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Predicting the clinical trajectory of feeding and swallowing abilities in CHARGE syndrome

To date, the feeding and oral-motor abilities of patients with CHARGE syndrome (CS) have not been longitudinally assessed. This study aims to investigate the level of these abilities at different ages and evaluate how they evolve during growth. We retrospectively analysed oral-motor features of 16 p...

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Autores principales: Onesimo, R., Sforza, E., Giorgio, V., Rigante, D., Kuczynska, E., Leoni, C., Proli, F., Agazzi, C., Limongelli, D., Cerchiari, A., Tartaglia, M., Zampino, G.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Berlin Heidelberg 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10167171/
https://www.ncbi.nlm.nih.gov/pubmed/36800035
http://dx.doi.org/10.1007/s00431-023-04841-4
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author Onesimo, R.
Sforza, E.
Giorgio, V.
Rigante, D.
Kuczynska, E.
Leoni, C.
Proli, F.
Agazzi, C.
Limongelli, D.
Cerchiari, A.
Tartaglia, M.
Zampino, G.
author_facet Onesimo, R.
Sforza, E.
Giorgio, V.
Rigante, D.
Kuczynska, E.
Leoni, C.
Proli, F.
Agazzi, C.
Limongelli, D.
Cerchiari, A.
Tartaglia, M.
Zampino, G.
author_sort Onesimo, R.
collection PubMed
description To date, the feeding and oral-motor abilities of patients with CHARGE syndrome (CS) have not been longitudinally assessed. This study aims to investigate the level of these abilities at different ages and evaluate how they evolve during growth. We retrospectively analysed oral-motor features of 16 patients with molecularly confirmed CS (age range 4–21 years old; mean 11 years; SD 6 years; median 10 years). Nearly 100% of CS new-borns had weak sucking at birth, and half of them demonstrated poor coordination between breathing and swallowing. Over time, the percentages of children with tube feeding dependence (60% at birth) faced a slow but steady decrease (from 33% at 6 months, 25% at 12 months, to 13% at school age) in tandem with the decreasing risk of aspiration. The ability of eating foods requiring chewing was achieved at school age, after the acquisition of an adequate oral sensory processing. A mature chewing pattern with a variety of food textures was not achieved by more than half of patients, including those requiring artificial enteral nutrition. Most patients started prolonged oral-motor treatments with speech language therapists in early childhood. Conclusions: Although feeding and swallowing disorders are constant features in CS patients, a slow and gradual development of feeding abilities occurs in most cases. Rehabilitation plays a key role in overcoming structural and functional difficulties and attaining appropriate eating skills. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s00431-023-04841-4.
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spelling pubmed-101671712023-05-10 Predicting the clinical trajectory of feeding and swallowing abilities in CHARGE syndrome Onesimo, R. Sforza, E. Giorgio, V. Rigante, D. Kuczynska, E. Leoni, C. Proli, F. Agazzi, C. Limongelli, D. Cerchiari, A. Tartaglia, M. Zampino, G. Eur J Pediatr Research To date, the feeding and oral-motor abilities of patients with CHARGE syndrome (CS) have not been longitudinally assessed. This study aims to investigate the level of these abilities at different ages and evaluate how they evolve during growth. We retrospectively analysed oral-motor features of 16 patients with molecularly confirmed CS (age range 4–21 years old; mean 11 years; SD 6 years; median 10 years). Nearly 100% of CS new-borns had weak sucking at birth, and half of them demonstrated poor coordination between breathing and swallowing. Over time, the percentages of children with tube feeding dependence (60% at birth) faced a slow but steady decrease (from 33% at 6 months, 25% at 12 months, to 13% at school age) in tandem with the decreasing risk of aspiration. The ability of eating foods requiring chewing was achieved at school age, after the acquisition of an adequate oral sensory processing. A mature chewing pattern with a variety of food textures was not achieved by more than half of patients, including those requiring artificial enteral nutrition. Most patients started prolonged oral-motor treatments with speech language therapists in early childhood. Conclusions: Although feeding and swallowing disorders are constant features in CS patients, a slow and gradual development of feeding abilities occurs in most cases. Rehabilitation plays a key role in overcoming structural and functional difficulties and attaining appropriate eating skills. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s00431-023-04841-4. Springer Berlin Heidelberg 2023-02-17 2023 /pmc/articles/PMC10167171/ /pubmed/36800035 http://dx.doi.org/10.1007/s00431-023-04841-4 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Research
Onesimo, R.
Sforza, E.
Giorgio, V.
Rigante, D.
Kuczynska, E.
Leoni, C.
Proli, F.
Agazzi, C.
Limongelli, D.
Cerchiari, A.
Tartaglia, M.
Zampino, G.
Predicting the clinical trajectory of feeding and swallowing abilities in CHARGE syndrome
title Predicting the clinical trajectory of feeding and swallowing abilities in CHARGE syndrome
title_full Predicting the clinical trajectory of feeding and swallowing abilities in CHARGE syndrome
title_fullStr Predicting the clinical trajectory of feeding and swallowing abilities in CHARGE syndrome
title_full_unstemmed Predicting the clinical trajectory of feeding and swallowing abilities in CHARGE syndrome
title_short Predicting the clinical trajectory of feeding and swallowing abilities in CHARGE syndrome
title_sort predicting the clinical trajectory of feeding and swallowing abilities in charge syndrome
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10167171/
https://www.ncbi.nlm.nih.gov/pubmed/36800035
http://dx.doi.org/10.1007/s00431-023-04841-4
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