Feeding Problems in Patients with Noonan Syndrome: A Narrative Review

Noonan syndrome (NS) belongs to the group of Noonan syndrome spectrum disorders (NSSD), which is a group of phenotypically related conditions. Feeding problems are often present not only in infancy but also in childhood, and even beyond that period. We describe the different aspects of feeding probl...

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Bibliographic Details
Published in:Journal of clinical medicine 2022-01, Vol.11 (3), p.754
Main Authors: Tiemens, Dagmar K, van Haaften, Leenke, Leenders, Erika, van Wegberg, Annemiek M J, Gunther Moor, Bregtje, Geelen, Joyce, Draaisma, Jos M T
Format: Article
Language:English
Subjects:
Age groups
Clinical medicine
Dysphagia
Esophagus
Failure to thrive
Food
Genes
Kinases
Natural history
Ostomy
Patients
Pediatrics
Resource recovery
Review
Teenagers
Vomiting
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Summary:Noonan syndrome (NS) belongs to the group of Noonan syndrome spectrum disorders (NSSD), which is a group of phenotypically related conditions. Feeding problems are often present not only in infancy but also in childhood, and even beyond that period. We describe the different aspects of feeding problems using a (theoretical) concept proposed in 2019. More than 50% of infants with NS develop feeding problems, and up to half of these infants will be tube-dependent for some time. Although, in general, there is a major improvement between the age of 1 and 2 years, with only a minority still having feeding problems after the age of 2 years, as long as the feeding problems continue, the impact on the quality of life of both NS infants and their caregivers may be significant. Feeding problems in general improve faster in children with a pathogenic or variant. The mechanism of the feeding problems is complex, and may be due to medical causes (gastroesophageal reflux disease and delayed gastric emptying, cardiac disease and infections), feeding-skill dysfunction, nutritional dysfunction with increased energy demand, or primary or secondary psychosocial dysfunction. Many of the underlying mechanisms are still unknown. The treatment of the feeding problems may be a medical challenge, especially when the feeding problems are accompanied by feeding-skill dysfunction and psychosocial dysfunction. This warrants a multidisciplinary intervention including psychology, nutrition, medicine, speech language pathology and occupational therapy.
ISSN:2077-0383
2077-0383
DOI:10.3390/jcm11030754