Successful early dietary intervention avoids obesity in patients with Prader-Willi syndrome: a ten-year follow-up.

摘要: UNLABELLED: Hyperphagia is a frequent symptom in patients with Prader-Willi syndrome (PWS) and results marked obesity the risk of metabolic cardiovascular complications. AIM: To investigate whether early diagnosis PWS strict dietary intervention prevents excessive weight gain PWS. PATIENTS AND METHODS: A fat reduced modified carbohydrate diet consisting 10 kcal/ cm height was provided to nine (seven female, two male) diagnosed (group A). Patients were prospectively followed at our center follow-up visits every three months. Eight late served as controls B). Body mass index (BMI) SDS compared between these groups over ten-year period. RESULTS: At age years BMI significantly lower group (-2.9 vs -1.2, p <0.05, -0.1 +1.8, < 0.05). After ten increased +1.2 A, but still than B (BMI +2.4), <0.005. without restrictive taller on (height -2.8 -1.3, CONCLUSION: Early treatment starting second year life continued until effective avoiding PWS, shorter stature. Therefore growth hormone may be useful additional patients.