scholarly journals Indomethacin increases liver damage in a murine model of liver injury from alpha-1-antitrypsin deficiency

Hepatology ◽  
2006 ◽  
Vol 44 (4) ◽  
pp. 976-982 ◽  
Author(s):  
David A. Rudnick ◽  
Olga Shikapwashya ◽  
Keith Blomenkamp ◽  
Jeffrey H. Teckman
Keyword(s):  
Liver Injury ◽  
Liver Damage ◽  
Murine Model ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

scholarly journals Oxidative stress contributes to liver damage in a murine model of alpha-1-antitrypsin deficiency

2012 ◽  
Vol 237 (10) ◽  
pp. 1163-1172 ◽  
Author(s):  
Nancy Y Marcus ◽  
Keith Blomenkamp ◽  
Muneeb Ahmad ◽  
Jeffrey H Teckman
Keyword(s):  
Oxidative Stress ◽  
Liver Damage ◽  
Murine Model ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

scholarly journals Pathophysiological aspects of liver damage in children with alpha-1-antitrypsin deficiency

2020 ◽  
Vol 65 (1) ◽  
pp. 11-21
Author(s):  
G. V. Volynets ◽  
A. V. Nikitin
Keyword(s):  
Liver Disease ◽  
Liver Damage ◽  
Autosomal Recessive ◽  
Neonatal Period ◽  
Autosomal Recessive Disease ◽  
Clinical Manifestations ◽  
Homozygous Mutation ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

Alpha-1-antitrypsin deficiency is an autosomal recessive disease characterized by both liver damage and lung disease in children and adults because of a decrease in the serum protein content due to the mutations in the PI (proteinase inhibitor) gene. The majority of liver diseases are associated with a homozygous mutation of the Z allele. There are many variations of clinical manifestations of the liver disease in children with the PI*ZZ genotype.  In the neonatal period, liver disease is usually cholestatic; and it is accompanied by a prolonged cholestatic jaundice, skin itching, which can be determined only later (after 6 months), decreased appetite and bad weight gain, hepato- and splenomegaly. The article describes the pathophysiology of liver damage in children with alpha-1-antitrypsin deficiency. The authors provide their recommendations for the management of children with suspected and confirmed alpha-1-antitrypsin deficiency.

scholarly journals Characteristics of hepatocellular carcinoma in a murine model of alpha-1-antitrypsin deficiency

2010 ◽  
Vol 40 (6) ◽  
pp. 641-653 ◽  
Author(s):  
Nancy Y. Marcus ◽  
Elizabeth M. Brunt ◽  
Keith Blomenkamp ◽  
Faiza Ali ◽  
David A. Rudnick ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Murine Model ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin
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