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α1-Antitrypsin deficiency, liver disease and emphysema

✍ Scribed by Helen Parfrey; Ravi Mahadeva; David A. Lomas

Book ID
117755940
Publisher
Elsevier Science
Year
2003
Tongue
English
Weight
327 KB
Volume
35
Category
Article
ISSN
1357-2725

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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