 5/10 e fibrose portal densa foi achada em todos os pacientes. Hiperplasia nodular regenerativa foi encontrada em 30% dos pacientes. CONCLUSÃO: A EHP parece ser negligenciada e subdiagnosticada no Brasil, devido as suas similaridades com esquistossomose. Em nosso estudo, fibrose portal densa, obliteração dos ramos da veia porta, atrofia do parênquima, dilatação sinusoidal e hiperplasia nodular do parênquima foram os principais achados histopatológicos e foram semelhantes aos descritos em outros países.

Increased Prevalence of Left Lobe Atrophy in Long-standing Extrahepatic Portal Vein Obstruction.

OBJECTIVES: The aim of this study was to describe long-term changes in standard blood tests and ultrasound (US) findings in pediatric patients with Extrahepatic Portal Vein Obstruction (EHPVO) who have not undergone Meso-Rex Bypass (MRB) surgery. METHODS: US, laboratory, and endoscopic data of 77 patients were analyzed and compared at 2 different points in time: at initial workup and at most recent follow-up. Differences were assessed using McNemar and Wilcoxon tests, while correlations were evaluated using generalized estimating equations. RESULTS: Upper gastrointestinal bleeding was the most frequent initial manifestation of EHPVO, in 45.4% of subjects. The mean age at diagnosis was 4.3 years. Seventy-four percentage had a previous history of umbilical catheterization and/or neonatal sepsis. Over time, there was a significant increase in the prevalence of leukopenia, thrombocytopenia, and, interestingly, of left lobe atrophy (LLA), even though the number of esophageal varices and the need for endoscopic interventions reduced. A significant correlation was found between history of umbilical catheterization and LLA, splenomegaly and LLA, and gallbladder wall thickening and LLA. Overall, the number of patients with cholelithiasis at initial workup was low (and therefore, not analyzed with inferential statistics); however, we observed a relative increase over time. CONCLUSIONS: In this cohort of patients with EHPVO followed over more than 8 years without treatment with MRB, we report the novel finding of a significant increase in the prevalence of LLA over time. LLA correlated with past medical history of umbilical catheterization, and findings of splenomegaly and gallbladder wall thickening. We propose that that LLA should be further explored as a marker of portal hypertension, particularly in the context of a history of umbilical catheterization.

Reconstructing spleno-mesenterico-portal cofluence by bifurcated allogeneic vein in local advanced pancreatic cancer-a feasible method to avoid left-sided portal hypertension.

BACKGROUND: Left-sided portal hypertension is usually found in patients undergoing pancreaticoduodenectomy (PD) with spleno-mesenterico-portal (S-M-P) confluence resection. This study is to explore the outcomes of S-M-P confluence reconstruction after resection by using bifurcated allogeneic vein. METHODS: Clinicopathologic data of patients who underwent extensive PD with S-M-P confluence resection for carcinoma of pancreatic head/uncinate process in our hospital between December 2011 and August 2018 were retrospectively reviewed and clinical outcomes of vein reconstruction after resection were analyzed. RESULTS: Of the 37 patients enrolled, S-M-P reconstruction by bifurcated allogeneic vein was performed in 24 cases (group 1) and simply splenic vein ligation in 13 cases (group 2). Items including pathological results, blood loss, and complications were comparable between the two groups, operation time was longer in group 1 (573.8 vs. 479.2 min, p = 0.018). Significantly decreased platelet count (205.9 vs. 133.1 × 109 /L, p = 0.001) and increased splenic volume (270.9 vs. 452.2 ml, p < 0.001) were observed in group 2 at 6 months after operation. The mean splenic hypertrophy ratio was 1.06 in group 1 and 1.63 in group 2, respectively (p < 0.001). There were four patients with varices were found in group 2, none in group 1. CONCLUSIONS: Without increased complications, reconstructing S-M-P confluence by bifurcated allogeneic vein after resection may help to avoid left-sided portal hypertension.

Portal hypertension and hepatocellular carcinoma: Des liaisons dangereuses .

BACKGROUND AND AIMS: Portal hypertension (PHT) and hepatocellular carcinoma (HCC) are major complication of cirrhosis which significantly contribute to morbidity and mortality. In this review, we aim to describe the consequences of both angiogenesis and inflammation in the pathogenesis of PHT and HCC, but also the difficulty to propose adapted treatment when PHT and HCC coexist in the same patients. METHODS: Studies for review in this article were retrieved from the PubMed database using literature published in English until March 2021. RESULTS: Portal hypertension occurs secondary to an increase of intrahepatic vascular resistances, the opening of portosystemic collateral vessels and the formation of neovessels, related to vascular endothelial growth factor (VEGF). Recently, bacterial translocation-mediated inflammation was also identified as a major contributor to PHT. Interestingly, VEGF and chronic inflammation also contribute to HCC occurrence. As PHT and HCC often coexist in the same patient, management of PHT and its related complications as well as HCC treatment appear more complex. Indeed, PHT-related complications such as significant ascites may hamper the access to HCC treatment and the presence of HCC is also independently associated with poor prognosis in patients with acute variceal bleeding related to PHT. Due to their respective mechanism of action, the combination of Atezolizumab and Bevacizumab for advanced HCC may impact the level of PHT and its related complications and to date, no real-life data are available. CONSLUSIONS: Appropriate evaluation and treatment of PHT remains a major issue in order to improve the outcome of HCC patients.

Recurrence of Primary Sclerosing Cholangitis After Liver Transplant in Children: An International Observational Study.

BACKGROUND AND AIMS: Recurrent primary sclerosing cholangitis (rPSC) following liver transplant (LT) has a negative impact on graft and patient survival; little is known about risk factors for rPSC or disease course in children. APPROACH AND RESULTS: We retrospectively evaluated risk factors for rPSC in 140 children from the Pediatric PSC Consortium, a multicenter international registry. Recipients underwent LT for PSC and had >90 days of follow-up. The primary outcome, rPSC, was defined using Graziadei criteria. Median follow-up after LT was 3 years (interquartile range 1.1-6.1). rPSC occurred in 36 children, representing 10% and 27% of the subjects at 2 years and 5 years following LT, respectively. Subjects with rPSC were younger at LT (12.9 vs. 16.2 years), had faster progression from PSC diagnosis to LT (2.5 vs. 4.1 years), and had higher alanine aminotransferase (112 vs. 66 IU/L) at LT (all P < 0.01). Inflammatory bowel disease was more prevalent in the rPSC group (86% vs. 66%; P = 0.025). After LT, rPSC subjects had more episodes of biopsy-proved acute rejection (mean 3 vs. 1; P < 0.001), and higher prevalence of steroid-refractory rejection (41% vs. 20%; P = 0.04). In those with rPSC, 43% developed complications of portal hypertension, were relisted for LT, or died within 2 years of the diagnosis. Mortality was higher in the rPSC group (11.1% vs. 2.9%; P = 0.05). CONCLUSIONS: The incidence of rPSC in this cohort was higher than previously reported, and was associated with increased morbidity and mortality. Patients with rPSC appeared to have a more aggressive, immune-reactive phenotype. These findings underscore the need to understand the immune mechanisms of rPSC, to lay the foundation for developing new therapies and improve outcomes in this challenging population.