Characterizing Hepatitis Delta in Spain and the gaps in its management.

BACKGROUND AND AIMS: Chronic hepatitis D (CHD) is a severe form of chronic viral hepatitis. The estimated HDV prevalence in Spain is around 5% of patients with hepatitis B. Reimbursement of new antiviral therapies (Bulevirtide, BLV) was delayed in our country until February 2024. We aimed to characterize the clinical profile of patients with HDV/HBV infection in Spain and current barriers in their management at the time of BLV approval. METHOD: Multicenter registry including patients with positive anti-HDV serology actively monitored in 30 Spanish centers. Epidemiological, clinical and virological variables were recorded at the start of follow-up and at the last visit. RESULTS: We identified 329 anti-HDV patients, 41% were female with median age 51 years. The most common geographical origin was Spain (53%) and East Europe (24%). Patients from Spain were older and had HCV and HIV coinfection probably associated to past drug injection (p<0.01). HDV-RNA was positive in 138 of 221 assessed (62%). Liver cirrhosis was present at diagnosis in 33% and it was more frequent among viremic patients (58% vs 25%, p<0.01). After a median follow-up of 6 (3-12) years, 44 (16%) resolved infection (18 spontaneously and 26 after Peg-INF). An additional 10% of patients developed cirrhosis (n=137) during follow-up (45% had portal hypertension and 14% liver decompensation). Liver disease progression was associated to persisting viremia. CONCLUSION: One-third of the patients with CHD already have cirrhosis at diagnosis. Persistence of positive viremia is associated to rapid liver disease progression. Importantly, barriers to locally determine/quantify HDV-RNA were present.

Enfermedad de Crohn perianal: implicaciones clínicas, pronósticas y consumo de recursos / Perianal Crohn's disease: clinical implications, prognosis and use of resources

Objetivo: conocer la prevalencia de la enfermedad perianal, los factores fenotípicos asociados, su influencia sobre el pronóstico y el impacto en el uso de recursos sanitarios en los pacientes con enfermedad de Crohn.Métodos: estudio observacional retrospectivo unicéntrico en el que incluimos 430 pacientes con enfermedad de Crohn en seguimiento en una consulta monográfica de enfermedad inflamatoria intestinal. Analizamos datos demográficos y fenotípicos de la enfermedad de Crohn, tratamientos farmacológicos y quirúrgicos, pruebas complementarias realizadas e ingresos hospitalarios, realizando estudio comparativo entre los pacientes sin enfermedad perianal y con enfermedad perianal, así como entre las formas simples y complejas.Resultados: la prevalencia de la enfermedad perianal fue del 40,2 % y sus manifestaciones más frecuentes fueron fístulas y abscesos. Su presencia se asoció a la afectación rectal y la existencia de manifestaciones extraintestinales. Los pacientes con enfermedad perianal precisaron con más frecuencia tratamiento inmunosupresor y biológico e ingresos, pero no más cirugía abdominal. Entre los pacientes con enfermedad perianal también fue más frecuente la necesidad de biológicos por la enfermedad luminal (42,8 % vs. 30,7 %). Además, condicionó un mayor consumo de exploraciones dirigidas al estudio de la enfermedad perianal y recto colonoscopias, pero no de entero-resonancia magnética (entero-RM)/entero-tomografía axial computarizada (entero-TAC).Conclusiones: la enfermedad perianal tiene una alta prevalencia en los pacientes con enfermedad de Crohn, sobre todo cuando existe afectación rectal. Se asocia a un peor pronóstico y requiere con más frecuencia tratamientos biológicos tanto por la evolución perianal como luminal, especialmente en la enfermedad perianal compleja. Esto condiciona más necesidad de ingresos hospitalarios y realización de exploraciones complementarias. (AU)

Perianal Crohn's disease: clinical implications, prognosis and use of resources.

OBJECTIVE: to investigate the prevalence of perianal disease, the associated phenotypical factors, its influence on prognosis and its impact on the use of health resources for patients with Crohn's disease. METHODS: a unicentric retrospective observational study was performed with 430 patients with Crohn's disease tracked through a monographical consultation of intestinal inflammatory disease. Demographic and phenotypical data of Crohn's disease, pharmacological and surgical treatments, complementary tests carried out and hospital admissions were analyzed. A comparative study between those patients without perianal disease and those with perianal disease was performed, both in simple form and complex form. RESULTS: the prevalence of perianal disease was 40.2 %, and fistulas and abscesses were the most frequent manifestations. These appearances were associated with an affected rectum and the existence of extra-intestinal manifestations. The patients with perianal disease most frequently required immuno-suppressant and biological treatment, but no further abdominal surgery. Amongst the patients with perianal disease, the need for biologics was more frequent for luminal disease (42.8 % vs 30.7 %). Furthermore, more explorations were needed, aimed at the study of perianal disease and recto-colonoscopies, although more magnetic resonance (MR)/computed tomography (CT) enterographies were not required. CONCLUSIONS: perianal disease has a high prevalence among patients with Crohn's disease, especially when the rectum is affected. It is associated with a worse prognosis and more frequently requires biological treatments due to perianal and luminal evolution, especially in cases of complex perianal disease. This condition calls for more hospital admissions and complementary tests.

Wilson's disease: Revisiting an old friend.

Wilson's disease (WD) is a rare condition caused by copper accumulation primarily in the liver and secondly in other organs, such as the central nervous system. It is a hereditary autosomal recessive disease caused by a deficiency in the ATP7B transporter. This protein facilitates the incorporation of copper into ceruloplasmin. More than 800 mutations associated with WD have been described. The onset of the disease frequently includes manifestations related to the liver (as chronic liver disease or acute liver failure) and neurological symptoms, although it can sometimes be asymptomatic. Despite it being more frequent in young people, WD has been described in all life stages. Due to its fatal prognosis, WD should be suspected in all patients with unexplained biochemical liver abnormalities or neurological or psychiatric symptoms. The diagnosis is established with a combination of clinical signs and tests, including the measurement of ceruloplasmin, urinary copper excretion, copper quantification in liver biopsy, or genetic assessment. The pharmacological therapies include chelating drugs, such as D-penicillamine or trientine, and zinc salts, which are able to change the natural history of the disease, increasing the survival of these patients. In some cases of end-stage liver disease or acute liver failure, liver transplantation must be an option to increase survival. In this narrative review, we offer an overview of WD, focusing on the importance of clinical suspicion, the correct diagnosis, and treatment.

Daño hepático inducido por mesterolona: a propósito de un caso / Drug-induced liver injury due to mesterolone: A case report

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Analysis of the burden and variability in the management of NAFLD patients in the clinical practice: unifying the required criteria

Aim: to assess the prevalence of non-alcoholic fatty liver disease (NAFLD) in the gastroenterology outpatient clinic and describe the use of the resources accordingly. Methods: a prospective and observational study of 403 patients seen in the gastroenterology outpatient clinic to rule out liver disease during three randomized months in 2016. The overall prevalence of NAFLD, disease severity, heterogeneity of the final diagnosis, the use of medical resources and their respective cost were analyzed. Results: the main reason for consultation was hypertransaminasemia (42.9%, 173/403), followed by hepatitis C virus (HCV) (28.5%, 115/403). NAFLD was identified as the definitive diagnosis in 29.8% (120/403) of the cohort, 69.2% (83/120) derived by hypertransaminasemia and 24.2% (29/120) by steatosis. Laboratory tests were performed in 96.7% (116/120), abdominal ultrasound in 88.3% (106/120), viral serology in 79.2% (95/120) and autoimmunity in 70% (84/120) of patients with NAFLD. Liver fibrosis was not assessed in 87.5% of cases. In a post-hoc analysis, 12.1% (17/120) had advanced fibrosis by FIB-4. On ultrasound, 65% (73/106) had hepatic steatosis and 15% (17/106) chronic liver disease (significant fibrosis). The mean time for diagnosis was 2.23 +/- 0.8 visits. The terminology used to define the clinical diagnosis was heterogeneous as follows: a) 48.3% (58/120) hepatic steatosis; b) 15% (18/120) non-alcoholic steatohepatitis; c) 15.8% (19/120) fatty liver; d) 13.3% (16/120) metabolic syndrome; and e) 7.5% (9/120) dual liver disease (fatty liver and alcohol). A pharmacological intervention was performed in six patients, a liver biopsy in two patients and another six were referred to another specialist. The average cost per patient until diagnosis was €570.78, which included analytical, autoantibodies, viral serology and abdominal ultrasound, with a mean of 2.5 consultations. Thus, the total expense in patients with NAFLD was €68,493.6. Conclusion: NAFLD is a frequent cause of hypertransaminasemia. However, the heterogeneity in the management and terminology of the disease makes it necessary to initiate medical training actions in order to unify the criteria for disease control

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Analysis of the burden and variability in the management of NAFLD patients in the clinical practice: unifying the required criteria.

AIM: to assess the prevalence of non-alcoholic fatty liver disease (NAFLD) in the gastroenterology outpatient clinic and describe the use of the resources accordingly. METHODS: a prospective and observational study of 403 patients seen in the gastroenterology outpatient clinic to rule out liver disease during three randomized months in 2016. The overall prevalence of NAFLD, disease severity, heterogeneity of the final diagnosis, the use of medical resources and their respective cost were analyzed. RESULTS: the main reason for consultation was hypertransaminasemia (42.9%, 173/403), followed by hepatitis C virus (HCV) (28.5%, 115/403). NAFLD was identified as the definitive diagnosis in 29.8% (120/403) of the cohort, 69.2% (83/120) derived by hypertransaminasemia and 24.2% (29/120) by steatosis. Laboratory tests were performed in 96.7% (116/120), abdominal ultrasound in 88.3% (106/120), viral serology in 79.2% (95/120) and autoimmunity in 70% (84/120) of patients with NAFLD. Liver fibrosis was not assessed in 87.5% of cases. In a post-hoc analysis, 12.1% (17/120) had advanced fibrosis by FIB-4. On ultrasound, 65% (73/106) had hepatic steatosis and 15% (17/106) chronic liver disease (significant fibrosis). The mean time for diagnosis was 2.23 ± 0.8 visits. The terminology used to define the clinical diagnosis was heterogeneous as follows: a) 48.3% (58/120) hepatic steatosis; b) 15% (18/120) non-alcoholic steatohepatitis; c) 15.8% (19/120) fatty liver; d) 13.3% (16/120) metabolic syndrome; and e) 7.5% (9/120) dual liver disease (fatty liver and alcohol). A pharmacological intervention was performed in six patients, a liver biopsy in two patients and another six were referred to another specialist. The average cost per patient until diagnosis was €570.78, which included analytical, autoantibodies, viral serology and abdominal ultrasound, with a mean of 2.5 consultations. Thus, the total expense in patients with NAFLD was €68,493.6. CONCLUSION: NAFLD is a frequent cause of hypertransaminasemia. However, the heterogeneity in the management and terminology of the disease makes it necessary to initiate medical training actions in order to unify the criteria for disease control.