(P27-028-23) Ingestive Behaviors, Diet Quality, and Gut Microbiota in Children With Angelman Syndrome (AS)

Objectives: Given the limited research on dietary intakes and the gut microbiome in AS, we sought to characterize the diet and gut microbiome differences between children with AS and a sibling control.

Methods: This study included 21 families of children with AS and a non-AS sibling dyads aged 3-14 years. Caregivers completed a web-based survey with a demographic and clinical battery. A subset (n=11) completed 3 24-hr dietary recalls for each child and another subset (n=17) completed 3 at-home stool collections from each child. Healthy Eating Index (HEI) was estimated to assess diet quality and paired t-tests were conducted for sibling comparison. Stool samples were sequenced using 16S RNA and analyzed via linear discriminant analyses.

Results: Children with AS (67% female) and their non-AS sibling (57% female) were 3 years apart on average (range: 0-8y) and mostly White (86%) and non-Hispanic (95%). There was no difference in age between AS (mean 6.4y) and non-AS (mean 7.2y) siblings. A large portion (62%) of children with AS had difficulties with breast/bottle feeding when young, 71% had been ever hospitalized (mainly due to seizure complications), 33% had a restricted diet, and 19% had food allergies. The mean overall HEI scores for all children, including siblings, mirrored the U.S. average for these age ranges and did not differ between AS and non-AS siblings (55 vs 49, respectively). The HEI score for dairy was marginally lower for children with AS compared to their siblings (p=0.05). In terms of the microbiome, children with AS did not differ substantially from their siblings in alpha diversity. However, children with AS had statistically significant elevations in Bacteroidota members Prevotella copri, Parabacteroides merdae and P. distasonis over their siblings. In contrast, siblings displayed elevations in clostridial members of family Lachnospiraceae.

Conclusions: While diet quality did not differ, significant differences in gut microbiota were found between children with AS and a sibling control, with AS groups displaying larger populations of likely propiogenic microbes and siblings larger populations of likely butyrogens. This hypothesis-generating study will help inform future work and bring us one step closer to optimizing treatment for individuals with AS.

Funding Sources: Angelman Syndrome Foundation