The risks of poor transition include delayed and inappropriate transfer that can result in
disengagement with healthcare. Structured transition care can improve control of chronic …

Nonselective β-blocker therapy and endoscopic variceal ligation reduce the incidence of
variceal hemorrhage in cirrhotic adults, but their use in children is controversial. There are …

Background Biliary atresia is an obliterative cholangiopathy of infancy that is fatal if
untreated. Surgical treatment, the Kasai portoenterostomy, may restore bile flow and clear …

ARC syndrome (OMIM 208085) is an autosomal recessive multisystem disorder
characterized by neurogenic arthrogryposis multiplex congenita, renal tubular dysfunction …

The long-term histological outcome after pediatric liver transplantation (OLT) is not yet fully
understood. De novoautoimmune hepatitis, consisting of histological chronic hepatitis …

Glycogen storage disease type III (GSDIII) is a rare disorder of glycogenolysis due to AGL
gene mutations, causing glycogen debranching enzyme deficiency and storage of limited …

Abstract Hereditary tyrosinaemia type 1 (HT-1) is a rare genetic disease caused by
mutations in the gene for the enzyme fumarylacetoacetase. It usually presents with liver …

Bleeding problems are associated with defects in platelet α-granules, yet little is known
about how these granules are formed and released. Mutations affecting VPS33B, a novel …

Hepatopulmonary syndrome (HPS) in stable patients with cirrhosis can easily be
overlooked. We report on the presenting symptoms, disease progression, and outcomes …

Acute liver failure (ALF) in infancy and childhood is a life-threatening emergency. Few
conditions are known to cause recurrent acute liver failure (RALF), and in about 50% of …