Effects of Bifidobacterium animalis Subsp. lactis (BPL1) Supplementation in Children and Adolescents with Prader-Willi Syndrome: A Randomized Crossover Trial.

Montse Amat-Bou,Carles Lerin,Marta Ramon-Krauel,Josep C Jiménez-Chillarón,Laia Villalta,Maria Elias,Daniel Ramón,Sonika Garcia-Ribera,Eric Climent,David Sanchez-Infantes,Irene Piquer-Garcia,Empar Chenoll,Raquel Corripio,Lourdes Ibañez

doi:10.3390/nu12103123

Abstract

Prader–Willi syndrome (PWS) is a rare genetic disorder characterized by a wide range of clinical manifestations, including obesity, hyperphagia, and behavioral problems. Bifidobacterium animalis subsp. lactis strain BPL1 has been shown to improve central adiposity in adults with simple obesity. To evaluate BPL1′s effects in children with PWS, we performed a randomized crossover trial among 39 patients (mean age 10.4 years). Participants were randomized to placebo–BPL1 (n = 19) or BPL1–placebo (n = 20) sequences and underwent a 12-week period with placebo/BPL1 treatments, a 12-week washout period, and a 12-week period with the crossover treatment. Thirty-five subjects completed the study. The main outcome was changes in adiposity, measured by dual-energy X-ray absorptiometry. Secondary outcomes included lipid and glucose metabolism, hyperphagia, and mental health symptoms. Generalized linear modeling was applied to assess differences between treatments. While BPL1 did not modify total fat mass compared to placebo, BPL1 decreased abdominal adiposity in a subgroup of patients older than 4.5 years (n = 28). BPL1 improved fasting insulin concentration and insulin sensitivity. Furthermore, we observed modest improvements in some mental health symptoms. A follow-up trial with a longer treatment period is warranted to determine whether BPL1 supplementation can provide a long-term therapeutic approach for children with PWS (ClinicalTrials.gov NCT03548480).

Highlights

Prader–Willi syndrome (PWS) is a rare genetic disorder characterized by a wide range of clinical manifestations, including altered body composition, hyperphagia, and severe behavioral problems [1]
We recently reported the gut microbiota associated with obesity in children with PWS, finding similar alterations to those found in simple obesity [21]
Our study with the BPL1 strain in subjects with PWS demonstrates that this probiotic exerts beneficial effects even in the context of genetic obesity

Summary

Introduction

Prader–Willi syndrome (PWS) is a rare genetic disorder characterized by a wide range of clinical manifestations, including altered body composition, hyperphagia, and severe behavioral problems [1] It is considered the most common cause of genetic obesity due to a combination of low energy expenditure and high energy intake [2,3,4]. Patients present hypotonia, feeding difficulties, and failure to thrive during the first months of life, and transition toward increased appetite, interest in food, and excessive adiposity at approximately 4.5 years of age Later on, they typically develop hyperphagia, morbid obesity if uncontrolled, and mental health problems, including anxiety and social problems [5,6], with patients with maternal disomy at higher risk for more severe problems [7]

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