Alpha-1-antitrypsin deficiency in children: liver disease is not reflected by low serum levels of alpha-1-antitrypsin - a study on 48 pediatric patients.

Background: Alpha-1-antitrypsin (alpha1-AT) is an important protease inhibitor. The phenotypes are characterized by a low total serum alpha1-AT or by an abnormal protein accumulating in the hepatocytes. The aim of our study was to examine a correlation of total serum alpha1-AT, phenotype, and liver involvement in pediatric patients.

Percutaneous transjugular intrahepatic stent shunt for treatment of intractable varicose bleeding in paediatric patients.

Unlabelled: Two 10-year and 11-year-old children with oesophageal and gastric varicose haemorrhage unresponsive to medical treatment and repeated endoscopic sclerotherapy underwent percutaneous transjugular intrahepatic portosystemic shunting (TIPSS). A newly developed introducing system was used. The procedure was performed to avoid the increased risk of emergency liver transplantation in children with hepatic failure.