Hepatology and Pancreatic Science

Paediatric Autoimmune Hepatitis (AIH) is a rare but serious chronic liver disease that primarily affects children and adolescents. It is characterized by the immune system mistakenly attacking the liver cells, leading to inflammation and potential long-term damage. Over the years, significant advancements have been made in the diagnosis, management, and long-term success outcomes for Paediatric AIH. This article explores these recent achievements, highlighting the strides made in understanding the disease, improving diagnostic methods, enhancing treatment approaches, and ultimately improving the prognosis for affected children. The diagnosis of Paediatric AIH has evolved with the emergence of advanced laboratory techniques and diagnostic criteria. While the disease remains challenging to diagnose due to its diverse clinical presentation, several recent achievements have improved the accuracy of identification.

The liver, often referred to as the body's metabolic powerhouse, plays a crucial role in maintaining overall health and wellbeing. Responsible for processing nutrients, detoxifying harmful substances, and regulating numerous metabolic functions, the liver is vulnerable to a variety of disorders that can significantly impact its function. Therapeutic diets tailored to address specific liver disorders have emerged as a vital component of managing these conditions and promoting liver health. In this comprehensive exploration, we delve into the world of therapeutic diets for liver disorders, examining their importance, key considerations, and the impact they have on patients' lives. Liver disorders encompass a wide spectrum of conditions, ranging from fatty liver disease, hepatitis, cirrhosis, to more rare genetic disorders like Wilson's disease and hemochromatosis.

Cholestatic liver conditions encompass a group of disorders characterized by impaired bile flow, leading to a build-up of bile acids and other substances in the liver. Among these conditions, Alagille Syndrome (ALGS) stands out as a rare genetic disorder that affects various organ systems, with the liver being a central target. ALGS, also known as Alagille-Watson Syndrome or arteriohepatic dysplasia, was first described by Dr. Daniel Alagille in 1969. It is an autosomal dominant disorder caused by mutations in the JAG1 or NOTCH2 genes, affecting multiple organs including the liver, heart, skeleton, and eyes. This paper delves into the clinical characteristics of Alagille Disorder in individuals with cholestatic liver conditions, shedding light on its presentation, diagnosis, and potential therapeutic strategies.

Liver transplantation is a life-saving procedure for individuals with end-stage liver disease, acute liver failure, or certain liver-related malignancies. While the primary goal of this procedure is to restore liver function and overall health, liver transplant recipients often encounter various metabolic changes that can have significant implications for their body composition, including the accumulation of fat. Understanding the metabolic processes and factors determining the amount of fat in the bodies of liver transplant recipients is crucial for optimizing their long-term health and well-being. Liver transplantation involves the replacement of a dysfunctional liver with a healthy donor liver. This procedure not only affects the direct functions of the liver but also has far-reaching impacts on the body's metabolism.