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

IF 3 3区 医学 Q1 PEDIATRICS
Eric Dumont, Dagmar K Tiemens, Jos M T Draaisma, Lotte E R Kleimeier, Debbie van Druten, Sandra Mulkens
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引用次数: 0

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.

What is known: • More than 50% of the infants with Noonan syndrome-like RASopathy have serious feeding/eating problems. • Most of them temporarily need tube feeding.

What is new: • Ultimately, these feeding/eating problems may develop into an avoidant/restrictive food intake disorder. • Behavioral therapy (SLIK program) can effectively manage complex feeding/eating problems such as avoidant/restrictive food intake disorder in patients with a Noonan syndrome-like RASopathy. • There were no significant differences found in the history of comorbid features, feeding skill (dys)function, avoidant/restrictive food intake disorder characteristics, or treatment outcomes.

患有努南综合征样RASopathy和回避/限制性食物摄入障碍的儿童是否从行为治疗中受益?
患有努南综合征样rasopathy的儿童由于早期并发器质性损伤(如胃肠道问题或其他器质性问题)而出现喂养问题的风险增加。他们的进食问题最终通常可以归类为回避/限制性食物摄入障碍,行为疗法是首选的治疗方法。这项研究的研究问题是,这种治疗是否会导致与没有这些RASopathies的儿童相似的结果。我们回顾性调查了在回避/限制性食物摄入障碍三级治疗中心治疗的遗传确诊的努南综合征样RASopathy患者的特征和治疗结果,并将其与没有努南综合征样RASopathy的回避/限制性食物摄入障碍儿童的匹配病例对照组进行了1:2的比较。在治疗开始和治疗结束后,两组在食物摄入测量和喂养技能/功能障碍方面都有显著改善,SDS体重/身高增加,管依赖性减少。我们发现患有和不患有Noonan综合征样RASopathy的儿童之间没有显著的治疗结果,也没有共病特征。结论:有Noonan综合征样RASopathy和回避/限制性食物摄入障碍的患者与没有Noonan综合征样RASopathy的患者一样,从认知行为治疗中获益。已知情况:•50%以上患有努南综合征样RASopathy的婴儿有严重的喂养/饮食问题。•多数患儿暂时需要管饲。最新进展:•最终,这些喂养/进食问题可能发展为回避/限制性食物摄入障碍。•行为疗法(SLIK计划)可以有效地管理复杂的进食问题,如努南综合征样RASopathy患者的回避/限制性食物摄入障碍。•在共病特征、喂养技能(日)功能、回避/限制性食物摄入障碍特征或治疗结果的历史上没有发现显著差异。
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来源期刊
CiteScore
5.90
自引率
2.80%
发文量
367
审稿时长
3-6 weeks
期刊介绍: The European Journal of Pediatrics (EJPE) is a leading peer-reviewed medical journal which covers the entire field of pediatrics. The editors encourage authors to submit original articles, reviews, short communications, and correspondence on all relevant themes and topics. EJPE is particularly committed to the publication of articles on important new clinical research that will have an immediate impact on clinical pediatric practice. The editorial office very much welcomes ideas for publications, whether individual articles or article series, that fit this goal and is always willing to address inquiries from authors regarding potential submissions. Invited review articles on clinical pediatrics that provide comprehensive coverage of a subject of importance are also regularly commissioned. The short publication time reflects both the commitment of the editors and publishers and their passion for new developments in the field of pediatrics. EJPE is active on social media (@EurJPediatrics) and we invite you to participate. EJPE is the official journal of the European Academy of Paediatrics (EAP) and publishes guidelines and statements in cooperation with the EAP.
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