Growth failure, delayed puberty and malnutrition are common in children with Alagille syndrome. Children with AGS have worse growth failure than children with other chronic liver diseases, such as biliary atresia. Although the exact cause of this growth failure is unknown, possible causes are:
Proper nutrition for children with Alagille syndrome is necessary to prevent and treat growth failure and to prevent and treat nutritional deficiencies. Children with AGS may be evaluated and monitored by a registered dietitian (RD) to assess their growth and nutritional status as well as to make individualized dietary recommendations. At The Children's Hospital of Philadelphia, these services are provided by the Nutrition Services Program. Children who are unable to consume adequate calories or who have poor growth may require a nasogastric tube (NG tube) or gastrostomy tube (G-tube) to improve their nutrition and growth. Children's Hospital provides these services through its Pediatric Enteral Access Center.
Children with Alagille syndrome who undergo liver transplantation do not have as much "catch-up" growth after transplantation as children with other liver diseases, which may suggest that poor growth is due to the primary gene defect in Jagged1. Unlike other pediatric liver diseases, Alagille syndrome can also involve organ systems — such as the kidney or heart — which may impair growth. More research is needed to learn why children with AGS do not grow as much after liver transplantation as children with other liver diseases grow.
Your child's physician and dietitian will work with your child to ensure that he or she gets the best possible nutrition to support growth and development. It is important for children with AGS to consume adequate calories and maintain normal serum levels of vitamins.
Reviewed by: Binita M. Kamath, MD
Date: January 2009