{"title":"Clinical, Histopathological, and Immunophenotypic Spectrum of Hepatic Epithelioid Hemangioendothelioma: Eight Years’ Data of a Tertiary Care Center from North India","authors":"Gunjangeet Kaur , Suvradeep Mitra , Adarsh Barwad , Debajyoti Chatterjee , Treshita Dey , Divya Khosla , Uma N. Saikia , Lileshwar Kaman , Usha Dutta , Ajay Duseja , Ashim Das","doi":"10.1016/j.jceh.2024.102429","DOIUrl":"10.1016/j.jceh.2024.102429","url":null,"abstract":"<div><h3>Background/Aims</h3><div>Epithelioid hemangioendothelioma (EHE) is an uncommon vascular tumor that commonly affects the liver. Hepatic EHE (HEHE) presents with variable clinical and histopathological features. We describe detailed clinico-histopathological features, differential diagnosis, and treatment outcomes of the cases of HEHE diagnosed in our center.</div></div><div><h3>Methods</h3><div>All cases of HEHE diagnosed in our institute in the last eight years (2016–2023) were reviewed (n = 8; 11 samples) (total 36 cases of EHE; 22.2%). The clinical features, radiology, histopathology, immunophenotype, molecular features, and treatment outcomes of all cases were evaluated.</div></div><div><h3>Results</h3><div>The median age of presentation was 49.5 years with a female: male ratio of 7:1. Abdominal pain was the commonest presentation. Approximately two-thirds of the patients had multifocal lesions. Histopathology showed purely epithelioid, predominantly epithelioid, and predominantly spindle-cell morphology in 50%, 25%, and 25%, respectively. All cases showed typical myxohyaline/fibrous stroma and organized thrombi of the portal/central veins. CD31 was the most commonly used immunostain with positivity in all cases. CAMTA1 break-apart fluorescence <em>in situ hybridization</em> was positive in 75% of cases, while none showed TFE3 immunopositivity. Chemotherapy was the most commonly employed therapy (n = 5) followed by surgery (n = 2). The median duration of follow-up was 26 months. Five patients were alive with disease (two patients ≥3 years), one patient died of sudden cardiac death, and two patients were lost to follow-up. Two patients developed metastatic disease at follow-up.</div></div><div><h3>Conclusions</h3><div>We describe the clinico-histopathological features and differential diagnosis of HEHE. This appears to be the largest case series of HEHE from India.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"15 1","pages":"Article 102429"},"PeriodicalIF":3.3,"publicationDate":"2024-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142587175","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Snigdha Verma, Vikrant Sood, Bikrant B. Lal, Rajeev Khanna, Seema Alam
{"title":"Metabolic Liver Diseases Presenting as Pediatric Onset Hypoglycemia: A Hepatologist's Primer","authors":"Snigdha Verma, Vikrant Sood, Bikrant B. Lal, Rajeev Khanna, Seema Alam","doi":"10.1016/j.jceh.2024.102425","DOIUrl":"10.1016/j.jceh.2024.102425","url":null,"abstract":"<div><div>Hypoglycemia, especially when recurrent or persistent, is an important indicator of inborn metabolic errors. Although commonly encountered by hepatologists, it continues to be a pandora's box as no consensus on the exact definition and diagnostic work up exists. Here, we present four interesting pediatric cases of varied age groups, presenting with hypoglycemia as their major symptomatology. We also attempted to provide a systematic diagnostic guide for a refined and targeted approach to inherited metabolic liver diseases presenting with hypoglycemia.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"15 1","pages":"Article 102425"},"PeriodicalIF":3.3,"publicationDate":"2024-10-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142573049","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Monalisa Sahu , Dibyalochan Praharaj , Ajeet S. Bhadoria
{"title":"Vaccination Strategies for a Liver Transplant Recipient","authors":"Monalisa Sahu , Dibyalochan Praharaj , Ajeet S. Bhadoria","doi":"10.1016/j.jceh.2024.102421","DOIUrl":"10.1016/j.jceh.2024.102421","url":null,"abstract":"<div><div>Patients with cirrhosis and liver transplant recipients are at increased risk of infections. Malnutrition, multiple hospital admissions, immune dysfunction related to cirrhosis, and immunosuppressive agents used for liver transplantation predispose the recipient to various life-threatening infections. Some of these infections are preventable with vaccines. With the COVID-19 pandemic, there has been an accelerated research in vaccination technology and platforms, which in turn may also improve awareness of physicians regarding this healthy and often ignored aspect of management of patients with cirrhosis and transplant recipients. The organ transplant candidates should complete the recommended vaccination schedule as early as possible (especially patients with compensated cirrhosis) or at least during their pretransplant work-up so as to prevent or reduce the severity of various infections.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"15 2","pages":"Article 102421"},"PeriodicalIF":3.3,"publicationDate":"2024-10-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142663024","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Lasse Pedersen , Lotte L. Eriksen , Frederik H. Brix , Hendrik Vilstrup , Bent Deleuran , Thomas D. Sandahl , Sidsel Støy
{"title":"The FGL-1/LAG-3 Axis is Associated With Disease Course in Alcohol-associated Hepatitis: A Preliminary Report","authors":"Lasse Pedersen , Lotte L. Eriksen , Frederik H. Brix , Hendrik Vilstrup , Bent Deleuran , Thomas D. Sandahl , Sidsel Støy","doi":"10.1016/j.jceh.2024.102424","DOIUrl":"10.1016/j.jceh.2024.102424","url":null,"abstract":"<div><h3>Background</h3><div>Alcohol-associated hepatitis (AH) has a short-term mortality rate of up to 40% primarily related to impaired hepatocyte regeneration and uncontrolled liver inflammation. The acute phase protein fibrinogen-like protein 1 (FGL-1) produced by hepatocytes stimulates hepatocyte proliferation by autocrine signaling. FGL-1 also is a ligand for the inhibitory T cell receptor lymphocyte activation gene 3 (LAG-3). In these ways, FGL-1 and LAG-3 have beneficial interactions that could be interrupted in AH.</div></div><div><h3>Aims</h3><div>We aimed to characterize FGL-1 and LAG-3 in patients with AH and describe their relationship with the disease state and course.</div></div><div><h3>Methods</h3><div>Thirty-two patients with AH were included at diagnosis and followed up for 3 years. We measured the hepatic gene expression of FGL-1 and LAG-3 using RNA sequencing, plasma FGL-1 and soluble (s)LAG-3 using ELISA, and LAG-3<sup>+</sup>CD8<sup>+</sup> T cells using flow cytometry. Healthy persons (HC) and patients with stable alcohol-associated cirrhosis served as controls.</div></div><div><h3>Results</h3><div>At diagnosis of AH, liver FGL-1 mRNA was increased when compared to HC, whereas plasma FGL-1 was unchanged. In contrast, liver LAG-3 mRNA was reduced in AH. Plasma sLAG-3 levels and the frequency of LAG-3<sup>+</sup>CD8<sup>+</sup> T cells were as in HC. However, those patients who had the lowest plasma FGL-1 and the lowest frequency of LAG-3<sup>+</sup>CD8<sup>+</sup> T cells at diagnosis had the highest disease severity and mortality.</div></div><div><h3>Conclusions</h3><div>Our data suggest that an impaired FGL-1/LAG-3 axis may be involved in the pathogenesis and course of AH.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"15 1","pages":"Article 102424"},"PeriodicalIF":3.3,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142573047","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Narendra S. Choudhary, Kunwar A. Singh, Swapnil Dhampalwar, Neeraj Saraf, Virendra Singh
{"title":"Acute Hepatitis C as an Acute Cause of Acute-on-chronic Liver Failure in Alcohol-associated Liver Disease","authors":"Narendra S. Choudhary, Kunwar A. Singh, Swapnil Dhampalwar, Neeraj Saraf, Virendra Singh","doi":"10.1016/j.jceh.2024.102423","DOIUrl":"10.1016/j.jceh.2024.102423","url":null,"abstract":"","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"15 1","pages":"Article 102423"},"PeriodicalIF":3.3,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142573048","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Xinjuan Huang , Ruoling Yu , Xinyun Tan , Manjie Guo , Yuanqin Xia , Huihui Zou , Xuelian Liu , Chunxiang Qin
{"title":"Comparison of NAFLD, MAFLD, and MASLD Prevalence and Clinical Characteristics in Asia Adults","authors":"Xinjuan Huang , Ruoling Yu , Xinyun Tan , Manjie Guo , Yuanqin Xia , Huihui Zou , Xuelian Liu , Chunxiang Qin","doi":"10.1016/j.jceh.2024.102420","DOIUrl":"10.1016/j.jceh.2024.102420","url":null,"abstract":"<div><h3>Background/Aims</h3><div>The principal limitations of the term non-alcoholic fatty liver disease (NAFLD) are the reliance on exclusionary confounder terms and the use of potentially stigmatizing language. Within three years, NAFLD went through two name changes, from NAFLD to metabolic-dysfunction-associated fatty liver disease (MAFLD) to metabolic dysfunction-associated steatotic liver disease (MASLD). However, there is no Asian consensus statement on the renaming of MASLD, and evidence on the epidemiology and characteristics in the Asia population under different diagnostic criteria remain limited. This study aimed to fill these gaps by analyzing the prevalence and characteristics of MASLD, NAFLD, and MAFLD in an Asian population.</div></div><div><h3>Methods</h3><div>A retrospective, cross-sectional study was conducted in regional China with participants from the health management database in 2017–2022. Demographic and laboratory metabolic profile and body composition data were obtained. Hepatic steatosis were diagnosed by ultrasound. The likelihood of having fibrosis was assessed using the NAFLD fibrosis score (NFS). Recently proposed criteria for metabolic dysfunction-associated steatotic liver disease (MASLD) were applied.</div></div><div><h3>Results</h3><div>A total of 20,226 subjects were included for final analysis. 7465 (36.91%) participants were categorized as MASLD patients, 10,726 (53.03%) participants were MAFLD, and 7333 (36.26%) participants were NAFLD. Compared with MAFLD, body composition of MASLD and NAFLD patients were obviously different. MASLD patients were older, had a higher body mass index and percentage of male gender, and had a higher ALT, diastolic blood pressure, triglyceride, and waist circumference but lower High-Density Lipoprotein Cholesterol (HDL-C) than non-MASLD patients. Using binary regression analysis, we found for the first time that putative bone mass (OR = 4.62, 95CI% 3.12–6.83) is associated with the risk of developing MASLD. The area under the receiver operating curve (AUC) for predicting cardiovascular outcomes (CV) was 0.644 for MAFLD and 0.701 for MASLD.</div></div><div><h3>Conclusion</h3><div>MASLD (36.91%) prevalence was closed to NAFLD (36.26%) and lower than MAFLD (53.03%). Presumed bone mass might be the predictor of disease progression in MASLD patients. MASLD better identifies patients likely to have a higher risk of metabolic disorders or CV events.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"15 1","pages":"Article 102420"},"PeriodicalIF":3.3,"publicationDate":"2024-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142587174","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mohamed Abuelazm , Ahmed Fares , Mahmoud M. Elhady , Ahmed Mazen Amin , Ubaid Khan , Ibrahim Gowaily , Fouad Jaber
{"title":"Branched-Chain Amino Acid Supplements for Sarcopenia in Liver Cirrhosis: A Systematic Review and Meta-analysis","authors":"Mohamed Abuelazm , Ahmed Fares , Mahmoud M. Elhady , Ahmed Mazen Amin , Ubaid Khan , Ibrahim Gowaily , Fouad Jaber","doi":"10.1016/j.jceh.2024.102417","DOIUrl":"10.1016/j.jceh.2024.102417","url":null,"abstract":"<div><h3>Background</h3><div>Sarcopenia, a key aspect of malnutrition in liver cirrhosis (LC), affects 30–70% of LC patients. Given the inconsistent results from RCTs on branched-chain amino acids (BCAAs) for treating sarcopenia in LC, we conducted a systematic review and meta-analysis to assess the efficacy and safety of BCAAs for sarcopenia management in LC patients.</div></div><div><h3>Methods</h3><div>A systematic review and meta-analysis synthesizing evidence from RCTs obtained from PubMed, Embase, Cochrane, Scopus, and Web of Science from inception to April 2024. We used the fixed-effects model to report dichotomous outcomes using risk ratio (RR) and continuous outcomes using mean difference (MD), with a 95% confidence interval (CI). PROSPERO ID: <span><span>CRD42024542761</span><svg><path></path></svg></span>.</div></div><div><h3>Results</h3><div>Five RCTs with 434 patients were included. BCAAs were significantly associated with decreased liver frailty index change (MD: −0.14 with 95% CI [-0.28, −0.01], <em>P</em> = 0.03). However, there was no significant difference between BCAAs and the control group regarding hand grip strength change (MD: 0.98 with 95% CI [-0.45, 2.41], <em>P</em> = 0.18). Also, BCAAs were associated with increased body mass index (BMI) change (MD: 0.99 with 95% CI [0.16, 1.82], <em>P</em> = 0.02) and increased QoL (standardized mean difference : 0.27 with 95% CI [0.03, 0.52], <em>P</em> = 0.03). However, there was no significant difference between BCAAs and the control group in model for end-stage liver disease (MELD) score change (MD: 0.65 with 95% CI [-1.20, 2.50], <em>P</em> = 0.49), skeletal muscle index change (MD: 0.21 with 95% CI [-0.23, 0.65], <em>P</em> = 0.35), and gait speed change (MD: 0.10 with 95% CI [-0.15, 0.34], <em>P</em> = 0.43).</div></div><div><h3>Conclusion</h3><div>BCAA supplementation in cirrhotic patients with sarcopenia reduced the liver frailty index, increased BMI and QoL, but did not affect handgrip strength, skeletal muscle index, gait speed, or MELD score. Outcome heterogeneity and study bias were noted, highlighting the need for further RCTs to confirm these results.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"15 1","pages":"Article 102417"},"PeriodicalIF":3.3,"publicationDate":"2024-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142698643","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"An Innovative Approach to Assessing the Psychosocial Impacts on Liver Transplant Recipients: The Prediction-by-correspondence Analysis","authors":"Se-Kang Kim , Rachel A. Annunziato","doi":"10.1016/j.jceh.2024.102418","DOIUrl":"10.1016/j.jceh.2024.102418","url":null,"abstract":"<div><h3>Background</h3><div>Innovative analytic techniques are applied to the psychological functioning of liver transplant (LT) recipients to comprehend its effect on post-transplant survival, hypothesizing that adherence will be predicted by psychosocial functioning.</div></div><div><h3>Methods</h3><div>The psychosocial functioning of 248 LT recipients (88 females) aged 19 to 74 is assessed using the Stanford Integrated Psychosocial Assessment for Transplantation (SIPAT). In addition, the Medication Level Variability Index (MLVI) and biopsy-proven rejection are utilized to evaluate successful adherence. The Z-scores of the SIPAT scores are used to transform them into an ordinal variable with excellent, good, minimally acceptable, and poor categories. We employ a modified version of correspondence analysis to predict the binary MLVI and rejection, which signify either success or failure in adherence, using ordinal MLVI categories as predictors.</div></div><div><h3>Results</h3><div>The excellent SIPAT category for LT beneficiaries was strongly related to adherence, whereas the minimally acceptable SIPAT was strongly related with failure in adherence. Females, ages 19–50, and ages 67–74 were associated with adherence (r = 0.49–1.00), whereas males and ages 56–60 were associated with failure in adherence (r = 0.43–0.91)</div></div><div><h3>Conclusion</h3><div>The clinical implications and utility of the novel analytic approaches introduced in the study are discussed.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"15 1","pages":"Article 102418"},"PeriodicalIF":3.3,"publicationDate":"2024-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142444588","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Arghya Samanta , Anshu Srivastava , Sangram S. Patel , Moinak Sen Sarma , Ujjal Poddar , Prabhakar Mishra
{"title":"“Parvovirus B19–related Acute Hepatitis: Clinical Spectrum and Outcome in Children”","authors":"Arghya Samanta , Anshu Srivastava , Sangram S. Patel , Moinak Sen Sarma , Ujjal Poddar , Prabhakar Mishra","doi":"10.1016/j.jceh.2024.102416","DOIUrl":"10.1016/j.jceh.2024.102416","url":null,"abstract":"<div><h3>Background/Aims</h3><div>Acute liver injury is a common manifestation of parvovirus B19 (PVB19) infection in immunocompromised patients. However, literature in immunocompetent children is scarce. We aimed to study the clinicolaboratory features and outcome of hepatic involvement by PVB19 infection in hospitalized children.</div></div><div><h3>Methods</h3><div>We retrospectively analyzed our prospectively kept database of all children (<18 years old) admitted with acute viral hepatitis (AVH), acute liver failure (ALF) or acute-on-chronic liver failure (ACLF), and PVB19 infection between January 2010 and December 2023. Clinical features, laboratory parameters, and complications were evaluated. Poor outcome was defined as death or liver transplantation.</div></div><div><h3>Results</h3><div>A total of 35 children (19 boys [54%], median age: 7.25 [interquartile range: 4–10.8] years) with PVB19-related hepatitis were studied (28 [80%] isolated PVB19 infection and 7 [20%] coinfections [3 with Epstein–Barr virus, 2 with hepatitis A, and 1 each with hepatitis-E and cytomegalovirus]). AVH (17, 49%) was the most common presentation, followed by ALF (13, 37%) and acute insult in ACLF (5, 14%). Patients with coinfection had significantly higher bilirubin (14.6 [9.4–21.5] vs 6.8 [4.3–10.9] mg/dl; <em>P</em>=0.004) and transaminases (ALT: 697 [428–1296] vs. 277 [157–478] U/L; <em>P</em>=0.02) but similar mortality (1/7 vs 6/23; <em>P</em>=1.0) than PVB19 alone. Nine cases (25.7%) had extrahepatic complications (hemophagocytic lymphohistiocytosis [HLH]: 3, acute kidney injury: 3, aplastic anemia: 2, and myocarditis: 1). Poor outcome occurred in 38% (5/13) ALF, 11.7% (2/17) AVH (HLH: 1, myocarditis: 1), and none (0/5) of the ACLF cases.</div></div><div><h3>Conclusion</h3><div>PVB19 should be considered in children presenting with indeterminate acute liver injury, especially in younger children or those with complications such as aplastic anemia, HLH, or myocarditis.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"15 1","pages":"Article 102416"},"PeriodicalIF":3.3,"publicationDate":"2024-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142444587","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}