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7 patients with phenylketonuria who developed neurological disability in adolescence or early adult life are described. 4 had been diagnosed by routine neonatal screening and started a low phenylalanine diet in infancy. 3 were diagnosed in early childhood because of developmental delay, and then started dietary treatment. Dietary control deteriorated in(More)
1 The influence of carbamazepine (CBZ) therapy on saliva antipyrine kinetics, urinary 6 beta-hydroxycortisol excretion and plasma gamma-glutamyltranspeptidase activity was determined in nine children aged 6-14 years. 2 During 5 weeks of CBZ therapy the mean (+/- s.d.) antipyrine clearance increased from 65 +/- 12 ml kg-1 h-1 to 143 +/- 34 ml kg-1 h-1 (P(More)
*The UK incidence of anaphylactic reactions is increasing. *Patients who have an anaphylactic reaction have life-threatening airway and, or breathing and, or circulation problems usually associated with skin or mucosal changes. *Patients having an anaphylactic reaction should be treated using the Airway, Breathing, Circulation, Disability, Exposure (ABCDE)(More)
In patients with phenylketonuria, blood phenylalanine concentration during childhood is the major determinant of cognitive outcome. Guidelines provide age-related recommendations for treatment. To ascertain the extent to which these aims are achievable, we audited results from four centres for the years 1994-2000. The median proportion of samples with(More)
1 Saliva carbamazepine (CBZ) pharmacokinetics were determined in six children aged 7-11 years at the start and after 5 weeks of CBZ therapy. 2 A single oral dose of CBZ, 14.7 +/- 2.3 mg kg -1, was administered and mixed saliva was collected at intervals during the next 36 h. CBZ therapy was then continued using the same total daily dose divided into two(More)
Saliva carbamazepine and phenytoin samples were used to monitor treatment in 35 children aged between 2 and 14 years during a 2-year period. All phenytoin levels and over half the carbamazepine levels that were above the therapeutic range were associated with adverse effects. Dose and carbamazepine saliva levels were significantly related but no such(More)