P-491: Hyperphagia symptoms and severity variation in patients with Bardet bidel and Alstrom syndrome treated with setmelanotide at Elizalde’s children hospital, Argentina

Introduction

Hyperphagia is the extreme unsatisfied drive to consume food and it is a hallmark of monogenic obesity. Hyperphagic drive for food, behavior and severity can be evaluated with Dykens’ Questionnaire and other scales that quantify hyperphagia symptoms. Setmelanotide is an agonist of the Melanocortin 4 Receptor (MC4R) that has been approved for long-term weight control in patients > 2 years old with melanocortin pathway dysfunctions obesity syndromes

Objectives

To describe changes in hyperphagia behavior, symptoms and severity in patients treated with Setmelanotide at the Elizalde´s Children Hospital

Methods

Dyken´s Hyperphagia Questionnaire, and two questionnaires about hyperphagia symptoms and hyperphagia impact were performed in 5 patients (4 with BBS and 1 with Almstrom Syndrome) treated with Setmelanotide in the Nutrition Unit of Elizalde´s Hospital. The survey was completed by parents of children between 6 and 12 years and by the patient when they were older than 12 years old

Results

Conclusions

Hyperphagia questionnaires are useful tools to follow treatment response.

Dykens´Hyperphagia Questionnaire is the most used tool for assessing hyperphagia and measuring changes in hyperphagic symptoms over time.

Setmelanotide reduced hyperphagia in pediatric patients with BBS and Almstrom Syndrome