α1-ANTITRYPSIN DEFICIENCY AND LIVER DISEASE
✍ Scribed by Lake-Bakaar, G.; Dooley, J.S.
- Book ID
- 122047039
- Publisher
- The Lancet
- Year
- 1982
- Tongue
- English
- Weight
- 161 KB
- Volume
- 320
- Category
- Article
- ISSN
- 0140-6736
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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
We present our experience with 18 pediatric patients with a,-antitrypsin deficiency of the PiZZ phenotype. Fifteen patients (83%) presented with neonatal cholestatic jaundice at a mean age of 2 f 0.6 months (2S.D.). The ma1e:female ratio was 15:3, indicating a male predominance. All metabolic, infec