10.1093/ibd/izy200_video1izy200.video15794817619001.

Three guidelines in Wilson disease (WD) have been issued to date: by the American Association for the Study of Liver Diseases (AASLD) in 2003 with revision in 2008, by the European Association for the Study of the Liver (EASL) in 2012, and most recently by the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) in 2018. The following review aims to compare and contrast the approach to diagnosis and management of WD outlined in each guidance. Diagnostic criteria for WD are variable, with the AASLD proposing a clinical/biochemical algorithmic approach, while EASL and ESPGHAN favor use of the Leipzig score. Screening of first-degree relatives differs in modality: clinical and genetic testing in AASLD and ESPGHAN, versus genetic testing alone in EASL. There is general consensus regarding treatment of WD, though ESPGHAN favors zinc over chelators in maintenance phase and for asymptomatic patients. Liver transplantation is indicated in cases of acute liver failure (ALF) due to WD, but not primarily for neuropsychiatric disease in all guidelines. EASL and ESPGHAN advocate for use of the revised King's score to guide transplant listing. There are limited recommendations on special circumstances including pregnancy, surgery, and malignancy risk in WD. Though current recommendations address the management of liver disease due to WD, future guidelines may include a more detailed discussion of neurological and psychiatric manifestations of WD.

Background and aims Non-alcoholic fatty liver disease (NAFLD) is strongly associated with cardiovascular disease in the general population. Both conditions seem more frequent in patients with inflammatory bowel disease (IBD). We aimed to assess the effect of NAFLD and liver fibrosis on intermediate-high cardiovascular risk in IBD. Methods We prospectively included IBD patients undergoing a routine screening program for NAFLD by transient elastography (TE) with associated controlled attenuation parameter (CAP). NAFLD and significant liver fibrosis were defined as CAP >275 dB/m and liver stiffness measurement (LSM) by TE ≥8 kPa, respectively. Cardiovascular risk was assessed with the atherosclerotic cardiovascular disease (ASCVD) risk estimator and categorized as low if <5%, borderline if 5%–7.4%, intermediate if 7.5%–19.9% and high if ≥20% or if previous cardiovascular event. Predictors of intermediate-high cardiovascular risk were investigated by multivariable logistic regression analysis. Results Of 405 patients with IBD included, 278 (68.6%), 23 (5.7%), 47 (11.6%) and 57 (14.1%) were categorized as at low, borderline, intermediate and high ASCVD risk, respectively. NAFLD and significant liver fibrosis were found in 129 (31.9%) and 35 (8.6%) patients, respectively. After adjusting for disease activity, significant liver fibrosis and BMI, predictors of intermediate-high ASCVD risk were NAFLD (adjusted odds ratio [aOR] 2.97, 95% confidence interval [CI] 1.56–5.68), IBD duration (aOR 1.55 per 10 years, 95% CI 1.22–1.97), and ulcerative colitis (aOR 2.32, 95% CI 1.35–3.98). Conclusions Assessment of cardiovascular risk should be targeted in IBD patients with NAFLD, particularly if they have longer IBD duration and ulcerative colitis.

Background People living with human immunodeficiency virus (PLWH) are at increased risk of cirrhosis and esophageal varices. Baveno VI criteria, based on liver stiffness measurement (LSM) and platelet count, have been proposed to avoid unnecessary esophagogastroduodenoscopy (EGD) screening for esophageal varices needing treatment (EVNT). This approach has not been validated in PLWH. Methods PLWH from 8 prospective cohorts were included if they fulfilled the following criteria: (1) compensated advanced chronic liver disease (LSM >10 kPa); (2) availability of EGD within 6 months of reliable LSM. Baveno VI (LSM <20 kPa and platelets >150 000/μL), expanded Baveno VI (LSM <25 kPa and platelets >110 000/μL), and Estudio de las Hepatitis Víricas (HEPAVIR) criteria (LSM <21 kPa) were applied to identify patients not requiring EGD screening. Criteria optimization was based on the percentage of EGDs spared, while keeping the risk of missing EVNT <5%. Results Five hundred seven PLWH were divided into a training (n = 318) and a validation set (n = 189). EVNT were found in 7.5%. In the training set, Baveno VI, expanded Baveno VI, and HEPAVIR criteria spared 10.1%, 25.5%, and 28% of EGDs, while missing 0%, 1.2%, and 2.2% of EVNT, respectively. The best thresholds to rule out EVNT were platelets >110 000/μL and LSM <30 kPa (HIV cirrhosis criteria), with 34.6% of EGDs spared and 0% EVNT missed. In the validation set, HEPAVIR and HIV cirrhosis criteria spared 54% and 48.7% of EGDs, while missing 4.9% and 2.2% EVNT, respectively. Conclusions Baveno VI criteria can be extended to HEPAVIR and HIV cirrhosis criteria while sparing a significant number of EGDs, thus improving resource utilization for PLWH with compensated advanced chronic liver disease.

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