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Physical growth in patients with phenylketonuria
Author(s) -
Dhondt J. L.,
Largillière C.,
Moreno L.,
Farriaux J. P.
Publication year - 1995
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf00711748
Subject(s) - medicine , humanities , philosophy
The data are from 94 children (48 males and 46 females) with PKU born in the period from 1969 to 1987 and treated by the same clinical team. They were identified as having phenylketonuria at the neonatal period and treated because of permanent hyperphenylalaninaemia above 12mg/dl (730/2mol/L). They have required a phenylalanine-restricted diet to maintain their blood phenylalanine below 8mg/dl (485,umol/L). Patients were receiving Lofenalac (Mead-Johnson) until 6 months of age, then Aminogram (Glaxo) (patients born before 1983) or PKU 2 (Milupa). At 8 years of age, a relaxed diet progressively replaced the phenylalanine-restricted diet. However, we tried to maintain phenylalanine concentrations between 15 and 20 mg/dl (910-1200 /tmol/L). Growth parameters were obtained at each clinic visit and were compared to the values established according to sex and age in normal French children.