Liver glycerokinase deficiency in man with hyperglycerolaemia and hypertriglyceridaemia
โ Scribed by D. POMETTA; A. SUENRAM; N. VON DER WEID; J. J. WIDMANN
- Book ID
- 114726424
- Publisher
- John Wiley and Sons
- Year
- 1984
- Tongue
- English
- Weight
- 372 KB
- Volume
- 14
- Category
- Article
- ISSN
- 0014-2972
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Pis-) children have been followed prospectively, of whom 1 PiSZ and 5 PiZ children died in early childhood. We now report clinical and biochemical signs of liver disease in adolescence and the prognosis of neonatal liver disease up to the age of 18 years. The tu,ATD subjects were offered a clinical
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