The State of Weight in Cystic Fibrosis: Understanding Nutritional Status and Individualizing Nutritional Care in the Modulator Era

囊性纤维化患者的体重状况:在调节剂时代了解营养状况并制定个体化营养护理方案

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Abstract

There is a well-established association between cystic fibrosis (CF) and malnutrition. Several comorbid conditions have also been associated with undernutrition in people with cystic fibrosis (PwCF). Highly effective modulator therapy has allowed for a paradigm shift altering disease progression and management. Modulator use has even been associated with acceleration of weight trajectory causing overnutrition, which can lead to cardiovascular and metabolic comorbid conditions. This review explores how nutritional status is evolving in the era of cystic fibrosis transmembrane conductance regulator (CFTR) modulators in people with CF, specifically in children. By synthesizing current research, we aim to support pediatric healthcare providers and nutritionists in delivering tailored, proactive nutritional care in this new therapeutic landscape.

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