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Paediatric liver disorders in Singapore
Insights
Pediatric liver diseases share adult pathologies but differ in causes and prevalence. This review covers genetic, neoplastic, and acquired conditions in children, from newborns to older children.
Area of Science:
- Pediatric Hepatology
- Childhood Liver Diseases
- Gastroenterology
Background:
- Infant and child livers are susceptible to adult pathologies, with distinct prevalence and etiological factors.
- Genetic metabolic disorders (e.g., galactosemia, phenylketonuria) and neoplasms (e.g., hepatoblastoma, hemangioma) frequently manifest in newborns.
- Acquired conditions like hepatitis and biliary atresia, along with Reyes encephalopathy and choledochal cysts, present unique challenges in pediatric liver care.
Purpose of the Study:
- To provide a comprehensive overview of liver pathologies in infants and children.
- To highlight differences in prevalence and causes compared to adult liver diseases.
- To discuss the diagnosis and management of specific pediatric liver conditions.
Main Methods:
- Review of common and significant liver diseases in the pediatric age group.
- Discussion of genetic, neoplastic, and acquired liver lesions.
- Exploration of conditions like Reyes encephalopathy, choledochal cysts, portal hypertension, and hepatic fibrosis.
- Consideration of specific etiologies such as beta-thalassemia and tropical liver infections.
Main Results:
- Genetic metabolic disorders are prominent in newborns, alongside neoplasms like hepatoblastoma and hemangioma.
- Hepatitis and biliary atresia are key acquired lesions requiring careful differentiation and management.
- Reyes encephalopathy, choledochal cysts, portal hypertension, and congenital hepatic fibrosis represent significant pediatric liver concerns.
- Certain tropical liver diseases are now rare in developed settings like Singapore.
Conclusions:
- Pediatric liver disease encompasses a broad spectrum from congenital to acquired conditions.
- Early diagnosis and appropriate management are crucial for favorable outcomes in children.
- Understanding the unique epidemiological and etiological profiles of pediatric liver diseases is essential for effective clinical practice.
Abstract:
The liver in an infant or child is as liable to the same pathologies afflicting the adult liver but with certain differences in prevalence and causes. Genetic disorders are more likely to present in the paediatric age group where many involve metabolic processes such as galactosemia, phenylketonuria, glycogen storage disease and others. Many of these present in the newborn period. However, neoplasms and hamartomas also present in the newborn period, such as congenital neuroblastoma with an enormously enlarged liver, hepatoblastoma and haemangioma. The latter may present with intractable cardiac failure as a result of considerable shunting of blood. Acquired liver lesions often present in the newborn period or early infancy and this includes hepatitis and biliary atresia. The difficulties in the differentiation of the two lesions will be discussed together with the management of biliary atresia. As the child grows older, Reyes encephalopathy with microvesicular fat in the liver is not uncommon. The pathophysiology of Reyes encephalopathy as seen locally will be described. The choledochal cyst with direct (Caroli's disease) or indirect effect on the liver will be described. Problems of childhood portal hypertension as well as congenital hepatic fibrosis will be described. Hemosiderosis of the liver is chiefly seen in homozygous beta-thalassaemia patients who have been kept alive with repeated blood transfusions. Amoebic and pyogenic hepatitis, fatty liver due to protein malnutrition, biliary ascariasis, etc, which are common in tropical and subtropical countries are rarely seen now in Singapore children.