Background: Prader Willi Syndrome (PWS) patients under treatment with growth hormone (GH) have a better body composition and cognitive performance than untreated patients. The 2013 guideline recommended to start this treatment as soon as possible, for that reason 14 toddlers have been included in our center. Classically a 25% decrease in the caloric intake of PWS patients has been recommended to avoid obesity. However, from clinical practice observation, we hypothesized that the caloric needs in these patients could be higher the first 2 years of age.
Objectives: To know the variation of BMI in patients with PWS under 2 years of age treated with GH and to determine their caloric intake.
Methods: Retrospective observational study comparing PWS patients under 2 years of age treated with GH in our hospital (G1) with a control group of a historical cohort of PWS patients with who did not receive treatment with GH before the age of 2 (G2). The caloric intake of G1 was obtained by a 24h intake questionnaire.
Results: The analyzed data correspond to 12 patients in G1 (58.3% males, 66.7% paternal deletion and 33.3% uniparental disomy) and 6 patients in G2 (50% males, 50% paternal deletion, 33.3% uniparental disomy and 16.7% genomic imprinting). The median age of onset of GH was 10.75 months (IQR 9-16) in G1 and 28.2 (IQR 27-34) in G2. The caloric intake of G1 patients was 663+/-122.35, 701.8+/-80.97, 711.8+/-105.76, 791.8+/-92 and 910.4+/-128.92 kcal per day on average at 9, 12, 15, 18 and 24 months respectively. Weight, height and BMI were compared in both groups at 9, 12, 15, 18 and 24 months. G1 patients show higher height in all the determinations. BMI tendency in G2 was to increase, whereas in G1 was to decrease progressively (P<0.05).
Conclusions: GH treatment in PWS patients under 2 years of age affects their BMI. It is important to adjust the caloric intake of these patients in order to adequate to their current needs. It is required to verify this results in longitudinal studies.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology