Presence Of Portal Hypertension Research Articles

HPB SO64 - Outcomes of liver resection for hepatocellular cancer with or without liver functional assessment using Tc 99m Mebrofenate SPECT-CT, portal pressures: A comparative study

Abstract Background Presence of background liver disease increases the post operative morbidity and mortality among patients undergoing liver resection for hepatocellular carcinoma (HCC). These patients need evaluation of background liver function and portal hypertension, in addition to the conventional risk assessment. The current study aims to look at the outcomes of liver resections performed for HCC cases comparing cohorts with conventional risk assessment (CA) and those with functional assessment (FA) of background liver. Method A retrospective study including all liver resections for HCC cases performed in our tertiary care hepatobiliary unit between 2013 and 2022 (10 years) was conducted. A pathway for liver functional assessment using Tc 99m Mebrofenate SPECT-CT, and portal pressures (cut-off values of 2.79/min/m2 and 10mmHg, respectively) was included in the departmental protocol in December 2018. Data on demographics, underlying degree of liver cirrhosis, types of liver resection and post-operative outcomes including post-hepatectomy liver failure (PHLF) were collected and reported using descriptive statistics. Results 200 liver resections, performed for HCC between 2013 and 2022, were divided into a conventional (126 patients) and functional (74 patients) assessment groups. Median age (68 years) was similar. Background liver cirrhosis was present in 27 (21%) vs 19 (26%) cases, and major resections were performed in 47 (37%) and 10 (13%) cases in the CA and FA groups respectively. In the CA group, 90-day mortality was 7% (compared to FA group (1%) (p=0.26). None of the patients in FA group was found to have PHLF whereas 3 patients each had PHLF grade B and C in the conventional group. Conclusion Formal functional assessment of the background liver by evaluating the function of future liver remnant and presence of portal hypertension helps in selection of patients for appropriate surgical treatment options and improve the post-operative outcomes.

Perioperative outcomes after hepatectomy for hepatocellular carcinoma among patients with cirrhosis, fatty liver disease, and clinically normal livers

IntroductionDespite superior outcomes with liver transplantation, cirrhotic patients with HCC may turn to other forms of definitive treatment. To understand perioperative outcomes, we examined perioperative mortality and major morbidity after hepatectomy for HCC among cirrhotic and non-cirrhotic patients. Methodology: The American College of Surgeons National Surgical Quality Improvement Project (ACS-NSQIP) database was queried for liver resection for HCC. Multivariable logistic regression was performed to determine the association between liver texture and risk of major non-infectious morbidity, post-hepatectomy liver failure (PHLF) and 30-day mortality. ResultsFrom 2014 to 2018, 2203 patients underwent hepatectomy: 58.6 % cirrhotic, 12.8 % fatty and 28.6 % normal texture. Overall 30 day-mortality was 2.1 % (n = 46), although higher among fatty liver (2.8 %) and cirrhotic (2.6 %; p = 0.025) patients. The incidence of PHLF was 6.9 %, with hepatectomy type, cirrhosis, and platelet count as major risk factors. Age, resection type, and platelet count were associated with major complications. Trisegmentectomy and right hepatectomy (OR = 3.60, OR = 3.46, respectively) conferred a greater risk of major noninfectious morbidity compared to partial hepatectomy. Among cirrhotics alone, hepatectomy type, platelet count, preoperative sepsis and ASA class were associated with major morbidity. DiscussionHepatic parenchymal disease/texture and function, presence of portal hypertension, and the extent of the liver resection are critical determinants of perioperative risk among HCC patients.

Histological characterization of liver involvement in systemic mastocytosis.

Systemic mastocytosis (SM) is characterized by the accumulation of atypical mast cells (MCs) in organs. Liver histology of SM has been marginally described and accurate histological classification is critical, given the consequences of aggressive SM diagnosis. We aimed to describe the histological features associated with liver SM using updated tools. Using the database of the French Reference Centre for Mastocytosis, we retrospectively identified patients with a liver biopsy (LB) and a diagnosis of SM. All LB procedures were performed according to the local physician in charge and centrally reviewed by an expert pathologist. A total of 28 patients were included: 6 had indolent SM, 9 had aggressive SM, and 13 had SM with an associated hematologic neoplasm. Twenty-five (89%) patients presented hepatomegaly, and 19 (68%) had portal hypertension. The LB frequently showed slight sinusoid dilatation (82%). Fibrosis was observed in 3/6 indolent SM and in almost all advanced SM cases (21/22), but none of them showed cirrhosis. A high MC burden (>50 MCs/high-power field) was correlated with elevated blood alkaline phosphatase levels (p = .030). The presence of portal hypertension was associated with a higher mean fibrosis grade (1.6 vs. 0.8 in its absence; p = .026). In advanced SM, the presence of nodular regenerative hyperplasia (NRH) was associated with decreased overall survival (9.5 vs. 46.3 months, p = .002). MC infiltration induced polymorphic hepatic lesions and the degree of fibrosis is associated with portal hypertension. NRH identifies a poor prognosis subgroup of patients with advanced SM. Assessing liver histology can aid in SM prognostic evaluation.

Лечение хронического гепатита D в Краснодарском крае – опыт реальной практики

Objective. To analyze the results of treatment of patients with chronic hepatitis D (CHD) in Krasnodar Krai in the conditions of real practice using bulevirtide, an HBV/HDV entry inhibitor. Patients and methods. Interim treatment outcomes were studied in 11 patients with CHD, predominantly at the cirrhotic stage (82%), with the presence of portal hypertension (55%), liver failure (n = 3), and hepatocellular carcinoma (n = 2, detected during treatment). Patients received bulevirtide as monotherapy (n = 7) or in combination with peginterferon (n = 4); evaluation periods ranged from 24–96 weeks of treatment. Results. All patients with known baseline HDV RNA level (n = 5) achieved virologic response, HDV RNA level reduction was -1.8 and -5.5 log10 after 24 and 96 weeks of monotherapy, -2.0–4.9 log10 after 24–72 weeks of combined therapy, with aviremia in 1 patient. In patients with unknown baseline HDV RNA level (n = 6) its dynamics during treatment corresponded to virologic response in 4 patients (decrease from -1.5 to -4.0 log10 in the period from 12/24/48 to 72 weeks of monotherapy, with aviremia in 1 patient). Efficacy improved as treatment continued to 72–96 weeks in 5 of 7 patients (4 received monotherapy, 1 received combination therapy). The overall virologic response rate was 82%, aviremia 18%, and biochemical response (ALT level reduction/normalization) 55%/45%, respectively. All patients with liver dysfunction (n = 3) showed virological and biochemical response, including aviremia and ALT normalization (1 and 2 patients, respectively). Treatment was characterized by good tolerability, absence of severe and serious adverse reactions, cases of treatment withdrawal, stability of liver function indices, including cirrhosis with dysfunction. In 1 patient after hepatectomy for HCC there was a temporary deterioration of liver function with the transition of class A to class B according to Child-Pugh, unrelated to treatment. Conclusion. Interim results of HDV treatment in real practice have demonstrated efficacy, safety and good tolerability of bulevirtide in monotherapy or combination therapy, including in patients with liver dysfunction. Further development of an optimal treatment algorithm is necessary, taking into account the results of ongoing studies, in order to improve patient prognosis and achieve cure of HDV infection. Key words: bulevirtide, hepatocellular carcinoma, decompensated cirrhosis, monotherapy, combination therapy, chronic hepatitis D, liver cirrhosis

Long-Term Impact of Direct-Acting Antivirals on Liver Fibrosis and Survival in HCV-Infected Liver Transplant Recipients.

(1) Background: Little is known about the long-term impact of sustained virological response (SVR) on fibrosis progression and patient survival in liver transplantation (LT) recipients treated with direct-acting antivirals (DAAs). We investigated liver fibrosis evolution and patient survival in hepatitis C virus (HCV)-infected patients receiving DAAs after LT. (2) Methods: All consecutive HCV-infected patients treated with DAAs after LT between May 2014 and January 2019 were considered. The clinical and virological features were registered at the baseline and during the follow-up. The liver fibrosis was assessed by liver biopsy and/or transient elastography (TE) at the baseline and at least 1 year after the end of treatment (EoT). (3) Results: A total of 136 patients were included. The SVR12 was 78% after the first treatment and 96% after retreatment. After the SVR12, biochemical tests improved at the EoT and remained stable throughout the 3-year follow-up. Liver fibrosis improved after the SVR12 (p < 0.001); nearly half of the patients with advanced liver fibrosis experienced an improvement of an F ≤ 2. The factors associated with lower survival in SVR12 patients were the baseline platelet count (p = 0.04) and creatinine level (p = 0.04). (4) Conclusions: The long-term follow-up data demonstrated that SVR12 was associated with an improvement in hepatic function, liver fibrosis, and post-LT survival, regardless of the baseline liver fibrosis. The presence of portal hypertension before the DAAs has an impact on patient survival, even after SVR12.

Cystic fibrosis liver disease in children - A review of our current understanding.

Cystic fibrosis is the second most common genetic disease in infancy. It is the result of a mutated channel protein, the CFTR, which secretes chloride ions, fluidifying secretions. Recent improvements in the treatment have increased life expectancy in these patients. Nevertheless, liver involvement remains the third cause of death. Unfortunately, our understating of the physiopathology is still deficient. Biliary obstruction secondary to the presence of thick secretions is considered to lead to cirrhosis. However, treatment with ursodeoxycolic acid has not changed the natural history. Furthermore, the presence of portal hypertension in the absence of cirrhosis cannot be explained. Recently, the role of CFTR as modulator of immune tolerance has been proposed, which could explain the presence of a persistent portal inflammation leading to fibrosis, and the gut-liver axis would also have a role in disease presentation and progression.

Impact of liver cirrhosis, the severity of cirrhosis, and portal hypertension on the outcomes of minimally invasive left lateral sectionectomies for primary liver malignancies

The impact of cirrhosis and portal hypertension on perioperative outcomes of minimally invasive left lateral sectionectomies remains unclear. We aimed to compare the perioperative outcomes between patients with preserved and compromised liver function (noncirrhotics versus Child-Pugh A) when undergoing minimally invasive left lateral sectionectomies. In addition, we aimed to determine if the extent of cirrhosis (Child-Pugh A versus B) and the presence of portal hypertension had a significant impact on perioperative outcomes. This was an international multicenter retrospective analysis of 1,526 patients who underwent minimally invasive left lateral sectionectomies for primary liver malignancies at 60 centers worldwide between 2004 and 2021. In the study, 1,370 patients met the inclusion criteria and formed the final study group. Baseline clinicopathological characteristics and perioperative outcomes of these patients were compared. To minimize confounding factors, 1:1 propensity score matching and coarsened exact matching were performed. The study group comprised 559, 753, and 58 patients who did not have cirrhosis, Child-Pugh A, and Child-Pugh B cirrhosis, respectively. Six-hundred and thirty patients with cirrhosis had portal hypertension, and 170 did not. After propensity score matching and coarsened exact matching, Child-Pugh A patients with cirrhosis undergoing minimally invasive left lateral sectionectomies had longer operative time, higher intraoperative blood loss, higher transfusion rate, and longer hospital stay than patients without cirrhosis. The extent of cirrhosis did not significantly impact perioperative outcomes except for a longer duration of hospital stay. Liver cirrhosis adversely affected the intraoperative technical difficulty and perioperative outcomes of minimally invasive left lateral sectionectomies.

Clinical manifestations and approach to the management of patients with common variable immunodeficiency and liver disease.

The clinical spectrum of common variable immunodeficiency (CVID) includes predisposition to infections, autoimmune/inflammatory complications and malignancy. Liver disease is developed by a proportion of patients with CVID, but limited evidence is available about its prevalence, pathogenesis and prognostic outcome. This lack of evidence leads to the absence of guidelines in clinical practice. In this study, we aimed at defining the characteristics, course and management of this CVID complication in Spain. Spanish reference centers were invited to complete a cross-sectional survey. Thirty-eight patients with CVID-related liver disease from different hospitals were evaluated by a retrospective clinical course review. In this cohort, abnormal liver function and thrombocytopenia were found in most of the patients (95% and 79% respectively), in keeping with the higher incidence of abnormal liver imaging and splenomegaly. The most common histological findings included nodular regenerative hyperplasia (NRH) and lymphocytic infiltration, which have been associated with portal hypertension (PHTN) leading to a poorer prognosis. Autoimmune/inflammatory complications occurred in 82% of the CVID patients that developed liver disease and 52% of the patients treated with immunomodulators showed a reduction in the liver function tests' abnormalities during treatment. Among the experts that conducted the survey, there was 80% or more consensus that the workup of CVID-related liver disease requires liver profile, abdominal ultrasound and transient elastography. The majority agreed that liver biopsy should be essential for diagnosis. There was 94% consensus that endoscopic studies should be performed in the presence of PHTN. However, there was 89% consensus that there is insufficient evidence on the management of these patients. Liver disease varies in severity and may contribute substantially to morbidity and mortality in patients with CVID. Hence the importance of close follow-up and screening of this CVID complication to prompt early targeted intervention. Further research is needed to evaluate the pathophysiology of liver disease in patients with CVID to identify personalized treatment options. This study emphasizes the urgent need to develop international guidelines for the diagnosis and management of this CVID complication.

Evaluation of Small Intestinal Permeability in Patients with Overlap Syndrome (Autoimmune Hepatitis/Primary Biliary Cholangitis)

Аim: to evaluate the state of small intestine permeability by the “double sugar test” in patients with overlap syndrome (autoimmune hepatitis / primary biliary cholangitis (AIH / PBC)).Materials and methods. Prospectively, 56 people were included in the study. Of these, 26 were diagnosed with AIH/PBC, 30 were in the control group. The diagnosis was made in accordance with the current recommendations. The average age of patients was 49.7 ± 13.8 years, healthy volunteers — 48.6 ± 9.2 years. The determination of the permeability of the small intestine was carried out by a “double sugar test” (the ratio of lactulose/mannitol in urine), using the method of high-performance liquid chromatography — mass spectrometry.Results. In patients with AIH/PBC, an increase in intestinal permeability was found — 0.20 [0.09; 0.30] (p &lt; 0.001) compared with the control group 0.01 [0.01; 0.02]. We divided patients at the stage of liver damage. An increased small intestinal permeability was revealed: hepatitis stage — 0.19 [0.13; 0.30] (p &lt; 0.001), liver cirrhosis stage — 0.18 [0.09; 0.30] (p &lt; 0.05) compared with the control group. In the early stages of disease (1 month from the onset of the disease) had an increased lactulose/mannitol ratio — 0.13 [0.05; 0.26] (p &lt; 0.001) compared to the control group. In the presence of portal hypertension (PH), small intestinal permeability was increased — 0.18 [0.09; 0.30] (p &lt; 0.001) compared with the control group.Conclusions. An increase in small bowel permeability was found in patients with overlapping syndrome. All patients had increased intestinal permeability (regardless of the presence of extrahepatic manifestations).

The Role of Shear-Wave Elastography of the Spleen in Ruling out the Presence of High-Risk Varices in Non-Alcoholic Fatty Liver Disease (NAFLD)

The progression of liver fibrosis and the presence of portal hypertension are two key points in the follow-up and severity assessment of patients with chronic liver disease. Objective evaluation of such aspects has proven to be difficult due to the lack of reproducible and standardized non-invasive methods. Therefore, the aim of this study was to evaluate whether spleen stiffness (SS) can rule out the presence of high-risk varices (HRVs) in patients with non-alcoholic fatty liver disease (NAFLD). We designed a prospective follow-up of a cohort of 48 consecutive patients diagnosed with compensated advanced chronic liver disease (cACLD) due to NAFLD, between January 2020 and January 2021. After clinical evaluation, laboratory testing, ultrasonography (US), and shear-wave elastography (2D-SWE.GE) of both the liver and the spleen, patients were endoscopically screened for esophageal varices, gastric varices, and portal hypertensive gastropathy. Correlations and predictors were assessed. After univariate, multivariate, and predictive analyses, SS could be referred to as an independent predictor for high-risk varices (AUROC 0.987, p &lt; 0.001, OR 4.985, 95% CI: 1.57–15.73, p = 0.006), with a calculated cutoff value of 17.95 kPa. These results are consistent with those of other, similar studies using both 2D-SWE.GE and a similar module (2D-SWE.SSI) in patients with metabolic liver disease. When confirmed by subsequent larger studies, SS could potentially become a useful non-invasive tool in the assessment of clinically significant portal hypertension in patients with advanced fatty liver disease.

Local tumor control of intermediate and advanced stage hepatocellular carcinoma after local ablative treatment with image-guided interstitial high-dose-rate brachytherapy: A subgroup analysis of 286 HCC nodules

Image-guided interstitial high-dose-rate brachytherapy (iBT) has been demonstrated to offer high local tumor control rates (LTC) of >90% after local ablation of intermediate and advanced hepatocellular carcinoma (HCC; BCLC B and C). The purpose of this study was to show the efficacy of iBT stratified by subgroups and to identify clinical characteristics associated with superior local tumor control (LTC) based on a highly heterogenous patient population METHODS AND MATERIALS: A cumulative number of 286 HCC nodules in 107 patients were retrospectively analyzed. Clinical and imaging follow-ups were conducted every 3 months after treatment. Analyzed clinical factors were: etiology, presence of liver cirrhosis, radiographic features, lesion size, pretreatment, administered dose, presence of portal hypertension, portal vein thrombosis, and level of alpha-fetoprotein (AFP). LTC rate was 88.8% for a median follow-up of 14.3 months (range 3-81 months; 95% CI: 85-92%). Median minimal enclosing tumor dose (D100) was 16.1 Gy (range 7.1-30.3 Gy; reference dose 15 Gy). Subgroup analysis showed significant fewer local recurrences for alcoholic liver disease (ALD)-related HCCs compared to those related to other causes of liver cirrhosis (nonalcoholic fatty liver disease, virus-related liver cirrhosis and other causes) (p = 0.015). LTC was significantly lower after prior surgical resection (p = 0.046). No significant variance was observed for the applied D100 in each group or for all other clinical factors tested. IBT achieves high LTC rates across treated subgroups. However, further studies should particularly address the possible impact of underlying etiology on local recurrence with emphasis on a possible higher radiosensitivity of ALD-related HCCs.

Porto-sinusoidal vascular disease: a new definition of an old clinical entity.

Porto-sinusoidal vascular disease (PSVD) is defined as a vascular liver disease characterized by the absence of cirrhosis and the presence of characteristic histological features, with or without the presence of portal hypertension (PH). Half of the patients with PSVD also have associated disease that may contribute to the development of PSVD. Patients usually remain asymptomatic until complications of PH arise. Variceal bleeding and portal vein thrombosis are major complications associated with PSVD. The treatment is focused on managing complications of PH, mainly through primary prophylaxis of variceal bleeding and treatment of portal vein thrombosis. Currently, there is insufficient evidence to support the use of anticoagulants for thrombosis prevention in these patients. Despite the increase of recognition of PSVD, further research is needed to enable early disease diagnosis, establish optimal screening methods, and develop strategies to slow down disease progression.

S1334 Pharmacotherapy for Primary Biliary Cholangitis: An Assessment of Medication Candidacy and Rates of Treatment

Introduction: Numerous trials have shown that Ursodeoxycholic Acid (UDCA) is effective in improving biochemical indices, delaying progression, and improving survival in patients diagnosed with Primary Biliary Cholangitis (PBC). The alternative therapy, Obeticholic Acid (OCA), is recommended for patients who cannot tolerate UDCA or who have an inadequate response to UDCA monotherapy. Despite the efficacy of these medications, prior investigations suggest that as many as 30% of patients with PBC may have never received treatment. This study aims to characterize usage rates of UDCA and second-line therapies among patients with PBC at a large urban health system with an academic liver transplant program. Methods: This was an observational, cross-sectional study. Patients were identified according to the ICD-10 code for PBC (ICD-10-CM: K74.3). All patients with a diagnosis of PBC who had any records within the health system were included. Review of medical records was performed to confirm the diagnosis of PBC (defined by AASLD practice guidelines) and determine which medications had been prescribed for treatment of PBC, as well as candidacy for second-line therapies. Results: 495 patients met inclusion criteria for this study. Of these, 91% self-identified as female, 7% as male, and 2% did not report. 78% self-identified as white/Caucasian, 7% as black/African American, 3% as Asian, 2% as Hispanic, and 9% did not report. Results for medication candidacy and usage are shown in the attached Figure. Notably, 95% of all patients were taking UDCA for treatment of their PBC. 67% of patients had PBC that was well-controlled on UDCA monotherapy. 8% of patients were taking OCA (either as combination or monotherapy). 3% of patients had a persistently elevated alkaline phosphatase despite appropriate treatment with UDCA and would benefit from the addition of OCA but had not been offered the medication. Only 3% of patients were not on any medication for management of PBC. Conclusion: Despite prior investigations suggesting that a large proportion of PBC patients may be unmedicated for the disease, the data presented here suggest that PBC patients are generally being managed according to guidelines in our health system. The majority of patients have disease that is well-controlled with UDCA monotherapy. However, OCA remains an important adjunctive/alternative therapy in certain cases, and physicians should be aware of indications for its use.Figure 1.: Medication candidacy and usage among patients diagnosed with PBC. UDCA – Ursodeoxycholic Acid; OCA – Obeticholic Acid; ALP – Alkaline Phosphatase; ULN – Upper Limit of Normal. Shown are all patients with a diagnosis of PBC who have records within the health system. Patients were stratified on the basis of medication usage and candidacy for OCA. The number of patients in each category is displayed in parentheses. OCA candidacy is considered to be one or more of the following: failure of ALP to decrease to within 1.67 times the upper limit of normal after one year of therapy with UDCA at appropriate weight-based dosing; inability to tolerate UDCA at appropriate weight-based dosing. * Severe liver disease is defined as one or more of the following: Child-Pugh B/C categorization; presence of portal hypertension; history of liver decompensation. Severe liver disease is a contraindication to the use of OCA

Comparison of the long-term outcomes of patients with hepatocellular carcinoma within the Milan criteria treated by ablation, resection, or transplantation.

Liver transplantation (LT), resection (LR), and ablation (LA) are three curative-intent treatment options for patients with early hepatocellular carcinoma (HCC). We aimed to develop a prognostic calculator to compare the long-term outcomes following each of these therapies. A total of 976 patients with HCC within the Milan criteria who underwent LT, LR, and LA between 2009 and 2019 from four institutions were evaluated. Multistate competing risks prediction models for recurrence-free survival (RFS), recurrence within the Milan criteria (RWM), and HCC-specific survival (HSS) were derived to develop a prognostic calculator. During a median follow-up of 51 months, 420 (43%) patients developed recurrence. In the multivariate analysis, larger tumor size, multinodularity, older age, male, higher alpha-fetoprotein (AFP), higher albumin-bilirubin (ALBI) grade, and the presence of portal hypertension were significantly associated with higher recurrence and decreased survival rates. The RFS and HSS were both significantly higher among patients treated by LT than by LR or LA and significantly higher between patients treated by LR than by LA (all p< 0.001). For multinodular HCC ≤3cm, although LT had better RFS and HSS than LR or LA, LA was noninferior to LR. An online prognostic calculator was then developed based on the preoperative clinical factors that were independently associated with outcomes to evaluate RFS, RWM, and HSS at different time intervals for all three treatment options. Although LT resulted in the best recurrence and survival outcomes, LR and LA also offered durable long-term alternatives. This prognostic calculator is a useful tool for clinicians to guide an informed and personalized discussion with patients based on their tumor biology and liver function.

90-day Case-Fatality in Critically ill Patients with Chronic Liver Disease Influenced by Presence of Portal Hypertension, Results from a Multicentre Retrospective Cohort Study

Critical illness in patients with chronic liver disease (CLD) is increasing in occurrence, and by virtue of its adverse effect on prognosis, its presence may influence the decision to offer admission to intensive care units (ICU). Our objective was to examine the determinants and outcome of patients with CLD admitted to ICU. A retrospective cohort of patients admitted to four adult ICUs in Queensland, Australia from 2017 to 2019. Patients with mild or moderate-severe CLD were defined by the absence and presence of portal hypertension, respectively, and were was determined using granular ICU and state-wide administrative databases. The primary outcome was 90-day all cause case-fatality. We included 3836 patients in the analysis, of which, 60 (2%) had mild liver disease and 132 (3%) had moderate-severe liver disease . Patients with CLD had higher incidence of other co-morbidities with the median adjusted-Charlson co-morbidity index (CCI) was 1 (interquartile range; IQR 0-3) for no CLD, 2 (IQR 1.5-4) for mild CLD, and 3 (IQR 2-5) for moderate-severe CLD. Case-fatality rates at 90 days was 17% for no CLD, 25% for mild CLD, and 41% for moderate-severe CLD. Among those with mild and moderate-severe CLD, an increased co-morbidity burden as measured by an adjusted CCI score of low (0-3), medium (4-5), high (6-7) and very high (>7) resulted in increasing case-fatality rates of 24-40%, 11-28.5%, 33-62%, and 50% respectively. Moderate-severe CLD, but not mild CLD, was independently associated with increased case-fatality at 90 days (Odds Ratio 1.58; 95% confidence interval 1.01-2.48; p = 0.004) after adjusting for medical co-morbidities and severity of illness using logistic regression analysis. Although patients with moderate-severe CLD have an increased risk for 90-day case-fatality, patients with mild CLD are not at higher risk for death following ICU admission.

A HRG novel mutation associated with idiopathic portal hypertension: Case report and literature review

Idiopathic portal hypertension (IPH) is defined as the presence of portal hypertension in the absence of a common cause. IPH can have several etiologies, one of which is a genetic disorder. Some genetic mutations, such as KCNN3 and DGUOK, were shown to be related to IPH pathogenesis. This is the first case report of a 22-year-old man who was diagnosed with IPH with a novel heterozygous mutation in the histidine-rich glycoprotein gene (c.545G > C, p.R182T). Using bioinformatics analysis and the protein quantification method, we showed that this novel mutation has a pathogenetic role in IPH. Our study broadens the mutation spectrum of the histidine-rich glycoprotein gene and provides new ideas for IPH etiology.

The severity of portal hypertension by a non-invasive assessment: acoustic structure quantification analysis of liver parenchyma

BackgroundAcoustic structure quantification (ASQ) has been applied to evaluate liver histologic changes by analyzing the speckle pattern seen on B-mode ultrasound. We aimed to assess the severity of portal hypertension (PHT) through hepatic ultrasonography.MethodsSixty patients diagnosed with PHT and underwent surgical treatment with portosystemic shunts were enrolled. Portal pressure (PP) was measured intraoperatively. Patients were divided into subgroups according to the severity of gastroesophageal varices and Child–Pugh class. Three difference ratio (Cm2) values on ASQ histogram mode were analyzed for their relationships with PP, degree of gastroesophageal varices and Child–Pugh liver function. Thirty healthy volunteers matched with the patients for gender and age were enrolled as controls. Comparisons among groups and correlation of the parameters with PP were analyzed. Area under the receive operating characteristic curve was used to evaluate the predicting value of ASQ parameters.ResultsIn the patients, the ASQ parameters peak Cm2 (Cm2max), mean Cm2 (Cm2mean) and the highest occurred Cm2 value of the obtained red curve (RmaxCm2) were all greatly increased (P < 0.0001, P < 0.0001, P = 0.027). Multiple comparisons indicated that, regardless of Child–Pugh class and degree of gastroesophageal varices, the patients had significantly increased Cm2max and Cm2mean compared with the controls (all P < 0.0001). No differences among subgroups were observed. Cm2max was significantly statistically correlated with PP (r = 0.3505, P < 0.01), degree of varices (r = 0.4998, P < 0.0001). Youden’s index for Cm2max with a cut-off value of 140.3 for predicting the presence of PHT, gastroesophageal varices and liver function equal to or worse than Child–Pugh class B were 0.8, 0.91 and 0.84, respectively.ConclusionsASQ analysis of ultrasonographic images may have a role in the evaluation of the severity of PHT by detecting liver histologic changes in the speckle pattern caused by cirrhosis.

A Brief Review on Hepatopulmonary Syndrome

Hepatopulmonary Syndrome is defined as decreased saturation of arterial oxygen due to dilatation of pulmonary vasculature in the presence of portal hypertension or advanced liver disease. In the year 1977, hepatopulmonary syndrome was first proposed based on the clinical findings and autopsy. In the patients with chronic liver disease, pulmonary manifestations like pulmonary vascular dilatation were observed in the autopsies.In the liver disease setting, dyspnea is usually present in the patient. The onset of dyspnea is gradual and aggravates by exertion. Most of the patients are asymptomatic during the early stages. This review article mainly focuses on the epidemiology, pathophysiology, clinical manifestations, diagnosis along with the treatment and management. A multidisplinary approach is required with the involvement of hepatologists, pulmonologists, primary clinicians, addiction medicine specialist, transplant surgeons and clinical pharmacists in the management of HPS.

Diagnostic Value of Portal Vein Velocity for Portal Hypertension in Patients with Hepatitis B Virus-related Cirrhosis.

Portal vein velocity (PVV) has shown a reasonable correlation with the presence of portal hypertension in patients with cirrhosis. This study aims to evaluate the value of PVV for diagnosing clinically significant portal hypertension (CSPH) and predicting the risk of variceal hemorrhage (VH) in patients with hepatitis B virus (HBV)-related cirrhosis. A cohort of 166 consecutive adult patients with HBV-related cirrhosis was recruited in this retrospective study from two high-volume liver centers in China between April 2015 and April 2017. The performance of PVV and other non-invasive parameters for diagnosing CSPH and predicting the risk of VH was studied. PVV demonstrated the best performance for diagnosing CSPH (defined as an HVPG ≥10 mmHg) in patients with HBV-related cirrhosis among the included non-invasive predictors with the area under the receiver operating characteristic curve (AUC), specificity, and sensitivity of 0.745, 50%, and 93.5%, respectively. Other non-invasive markers, including APRI, AAR, LS, FIB-4, and diameter of the portal vein, did not show sufficient performance with the AUCs of 0.565, 0.560, 0.544, 0.529, and 0.474, respectively. With regard to predicting the risk of VH (defined as an HVPG ≥12 mmHg), PPV also exhibited a moderate performance with an AUC of 0.762, which was superior to that of the aforementioned markers. By using two cutoff values of PVV to rule-out (11.65 cm/s) and rule-in (20.20 cm/s) CSPH, 30 (33.7%) patients showed definite results categories, with 23 (76.7%) patients were well classified and 7 (23.3%) were misclassified. Fifty-nine (66.3%) patients were with indeterminate results. By using PVV values of 13.10 cm/s and 21.40 cm/s to rule-out and rule-in HVPG ≥ 12mmHg, 34 (38.2%) patients has definite results, among whom 26 (76.5%) were well classified and 8 (23.5%) were misclassified. And 55 (61.8%) patients required further evaluation. PPV is insufficient to serve as a non-invasive parameter for identifying CSPH and predicting the risk of VH in patients with HBV-related cirrhosis.

Особливості діагностики енцефалопатії у хворих з синдромом портальної гіпертензії

Актуальність. Печінкова енцефалопатія — патологічні зміни функцій головного мозку незапального генезу, що проявляються синдромом вторинних неврологічних і когнітивних розладів, спричинених комплексом глибоких метаболічних порушень, в результаті гострої печінкової недостатності, хронічних захворювань печінки або портосистемного шунтування крові. Початкова стадія енцефалопатії не має клінічних неврологічних симптомів, але визначається у 20–80 % хворих на цироз печінки і на відміну від кінцевих стадій є оборотною. Мета дослідження: оцінити ефективність тесту зв’язку чисел, флікер-тесту та електроенцефалографії в діагностиці енцефалопатії у хворих з печінковою та позапечінковою формою портальної гіпертензії. Матеріали та методи. Синдром печінкової енцефалопатії вивчено у 50 хворих з печінковою формою портальної гіпертензії, 30 хворих з позапечінковою формою портальної гіпертензії та 25 осіб без ознак портальної гіпертензії. Для діагностики використовували тест зв’язку чисел, флікер-тест та електроенцефалографію. Результати. Тест зв’язку чисел дозволив встановити ознаки печінкової енцефалопатії майже в усіх хворих незалежно від наявності та форми портальної гіпертензії. Однак прояви печінкової енцефалопатії за частотою і за вираженістю переважали у хворих з печінковою портальною гіпертензією. Проведення флікер-тесту дозволило діагностувати мінімальну печінкову енцефалопатію у 96 % хворих з печінковою портальною гіпертензією та 60 % хворих з позапечінковою портальною гіпертензією. Виконання електроенцефалографії визначило наявність печінкової енцефалопатії у 60 % хворих з печінковою портальною гіпертензією, 50 % хворих з позапечінковою портальною гіпертензією та 12 % хворих без портальної гіпертензії. Висновки. Зіставлення ефективності діагностичних тестів показало, що простий у виконанні і доступний тест зв’язку чисел є найбільш ефективним, однак і інші тести мають свої позитивні сторони в клінічній оцінці печінкової енцефалопатії у хворих з портальною гіпертензією.