alpha-1-antitrypsinbile ductsA child is reported whose alpha‐1 ‐antitrypsin phenotype is SZ and who has chronic cholestatic liver disease that began in the neonatal period. Liver biopsy demonstrated paucity of the interlobular bile ducts, marked hepatocellular deposition in periportal areas of PAS...
alpha1-Antitrypsin and alpha1-Antitrypsin Phenotyping(redirected from a1-AT phenotype) α1-Antitrypsin and α1-Antitrypsin Phenotyping Synonym/acronym: α1-antitrypsin: A1AT, α1-AT, AAT; α1-antitrypsin phenotyping: A1AT phenotype, α1-AT phenotype, AAT phenotype, Pi phenotype. Common use To...
Alpha1-antitrypsin deficiency with cirrhosis associated with the protease inhibitor phenotype SZ. Following the incidental finding of hepatomegaly in a 39 year old white man from the Dominican Republic, a liver biopsy disclosed chronic active hepatitis and cirrhosis. Six years later, after presenting ...
The effects of anticoagulants on the determination of both trypsin inhibitory capacity and the concentration of alpha 1-antitrypsin measured by radial immunodiffusion, and on the alpha 1-antitrypsin phenotype were investigated. These results were compared with those obtained for serum. The following anti...
This report describes the clinical characteristics of a group of 59 individuals with the PI*SZ phenotype and alpha 1-antitrypsin (alpha 1-AT) deficiency, identified during recruitment of a registry for subjects with severe alpha 1-antitrypsin deficiency. Currently, 1,129 individuals with levels of...
Alpha-1 antitrypsin deficiency in liver disease in children: phenotypes, manifestations and prognosis Among 424 children with liver disease, 20 had alpha1-antitrypsin deficiency associated with protease inhibitor ZZ phenotype. This disorder manifested itself as cholestasis in early infancy in 19 children....
Nine had ALD, 8 had NASH, 2 had PBC, and one each had alpha-1 antitrypsin deficiency, PSC, Hepatitis C and secondary biliary ... H Dhaliwal,A Smith,D Tripathi - Gut 被引量: 0发表: 2015年 A 32 year old male with idiopathic hepatic encephalopathy and necrotic lower extremity A 32 ...
Lman1 −/− mice exhibit ER accumulation of another LMAN1 cargo, alpha-1 antitrypsin (A1AT), with an intermediate level of A1AT ER retention observed in Lman1 cgt/cgt mice. Finally, the previously reported strain-specific, partially penetrant, perinatal lethality of LMAN1-deficient mice (...
Alpha-1-antitrypsin deficiency: smoking, decline in lung function and implications for therapeutic trials Background: Alpha-1-antitrypsin (AT) deficiency is a hereditary disorder associated with pulmonary emphysema. AT replacement therapy has been available for... DCS Hutchison,D Cooper - 《Respir Med...
Kelly, J. KVasudev, K. SLee FI, Kelly JK, Vasudev KS (1983) Hepatic changes in a patient with alpha-l-antitrypsin deficiency (MZ phenotype). Portal tract elastosis and noncirrhotic portal hypertension. Arch Pathol Lab Med 107: 453–455...