Cruveilhier-Baumgarten syndrome presents a rare manifestation of portal hypertension characterized by a portosystemic shunt through a dilated paraumbilical vein, typically accompanied by classical signs such as caput medusae and a venous hum. We report a compelling case of a 41-year-old male presenting with portal hypertension, exhibiting clinical and radiological features of Cruveilhier-Baumgarten syndrome but notably lacking the characteristic venous hum. Clinical examination revealed moderate splenomegaly with prominent dilated veins and venous thrill but no caput medusae. Laboratory investigations indicated thrombocytopenia and esophageal varices on upper GI endoscopy. Imaging studies confirmed portal hypertension with findings consistent with Cruveilhier-Baumgarten syndrome, including a dilated paraumbilical vein and splenic artery aneurysms, along with the unexpected absence of a venous hum. Despite the classical radiological features, our patient did not present with hematemesis, possibly attributed to the presence of paraumbilical veins. This case highlights the diagnostic challenges and atypical presentations of Cruveilhier-Baumgarten syndrome, emphasizing the importance of comprehensive clinical evaluation and imaging modalities in its diagnosis and management. Management strategies primarily focus on addressing portal hypertension and underlying liver disease. This case underscores the need for further research to elucidate the varied clinical presentations and pathophysiology of Cruveilhier-Baumgarten syndrome variants, enhancing our understanding and management of this rare entity.

BACKGROUND: Autoimmune hepatitis (AIH) is uncommon and predominantly affects females. Data on AIH from India are scanty. We retrospectively analyzed the spectrum and outcome of adults with AIH and compared it between male and female patients.
METHODS: AIH was diagnosed using a simplified AIH score. For suspected seronegative AIH, the revised score was used. Standard therapies for AIH and portal hypertension were administered and response was assessed at six months. Relapse rates and five-year mortality were also evaluated.
RESULTS: Of the 157 patients with AIH, 85 (male: female 25: 60) were included in the study. The median age at diagnosis was 46 (interquartile range (IQR) 32-55.5) years in males vs 45 (IQR 34.2-54) years in females (p=0.91). A similar proportion of male and female patients presented with cirrhosis, acute severe AIH, or AIH-related acute on chronic liver failure (ACLF); Extra-hepatic autoimmune diseases were less common in male patients (16% vs 35.5% p=0.02). Other laboratory and histological features were comparable in both groups. During the median follow-up period of 51 months (IQR 45-67 months). The biochemical and clinical response at six months were seen in 64% of male patients and 63.3% of female patients (p= 0.57). Of patients, 75% relapsed in the male AIH group (12 of 16 patients) after initial remission compared to 42% in the female group (p=0.02). Five-year mortality was 14.1%, and no patient developed hepatocellular carcinoma.
CONCLUSIONS: Male and female patients with AIH have similar clinical, biochemical, and histological profiles. More male patients relapsed after an initial response to therapy.

Objectives This study aimed to identify the causes, clinical characteristics, and 28-day in-hospital mortality predictors in patients with acute-on-chronic liver failure (ACLF). Methods A cross-sectional study enrolled sixty-four patients aged 18-70 years with acute-on-chronic liver failure. The study was conducted at the Gastroenterology Department, Lahore General Hospital. The study classified ACLF according to the criteria of the European Association for the Study of the Liver - Chronic Liver Failure (EASL-CLIF). Patients were followed for 28 days for mortality outcomes. The outcomes between Survivor and Non-survivor groups were compared using the Chi-Square/Fisher\'s Exact Test for categorical variables and the Mann-Whitney U test for continuous variables. Results In this study, age and duration of chronic liver disease were not significantly different between survivors and non-survivors. The etiology of liver disease and ACLF causes had no impact on 28-day mortality. Non-survivors had lower mean arterial pressure, and higher mortality was linked with lower Glasgow Coma Scale scores, upper gastrointestinal bleeding, and Grade IV hepatic encephalopathy. Significant differences in bilirubin, serum creatinine, urea, and C-reactive protein levels were observed at 28 days. Survival rates were highest with single organ failure (35.94%) and decreased with multiple organ failures. The overall survival rate was 51.56%. Predictive validity for mortality was assessed using the Area Under the Curve (AUC), with Child-Turcotte-Pugh (CTP) at 0.679, Model for End-Stage Liver Disease (MELD) at 0.819, and Chronic Liver Failure-Sequential Organ Failure Assessment (CLIF-SOFA) at 0.771. Conclusion This study concludes that in acute-on-chronic liver failure, factors like age, gender, and disease etiology do not significantly predict 28-day mortality. Key mortality indicators include clinical parameters such as lower Glasgow Coma Scale scores, hepatic encephalopathy Grade IV, and laboratory findings like elevated bilirubin and serum creatinine. The MELD score is the most compelling prognostic tool.

UNASSIGNED: Chronic liver disease (CLD) is one of the important causes of morbidity and mortality in our country, and since the damage to the liver is irreversible, we have to look for many severity markers or predictors for the prognosis of the patient. In this study, we have tried to correlate the level of serum uric acid (UA) with the severity of CLD presented as a Child-Pugh score.
UNASSIGNED: A cross-sectional observational study was conducted at Vijayanagar Institute of Medical Science (VIMS), Ballari, Karnataka, from October 2015 to June 2017 in the Department of General Medicine. Fifty patients diagnosed with CLD, aged between 18 and 65 years, of either gender, were enrolled in the study. Serum UA levels were measured, and liver function and coagulation parameters were assessed. A statistical analysis was performed to evaluate the association between serum UA levels, liver function test, and coagulation parameters.
UNASSIGNED: In our study, the mean serum UA level was 6.52 mg/dl and was raised in patients with CLD in correlation to its severity. Alcoholic liver disease (ALD) was the most common etiology for CLD (80%) followed by hepatitis B (Hep B) virus infection (12%) and hepatitis C (Hep C) virus infection (6%). Serum UA levels increased as the Child-Turcotte-Pugh (CTP) score increased. The mean UA level in CTP class C was 8.29 mg/dl. Various parameters such as serum aspartate aminotransferase (AST), alanine transaminase (ALT), alkaline phosphatase, total bilirubin, international normalized ratio (INR), calcium, and albumin were significantly associated with serum UA levels in CLD patients.
UNASSIGNED: The correlation between rising blood UA levels and the Child-Pugh score shows that UA estimate may be a valid and affordable indicator for assessing the extent of liver cirrhosis in CLD.

OBJECTIVE: Hepatocellular carcinoma (HCC) affects millions of people each year and is associated with high mortality and morbidity. Sarcopenia, a condition of muscle wasting, and decreased muscle performance is common among aging adults, and is associated with poor clinical outcomes. Individuals with HCC and chronic liver disease (CLD) are at high risk of sarcopenia because of the adverse effects of chronic inflammation, endocrine dysfunction, and hyperammonemia on muscle metabolism and adequate nutrition. Our aim is to review the clinical relationship between HCC and sarcopenia, and the assessment and management of these patients.
METHODS: A narrative review based on a literature search using PubMed. Keywords related to HCC and sarcopenia were used to identify relevant articles, primarily those published 2018-2023. The information was synthesized to provide a narrative review focused on the most recent literature.
UNASSIGNED: Sarcopenia frequently co-exists with HCC and increases risk for adverse clinical outcomes such as symptom burden, quality of life (QoL), survival, and side effects of antineoplastic therapy. Tools are available to screen, assess and manage patients with HCC, and although there is no specific pharmacologic agent approved for sarcopenia in the United States, multimodal therapy is feasible in daily practice. Comprehensive management by an interdisciplinary team should include nutritional counseling, an exercise regimen and control of symptoms affecting nutrition and function.
CONCLUSIONS: Sarcopenia has adverse effects on prognosis and tolerability of surgical and medical therapy in HCC. Patients with CLD and/or HCC would benefit from early identification, assessment, and therapeutic intervention. Management should be comprehensive, interdisciplinary, and include both pharmacologic and non-pharmacologic treatments. Further research is needed to identify individual agents that may mitigate muscle wasting and trials are needed to evaluate the benefit of multimodal therapy in HCC.

It is well established that direct oral anticoagulants (DOACs) are the cornerstone of anticoagulant strategy in atrial fibrillation (AF) and venous thromboembolism (VTE) and should be preferred over vitamin K antagonists (VKAs) since they are superior or non-inferior to VKAs in reducing thromboembolic risk and are associated with a lower risk of intracranial hemorrhage (IH). In addition, many factors, such as fewer pharmacokinetic interactions and less need for monitoring, contribute to the favor of this therapeutic strategy. Although DOACs represent a more suitable option, several issues should be considered in clinical practice, including drug-drug interactions (DDIs), switching to other antithrombotic therapies, preprocedural and postprocedural periods, and the use in patients with chronic renal and liver failure and in those with cancer. Furthermore, adherence to DOACs appears to remain suboptimal. This narrative review aims to provide a practical guide for DOAC prescription and address challenging scenarios.

Chronic liver disease (CLD) is a persistent public health burden, with over one billion cases reported worldwide. In most cases, the progression of CLD is slow and undulating with end-stage liver disease developing at variable time points depending on the underlying etiology of the disease. The concept of reversibility or halting progression to end stage liver disease is recent and various medications are in the pipeline which influence the progression of CLD. Non-invasive tests for monitoring of CLD may have the potential to avoid the morbidity and mortality related to invasive procedures. However, their applicability and validation in pediatrics requires further development and a coordinated effort by large pediatric liver centres. Recent advances in metabolomics and modern molecular technologies have led to an understanding of the interaction between gut microbiome liver axis and gut dysbiosis contributing to liver diseases. In the future, modifying the gut microbiome has the potential to change the outcome and significantly reduce the morbidity associated with CLD. This article focuses on newer modalities and concepts in the management of CLD, which may help develop strategies to prevent its progression to end-stage liver disease and associated morbidity/mortality.

Background Zinc, an essential trace element, plays a vital role in cellular metabolism, and the liver is the main organ responsible for its metabolism. Because serum zinc levels are found to be lowered in chronic liver diseases, it has been hypothesized to be a precipitating factor for the development of hepatic encephalopathy. Methodology This prospective, observational study included patients with decompensated cirrhosis of the liver who were admitted to the medical intensive care unit of a tertiary care institute in northern India between September 2021 and April 2023. The diagnosis was based on history and detailed clinical examination. The serum zinc levels of patients were estimated using atomic absorption spectrometry at admission and compared to that of healthy controls. Serum zinc levels were correlated with the severity of liver disease and hepatic encephalopathy among the cases. Results A total of 100 cases of decompensated cirrhosis of the liver and 50 healthy controls were included. The mean serum zinc level of the cases was 40.5 ± 10.0 µg/dL which was significantly lower than the mean serum zinc level (104.0±9.1 µg/dL) of controls (p < 0.0001). Serum zinc level was significantly lower in patients with higher grades of hepatic encephalopathy (p = 0.000). Similarly, serum zinc level was significantly reduced among patients with higher Child-Pugh and Model for End-stage Liver Disease scores. Conclusions Serum zinc level is significantly reduced in patients with decompensated cirrhosis of the liver, and lower serum zinc level is associated with the increased severity of the disease and higher grades of hepatic encephalopathy. In patients with decompensated cirrhosis of the liver, maintenance of adequate serum zinc levels may prevent hepatic encephalopathy.

Chronic liver disease (CLD) entails elevated risk of COVID-19 severity and mortality. The effectiveness of the booster dose of inactivated SARS-CoV-2 vaccine in stimulating antibody response in CLD patients is unclear. Therefore, we conducted a cross-sectional study involving 237 adult CLD patients and 170 healthy controls (HC) to analyze neutralizing antibodies (NAbs) against SARS-CoV-2 prototype and BA.4/5 variant, anti-receptor binding domain (RBD) IgG, and total anti-SARS-CoV-2 antibodies. Serum levels of the total anti-SARS-CoV-2 antibodies, anti-RBD IgG and inhibition efficacy of NAbs were significantly elevated in CLD patients after the booster dose compared with the pre-booster dose, but were relatively lower than those of HCs. Induced humoral responses decreased over time after booster vaccination. The neutralization efficiency of the serum against BA.4/5 increased but remained below the inhibition threshold. All four SARS-CoV-2 antibodies, including total anti-SARS-CoV-2 antibodies, anti-RBD IgG and NAbs against prototype and BA.4/5, were lower in patients with severe CLD than those with non-severe CLD. After booster shot, age and time after the last vaccine were the risk factors for seropositivity of NAb against BA.4/5 in CLD patients. Additionally, white blood cell counts and hepatitis B core antibodies were the protective factors, and severe liver disease was the risk factor associated with seropositivity of total anti-SARS-CoV-2 antibodies. Overall, our data uncovered that antibody responses were improved in CLD patients and peaked at 120 days after the booster vaccines. All antibodies excepting total anti-SARS-CoV-2 antibodies declined after peak. CLD patients exhibited impaired immunologic responses to vaccination and weakened NAbs against BA.4/5, which hindered the protective effect of the booster shot against Omicron prevalence. Cellular immune responses should be further evaluated to determine the optimal vaccine regimen for CLD patients.

Background Portal hypertension leads to the formation of portosystemic collateral veins, of which esophageal varices (EV) are the most severe complications and have the greatest clinical impact. The possibility of identifying cirrhotic patients with varices by non-invasive tests is appealing, as they can lead to reduced healthcare costs and can be done in resource-limited settings. In this study, we investigated ammonia as a potential non-invasive predictor of EV. Methods This was a single-center cross-sectional observational study that was done at a tertiary health care hospital in north India. It included 97 chronic liver disease patients irrespective of etiology after excluding patients with portal vein thrombosis and hepatocellular carcinoma to participate in endoscopic screening for the presence of EV and correlate it with various non-invasive markers like serum ammonia levels, thrombocytopenia and aspartate aminotransferase to platelet ratio index (APRI ). On the basis of endoscopy, enrolled patients were divided into two groups, i.e., group A consisting of large varices (grade III and grade IV) and group B consisting of patients with low-grade varices and no varices (grade II, grade I, and no varices). Results This study included 97 patients, out of which 81 patients have varices on endoscopy, and mean serum ammonia levels were found to be significantly higher in cases with varices (135 ±69.70 ) vs. those without varices (94±43) (p value=0.026). Further, on comparing serum ammonia values between patients with large varices (Grade III/IV) (Group A) with a mean value of 176 ± 83 vs. Grade I/II/No varices (Group B) with a mean value of 107±47, which were significantly higher in Group A patients (<0.001). In our study, we also found a correlation between blood urea level as a non-invasive predictor of varices, but no statistically significant relation was found between thrombocytopenia and APRI. Conclusion This study found that serum ammonia can be used as a useful marker for the prediction of EV and can also be used to determine the severity of varices. Apart from ammonia, serum urea levels can also prove to be a good non-invasive marker for the prediction of varices although further multicentric studies are warranted to reach this conclusion.