Chronic liver disease (CLD) is a major global health problem, affecting millions of people worldwide. According to the Global Burden of Disease Study, liver diseases accounted for over 2 million deaths and 49 million disability-adjusted life years (DALYs) in 2019 [1]. The main chronic liver conditions contributing to mortality include cirrhosis, liver cancer, and viral hepatitis. The burden of viral hepatitis may be less of a contributing factor now due to treatment availability for Hepatitis C but is likely to be offset by the increasing burden of Metabolic Associated Steatotic Liver Disease (MA-SLD) previously known as non-alcoholic fatty liver disease (NAFLD) [2]. The increasing incidence of obesity and metabolic syndrome in children along with other chronic liver diseases is undoubtedly going to contribute to increased mortality in the future years. The burden of CLD is significant and not only does it have a profound impact on individuals and families, but also on economy of healthcare systems. Hence the management of CLD requires a multidisciplinary approach involving pediatric hepatologists, dietitians, surgeons, and other allied healthcare professionals i.e., physiotherapists, family support workers, psychologists, play therapists etc.

Early recognition of conditions causing CLD and appropriate investigations with timely management to prevent and halt the progression in those conditions is essential to improve the long-term outcome of children and families. In some children, even with supportive management, there is inevitable progression of liver disease and scarring within the liver to cirrhosis.

Cirrhosis is characterized by varying degree of fibrosis replacing the healthy parenchyma, secondary to long standing damage and inflammation. It is broadly classified as an early asymptomatic stage i.e., compensated CLD and a later stage of decompensated CLD characterized by appearance of ascites, jaundice, hepatic encephalopathy and gastrointestinal bleeding [3]. When an acute insult occurs in a diseased liver (previously known or not) and causes liver decompensation, organ failures and poor outcome, it is labelled as Acute on chronic liver failure (ACLF) [4]. This needs urgent recognition as timely management of the acute insult can improve the outcome.

There is a hyperdynamic circulatory state with splanchnic vasodilation, systemic inflammation, immune dysfunction and portal hypertension with portosystemic shunting in cirrhosis. This is responsible for the various complications of cirrhosis including ascites, hepatic encephalopathy, variceal bleeding, acute kidney injury (AKI), hepatopulmonary syndrome (HPS), portopulmonary hypertension (PPHN), cirrhotic cardiomyopathy (CCM) and infections [3]. Other systemic manifestations may be etiology specific like lung involvement in alpha 1 antitrypsin deficiency, inflammatory bowel disease in sclerosing cholangitis, neurological complications in Wilson’s disease etc. A knowledge about these has relevance for patient evaluation and also for management and prognosis.

Children with CLD often have severe malnutrition, poor muscle mass and specific nutritional deficiencies at presentation, which need to be recognised and managed with a customized nutrition plan by a dietitian. This may involve nutritional rehabilitation with high caloric supplemental drinks enterally or with nasogastric tube feeds for a variable period depending on severity of malnutrition at the time of presentation. In extreme circumstances, parenteral nutrition may be needed to improve nutrition when malabsorption may prevent enteral nutrition intake. The nutritional management may have to continue even after liver transplantation (LT).

In some cases, LT may be necessary for children with end-stage liver disease or progressive liver failure. This involves replacing the diseased liver with a healthy liver from a deceased or living donor. The child will require lifelong immunosuppressive medications to prevent organ rejection. The expertise in management of the LT is at specialist centres with multidisciplinary teams taking care of the patients. There are several complications that can develop after LT and timely interventions can help in early recognition and treatment that can save the liver transplant from chronic damage. It is equally important for clinicians to recognise that there are certain liver conditions which preclude LT and hence after seeking specialist opinion, families should be counselled about the futility of the procedure in those select cases. This can only be possible if awareness amongst local clinicians is raised and early help sought from specialist centre thus reinforcing the importance of close ties between local pediatricians and specialist centres of expertise in best interest of the patient.

Patient education regarding the long-term complications, importance of compliance and follow-up and measures to prevent further liver damage is vital yet often neglected. CLD can have a significant impact on the child's emotional well-being and quality of life which can affect daily functioning and overall well-being. As a part of ongoing care for children with CLD, the psychosocial support, counselling, and access to support groups are essential to cope with the challenges of living with a chronic illness and will help to navigate the management into adulthood.

Over recent years, advances in genetics and molecular biology have improved our understanding of many conditions presenting as CLD and helped develop specific therapy for these disorders. There is a huge amount of research being done in CLD to prevent and halt progression of liver disease, and in the future, there is a possibility of using medications which can actually reverse liver disease and scarring associated with liver disease [5]. The role of gut microbiota and its manipulation to influence progression of liver disease remains an uncharted territory in improving long term management and outcome of CLD. The focus of management is shifting to early identification, reversal of causative factors and prevention rather than treatment of complications.

This special symposium on CLD in children, published in two issues of IJP covers a comprehensive range of topics on diagnosis, and management of CLD and its associated complications including LT [6,7,8,9,10,11,12,13]. The various national as well as international experts with the wealth of knowledge and experience have formulated an easy, practical approach for management of CLD in clinical practice. This includes medications to alleviate symptoms or treat underlying disease and slow down disease progression, nutritional support, and in some cases, LT. These complex decisions are best done in conjunction with a specialist centre with multidisciplinary team input.

In conclusion, CLD poses a significant burden on individuals, families, and healthcare systems globally. There is a need of a concerted effort from the patients, pediatricians, and pediatric hepatologists to identify early, prevent and manage CLD correctly in order to reduce mortality, improve quality of life, and alleviate the economic and social burden associated with this condition.