Chapter 14 – Alagille Syndrome
Abstract Alagille syndrome (ALGS) is an autosomal dominant, multisystem disorder which was first described in 1969 by Daniel Alagille as a constellation of clinical features in five different organ systems…
Abstract Alagille syndrome (ALGS) is an autosomal dominant, multisystem disorder which was first described in 1969 by Daniel Alagille as a constellation of clinical features in five different organ systems…
Abstract Hepatitis C virus (HCV) is an important cause of viral hepatitis in children and the actual number of infected children is clearly underestimated. HCV infection across the pediatric age…
Abstract Autoimmune hepatitis (AIH) is a progressive inflammatory disorder of unknown etiology, characterized histologically by interface hepatitis, serologically by the presence of non-organ specific autoantibodies, biochemically by elevated aminotransferases and…
Abstract Optimal care of children with viral hepatitis necessitates incorporation of recent advances in diagnosis, prevention, and treatment into clinical practice. Although primary viral infection of the liver has been…
Abstract Intestinal failure (IF) is defined as the end result of any gastrointestinal disorder in which functional intestinal mass is insufficient to allow adequate growth, hydration, and electrolyte balance in…
Abstract Elevation of the serum bilirubin level is a common finding during the first week of life. This can be a transient phenomenon that resolves spontaneously, or it can signify…
Abstract Inherited cholestasis of hepatocellular origin has long been described in the neonate or during the first year of life [1]. Many of these infants were categorized as having idiopathic…
Abstract Biliary atresia and related disorders of the biliary tract, such as choledochal cysts, must be considered in the differential diagnosis of prolonged conjugated hyperbilirubinemia in the newborn (neonatal cholestasis)….
Abstract Neonatal hepatitis refers to a heterogeneous group of disorders that result in a somewhat similar morphologic change in the liver of an infant younger than three months of age…
Abstract When first encountering an infant or child with cholestatic liver disease, it is essential that diagnostic evaluation be conducted promptly in order to recognize disorders amenable either to specific…