Phenylalanine free infant formula in patients with phenylketonuria: A retrospective study

Yılmaz Nas Ö.

SSIEM Annual Symposium 2023, Yerushalayim, İsrail, 29 Ağustos - 01 Eylül 2023, ss.217, (Özet Bildiri)

  • Yayın Türü: Bildiri / Özet Bildiri
  • Basıldığı Şehir: Yerushalayim
  • Basıldığı Ülke: İsrail
  • Sayfa Sayıları: ss.217
  • Ankara Yıldırım Beyazıt Üniversitesi Adresli: Evet

Özet

Background: We report a longitudinal, retrospective study on a phenylalanine (Phe)-free infant formula.

Methods: Data on blood Phe concentrations, growth, dietary intake, and tolerance were collected from diagnosis until 2 years of age. Parents completed a questionnaire on their child's experience with Phe-free infant formula (PKU Start: Vitaflo International Ltd).

Results: 25 children (12 males, 48%) with a median age of 3.2y (range: 1.6-5.9y) were included. 20 (80%) discontinued the formula at a median age of 1.7y (range: 1.1-3.0y), while 5 (20%) continued to receive some formula until a median age of 3.2y (range: 1.6-5.9y). The median blood Phe levels were within the target range of 120-360 μmol/L. Normal growth was observed from 0-6 months to 19-24 months of age, with median z-score increases for length- for-age (from -0.1 [IQR:1.6] to 0.6 [IQR:1.5]), weight-for-age (from -0.3 [IQR:1.7] to 0.9 [IQR:2.0]), and BMI-for-age (from -0.4 [IQR:1.5] to 2.0 [IQR:1.6]). Median total protein intake increased from 2.8 g/kg/day at 0-6 months to 3.3 g/kg/day at 19-24 months, with natural protein intake remaining stable at 0.4 g/kg/day. The formula provided a median of 74% (IQR: 28%) of total protein intake in 0-6 months, declining to 32% (IQR:27%) and 23% (IQR:24%) for 7-12 months and 13-18 months, respectively. Infant feeding difficulties (28%, n=7) and gastrointestinal symptoms (52%, n=13) were most common at age 0-6 months, while respiratory symptoms (16%, n=4) and infectious diseases (60%, n=15) were most common at age 7-12 months but decreased with time. The formula was well accepted by 80% (n=20) of infants, with initial difficulty reported in 20% (n=5) which improved with age. Some parents reported reflux and/or constipation (44%, n=11) during follow-up, but these were not attributed to the study formula.

Conclusion: The study formula was well accepted and tolerated with good metabolic control, and normal growth observed in infants/children with PKU.