Do children with a Noonan syndrome-like RASopathy and avoidant/restrictive food intake disorder benefit from behavioral therapy?

Abstract: Children with Noonan syndrome-like RASopathies are at increased risk for developing feeding problems due to comorbid organic impairments at an early age, such as gastrointestinal problems or other organicity. Their feeding problems can ultimately often be classified as avoidant/restrictive food intake disorder, for which behavioral therapy is the first-choice treatment. The research question in this study is whether this treatment leads to similar results as in children without these RASopathies. We retrospectively investigated patients with a genetically confirmed Noonan syndrome-like RASopathy who were treated for their disordered eating in a tertiary center for avoidant/restrictive food intake disorder on characteristics and treatment outcomes and compared them to a matched case–control group of children with avoidant/restrictive food intake disorder without Noonan syndrome-like RASopathy in a ratio of 1:2. Both groups improved substantially on food intake measures and feeding skills/dysfunction between the start of therapy and immediately after the therapy and showed an increase in SDS weight/height and a decrease in tube dependency. We found no significant treatment outcomes between children with and without Noonan Syndrome-like RASopathy, nor for comorbid features. Conclusion: Patients with Noonan syndrome-like RASopathy and avoidant/restrictive food intake disorder benefit equally well from cognitive behavioral therapy, as patients without a Noonan syndrome-like RASopathy. (Table presented.)