End-to-side portacaval shunts were carried out in three children with the liver disease of alpha-1-antitrypsin deficiency and complications of portal hypertension. Their clinical courses have been stable for 31/2 to almost 7 years. Postoperative liver biopsy material from two of the patients showed the typical histopathological changes caused by portal diversion, as well as an apparent reduction in the quantity of alpha-1-antitrypsin particles in the hepatocytes. The metabolic changes caused by portal diversion have apparently created a more favourable equilibrium between the synthesis and excretion of the abnormal alpha-1-antitrypsin. © 1983
Purpose : A congenital extrahepatic portosystemic shunt (CEPS) is a rare abnormality. The shunts are...
Objectivesα-1-Antitrypsin (A1AT) deficiency is a common genetic disease with an unpredictable and hi...
External biliary fistula (BF) or ileal bypass (IB) was performed in dogs at the time of or 2 weeks a...
Two children with glycogen storage disease were treated with portacaval transposition. The first is ...
Seven patients with Types I, III, or VI glycogen storage disease were treated with portal diversion ...
ALPHA-1-antitrypsin deficiency associated with chronic obstructive airway disease was recognized in ...
Complete portacaval shunt was used to treat 10 patients with glycogen storage disease. A favourable ...
Background/Aims: The response to the liver damage caused by portacaval shunt (PCS) is characterized ...
A 16-year-old girl with advanced cirrhosis and severe alpha1-antitrypsin deficiency of the homozygou...
The use of a completely diverting portacaval shunt for the purpose of lowering the serum concentrati...
The object of this work was to establish an experimental model in the rat to examine the relative ro...
A 17‐yr‐oId female received a liver transplant for type I glycogen storage disease. A year later, wh...
We investigated noninvasive follow-up markers for histologic liver fibrosis and portal hypertension ...
Portocaval shunt was performed in ten patients with homozygous and two with heterozygous familial hy...
The diagnosis and surgical management of extrahepatic portal hypertension in infants and children ha...
Purpose : A congenital extrahepatic portosystemic shunt (CEPS) is a rare abnormality. The shunts are...
Objectivesα-1-Antitrypsin (A1AT) deficiency is a common genetic disease with an unpredictable and hi...
External biliary fistula (BF) or ileal bypass (IB) was performed in dogs at the time of or 2 weeks a...
Two children with glycogen storage disease were treated with portacaval transposition. The first is ...
Seven patients with Types I, III, or VI glycogen storage disease were treated with portal diversion ...
ALPHA-1-antitrypsin deficiency associated with chronic obstructive airway disease was recognized in ...
Complete portacaval shunt was used to treat 10 patients with glycogen storage disease. A favourable ...
Background/Aims: The response to the liver damage caused by portacaval shunt (PCS) is characterized ...
A 16-year-old girl with advanced cirrhosis and severe alpha1-antitrypsin deficiency of the homozygou...
The use of a completely diverting portacaval shunt for the purpose of lowering the serum concentrati...
The object of this work was to establish an experimental model in the rat to examine the relative ro...
A 17‐yr‐oId female received a liver transplant for type I glycogen storage disease. A year later, wh...
We investigated noninvasive follow-up markers for histologic liver fibrosis and portal hypertension ...
Portocaval shunt was performed in ten patients with homozygous and two with heterozygous familial hy...
The diagnosis and surgical management of extrahepatic portal hypertension in infants and children ha...
Purpose : A congenital extrahepatic portosystemic shunt (CEPS) is a rare abnormality. The shunts are...
Objectivesα-1-Antitrypsin (A1AT) deficiency is a common genetic disease with an unpredictable and hi...
External biliary fistula (BF) or ileal bypass (IB) was performed in dogs at the time of or 2 weeks a...