Background: Prader Willi syndrome (PWS) results from the loss of paternally imprinted genes on chromosome 15q1115 and is characterized by neonatal hypotonia, short stature, hypogonadism, aggressive food-seeking behavior, hyperphagia, and obesity with difficult in losing weight only with nutritional approach.
Objective and hypotheses: We report our experience on sleeve gastrectomy in PWS and obese subjects during 12 months of follow-up.
Method: Three PWS (two males; aged 15.9±4.4 years; range 10.919.3) and six age matched obese controls (OB; one male; aged 15.6±2.8; range 10.518.5). PWS showed basal mean BMI 43.1±0.9, BMI S.D. 4.5±1.4, waist circumference 107±21 cm, HbA1c 44.7±8.3 mmol/mol, HOMA-IR 13.8±19.4. OB showed basal mean BMI 57.2±17.8 (P<0.05 vs PWS), BMI S.D. 7.5±2 (P<0.05 vs PWS), waist circumference 120±9.1 cm, HbA1c 36.4±2.4 mmol/mol, HOMA-IR 5.0±2.0 (P<0.01 vs PWS). One PWS has type 2 Diabetes treated with biguanides and GLP1 agonists and one OB showed IGT. None of pts was treated with GH. Ethic Committee and informed consents were obtained.
Results: After 12 months from surgery, PWS pt with type 2 Diabetes normalized glycaemia and HbA1c and stopped therapy, as pt obese with IGT. One PWS pt did not lose weight during follow-up. After 1 year PWS showed mean BMI 37.6±4.6, BMI S.D. 3.7±1.7, waist circumference 98±15.5 cm, HbA1c 38±6.2 mmol/mol, HOMA-IR 2.0±0.9. OB showed mean BMI 39.5±7.4, BMI S.D. 6.1±0.6 (P<0.05 vs PWS), waist circumference 106.8±8.8 cm, HbA1c 33.0±1.7 mmol/mol, HOMA-IR 3.5±2.3. One obese pt developed reflux and vomiting controlled by diet and anti-reflux therapy.
Conclusion: Our data, although preliminary showed that sleeve gastrectomy in adolescent with PWS, as in obese patients, improve positively BMI, and may normalize glycemic control and insulin resistance. No statistically difference were found among parameters but this result can be influenced by the very small sample and the widespread distribution of data. These results need to be confirmed on higher number of pts with a longer follow up.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology