Early assessment of evolution of liver disease associated with α1-antitrypsin deficiency in childhood
✍ Scribed by Gabriella Nebbia; Michèle Hadchouel; Michel Odievre; Daniel Alagille
- Book ID
- 119460012
- Publisher
- Elsevier Science
- Year
- 1983
- Tongue
- English
- Weight
- 388 KB
- Volume
- 102
- Category
- Article
- ISSN
- 1097-6833
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📜 SIMILAR VOLUMES
alpha-1-antitrypsin; PiZZ, a1-ATZ phenotype; ER, endoplasmic acts with the target P 1 -P 1 residues of the reactive center loop, reticulum; MHC, major histocompatibility complex. the loop inserts further into the gap in the A sheet, generating From the: Departments of Pediatrics, Cell Biology, and
Chronic liver disease affects up to 20% of children with alpha 1-antitrypsin deficiency owing to the PiZZ genotype. Previous observations of a familial occurrence and abnormal immune responses to liver antigens in these patients suggests that immunoregulatory genes may be involved in the pathogenesi