World Journal of Hepatology最新文献

{"title":"Predictors of survival in autoimmune liver disease overlap syndromes.","authors":"Dujinthan Jayabalan, Yi Huang, Luis Calzadilla-Bertot, Malik Janjua, Bastiaan de Boer, John Joseph, Wendy Cheng, Simon Hazeldine, Briohny W Smith, Gerry C MacQuillan, Michael C Wallace, George Garas, Leon A Adams, Gary P Jeffrey","doi":"10.4254/wjh.v16.i9.1269","DOIUrl":"10.4254/wjh.v16.i9.1269","url":null,"abstract":"<p><strong>Background: </strong>Survival in patients with autoimmune liver disease overlap syndromes (AILDOS) compared to those with single autoimmune liver disease is unclear.</p><p><strong>Aim: </strong>To investigate the survival of patients with AILDOS and assess the accuracy of non-invasive serum models for predicting liver-related death.</p><p><strong>Methods: </strong>Patients with AILDOS were defined as either autoimmune hepatitis and primary biliary cholangitis overlap (AIH-PBC) or autoimmune hepatitis and primary sclerosing cholangitis overlap (AIH-PSC) and were identified from three tertiary centres for this cohort study. Liver-related death or transplantation (liver-related mortality) was determined using a population-based data linkage system. Prognostic scores for liver-related death were compared for accuracy [including liver outcome score (LOS), Hepascore, Mayo Score, model for end-stage liver disease (MELD) score and MELD incorporated with serum sodium (MELD-Na) score].</p><p><strong>Results: </strong>Twenty-two AILDOS patients were followed for a median of 3.1 years (range, 0.35-7.7). Fourteen were female, the median age was 46.7 years (range, 17.8 to 82.1) and median Hepascore was 1 (range, 0.07-1). At five years post enrolment, 57% of patients remained free from liver-related mortality (74% AIH-PBC, 27% AIH-PSC). There was no significant difference in survival between AIH-PBC and AIH-PSC. LOS was a significant predictor of liver-related mortality (<i>P</i> < 0.05) in patients with AIH-PBC (<i>n</i> = 14) but not AIH-PSC (<i>n</i> = 8). A LOS cut-point of 6 discriminated liver-related mortality in AIH-PBC patients (<i>P</i> = 0.012, log-rank test, 100% sensitivity, 77.8% specificity) (Harrell's C-statistic 0.867). The MELD score, MELD-Na score and Mayo Score were not predictive of liver-related mortality in any group.</p><p><strong>Conclusion: </strong>Survival in the rare, AILDOS is unclear. The current study supports the LOS as a predictor of liver-related mortality in AIH-PBC patients. Further trials investigating predictors of survival in AILDOS are required.</p>","PeriodicalId":23687,"journal":{"name":"World Journal of Hepatology","volume":null,"pages":null},"PeriodicalIF":2.5,"publicationDate":"2024-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11438591/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142355055","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}

{"title":"Blood cell counts and nonalcoholic fatty liver disease: Evidence from Mendelian randomization analysis.","authors":"Bin Hu, Ai-Hong Wan, Xi-Qiao Xiang, Yuan-Hao Wei, Yi Chen, Zhen Tang, Chang-De Xu, Zi-Wei Zheng, Shao-Ling Yang, Kun Zhao","doi":"10.4254/wjh.v16.i8.1145","DOIUrl":"10.4254/wjh.v16.i8.1145","url":null,"abstract":"<p><strong>Background: </strong>Previous research has highlighted correlations between blood cell counts and chronic liver disease. Nonetheless, the causal relationships remain unknown.</p><p><strong>Aim: </strong>To evaluate the causal effect of blood cell traits on liver enzymes and nonalcoholic fatty liver disease (NAFLD) risk.</p><p><strong>Methods: </strong>Independent genetic variants strongly associated with blood cell traits were extracted from a genome-wide association study (GWAS) conducted by the Blood Cell Consortium. Summary-level data for liver enzymes were obtained from the United Kingdom Biobank. NAFLD data were obtained from a GWAS meta-analysis (8434 cases and 770180 controls, discovery dataset) and the Fingen GWAS (2275 cases and 372727 controls, replication dataset). This analysis was conducted using the inverse-variance weighted method, followed by various sensitivity analyses.</p><p><strong>Results: </strong>One SD increase in the genetically predicted haemoglobin concentration (HGB) was associated with a β of 0.0078 (95%CI: 0.0059-0.0096), 0.0108 (95%CI: 0.0080-0.0136), 0.0361 (95%CI: 0.0156-0.0567), and 0.0083 (95%CI: 00046-0.0121) for alkaline phosphatase (ALP), alanine aminotransferase (ALT), aspartate aminotransferase, and gamma-glutamyl transferase, respectively. Genetically predicted haematocrit was associated with ALP (β = 0.0078, 95%CI: 0.0052-0.0104) and ALT (β = 0.0057, 95%CI: 0.0039-0.0075). Genetically determined HGB and the reticulocyte fraction of red blood cells increased the risk of NAFLD [odds ratio (OR) = 1.199, 95%CI: 1.087-1.322] and (OR = 1.157, 95%CI: 1.071-1.250). The results of the sensitivity analyses remained significant.</p><p><strong>Conclusion: </strong>Novel causal blood cell traits related to liver enzymes and NAFLD development were revealed through Mendelian randomization analysis, which may facilitate the diagnosis and prevention of NAFLD.</p>","PeriodicalId":23687,"journal":{"name":"World Journal of Hepatology","volume":null,"pages":null},"PeriodicalIF":2.5,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11362901/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142112621","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}

{"title":"Impact of non-alcoholic fatty liver disease on coronavirus disease 2019: A systematic review.","authors":"Abdul Moeed, Muhammad Omar Larik, Muhammad Ahmed Ali Fahim, Hafsah Alim Ur Rahman, Lubna Najmi, Mah I Kan Changez, Muhammad Moiz Javed, Md Al Hasibuzzaman","doi":"10.4254/wjh.v16.i8.1185","DOIUrl":"10.4254/wjh.v16.i8.1185","url":null,"abstract":"<p><strong>Background: </strong>Many studies have revealed a link between non-alcoholic fatty liver disease (NAFLD) and coronavirus disease 2019 (COVID-19), making understanding the relationship between these two conditions an absolute requirement.</p><p><strong>Aim: </strong>To provide a qualitative synthesis on the currently present data evaluating COVID-19 and NAFLD.</p><p><strong>Methods: </strong>This systematic review was conducted in accordance with the guidelines provided by preferred reporting items for systematic reviews and meta-analyses and the questionnaire utilized the population, intervention, comparison, and outcome framework. The search strategy was run on three separate databases, PubMed/MEDLINE, Scopus, and Cochrane Central, which were systematically searched from inception until March 2024 to select all relevant studies. In addition, ClinicalTrials.gov, Medrxiv.org, and Google Scholar were searched to identify grey literature.</p><p><strong>Results: </strong>After retrieval of 11 studies, a total of 39282 patients data were pooled. Mortality was found in 11.5% and 9.4% of people in NAFLD and non-NAFLD groups. In all, 23.2% of NAFLD patients and 22% of non-NAFLD admissions diagnosed with COVID-19 were admitted to the intensive care unit, with days of stay varying. Ventilatory support ranged from 5% to 40.5% in the NAFLD cohort and from 3.1% to 20% in the non-NAFLD cohort. The incidence of acute liver injury showed significance. Clinical improvement on days 7 and 14 between the two classifications was significant. Hospitalization stay ranged from 9.6 days to 18.8 days and 7.3 days to 16.4 days in the aforementioned cohorts respectively, with 73.3% and 76.3% of patients being discharged. Readmission rates varied.</p><p><strong>Conclusion: </strong>Clinical outcomes except mortality consistently showed a worsening trend in patients with NAFLD and concomitant COVID-19. Further research in conducting prospective longitudinal studies is essential for a more powerful conclusion.</p>","PeriodicalId":23687,"journal":{"name":"World Journal of Hepatology","volume":null,"pages":null},"PeriodicalIF":2.5,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11362910/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142112626","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}

{"title":"Bridging the gap: Addressing disparities in hepatitis C screening, access to care, and treatment outcomes.","authors":"Maram Alenzi, Mohammad Almeqdadi","doi":"10.4254/wjh.v16.i8.1091","DOIUrl":"10.4254/wjh.v16.i8.1091","url":null,"abstract":"<p><p>Hepatitis C virus (HCV) is a significant public health challenge globally, with substantial morbidity and mortality due to chronic liver disease. Despite the availability of highly effective and well-tolerated direct-acting antiviral therapies, widespread disparities remain in hepatitis C screening, access to treatment, linkage to care, and therapeutic outcomes. This review article synthesizes evidence from various studies to highlight the multifactorial nature of these disparities, which affects ethnic minorities, people with lower socioeconomic status, individuals with substance use disorders, and those within correctional facilities. The review also discusses policy implications and targeted strategies needed to overcome barriers and ensure equitable care for all individuals with HCV. Recommendations for future research to address gaps in knowledge and evaluation of the effectiveness of interventions designed to reduce disparities are provided.</p>","PeriodicalId":23687,"journal":{"name":"World Journal of Hepatology","volume":null,"pages":null},"PeriodicalIF":2.5,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11362903/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142112622","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}

{"title":"Correlation between non-alcoholic fatty liver disease and metabolic parameters in persons with newly diagnosed type 2 diabetes mellitus.","authors":"Supriyo Mukherjee, Sushmita Mukherjee, Chun Shing Kwok, Anne Phillips","doi":"10.4254/wjh.v16.i8.1120","DOIUrl":"10.4254/wjh.v16.i8.1120","url":null,"abstract":"<p><strong>Background: </strong>There are limited studies investigating the association between type 2 diabetes mellitus (T2DM) and non-alcoholic fatty liver disease (NAFLD) in the region of Bihar, India.</p><p><strong>Aim: </strong>To estimate the prevalence of NAFLD in persons with newly diagnosed T2DM in the population of North Bihar, India.</p><p><strong>Methods: </strong>This single centre cross-sectional study was undertaken in the Research Centre for Diabetes Hypertension and Obesity, Samastipur, Bihar, India. Data were collected from persons newly diagnosed with T2DM or those diagnosed within 6 months of when the study was conducted between December 2022 to May 2023.</p><p><strong>Results: </strong>A total of 148 people with newly diagnosed T2DM were included (median age 47 years, 46.6% female) and 109 patients with liver disease on ultrasound evaluation. The persons with liver disease consumed more fats and oils (88.1% <i>vs</i> 74.4%, <i>P</i> = 0.042) and they had significantly greater body mass index (27.4 <i>vs</i> 23.0, <i>P</i> < 0.001), waist circumference (37 <i>vs</i> 33, <i>P</i> < 0.001), and waist-to-hip ratio (1.00 <i>vs</i> 0.70, <i>P</i> = 0.025). Females were associated with greater liver disease [odds ratio (OR): 3.09, 95% confidence interval (CI): 1.09-8.80, <i>P</i> = 0.32]. Waist circumference (OR: 1.42, 95%CI: 1.22-1.66, <i>P</i> < 0.001) and low-density lipoprotein cholesterol (OR: 1.01, 95%CI: 1.01-1.02, <i>P</i> = 0.048) were associated with any liver disease. The factors most associated with grade 2/3 liver disease was right upper quadrant pain or heaviness (OR: 5.22, 95%CI: 1.40-19.41, <i>P</i> = 0.14), greater income (OR: 3.58, 95%CI: 1.28-10.04, <i>P</i> = 0.015) and waist circumference (OR: 1.31, 95%CI: 1.02-1.69, <i>P</i> = 0.036).</p><p><strong>Conclusion: </strong>NAFLD is common in new/recently diagnosed T2DM and disease burden is high and common among patients who are either high consumers of fats and oils or have obesity-associated markers.</p>","PeriodicalId":23687,"journal":{"name":"World Journal of Hepatology","volume":null,"pages":null},"PeriodicalIF":2.5,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11362911/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142112624","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}

{"title":"Gadoxetic acid-enhanced magnetic resonance imaging in the assessment of hepatic sinusoidal obstruction syndrome in a mouse model.","authors":"Yuan-Yuan Chen, Li Yang, Jun Li, Sheng-Xiang Rao, Ying Ding, Meng-Su Zeng","doi":"10.4254/wjh.v16.i8.1167","DOIUrl":"10.4254/wjh.v16.i8.1167","url":null,"abstract":"<p><strong>Background: </strong>Neoadjuvant chemotherapy can cause hepatic sinusoidal obstruction syndrome (SOS) in patients with colorectal cancer liver metastases and increases postoperative morbidity and mortality.</p><p><strong>Aim: </strong>To evaluate T₁ mapping based on gadoxetic acid-enhanced magnetic resonance imaging (MRI) for diagnosis of hepatic SOS induced by monocrotaline.</p><p><strong>Methods: </strong>Twenty-four mice were divided into control (<i>n</i> = 10) and experimental (<i>n</i> = 14) groups. The experimental groups were injected with monocrotaline 2 or 6 days before MRI. MRI parameters were: T1 relaxation time before enhancement; T1 relaxation time 20 minutes after enhancement (T_1post); a reduction in T1 relaxation time (△T₁%); and first enhancement slope percentage of the liver parenchyma (ESP). Albumin and bilirubin score was determined. Histological results served as a reference. Liver parenchyma samples from the control and experimental groups were analyzed by western blotting, and organic anion transporter polypeptide 1 (OATP1) was measured.</p><p><strong>Results: </strong>T_1post, △T₁%, and ESP of the liver parenchyma were significantly different between two groups (all <i>P</i> < 0.001) and significantly correlated with the total histological score of hepatic SOS (<i>r</i> = -0.70, 0.68 and 0.79; <i>P</i> < 0.001). △T₁% and ESP were positively correlated with OATP1 levels (<i>r</i> = 0.82, 0.85; <i>P</i> < 0.001), whereas T_1post had a negative correlation with OATP1 levels (<i>r</i> = -0.83; <i>P</i> < 0.001).</p><p><strong>Conclusion: </strong>T₁ mapping based on gadoxetic acid-enhanced MRI may be useful for diagnosis of hepatic SOS, and MRI parameters were associated with OATP1 levels.</p>","PeriodicalId":23687,"journal":{"name":"World Journal of Hepatology","volume":null,"pages":null},"PeriodicalIF":2.5,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11362905/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142112625","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}

{"title":"<i>Rocahepevirus ratti</i>: An underrecognised cause of acute hepatitis.","authors":"George S Gherlan","doi":"10.4254/wjh.v16.i8.1084","DOIUrl":"10.4254/wjh.v16.i8.1084","url":null,"abstract":"<p><p>Zoonoses are responsible for many of all emerging infectious diseases as well as for those already established. <i>Rocahepevirus ratti</i> is a rat-originated virus related to the hepatitis E virus <i>(Paslahepevirus balayani)</i> but highly divergent genetically from this, with a high cross-species infection potential and zoonotic transmission. It can infect humans, leading to acute hepatitis, and is primarily transmitted through the consumption of contaminated water. <i>Rocahepevirus ratti</i> was first discovered in Germany in 2010. The first human case was described in 2017 in Hong Kong in an immune-compromised patient. The first case of chronic infection with <i>Rocahepevirus ratti</i> was described in 2023. A meta-analysis based on 38 studies published between 2000 and 2023 identified 21 cases in humans described up to this date and 489 infections in different animals. Raising awareness regarding this virus is essential, as there are probably many cases that remain undiagnosed, and the virus even has the ability to produce chronic infections in selected patients.</p>","PeriodicalId":23687,"journal":{"name":"World Journal of Hepatology","volume":null,"pages":null},"PeriodicalIF":2.5,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11362906/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142117093","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}

{"title":"Intermittent fasting and the liver: Focus on the Ramadan model.","authors":"Mohamed H Emara, Hanan Soliman, Ebada M Said, Hassan Elbatae, Mostafa Elazab, Shady Elhefnawy, Tarik I Zaher, Ahmed Abdel-Razik, Mohamed Elnadry","doi":"10.4254/wjh.v16.i8.1070","DOIUrl":"10.4254/wjh.v16.i8.1070","url":null,"abstract":"<p><p>Intermittent fasting (IF) is an intervention that involves not only dietary modifications but also behavioral changes with the main core being a period of fasting alternating with a period of controlled feeding. The duration of fasting differs from one regimen to another. Ramadan fasting (RF) is a religious fasting for Muslims, it lasts for only one month every one lunar year. In this model of fasting, observers abstain from food and water for a period that extends from dawn to sunset. The period of daily fasting is variable (12-18 hours) as Ramadan rotates in all seasons of the year. Consequently, longer duration of daily fasting is observed during the summer. In fact, RF is a peculiar type of IF. It is a dry IF as no water is allowed during the fasting hours, also there are no calorie restrictions during feeding hours, and the mealtime is exclusively nighttime. These three variables of the RF model are believed to have a variable impact on different liver diseases. RF was evaluated by different observational and interventional studies among patients with non-alcoholic fatty liver disease and it was associated with improvements in anthropometric measures, metabolic profile, and liver biochemistry regardless of the calorie restriction among lean and obese patients. The situation is rather different for patients with liver cirrhosis. RF was associated with adverse events among patients with liver cirrhosis irrespective of the underlying etiology of cirrhosis. Cirrhotic patients developed new ascites, ascites were increased, had higher serum bilirubin levels after Ramadan, and frequently developed hepatic encephalopathy and acute upper gastrointestinal bleeding. These complications were higher among patients with Child class B and C cirrhosis, and some fatalities occurred due to fasting. Liver transplant recipients as a special group of patients, are vulnerable to dehydration, fluctuation in blood immunosuppressive levels, likelihood of deterioration and hence observing RF without special precautions could represent a real danger for them. Patients with Gilbert syndrome can safely observe RF despite the minor elevations in serum bilirubin reported during the early days of fasting.</p>","PeriodicalId":23687,"journal":{"name":"World Journal of Hepatology","volume":null,"pages":null},"PeriodicalIF":2.5,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11362902/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142112627","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}

{"title":"Dynamics of glutamine synthetase expression in hepatic ischemia-reperfusion injury: Implications for therapeutic interventions.","authors":"Zhi-Hao Huang, Meng-Qi Dong, Feng-Yong Liu, Wei-Jie Zhou","doi":"10.4254/wjh.v16.i8.1177","DOIUrl":"https://doi.org/10.4254/wjh.v16.i8.1177","url":null,"abstract":"<p><strong>Background: </strong>Hepatic ischemia-reperfusion injury (IRI) poses a great challenge in liver surgery and transplantation because of oxidative stress and inflammatory responses. The changes in glutamine synthetase (GS) expression during hepatic IRI remain unclear.</p><p><strong>Aim: </strong>To investigate the dynamic expression of GS during hepatic IRI.</p><p><strong>Methods: </strong>Following hepatic ischemia for 1 h and reperfusion, liver tissue samples were collected at 0.5, 6, and 24 hours postreperfusion for fixation, embedding, sectioning. Hematoxylin and eosin staining and GS staining were performed.</p><p><strong>Results: </strong>GS expression rapidly decreases in hepatocytes around the central vein after IRI, reaching its lowest point at 6 hours postreperfusion, and then gradually recovers.</p><p><strong>Conclusion: </strong>GS is highly sensitive to IRI, highlighting its potential role as an indicator of liver injury states and a target for therapeutic intervention.</p>","PeriodicalId":23687,"journal":{"name":"World Journal of Hepatology","volume":null,"pages":null},"PeriodicalIF":2.5,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11423427/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142355035","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}

{"title":"Alpha-1 antitrypsin deficiency and Pi*Z allele as important co-factors in the development of liver fibrosis.","authors":"Ana Isabel Ferreira, Catarina Guimarães, Vitor Macedo Silva, Sofia Xavier, Joana Magalhães, José Cotter","doi":"10.4254/wjh.v16.i8.1099","DOIUrl":"10.4254/wjh.v16.i8.1099","url":null,"abstract":"<p><strong>Background: </strong>Alpha-1 antitrypsin deficiency (AATD) is a codominant autosomal hereditary condition that predisposes patients to the development of lung and/or liver disease, and Pi*Z allele is the most clinically relevant mutation.</p><p><strong>Aim: </strong>To evaluate the impact of clinical parameters and AATD phenotypes, particularly the Pi*Z allele, in liver fibrosis.</p><p><strong>Methods: </strong>Cross-sectional cohort study including consecutive patients with AATD followed in Pulmonology or Hepatology consultation.</p><p><strong>Results: </strong>Included 69 patients, 49.3% had Pi*MZ phenotype and 10.1% Pi*ZZ. An age ≥ 55 years, age at diagnosis ≥ 41 years and AAT at diagnosis < 77 mg/dL predicted a nonalcoholic fatty liver disease fibrosis score (NFS) not excluding advanced fibrosis [area under the curve (AUC) = 0.840, <i>P</i> < 0.001; AUC = 0.836, <i>P</i> < 0.001; AUC = 0.681, <i>P</i> = 0.025]. An age ≥ 50 years and age at diagnosis ≥ 41 years predicted a fibrosis-4 index of moderate to advanced fibrosis (AUC = 0.831, <i>P</i> < 0.001; AUC = 0.795, <i>P</i> < 0.001). Patients with hypertension, type 2 diabetes mellitus (DM), dyslipidaemia, metabolic syndrome, and regular alcohol consumption were more likely to have a NFS not excluding advanced fibrosis (<i>P</i> < 0.001, <i>P</i> = 0.002, <i>P</i> = 0.008, <i>P</i> < 0.001, <i>P</i> = 0.033). Patients with at least one Pi*Z allele and type 2 DM were 8 times more likely to have liver stiffness measurement ≥ 7.1 kPa (<i>P</i> = 0.040).</p><p><strong>Conclusion: </strong>Risk factors for liver disease in AATD included an age ≥ 50 years, age at diagnosis ≥ 41 years, metabolic risk factors, regular alcohol consumption, at least one Pi*Z allele, and AAT value at diagnosis < 77 mg/dL. We created an algorithm for liver disease screening in AATD patients to use in primary care, selecting those to be referred to Hepatology consultation.</p>","PeriodicalId":23687,"journal":{"name":"World Journal of Hepatology","volume":null,"pages":null},"PeriodicalIF":2.5,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11362909/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142112620","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}