Hepatitis autoinmune en la edad pediátrica Autoimmune hepatitis in pediatric patients

Introducción: la hepatitis autoinmune es una enfermedad inflamatoria de origen desconocido responsable de una destrucción progresiva del hígado y evolución hacia la cirrosis. Objetivo: el objetivo es evaluar las características de las hepatitis autoinmunes en la población infantil. Material y métodos: estudio retrospectivo de pacientes diagnosticados en nuestro servicio en los últimos 10 años. Las variables analizadas son: edad, sexo, forma de presentación, función hepática, inmunoglobulinas, autoinmunidad, histología, tratamiento, necesidad de trasplante y evolución clínica. Según la positividad de los auto-anticuerpos se clasifican en tipo I (ANA y/o ASMA) y tipo II (LKM-1). Resultados: se diagnostica a siete pacientes, 5 mujeres (71,5%) y 2 varones (28,5%); tipo I 5 y tipo II dos pacientes. La edad al diagnóstico es 21 meses a 12 años. En el tipo I la presentación clínica es como hepatitis aguda en 3 casos y 2 pacientes con insuficiencia hepática progresiva. Las tipo II se diagnostican tras un hallazgo analítico siendo asintomáticas. La elevación de transaminasas (x10 su valor) se observa en el 71,5% e hipergammaglobulinemia en el 85%. El tratamiento instaurado es azatioprina y corticosteroides con un tiempo medio de remisión de 14 meses. Dos pacientes recaen al retirar corticosteroides. Conclusión: las formas de presentación son variadas y puede ser indistinguible a una hepatitis viral. Se debe sospechar ante una elevación de las aminotransferasas y la presencia de hipergammaglobulinemia. Con buenos resultados el tratamiento recomendado sería azatioprina y corticosteroides. Existen altos porcentajes de recaídas al retirar la corticoterapia por lo que algunos pacientes precisarían de dosis mínimas para mantener la remisión.Background: autoimmune hepatitis (AIH) is an inflammatory disease of unknown origin that is responsible for progressive liver necrosis and ultimately cirrhosis. Objective: our aim was to evaluate the characteristics of autoimmune hepatitis presenting in the pediatric age. Material and methods: we conducted a retrospective study of all patients diagnosed with AIH in our hospital department during the last 10 years. Variables analyzed included age, sex, clinical presentation, hepatic function, immunoglobulins, autoimmunity markers, histology, treatment, need for transplant, and clinical evolution. According to the positive level of auto-antibodies, AIH patients were classified as type I AIH (ANA and/or smooth-muscle antibodis) and type II (anti-LKM-1). Results: seven patients were diagnosed in this period -5 girls (71.5%) and 2 boys (28.5%). Five patients presented with type-I serological markers, and two with type-II markers. Age range at diagnosis was from 21 months to 12 years. In the type-I group, 3 patients presented with acute hepatitis while 2 other patients were diagnosed from laboratory findings while asymptomatic. Elevated aminotransferase (10 times the normal level) was observed in 71.5%, and 85% had elevated immunoglobulins. Treatment with azathioprine and prednisone was started after diagnosis with an average time to remission of 14 months. Two patients relapsed following steroid withdrawal. Conclusion: AIH can have different forms of clinical presentation, and is sometimes indistinguishable from viral hepatitis. AIH must be ruled out in patients presenting with concomitant elevation of aminotransferases and immunoglobulins. The commonly accepted treatment is a combination of azathioprine and corticosteroids. A high percentage of patients experience a relapse of disease after steroids are withdrawn. Therefore, some patients will need to stay on combined therapy with minimal doses of steroids

Vedolizumab in Pediatric inflammatory bowel diseases: a retrospective multi-center experience from the paediatric IBD Porto group of ESPGHAN

Vedolizumab (VDZ) has proven as an effective medication in adult Inflammatory Bowel Disease (IBD). There has been increased off-label use of VDZ also in children but with very limited published experience. Therefore we aimed to describe the short-term effectiveness and safety of VDZ in children with IBD in the largest pediatric cohort to date

Quality of Life in Paediatric Crohn’s Disease: Data From the Imagekids Study

Background: The evaluation of health-related quality of life (HRQOL), using the validated disease-specific IMPACT-III questionnaire, has a key role in ascertaining the effect of disease on patients with Crohn’s disease (CD). We sought to describe HRQOL variations across a large prospective cohort of paediatric CD patients with varying disease experience. Methods: We used the prospectively collected data from the ImageKids study (a multicentre, multinational study designed to develop the pMEDIC and PICMI scores for magnetic resonance enterography) on children diagnosed with CD. IMPACT-III (35-item self-administered scale) was used to assess HRQOL in this cohort. Results: Data from 180 patients were analysed, 94 males (52.2%) with a mean age of 14.2±2.2y and a median of 27 month (IQR 0.05–4.2)of follow-up. According to wPCDAI, 29.0% of patients were in clinical remission, whereas 39%, 13%, and 19% had mild, moderate, and severe disease, respectively. IMPACT-III total score had a poor but significant correlation with degree of mucosal inflammation judged by the SES-CD (r = -0.285, p \u3c 0.0001). Correlation was strong with clinical activity judged by wPCDAI (r = -0.550, p \u3c 0.0001). Patients with higher disease activity had lower total IMPACT-III score, as did the 4 domains (wellbeing, emotional functioning, social functioning, and body image, Table I). Differences across wPCDAI groups were higher for wellbeing and lower for body-image domains (Figure 1). Patients with perianal disease had lower wellbeing (p = 0.026) and body image (p = 0.004) domain scores. Steroid treatment was associated more with lower emotional functioning score than enteral nutrition was (p = 0.028). Conclusions: In this ImageKids cohort, HRQOL was lower in patients with higher disease activity and in those with perianal disease. An awareness of which domains within IMPACT may be differentially affected by various therapies or disease characteristics could help the clinician by focusing interventions (ie, psychological) to address these areas of concern

Quality of Life in Paediatric Crohn’s Disease: Data From the Imagekids Study

Background: The evaluation of health-related quality of life (HRQOL), using the validated disease-specific IMPACT-III questionnaire, has a key role in ascertaining the effect of disease on patients with Crohn’s disease (CD). We sought to describe HRQOL variations across a large prospective cohort of paediatric CD patients with varying disease experience. Methods: We used the prospectively collected data from the ImageKids study (a multicentre, multinational study designed to develop the pMEDIC and PICMI scores for magnetic resonance enterography) on children diagnosed with CD. IMPACT-III (35-item self-administered scale) was used to assess HRQOL in this cohort. Results: Data from 180 patients were analysed, 94 males (52.2%) with a mean age of 14.2±2.2y and a median of 27 month (IQR 0.05–4.2)of follow-up. According to wPCDAI, 29.0% of patients were in clinical remission, whereas 39%, 13%, and 19% had mild, moderate, and severe disease, respectively. IMPACT-III total score had a poor but significant correlation with degree of mucosal inflammation judged by the SES-CD (r = -0.285, p \u3c 0.0001). Correlation was strong with clinical activity judged by wPCDAI (r = -0.550, p \u3c 0.0001). Patients with higher disease activity had lower total IMPACT-III score, as did the 4 domains (wellbeing, emotional functioning, social functioning, and body image, Table I). Differences across wPCDAI groups were higher for wellbeing and lower for body-image domains (Figure 1). Patients with perianal disease had lower wellbeing (p = 0.026) and body image (p = 0.004) domain scores. Steroid treatment was associated more with lower emotional functioning score than enteral nutrition was (p = 0.028). Conclusions: In this ImageKids cohort, HRQOL was lower in patients with higher disease activity and in those with perianal disease. An awareness of which domains within IMPACT may be differentially affected by various therapies or disease characteristics could help the clinician by focusing interventions (ie, psychological) to address these areas of concern

Corona virus disease 2019 and paediatric inflammatory bowel diseases: Global experience and provisional guidance (March 2020) from the Paediatric IBD Porto Group of European Society of Paediatric Gastroenterology, Hepatology, and Nutrition

Introduction: With the current coronavirus disease 2019 (COVID-19) pandemic, concerns have been raised about the risk to children with inflammatory bowel diseases (IBD). We aimed to collate global experience and provide provisional guidance for managing paediatric IBD (PIBD) in the era of COVID-19. Methods: An electronic reporting system of children with IBD infected with SARS-CoV-2 has been circulated among 102 PIBD centres affiliated with the Porto and Interest-group of ESPGHAN. A survey has been completed by major PIBD centres in China and South-Korea to explore management during the pandemic. A third survey collected current practice of PIBD treatment. Finally, guidance points for practice have been formulated and voted upon by 37 PIBD authors and Porto group members. Results: Eight PIBD children had COVID-19 globally, all with mild infection without needing hospitalization despite treatment with immunomodulators and/or biologics. No cases have been reported in China and South Korea but biologic treatment has been delayed in 79 children, of whom 17 (22%) had exacerbation of their IBD. Among the Porto group members, face-to-face appointments were often replaced by remote consultations but almost all did not change current IBD treatment. Ten guidance points for clinicians caring for PIBD patients in epidemic areas have been endorsed with consensus rate of 92% to 100%. Conclusions: Preliminary data for PIBD patients during COVID-19 outbreak are reassuring. Standard IBD treatments including biologics should continue at present through the pandemic, especially in children who generally have more severe IBD course on one hand, and milder SARS-CoV-2 infection on the other.</p