Unknown

Dataset Information

0

Characterization of rapid weight gain phenotype in children with narcolepsy.


ABSTRACT:

Objectives

To characterize the rapid weight gain (RWG) phenotype associated with the onset of childhood narcolepsy and to determine whether it could constitute a marker of severity of the disease.

Methods

RWG was defined using the BMI z-score slope reported to one year (>0.67 SD) from symptom onset to disease diagnosis. We compared the clinical, metabolic, and sleep characteristics between patients with or without RWG at diagnosis. Pharmacological management, anthropometric, and clinical progression were also evaluated during the follow-up.

Results

A total of 84 de novo narcoleptic pediatric patients were included; their median age at diagnosis was 12.0 years; 59.5% boys, 90.5% cataplexy, and 98.7% HLA-DQB1*06:02, 57% had RWG profile. RWG patients were younger at diagnosis than non-RWG patients, despite a shorter diagnostic delay. They had a higher BMI z-score and a higher prevalence of obesity at diagnosis, but not at symptom onset, and higher adapted Epworth Sleepiness Scale and Insomnia Severity Index scores than non-RWG patients. No differences on nocturnal polysomnography and multiple sleep latency tests were found between groups at disease diagnosis. After a median follow-up of 5 years, RWG patients still had a higher BMI z-score and a higher prevalence of obesity despite benefiting from the same therapeutic management and displaying improvement in sleepiness and school difficulties.

Conclusions

Narcoleptic RWG patients were younger, sleepier, and the prevalence of obesity was higher at diagnosis despite a shorter diagnostic delay than that of non-RWG patients. These patients had also a higher risk of developing a long-term obesity, despite a positive progression of their narcoleptic symptoms. RGW could then represent a maker of a more severe phenotype of childhood narcolepsy, which should inspire a prompt and more offensive management to prevent obesity and its complications.

SUBMITTER: Zhang M 

PROVIDER: S-EPMC9062543 | biostudies-literature | 2022 Jun

REPOSITORIES: biostudies-literature

altmetric image

Publications

Characterization of rapid weight gain phenotype in children with narcolepsy.

Zhang Min M   Thieux Marine M   Inocente Clara Odilia CO   Vieux Noemie N   Arvis Laura L   Villanueva Carine C   Lin Jian-Sheng JS   Plancoulaine Sabine S   Guyon Aurore A   Franco Patricia P  

CNS neuroscience & therapeutics 20220225 6


<h4>Objectives</h4>To characterize the rapid weight gain (RWG) phenotype associated with the onset of childhood narcolepsy and to determine whether it could constitute a marker of severity of the disease.<h4>Methods</h4>RWG was defined using the BMI z-score slope reported to one year (>0.67 SD) from symptom onset to disease diagnosis. We compared the clinical, metabolic, and sleep characteristics between patients with or without RWG at diagnosis. Pharmacological management, anthropometric, and c  ...[more]

Similar Datasets

| S-EPMC11251034 | biostudies-literature
| S-EPMC7604478 | biostudies-literature
| PRJEB31532 | ENA
| S-EPMC5338020 | biostudies-literature
| S-EPMC7731641 | biostudies-literature
| S-EPMC7534524 | biostudies-literature
| S-EPMC9540679 | biostudies-literature
| S-EPMC6866175 | biostudies-literature
| S-EPMC5363783 | biostudies-literature
| S-EPMC7994286 | biostudies-literature