RESUMO
Chronic liver disease is a growing problem that has substantial effects on public health. Many paediatric liver conditions are precursors of adult chronic liver disease, cirrhosis, and hepatocellular carcinoma. Clinical management of Wilson's disease, autoimmune liver disease, and chronic biliary disorders, such as biliary atresia, which remains the most common paediatric chronic liver disease and indication for liver transplantation, is similar in children and adults. In the past 10 or so years, paediatric hepatology has expanded into neighbouring clinical areas, such as metabolic liver diseases and systemic conditions with liver involvement. We aim to describe some of these disorders, and outline their natural history and possible differences between management in adults and children to stimulate further debate on the optimal transition of care between paediatric and adult specialists.
Assuntos
Hepatopatias , Adolescente , Criança , Doença Crônica , Progressão da Doença , Humanos , Hepatopatias/complicações , Hepatopatias/diagnóstico , Hepatopatias/fisiopatologia , Hepatopatias/terapiaRESUMO
Non-alcoholic fatty liver disease (NAFLD) is the most common paediatric liver disease with a prevalence of almost 10%; therefore, the majority of affected patients are under the care of general practitioners and non-specialists. The condition is caused by central obesity with insulin resistance with additional factors influencing inflammatory activity (steatohepatitis). Ongoing inflammation leads to fibrosis and end-stage liver disease, though this will usually occur after children have transitioned into adult care. However, their main morbidity and mortality is from type 2 diabetes and complications of atherosclerosis. The minority of children undergo biopsy but currently there is no other method to accurately assess the stage of disease. Management is focused at weight loss through a combination of diet and exercise. Here, we present a current review of paediatric NAFLD aimed at non-specialists, with practice points for implementation.
Assuntos
Hepatopatia Gordurosa não Alcoólica/diagnóstico , Hepatopatia Gordurosa não Alcoólica/etiologia , Biópsia , Criança , Diabetes Mellitus Tipo 2/complicações , Diagnóstico Diferencial , Humanos , Fígado/patologia , Hepatopatia Gordurosa não Alcoólica/terapia , Obesidade/complicações , Prognóstico , Encaminhamento e Consulta , Redução de PesoRESUMO
Neonatal jaundice lasting greater than 2 weeks should be investigated. Pale stools and dark or yellow urine are evidence of liver disease, which should be urgently investigated. The neonatal hepatitis syndrome has many causes, and a structured approach to investigation is mandatory. It should be possible to confirm or exclude biliary atresia within one week, so that definitive surgery is not delayed unnecessarily. Babies with the neonatal hepatitis syndrome should have vigorous fat-soluble vitamin supplementation, including parenteral vitamin K if coagulation is abnormal. The prognosis for infants with idiopathic neonatal hepatitis and multifactorial cholestasis is excellent.