Background:Steatotic liver disease (SLD) is prevalent among women with breast cancer, but the influence of hormonotherapy on its severity and progression remains uncertain. This study evaluated the prevalence, severity, and predictors of SLD in relation to hormone therapy. Methods:In this cross-sectional study, women with breast cancer were stratified by endocrine therapy exposure and evaluated with liver transient elastography and ultrasound. A subset of patients exposed to hormone therapy was prospectively followed for approximately 24 months to assess changes in liver stiffness over time. Results:Among 171 women [mean age 58±10 years; median follow-up 53 months (range, 1-315 months)], comorbidities included diabetes (26.9%), hypertension (53.2%), dyslipidemia (31.0%), and obesity (70.2%). Patients were divided into four groups: no hormone therapy (n=55, 32.2%), tamoxifen only (n=72, 42.1%), anastrozole only (n=16, 9.4%), and both drugs (n=28, 16.4%). SLD was present in 57.9% of participants, with no significant differences in steatosis prevalence (P=0.09) or liver stiffness (P=0.20) across groups. Liver stiffness ≥8 kPa occurred in 12.3%, and stiffness ≥12 kPa in 5.8%. Diabetes was independently associated with steatosis, and metabolic syndrome with advanced fibrosis, regardless of hormone therapy duration. Among 35 patients in the prospective sub-cohort, no significant changes in liver stiffness were detected after 24 months of continuous endocrine therapy. Conclusions:Over half of the participants had SLD, with advanced fibrosis in ~10%. Metabolic factors were independently associated with SLD development and progression, regardless of hormone therapy exposure. Patients in continuous use of endocrine therapy had no dynamic changes in liver stiffness after 24 months of follow-up.

GOALS:To investigate the prevalence of inflammatory bowel disease (IBD) in a cohort of Brazilian patients with primary sclerosing cholangitis (PSC) and evaluate clinical and prognostic factors associated with concomitant IBD. BACKGROUND:IBD is reported worldwide in 62% to 81% of patients with PSC, especially in males and north Europeans. Little is known about the association of IBD and PSC in population from multigenetic ethnic origin. STUDY:Data of PSC patients from the Brazilian Cholestasis Study Group database were retrospectively reviewed to compare demographic, clinical, laboratory, and transplant-free survival between those with and without IBD. RESULTS:After exclusion of 59 (14%) participants with overlap syndrome with autoimmune hepatitis, 359 individuals with PSC were included {56% male, median age 44 [interquartile range (IQR): 33 to 54] y}. IBD was investigated in 298 (83%) participants and diagnosed in 217 (73%), including ulcerative colitis (83%), Crohn's disease (13%), and indeterminate colitis (4%). Male sex frequency was similar in patients with and without IBD (58% vs. 47%, P =0.073). IBD was more frequently diagnosed in patients without obesity ( P =0.035), positive for antinuclear antibody ( P =0.006), and positive for anti-smooth muscle antibody ( P =0.046). IBD diagnosis occurred before, concomitant, or after PSC diagnosis in 59%, 22%, and 19% of cases, respectively. IBD was more frequently diagnosed before PSC in participants asymptomatic for liver disease ( P =0.017), without advanced liver disease ( P =0.017), before liver transplantation (LT) ( P <0.001), and positive for antinuclear antibody ( P =0.021). In a median follow-up of 69 months (IQR 31-124), LT occurred in 27.7% and cohort mortality was 11.4%. IBD was neither associated with the combined outcome of death or LT ( P =0.745) nor with transplant-free survival ( P =0.902). CONCLUSIONS:In Brazilian PSC patients, IBD frequency was similar to that reported in other populations but exhibited a balanced proportion between males and females. IBD diagnosis occurred before PSC in most patients, especially those without advanced liver disease. IBD presence was not associated with worse PSC prognosis.

INTRODUCTION AND OBJECTIVES:After Liver Transplantation (LT), patients often gain weight and develop metabolic comorbidities. This study aimed to assess the prevalence and severity of Metabolic Dysfunction-Associated Steatotic Liver Disease (MASLD) after LT and the incidence of New-Onset Diabetes After LT (NODALT). METHODS:Post-LT patients were recruited from an outpatient clinic. Hepatic ultrasound and 2D shear-wave elastography were performed. RESULTS:A total of 142 patients were included, 63 % male with median age 60 (Interquartile Range [IQR] 47‒68 years). Median follow-up time post-LT was 137 (IQR 77‒205 months). MASLD was diagnosed in 26 %. Patients with MASLD exhibited a higher prevalence of central obesity (92 % vs. 68 %, p = 0.006), diabetes (65 % vs. 38 %, p = 0.005), hypertriglyceridemia (51 % vs. 26 %, p = 0.008), and metabolic syndrome (69 % vs. 41 %, p = 0.006) compared to those without MASLD. MASLD was independently associated with hypertriglyceridemia (Odds Ratio [OR = 2.80], 95 % Confidence Interval [95 % CI 1.22‒6.43], p = 0.015) and post-LT diabetes (OR = 2.65, 95 % CI 1.15‒6.10, p = 0.022). NODALT occurred in 36 % and was independently associated with older age (OR = 1.05, 95 % CI 1.01‒1.08, p = 0.01), time from LT (OR = 1.01, 95 % CI 1.003‒1.016, p = 0.006), body mass index (OR = 1.12, 95 % CI 1.02‒1.22, p = 0.02) and MASLD (OR = 2.99, 95 % CI 1.13‒7.92, p = 0.028). Advanced fibrosis occurred in 9 % of MASLD patients, which was similar from patients without MASLD. CONCLUSION:After LT, the prevalence of MASLD was 26 % ant the incidence of NODALT was 36 %. MASLD was associated with diabetes and hypertriglyceridemia, while NODALT was linked to older age, longer follow-up post-LT and MASLD. These findings emphasize the importance of preventing post-LT metabolic complications.

BACKGROUND:Ursodeoxycholic acid (UDCA) is the standard treatment for primary biliary cholangitis (PBC), but a significant proportion of patients do not respond adequately, leading to increased risk of adverse outcomes. This study aims to develop a new and straightforward predictive score to identify PBC patients likely to achieve a complete response to UDCA. METHODS:A logistic regression analysis was conducted using a derivation cohort of PBC patients to identify pre-treatment variables associated with response to UDCA. This analysis led to the development of the ALP-A score, calculated as: Age at diagnosis divided by (alkaline phosphatase at diagnosis/upper limit of normal). ALP-A score accuracy was evaluated using the area under the ROC curve, validated with a large external cohort from Brazil. Additionally, the correlation between the ALP-A score and the previously validated UDCA response score (URS) was assessed. RESULTS:ALP-A score had good predictive power for adequate (AUC 0.794; 95% CI, 0.737-0.852) and deep (0.76; 95% CI, 0.69-0.83) UDCA response at 1 year of treatment. A cutoff score of 17 and 23 points was determined to be the optimal threshold for distinguishing adequate and deep responders, respectively, from non-responders. ALP-A score demonstrated a sensitivity of 73%, specificity of 71%, positive predictive value of 65%, negative predictive value of 78%, and overall accuracy of 72% for biochemical response. The URS displayed similar discriminative ability (AUC 0.798; 95% CI, 0.741-0.855). CONCLUSION:ALP-A score performs comparably to URS but offers the great advantage of simplicity for routine clinical use. It serves as a valuable tool to identify PBC patients less likely to respond to UDCA treatment, facilitating early consideration of alternative therapeutic approaches.

OBJECTIVE Primary biliary cholangitis is a chronic and progressive autoimmune liver disease, whose prognosis can be improved by normalizing alkaline phosphatase and bilirubin. While ursodeoxycholic acid (UDCA) is first line standard of care, approximately 40% of patients exhibit incomplete response. We aimed to identify prognostic markers for deep response to UDCA therapy at presentation. PATIENT AND METHODS Data from the Brazilian Cholestasis Study Group cohort were analyzed retrospectively. Patients were assessed for deep response, defined as normal alkaline phosphatase and bilirubin, after 1 year of UDCA treatment. Additionally, the performance of the UDCA response score in predicting deep response was evaluated. RESULTS A total of 297 patients were analyzed, with 57.2% achieving an adequate response according to the Toronto criteria, while 22.9% reached deep response. Cirrhosis (OR 0.460; 95% CI 0.225-0.942; p=0.034) and elevated baseline alkaline phosphatase levels (OR 0.629; 95% CI 0.513-0.770; p<0.001) were associated with reduced odds of deep response. The UDCA response score exhibited moderate discrimination power (AUROC=0.769) but lacked calibration. CONCLUSIONS Baseline ALP and liver fibrosis emerge as the most important prognostic factors to predict normalization of alkaline phosphatase and bilirubin after UDCA. The UDCA response score was inadequate for predicting deep response in the Brazilian PBC population.

INTRODUCTION AND OBJECTIVES:Autoimmune hepatitis (AIH) is a rare disease with a complex and not fully understood pathogenesis. Prognostic factors that might influence treatment response, relapse rates, and transplant-free survival are not well established. This study investigates clinical and biochemical markers associated with response to immunosuppression in patients with AIH. MATERIALS AND METHODS:This retrospective cohort study included 102 patients with AIH treated with immunosuppressants and followed at the Federal University of Minas Gerais, Brazil, from 1990 to 2018. Pretreatment data such as clinical profiles, laboratory, and histological exams were analyzed regarding biochemical response at one year, histological remission, relapse, and death/transplantation rates. RESULTS:Cirrhosis was present in 59 % of cases at diagnosis. One-year biochemical remission was observed in 55.7 % of the patients and was found to be a protective factor for liver transplant. Overall survival was 89 %. Patients with ascites at disease onset showed a higher aspartate aminotransferase (AST)/ alanine aminotransferase (ALT) ratio and elevated Model of end-stage liver disease (MELD) score. The presence of ascites was significantly associated with a 20-fold increase in mortality rate. CONCLUSIONS:AIH has a severe clinical phenotype in Brazilians, with high rates of cirrhosis and low remission rates. Early diagnosis and treatment are essential for achieving remission and reducing complications. The presence of ascites is significantly associated with mortality, emphasizing the importance of monitoring and prompt intervention. This study also stresses the need for further research on AIH in Latin America.

Conflict of interest: Yes, This work was supported by Brazilian Society of Hepatology and Instituto Brasileiro do Fígado - IBRAFIG. Introduction and Objectives: Previous studies have identified risk factors associated with recurrent primary sclerosing cholangitis (rPSC) after liver transplantation (LT) in Caucasians. There is paucity of data regarding rPSC in multiethnic Latin patients. Objectives: To investigate rPSC frequency and its associated risk factors in a highly admixed population from Brazil. Patients / Materials and Methods: The Brazilian Cholestasis Study Group database was retrospectively reviewed for including primary sclerosing cholangitis (PSC) patients who underwent LT. Primary outcome was rPSC. Results and Discussion: A total of 96 patients were included, 60% males, mean age 32 ± 13 years. After a follow-up of 90 months (interquartile range 39-154), rPSC occurred in 29 (30%) of the participants. There were no statistically significant associations between rPSC and age, gender, concurrent or de novo inflammatory bowel disease, MELD score at the time of LT or allograft rejection. The only factor associated with an increased risk of disease recurrence was time after LT. Conclusions: In Brazilian PSC patients who underwent LT, one-third had rPSC. Longer time after LT was associated with rPSC diagnosis.

Conflict of interest No Introduction and Objectives Hepatosplenic schistosomiasis (HSS) is one of the main causes of non-cirrhotic presinusoidal portal hypertension. Although liver stiffness in HSS is typically lower than in cirrhosis, it is still unknown if over the years liver injury related to periportal fibrosis, to metabolic disease or vascular complications can alter liver stiffness and impact in serious hepatic events. This study aims to determine whether metabolic factors, portal vein thrombosis (PVT), or changes in liver stiffness over time are associated with serious hepatic events (SHE) in HSS patients. Patients / Materials and Methods In this prospective study, adults with laboratory and radiologically confirmed HSS, without concurrent cirrhosis or other liver diseases, were included. All participants underwent initial transient elastography, followed by a second assessment after at least 3 years. The primary outcome was the occurrence of SHE, defined as upper variceal bleeding and/or ascites. Results and Discussion Among the 26 patients studied, 65.4% were male, with a mean age of 55 ± 9 years. The main metabolic comorbidities were obesity (27%), hypertriglyceridemia (8%), low HDL-c (4%), and diabetes (13%). Baseline liver stiffness measurement (LSM) was 9.9 kPa (±3.9) and the controlled attenuation parameter (CAP) was 238 dB/m (IQR 121-270). After a median follow-up of 59 months (IQR 51-64), serious hepatic events occurred in 46% of the patients. There was a non-significant median absolute increase in LSM of 1.4 kPa (IQR -1.5 to +3.6) and a median relative increase of +14% (IQR -17% to +50%), with no statistically significant differences in paired analysis (p = 0.140). No metabolic or anthropometric factors were associated with changes in liver stiffness. PVT occurred in 7 patients (26.9%) and was the only factor significantly associated with the occurrence of serious hepatic events (p = 0.026), although it did not significantly interfere with LSM (p = 0.842). Conclusions LSM remained relatively stable in HSS patients over a median follow-up of almost 5 years, proving to be a useful tool in distinguishing HSS from cirrhosis. SHE were primarily associated with PVT, which may further elevate portal pressure.

Conflict of interest No Introduction and Objectives Primary biliary cholangitis is a chronic and progressive autoimmune liver disease, whose prognosis can be improved by normalizing alkaline phosphatase and bilirubin. While ursodeoxycholic acid (UDCA) is first line standard of care, approximately 40% of patients exhibit incomplete response. We aimed to identify prognostic markers for deep response to UDCA therapy at presentation. Patients / Materials and Methods Data from the Brazilian Cholestasis Study Group cohort were analyzed retrospectively. Patients were assessed for deep response (defined as normalization of alkaline phosphatase and bilirubin) after 1 year of UDCA treatment. With the purpose of selecting the set of relevant variables related to the deep response for a parsimonious multivariate model, we applied the Varrank algorithm. Additionally, the performance of the UDCA response score in predicting deep response was evaluated. Results and Discussion A total of 297 patients were analyzed, with 57.2% achieving an adequate response according to the Toronto criteria, while 22.9% reached deep response. Cirrhosis (OR 0.460; 95% CI 0.225-0.942; p=0.034) and elevated baseline alkaline phosphatase levels (OR 0.629; 95% CI 0.513-0.770; p<0.001) were associated with reduced odds of deep response. The UDCA response score exhibited moderate discrimination power (AUROC=0.769) but lacked calibration. Conclusions Baseline ALP, and cirrhosis at diagnosis emerge as the most important prognostic factors to predict normalization of alkaline phosphatase and bilirubin after UDCA. The UDCA response score is inadequate for predicting deep response in the Brazilian PBC population.

BACKGROUND AND AIM:Primary sclerosing cholangitis (PSC) has been shown to recur after liver transplantation (LT). Some studies have identified certain clinical and laboratory variables associated with an increased risk for recurrent PSC (rPSC) in Caucasians. Furthermore, de novo cholangiocarcinoma (CCA) has been reported anecdotally in patients with rPSC. This study aims to assess the prevalence of rPSC, identify its associated risk factors, and investigate the occurrence of de novo CCA in a highly admixed population from Brazil. METHODS:All patients submitted to LT for PSC enrolled in the Brazilian Cholestasis Study Group database were retrospectively reviewed for the occurrence of rPSC and de novo CCA. RESULTS:Ninety-six (58 males, mean age 32 ± 13 years) patients with PSC underwent LT. After 90 (39-154) months of follow-up (FU), rPSC was observed in 29 (30%) subjects. There were no significant associations between rPSC and age, gender, concurrent or de novo inflammatory bowel disease, MELD score at the time of LT or allograft rejection. The only factor associated with an increased risk of disease recurrence was time after LT. Although survival was decreased in patients who developed rPSC, this difference was not significant. Only one female patient developed de novo CCA after rPSC, 11 years after LT. CONCLUSIONS:Recurrent PSC was observed in one-third of PSC LT patients in Brazil and was associated with longer time after LT. Despite its frequency, rPSC was not associated with a higher risk of graft loss or a significant reduction in posttransplant survival.

Introduction and Objectives: Cryptogenic chronic hepatitis is an increasing cause of liver transplantation and affects 5-15% of patients with chronic liver diseases. This study aimed to investigate possible underlying causes of presumed cryptogenic liver disease referred to specialized liver centers by general practitioners in Brazil and to propose a new diagnostic algorithm including metabolic-dysfunction-associated fatty liver disease (MAFLD) definition and lysosomal acid lipase deficiency (LAL-D) investigation. Materials and Methods: A retrospective multicentric Brazilian cohort of patients with presumed chronic cryptogenic hepatitis was reanalyzed with respect to their clinical, laboratory and histological data using Czaja´s algorithm (2011). Results: 326 patients [mean age 60 (46-68) years, 42.9% males] were initially included, 35.7% with cirrhosis. Forty-five individuals were excluded due to an incomplete etiological investigation. Using Czaja´s algorithm, diagnosis of nonalcoholic fatty liver disease could be established in 60 patients (21.3%), alpha-1-antitrypsin deficiency in 9 (3.2%), alcoholic liver disease in 7 (2.7%), autoimmune hepatitis in 5 (1.78%), hemochromatosis in 5 (1.78%), biliary-related hepatitis in 4 (1.4%), viral hepatitis in 4 (1.4%), Budd Chiari in 4 (1.4%), glycogenosis in 3 (1%), drug-induced liver injury in 2 (0.7%), and Wilson disease in 1 (0.35%). LAL-D was demonstrated in 3 individuals (1%). One hundred seventy-five patients remained with cryptogenic hepatitis (53.6%) (FIGURE A). During follow-up, 40 of those patients were submitted to liver transplantation and 19 (47.5%) were retrospectively diagnosed with non-alcoholic steatohepatitis after histopathological examination of the explanted liver. By including MAFLD in the first step of the new algorithm, 100 patients would have been diagnosed (34.9%), reducing the number of individuals without a diagnosis by 18.3% (FIGURE B). Conclusions: One-third of patients with initially presumed cryptogenic liver disease were diagnosed with MAFLD. Despite being a rare disease, LAL-D investigation should be considered for individuals with chronic liver disease of unknown etiology. An updated diagnostic algorithm is proposed for those individuals.

Introduction and Objectives: Primary biliary cholangitis (PBC) and autoimmune hepatitis (AIH) and PBC overlap syndrome (AIH/PBC) have been associated with a higher risk of hepatocellular carcinoma (HCC) and extra-hepatic malignancy (EHM). This study aimed to assess potential risk factors associated with cancer development in PBC and AIH/PBC patients. Materials and Methods: The Brazilian Cholestasis Study Group database was reviewed and analyzed. Results: Among the 752 PBC patients enrolled, 64 of them with AIH/PBC, and 87 cancers were identified in 79 patients, including 20 cases of HCC and 67 of EHM. Patients with HCC had a higher prevalence of cirrhosis (95% vs. 32.5%, p= <0.001), smoking (55% vs. 12.3%, p= <0.001, CREST syndrome (30% vs. 7.6%, p= 0.003) and prior azathioprine (30% vs. 8%, p= 0.005) and prednisone (35% vs. 14%, p= 0.018) previous use, compared with their counterparts. Patients with EHM had a higher prevalence of smoking (42.3% vs. 12.3%, p= <0.001), AMA positivity (96.6% vs 80.6%, p = <0.001), azathioprine use (21% vs 8%, p= 0.01) and concurrent other autoimmune diseases. In multivariate analysis, cirrhosis, obesity and prior azathioprine therapy were independent risk factors for HCC, while Sjogren syndrome and psoriasis were associated with EHM. Fibrates reduced EHM risk. Conclusions: The prevalence of EHM is higher when compared to HCC in PBC patients. Cirrhosis, obesity, prior azathioprine use, and concurrent autoimmune diseases were significantly associated with cancer in PBC, while fibrate use was apparently protective against EHM.

Abstract Background Nonalcoholic fatty liver disease (NAFLD) is prevalent among women with breast cancer. The impact of endocrine therapy on the severity and progression of NAFLD in the long term remains unclear. Aims To assess the prevalence and severity of NAFLD related to hormone therapy for breast cancer, and to investigate risk factors associated with its occurrence and progression. Methods Cross-sectional study recruited women with breast cancer. Abdominal ultrasound was used to detect liver steatosis, and transient elastography to evaluate fibrosis. Results 171 patients were enrolled – mean age 58 ± 10 years and follow-up period 1-315 months (median 53, interquartile range 25–102). Comorbidities: diabetes (26.9%), hypertension (53.2%), dyslipidemia (31.0%) and obesity (70.2%). Four groups were formed: 55 (32.2%) patients unexposed to hormone therapy, 72 (42.1%) exposed only to tamoxifen, 16 (9.4%) only to anastrozole, and 28 (16.4%) to both drugs. Liver steatosis was detected in 57.9%, with no significant differences between groups (p = 0.092). Liver stiffness was similar between groups: median 5.4 kPa (p = 0.200), 12.3% with liver stiffness ≥ 8 Kpa (p = 0.568) and 5.8% ≥12 Kpa (p = 0.177). Diabetes was independently associated with steatosis, and metabolic syndrome with advanced fibrosis, even after adjustment for hormone therapy duration. Conclusion More than half of patients had NAFLD, and approximately 10% had advanced fibrosis. Metabolic risk factors were independently associated with occurrence and progression of NAFLD, regardless of hormone therapy exposure. The risk of NAFLD induced by tamoxifen and anastrozole seems to have been previously overestimated.

BACKGROUND & AIMS:Genetic ancestry or racial differences in health outcomes exist in diseases associated with systemic inflammation (eg, COVID-19). This study aimed to investigate the association of genetic ancestry and race with acute-on-chronic liver failure (ACLF), which is characterized by acute systemic inflammation, multi-organ failure, and high risk of short-term death.METHODS:This prospective cohort study analyzed a comprehensive set of data, including genetic ancestry and race among several others, in 1274 patients with acutely decompensated cirrhosis who were nonelectively admitted to 44 hospitals from 7 Latin American countries.RESULTS:Three hundred ninety-five patients (31.0%) had ACLF of any grade at enrollment. Patients with ACLF had a higher median percentage of Native American genetic ancestry and lower median percentage of European ancestry than patients without ACLF (22.6% vs 12.9% and 53.4% vs 59.6%, respectively). The median percentage of African genetic ancestry was low among patients with ACLF and among those without ACLF. In terms of race, a higher percentage of patients with ACLF than patients without ACLF were Native American and a lower percentage of patients with ACLF than patients without ACLF were European American or African American. In multivariable analyses that adjusted for differences in sociodemographic and clinical characteristics, the odds ratio for ACLF at enrollment was 1.08 (95% CI, 1.03-1.13) with Native American genetic ancestry and 2.57 (95% CI, 1.84-3.58) for Native American race vs European American race CONCLUSIONS: In a large cohort of Latin American patients with acutely decompensated cirrhosis, increasing percentages of Native American ancestry and Native American race were factors independently associated with ACLF at enrollment.

Introduction and objectives: Primary biliary cholangitis (PBC) and autoimmune hepatitis (AIH) and PBC over-lap syndrome (AIH/PBC) have been associated with a higher risk of hepatocellular carcinoma (HCC) and extra-hepatic malignancy (EHM). This study aims to assess potential risk factors associated with cancer development in PBC and AIH/PBC. Materials and methods: The Brazilian Cholestasis Study Group database was reviewed to compare clinical and laboratory features of PBC patients with HCC and EHM with those without cancer. Results: Among the 752 PBC patients enrolled, 64 of them with AIH/PBC, 87 cancers were identified in 72 patients, including 20 cases of HCC and 67 of EHM. Patients with HCC had a higher prevalence of cirrhosis

Introduction:Cryptogenic chronic hepatitis is a growing cause of liver transplants, affecting 5%-15% of patients with chronic liver diseases. This study aimed to identify underlying causes of cryptogenic liver disease in a Brazilian cohort and propose a new diagnostic algorithm, including investigation for metabolic-dysfunction-associated fatty liver disease (MAFLD) and lysosomal acid lipase deficiency (LAL-D). Methods:A retrospective analysis was conducted on 326 patients with presumed cryptogenic hepatitis. Results:Using Czaja's algorithm, non-alcoholic fatty liver disease was diagnosed in 21.3% of patients, while alpha-1 antitrypsin deficiency, alcoholic liver disease, autoimmune hepatitis, hemochromatosis, biliary-related hepatitis, viral hepatitis, Budd-Chiari syndrome, glycogenosis, drug-induced liver injury, and Wilson's disease were diagnosed in smaller proportions (< 3.5% each). LAL-D was found in 1% of patients, and 53.6% of patients remained with cryptogenic hepatitis. The etiology of the liver disease in a subset of patients undergoing liver transplantation was updated post hoc based on explant histology, and non-alcoholic steatohepatitis was found in 52.5% of patients. By incorporating the concept of MAFLD, the new algorithm could diagnose 49.1% of patients, reducing the number of individuals without an etiological diagnosis by 11.4%. Conclusion:One-third of patients with initially presumed cryptogenic liver disease were diagnosed with MAFLD. LAL-D should be considered in patients with chronic liver disease of unknown etiology. The updated diagnostic algorithm proposed in this study could improve diagnostic accuracy and aid in the management of patients with cryptogenic hepatitis.

Introduction: Women with Polycystic Ovary Syndrome (PCOS) have a higher prevalence of Nonalcoholic Fatty Liver Disease (NAFLD) than the general population. PCOS and NAFLD have common metabolic risk factors, however, the role of diet in NAFLD development is still uncertain in PCOS women.Objective: To evaluate and compare the dietary patterns and nutritional intake in patients with PCOS with and without NAFLD.Method: Cross-sectional study that included patients with PCOS diagnosed according to Rotterdam criteria. All participants were submitted to abdominal ultrasound to investigate liver steatosis. Dietary profile was assessed by 24-hour food recall (24hR), and Food Frequency Questionnaire (FFQ). Diet quality was assessed by the Healthy Eating Index (HEI) adapted for the Brazilian population. Physical activity practice was also assessed.Results: 87 participants were included (average age 35.2 +/- 5.7 years), among whom, 67 (77%) had NAFLD. The group with PCOS and NAFLD presented higher body mass index (BMI) (34.9 +/- 4.5 vs. 30.4 +/- 4.9 kg/m2; p = 0.001), Waist Circumference (WC) (103 [97-113] vs. 95 [87.5-100] cm; p < 0.001) and were considered physically active less frequently than those without NAFLD (34.3% vs. 60%; p = 0.04). Food intake and dietary patterns assessed by 24hR, FFQ and HEI presented no difference between the groups.Conclusions: PCOS women with coexistent NAFLD had higher BMI, WC and were less physically active than those without NAFLD. Dietary evaluation showed that PCOS women with NAFLD had no significant difference in macro and micronutrients or food group intake and diet quality in comparison to those without NAFLD.

Background: Lysosomal acid lipase deficiency (LAL-D) is a rare genetic disease associated with the deregulation of lipid metabolism, leading to atherosclerosis, dyslipidemia, and hepatic steatosis, with potential progression to cirrhosis. Our study aims to assess the role of LAL-D in the setting of cryptogenic liver disease. Methods: A large multicenter cross-sectional study was conducted, which included 135 patients with cryptogenic liver disease from four liver centers in Brazil. All patients were submitted to the investigation of LAL enzyme activity on dried blood spots. Results: Three patients (two female) presented levels of LAL below the reference limit, compatible with LAL-D (2.2%). They had a mean age of 43.9 ± 10.1 years and a mean body-mass index (BMI) of 23.1 ± 1.7 kg/m2. The mean serum levels of glucose, HDL-cholesterol, and triglycerides were 89.7 ± 3.2, 21.7 ± 3.2, and 206.7 ± 25.5 mg/dL, respectively. All patients had duodenal polyposis with xanthomatous macrophages. LAL-D investigation should be considered for individuals with chronic liver disease of an unknown etiology, especially with a normal BMI, high triglycerides, and low-HDL-cholesterol levels. The identification of LAL-D patients is extremely important since enzyme replacement therapy with Sebelipase Alfa significantly increases their survival.

BACKGROUND:Mandatory colonoscopy in liver transplantation (LT) candidates is recommended but still controversial.OBJECTIVE:To investigate the frequency of colonoscopy lesions in order to support colorectal cancer (CRC) screening in a real-world pre-LT cohort.METHODS:Retrospective study conducted at a single-center included 632 subjects who underwent pre-transplantation colonoscopy.RESULTS:Median age was 56.9 years (yr.) old (82.3% were ≥50 yr.). Primary sclerosing cholangitis (PSC) occurred in 4.6%. Colonoscopy was abnormal in 438 (69.3%) by detection of polyps (37.7%), vascular changes (29.9%), diverticulosis (18.4%), inflammatory bowel disease features (5.2%) and CRC (0.6%). Histology was available in 66.8% of polyps: hyperplastic (47.8%), low-grade dysplasia (56.6%) and high-grade dysplasia (3.8%). High-risk adenomas occurred in 8.2% of the 594 subjects evaluated. Individuals ≥50 yr. were more likely to present abnormal colonoscopy and polyps. High-grade dysplasia and CRC were only found in individuals ≥50 yr. Patients with high-risk adenomas were more likely to be ≥50 yr.: there was no association between high-risk adenomas detection and liver disease etiology or PSC diagnosis.CONCLUSION:Most LT candidates presented abnormal colonoscopy examination, especially by polyps presence. All cases of high-grade dysplasia and CRC occurred in patients ≥50 yr., regardless of disease etiology.