Wesley Dixon , Kathleen E. Corey , Jay Luther , Russell P. Goodman , Esperance A. Schaefer
{"title":"Prevalence and Clinical Correlation of Cardiometabolic Risk Factors in Alcohol-Related Liver Disease and Metabolic Dysfunction and Alcohol Associated Liver Disease (MetALD)","authors":"Wesley Dixon , Kathleen E. Corey , Jay Luther , Russell P. Goodman , Esperance A. Schaefer","doi":"10.1016/j.jceh.2024.102492","DOIUrl":"10.1016/j.jceh.2024.102492","url":null,"abstract":"<div><h3>Background</h3><div>Recent introduction of new steatotic liver disease categorizations has necessitated updated epidemiologic studies. Specifically, recognition of (1) “MetALD” defined as where metabolic dysfunction–associated steatotic liver disease (MASLD) overlaps with alcohol use and (2) alcohol-related liver disease (ALD) without cardiometabolic risk factors (CMRFs) creates new clinical phenotypes with undefined prevalence.</div></div><div><h3>Methods</h3><div>We conducted a cross-sectional multicenter analysis of liver disease associated with alcohol use (ALD and MetALD). We included adults with an International Classification of Diseases (ICD) diagnosis of ALD or both metabolic dysfunction associated liver disease and alcohol use disorder assigned from 1/1/2000–1/1/2024.</div></div><div><h3>Results</h3><div>Among 4057 patients, only 118 (2.9%) did not have any CMRF (“pure ALD”). Compared to patients with CMRF, patients with pure ALD were more commonly female (56% [0 CRMF] vs. 48%, 45%, 38%, and 42% [1, 2, 3, and 4 CMRFs, respectively]; <em>P</em> < 0.01) and younger (54 vs. 53, 60, 68, and 67 years [1, 2, 3, and 4 CMRFs, respectively]; <em>P</em> < 0.01). Those with pure ALD had higher rates of cirrhosis (49% vs. 39%, 31%, 30%, 34% [1, 2, 3, and 4 CMRFs, respectively]; <em>P</em> < 0.01), hepatocellular carcinoma (10% vs. 6.9%, 5.7%, 4.3%, and 5.1% [1, 2, 3, and 4 CMRFs, respectively]), and death (21% vs. 15%, 17%, 18%, and 21% [1, 2, 3, and 4 CMRFs, respectively]; <em>P</em> = 0.04). Patients whose only CMRF was body mass index (BMI) 25–30 kg/m<sup>2</sup> did not differ significantly from patients with pure ALD. Factors associated with cirrhosis in univariable analysis included male sex (odds ratio [OR]: 1.47, confidence interval [CI]: 1.29–1.67), age (OR: 1.08 per 10 years, CI: 1.03–1.13), and diabetes (OR: 1.21, CI: 1.05–1.40) but not BMI 25–30 kg/m<sup>2</sup> (OR: 0.86, CI: 0.64–1.14). No differences in single-nucleotide polymorphisms (<em>PNPLA3, GCKR, TM6SF2, MBOAT7,</em> or <em>HSD17B12</em>) were identified between groups.</div></div><div><h3>Conclusions</h3><div>ALD without diagnosed metabolic disease is uncommon and associated with higher rates of cirrhosis, HCC, and all-cause mortality than ALD with concurrent CMRF. Having a BMI measuring 25–30 kg/m<sup>2</sup> did not impact these clinical outcomes, raising the question of optimal BMI cut-off for MetALD. Further investigating these novel disease categories is essential for better understanding their biology and clinical impact.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"15 3","pages":"Article 102492"},"PeriodicalIF":3.3,"publicationDate":"2024-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143100011","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":"Role of Locoregional Therapy on Survival After Living Donor Liver Transplantation for Hepatocellular Carcinoma--Experience from a High-volume Center","authors":"Vibha Varma, Phani K. Nekarakanti, Shaleen Agarwal, Rajesh Dey, Subash Gupta","doi":"10.1016/j.jceh.2024.102490","DOIUrl":"10.1016/j.jceh.2024.102490","url":null,"abstract":"<div><h3>Background</h3><div>Locoregional therapy (LRT) in patients with hepatocellular carcinoma (HCC) before liver transplantation (LT) has a role in improving the tumor biology and post-LT survival outcome apart from downstaging and bridging. We retrospectively analyzed our database of adult living donor liver transplants (LDLT) for HCC, to compare the survival outcomes in Group-1 (upfront-LT, HCC within Milan/UCSF/AFP<1000 ng/ml) and Group-2 (LT post-LRT, HCC beyond UCSF/irrespective of tumor burden with AFP>1000 ng/ml). We also explored the risk factors for recurrence on follow-up.</div></div><div><h3>Methods</h3><div>A study group (n = 506, Group-1-348, Group-2 = 158) of patients with HCC who underwent LDLT between July 2006 and December 2022, excluding incidental HCC (n = 42), patients with other histology (n = 13) and in-hospital mortality (n = 43), were analyzed. Study cohort (n = 341), after propensity score matching, was analyzed for survival outcomes (overall survival, OS and disease-free survival, DFS) and risk factors for recurrence between Group-1 (n = 156) and Group-2 (n = 158).</div></div><div><h3>Results</h3><div>Group-2 exhibited a trend towards better mean OS and DFS compared to Group-1 (OS-133 vs. 107-months, <em>P</em> = NS, DFS-118 vs. 102-months, <em>P</em> = NS). Long-term OS (10-year) for those within Milan and UCSF criteria was superior in Group-2, <em>P</em> = NS. Complete pathological response (cPR) after LRT (46.8%), significantly improved OS and DFS compared to those with partial response and stable disease; 152 vs. 94 vs. 49 months, <em>P</em> = 0.001, and 147 vs. 75 vs. 41 months, <em>P</em> = 0.006, respectively. Recipient age, size of tumor, and pre-LT serum alpha-fetoprotein (AFP) were independent predictors of cPR. Independent risk factors for recurrence included pre-LT AFP, tumors beyond UCSF, perineural invasion, and high-grade tumors.</div></div><div><h3>Conclusion</h3><div>Locoregional therapy in HCC offers significantly better OS and DFS in those who had a complete pathological response. Risk factors for recurrence post-LT were AFP level, beyond UCSF tumors, and high-grade HCC with PNI on histology.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"15 3","pages":"Article 102490"},"PeriodicalIF":3.3,"publicationDate":"2024-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143047009","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}
Mahmoudreza Moein , Bridgette Nixon , Michael Leyderman , Ali Bassir , Brenden Maloney , Abolfazl Jamshidi , Matin Moallem Shahri , Amin Bahreini , Alireza Golkarieh , Reza Saidi
{"title":"Long-Term Survival and Recurrence in HCC vs. Non-HCC Liver Transplant Recipients: A Two-Decade Longitudinal Analysis","authors":"Mahmoudreza Moein , Bridgette Nixon , Michael Leyderman , Ali Bassir , Brenden Maloney , Abolfazl Jamshidi , Matin Moallem Shahri , Amin Bahreini , Alireza Golkarieh , Reza Saidi","doi":"10.1016/j.jceh.2024.102489","DOIUrl":"10.1016/j.jceh.2024.102489","url":null,"abstract":"<div><h3>Background</h3><div>We aim to compare the long-term survival outcomes of patients who have received liver transplants (LTs) as a result of primary hepatocellular carcinoma (HCC).</div></div><div><h3>Methods and materials</h3><div>A retrospective registry analysis of the Scientific Registry of Transplant Recipients (SRTR) database was done for LTs that were performed in the United States from January 2000 to June 2023.</div></div><div><h3>Results</h3><div>A total of 143,717 LT cases have met both the inclusion and the exclusion criteria and were included in the final analysis. The most common primary diagnosis in the non-HCC cohort was hepatitis C virus (HCV) (14,813 cases, 27%), alcoholic cirrhosis (6631 cases, 12.1%) in the 2001–2010 cohort, alcoholic cirrhosis (18,370 cases, 20.7%), and non-alcoholic steatohepatitis (NASH) (13,997 cases,15.8%) in the 2011–2023 cohort. The data analysis showed a significant overall one- and five-year allograft survival improvement in the 2011–2023 time frame compared to the 2001–2010 group in both HCC and non-HCC patients. The allograft survival difference became more significant after the 5 years of follow-up with a 10% difference between the two time frames in both HCC and non-HCC groups. Patients who met and were selected based on Milan's criteria had significantly better outcomes in both cohorts. Five-year allograft and patient survival were also significantly higher in the patients who met Milan's criteria in 2011–2023 cohort, compared to 2001–2010 cohort (74.4% vs. 66.1%, <em>P</em>-value <0.001, and 76% vs. 68.7%, <em>P</em>-value <0.01, respectively). Acute and chronic rejections were significantly higher in the non-HCC groups in both time frames. It was 6.5% vs. 4.8%, <em>P</em> = 0.03 in 2001–2010, and 13.6% vs. 8.2%, <em>P</em> = 0.0007 in 2011–2023, for acute rejection and 10.8% vs. 6.7%, <em>P</em> = 0.0001 in 2001–2010, and 14.1% vs. 10.3%, <em>P</em> = 0.01 in 2011–2023, for chronic rejection.</div></div><div><h3>Conclusion</h3><div>Short- and long-term outcomes of LT are almost equal to the other causes of liver transplantation in the recent decade, which can significantly overcome the dilemma of doing LT in patients with HCC diagnosis, who need LT. Adhering to the Milan criteria is crucial for optimizing outcomes, as demonstrated by our study's findings, which highlight significantly better allograft and patient survival rates among those who meet these criteria.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"15 3","pages":"Article 102489"},"PeriodicalIF":3.3,"publicationDate":"2024-12-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143099997","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}
Marcio J.M. Amaral, Felipe S. Moura, Luan C.V. Lima, Matheus Souza
{"title":"Response and Adverse Event Rates With Placebo in Compensated MASH-related Cirrhosis: A Meta-analysis","authors":"Marcio J.M. Amaral, Felipe S. Moura, Luan C.V. Lima, Matheus Souza","doi":"10.1016/j.jceh.2024.102487","DOIUrl":"10.1016/j.jceh.2024.102487","url":null,"abstract":"","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"15 3","pages":"Article 102487"},"PeriodicalIF":3.3,"publicationDate":"2024-12-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143157026","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}
Sara Maher , Shimaa Atta , Manal Kamel , Olfat A. Hammam , Hend Okasha
{"title":"Therapeutic Potential and Mechanistic Insights of a Novel Synthetic α-Lactalbumin-Derived Peptide for the Treatment of Liver Fibrosis","authors":"Sara Maher , Shimaa Atta , Manal Kamel , Olfat A. Hammam , Hend Okasha","doi":"10.1016/j.jceh.2024.102488","DOIUrl":"10.1016/j.jceh.2024.102488","url":null,"abstract":"<div><h3>Background</h3><div>Liver fibrosis is a serious global health issue, but current treatment options are limited due to a lack of approved therapies capable of preventing or reversing established fibrosis.</div></div><div><h3>Aim</h3><div>This study investigated the antifibrotic effects of a synthetic peptide derived from α-lactalbumin in a mouse model of thioacetamide (TAA)-induced liver fibrosis.</div></div><div><h3>Methods</h3><div><em>In silico</em> analyses were conducted to assess the physicochemical properties, pharmacophore features, and docking interactions of the peptide. Mice with induced fibrosis were treated with three different doses of the synthetic peptide (2.5, 5, or 10 μg/kg, twice weekly for 8 weeks). Immunohistochemistry, antioxidant enzyme levels, IGF-1 levels, and expression of fibrosis-related genes were assessed.</div></div><div><h3>Results</h3><div>Peptide interacted with human prothrombin's many sites with varying binding affinities. Besides, ligand similarity analysis identified 26 thrombin inhibitors with high Tanimoto scores. The peptide exhibited antifibrotic effects with dose-dependent improvements. The upregulated expression of IGF-1 in all treated groups compared with the pathological untreated group. In contrast, fibrotic markers such as TIMP, PDGF-α, and TGF-β were upregulated in the untreated pathological group but downregulated in the peptide-treated groups. The assessment of IGF-1 concentration in sera demonstrated that the peptide-treated groups exhibited an increase in IGF-1 levels. Histopathological examination of peptide-treated groups showed normal hepatic architecture with hepatocytes arranged in thin plates. Immunohistochemical results of high dose peptide-treated group showed a few numbers of positive αSMA with mild proliferating cell nuclear antigen expression.</div></div><div><h3>Conclusion</h3><div>The synthetic α-lactalbumin peptide shows promise as an antifibrotic therapy. Its safety and effectiveness are supported by <em>in silico</em> and <em>in vivo</em> analyses. The peptide's pharmacophore characteristics and potential as a thrombin inhibitor combine with its ability to downregulate fibrotic markers and maintain liver tissue integrity. These findings concluded the potential of this peptide as a promising therapeutic candidate for liver fibrosis, warranting further investigation.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"15 3","pages":"Article 102488"},"PeriodicalIF":3.3,"publicationDate":"2024-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143046070","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}
Tara P. Tripathy , Ranjan Patel , Srikant Behera , Brahmadatta Pattnaik , Tanmay Dutta , Sudipta Mohakud , Sunita Gupta , Adarsh K. Mohapatra , Debananda Sahoo , Suprava Naik , Hemant K. Nayak , Rashmi R. Mohanty , Manas K. Panigrahi
{"title":"The Change in Liver Volume After Inferior Vena Cava and/or Hepatic Vein Venoplasty in Patients With Budd Chiari Syndrome With at Least One Patent Hepatic Vein Presenting With Ascites","authors":"Tara P. Tripathy , Ranjan Patel , Srikant Behera , Brahmadatta Pattnaik , Tanmay Dutta , Sudipta Mohakud , Sunita Gupta , Adarsh K. Mohapatra , Debananda Sahoo , Suprava Naik , Hemant K. Nayak , Rashmi R. Mohanty , Manas K. Panigrahi","doi":"10.1016/j.jceh.2024.102486","DOIUrl":"10.1016/j.jceh.2024.102486","url":null,"abstract":"<div><h3>Objective</h3><div>To assess the effects of inferior vena cava and/or hepatic vein (IVC±HV) venoplasty on liver volumetry and function in individuals with Budd Chiari syndrome (BCS) who present with ascites and at least one patent hepatic vein.</div></div><div><h3>Methods</h3><div>A retrospective analysis was conducted on the clinical data of 17 patients with BCS (6 males and 11 females, average age of 42.3 ± 11.9 years) who underwent IVC venoplasty for ascites caused by IVC blockage and at least one patent HV, either pre- or post-venoplasty. Liver function tests and abdominal CT scans were performed before the procedure and at three and six months post-venoplasty. The changes in liver function and volume before and after venoplasty were analyzed.</div></div><div><h3>Results</h3><div>Each of the 17 patients successfully underwent IVC±HV venoplasty. During the median follow-up period of six months, all patients survived. Comparisons with preoperative conditions showed significant improvements in ascites and liver function three and six months after the procedure (<em>P</em> < 0.05). The liver volumes measured before and at three- and six-months post-procedure were 2077.06 ± 185.53 cm³, 1742.00 ± 124.62 cm³, and 1632.71 ± 108.29 cm³, respectively. There was a significant decrease in liver volume between the pre-operative measurements and the three-month follow-up, as well as between the three-month and six-month follow-ups (<em>P</em> < 0.05).</div></div><div><h3>Conclusions</h3><div>IVC±HV venoplasty produced satisfactory clinical results in BCS patients. Following the intervention, there was a progressive decrease in hepatic congestion and an improvement in liver function which correlated with decrease in liver volume.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"15 3","pages":"Article 102486"},"PeriodicalIF":3.3,"publicationDate":"2024-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143045906","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":"Temporal Trends of Fungal Infections in Cirrhotic Patients: A Retrospective Cohort Study 2016–2020","authors":"Choday Silpa , Talal Alomar , Robert J. Wong","doi":"10.1016/j.jceh.2024.102469","DOIUrl":"10.1016/j.jceh.2024.102469","url":null,"abstract":"<div><h3>Background</h3><div>Patients with cirrhosis are susceptible to infections due to abnormalities in humoral and cell-mediated immunity. Fungal infections are associated with delayed diagnosis and high mortality rates, emphasizing the importance of performing fungal cultures and maintaining elevated levels of suspicion in this patient population.</div></div><div><h3>Methods</h3><div>This retrospective cohort study analyzes cirrhotic patients readmitted with bacterial and fungal infections and investigates outcomes, including in-hospital mortality and hospital resource utilization. Data was acquired from the Nationwide Readmission Database (NRD) from 2016 to 2020. Total hospital costs were calculated using HCUP Cost-to-Charge Ratio files and adjusted for inflation based on the Consumer Price Index (CPI) for medical care services in the U.S., with 2020 as the reference year. The NRD dataset lacks details like ascitic fluid cell counts, antifungal/antibacterial drugs used, and treatment responses, limiting the clinical insights that can be derived.</div></div><div><h3>Results</h3><div>The study analyzed 393,195 index hospitalizations. Among these, 102,505 account for 30-day and 157,079 account for 90-day readmissions. The 30-day and 90-day readmissions for spontaneous bacterial peritonitis (SBP) are 8478 and 15,690 respectively. The 30-day and 90-day readmissions for spontaneous fungal peritonitis (SFP) are 3106 and 5798 respectively. The mean age of patients was 57.9 years (standard deviation between 57.7 and 58.1). The mean length of stay (LOS) for SBP at 30 days is 9.4 days, while SFP has ranged from 14.9 to 32.3 days for various fungal infections. Aspergilloses have the longest LOS among SFP. There is an increased rate of mortality as well as hospital charges with SFP compared to SBP (<em>P</em> < 0.001). The 30-day index admission total charges for SBP are $42,258 and SFP are $51,739. The 30-day readmission total charges for SBP are 64, 266 and for SFP 89,913.</div></div><div><h3>Conclusions</h3><div>There is increased mortality, LOS, and hospital costs for SFP compared to SBP. It is important to consider SFP in the diagnostic workup for patients who do not respond to antibiotics. Early recognition and administration of antifungals can be associated with improved outcomes.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"15 3","pages":"Article 102469"},"PeriodicalIF":3.3,"publicationDate":"2024-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143028914","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}
Ankit Agrawal , Anshu Srivastava , Amita Moirangthem , Suzena M. Singh , Dhriti Kodethoor , Srinivas S. Vadlapudi , Moinak S. Sarma , Ujjal Poddar
{"title":"Glycerol-3-Phosphate Dehydrogenase 1 Deficiency and Steatotic Liver Disease in Children: Our Cases and Review of Literature","authors":"Ankit Agrawal , Anshu Srivastava , Amita Moirangthem , Suzena M. Singh , Dhriti Kodethoor , Srinivas S. Vadlapudi , Moinak S. Sarma , Ujjal Poddar","doi":"10.1016/j.jceh.2024.102484","DOIUrl":"10.1016/j.jceh.2024.102484","url":null,"abstract":"<div><h3>Introduction</h3><div>Glycerol-3-phosphate dehydrogenase 1 (GPD1) deficiency is an autosomal recessive disorder causing hypertriglyceridemia, hepatomegaly, fatty liver, and hepatic fibrosis in infancy. It is an under-recognized cause of pediatric steatotic liver disease (SLD) with only 36 cases reported worldwide.</div></div><div><h3>Method</h3><div>We analyzed the clinical profile of our five cases diagnosed by exome sequencing (ES) and reviewed the published cases till December 2023 using PubMed search.</div></div><div><h3>Results</h3><div>Five unrelated boys (6–24months) presented with hepatomegaly, hypertriglyceridemia, transaminase elevation, and fatty liver. ES revealed compound heterozygous mutations in two and homozygous mutation in three including two novel mutations (c.917T >C, c.905C > G). All received supportive therapy, and fenofibrate was successfully used in one case for progressive hypertriglyceridemia. Globally, 36 cases (age at diagnosis: 6 [1–164 months]) have been reported. Majority had hepatomegaly (94.4%), 22.2% each had splenomegaly and growth failure. Hypertriglyceridemia (97.2%) was nearly universal, 100% had fatty liver, and hypoglycaemia (11.2%) was uncommon. Liver biopsy (n = 18) demonstrated steatosis in all, fibrosis in 94.4%, and cirrhosis in 22.2%. During follow-up (11–376 months), hepatomegaly resolved in 35.2%, triglycerides, and transaminases normalized in 29.1% and 31.5%, respectively. No pancreatitis, cardiac events, or liver decompensation was reported.</div></div><div><h3>Conclusion</h3><div>GPD1 is an uncommon cause of SLD, raised transaminases and hypertriglyceridemia in young children. Diagnosis is confirmed by genetic testing. Supportive therapy, parental counseling about the disease nature and close follow-up is recommended.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"15 3","pages":"Article 102484"},"PeriodicalIF":3.3,"publicationDate":"2024-12-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143006176","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}
Sunil Shenvi, Prashantha S. Rao, Ameya Panchwagh, Mitul Shah, Shaesta N. Zaidi, Ravi Mohanka
{"title":"Successful Liver Transplantation from a Deceased Donor With Gilbert's Syndrome","authors":"Sunil Shenvi, Prashantha S. Rao, Ameya Panchwagh, Mitul Shah, Shaesta N. Zaidi, Ravi Mohanka","doi":"10.1016/j.jceh.2024.102470","DOIUrl":"10.1016/j.jceh.2024.102470","url":null,"abstract":"","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"15 3","pages":"Article 102470"},"PeriodicalIF":3.3,"publicationDate":"2024-12-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143006204","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":"“Post-Kasai Portoenterostomy Cholangitis: What Have We Learnt So Far?”","authors":"Arghya Samanta, Moinak S. Sarma","doi":"10.1016/j.jceh.2024.102471","DOIUrl":"10.1016/j.jceh.2024.102471","url":null,"abstract":"<div><div>Post-Kasai portoenterostomy (KPE) cholangitis is one of the most common complications that has a negative impact on liver function and native liver survival. Early diagnosis and judicious empiric antimicrobial management are, therefore, important to prevent further liver damage and decompensation. However, there is no consensus regarding the standard definition of post-KPE cholangitis, and established guidelines on evaluation and management are also lacking. Metagenomic next-generation sequencing, a new molecular diagnostic technique, has the potential for detecting broader spectrum of pathogens, especially in blood culture-negative patients. Antibiotic prophylaxis to prevent cholangitis has been widely used, but questions over the choice of antibiotics, route of administration, and optimal duration remain unsettled. The available evidence on the efficacy of antibiotic prophylaxis in preventing cholangitis has shown conflicting results. This review offers a summary of the current research on advances in diagnostic approaches, including molecular techniques, and therapeutic challenges in managing intractable cholangitis.</div></div>","PeriodicalId":15479,"journal":{"name":"Journal of Clinical and Experimental Hepatology","volume":"15 3","pages":"Article 102471"},"PeriodicalIF":3.3,"publicationDate":"2024-12-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143006084","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}