L’essentiel du PNDS « Obésités de causes rares »: Rare obesities: the essentials of the French National Diagnosis and Care Protocol

Abstract

Rare genetic obesities are related to a neuroendocrine disorder linked to a dysfunction of the energy balance at the hypothalamic level, i.e eating behaviour and energy expenditure. More than sixty genes playing a key role in the leptin/melanocortin pathway contribute to these rare forms of obesity. The National Program for Diagnosis and Care Program (French PNDS) for Obesity of Rare Causes, available on the website of Haute Autorité de Santé, establishes recommendations for assessment and management at all ages. Eating disorders and complications related to early and severe obesity are very often associated with neuropsychological and behavioral disorders, leading to complex clinical situations. These conditions render the clinical management particularly challenging. Patients’ early genetic diagnosis is critical to allow specialized, multidisciplinary and coordinated care, with a necessary interaction between the health and social sectors and families. It also allows for personalized medicine by providing access to new drug therapies and informed discussion of bariatric surgery by improving the analysis of the benefit/risk balance. In this respect, these rare forms of obesity are a model of care for more common obesity in very vulnerable populations with disabilities, such as people with NDD (neurodevelopmental disorders) for whom the prevalence of obesity and its complications is higher than in the general population. The prevention of worsening weight gain is then based on the control of the person’s environment with a limitation of access to food and the establishment of a reassuring daily eating environment to limit impulsivity as well as the practice of an adapted, sustained and supervised physical activity. All this requires an efficient and adapted coordination of the care pathway close to the person and throughout his life.© 2023 Société francophone nutrition clinique et métabolisme (SFNCM).Published by Elsevier Masson SAS. All rights reserved.