Nutritional management of people living with cystic fibrosis throughout life and disease continuum: Changing times, new challenges

Abstract

Cystic fibrosis (CF) is a genetic disease caused by mutations in the gene encoding for the ion channel cystic fibrosis transmembrane conductance regulator (CFTR). The management of CF disease has evolved in recent decades from treating downstream disease manifestations affecting the airways, the lungs and the gastrointestinal system to addressing the CFTR gene defect. The advent of CFTR modulators, which correct the functionality of the defective CFTR, contributes to reshaping the landscape of CF demographics, prognosis and therapies, including nutritional management. A spectrum of clinical manifestations is emerging within the same patient population where undernutrition and nutritional deficiencies coexist with excessive weight gain and metabolic derangements. Such contrasting presentations challenge current practices, require adjustments to traditional approaches, and involve more individualised interventions. This narrative review examines the current state of knowledge on the nutritional management of people living with cystic fibrosis from early life to adulthood in the era of CFTR modulation.

Anais Do Iii Congresso Luso-Brasileiro De Atenção Integral À Saúde (On-Line) - Resumos Expandidos

“…As manifestações clínicas digestivas da FC variam, abrangendo a doença pancreática exócrina, íleo meconial, constipação, síndrome de obstrução intestinal distal (SOID), doença hepática, supercrescimento bacteriano do intestino delgado e inflamação intestinal (BIRIMBERG-SCHWARTZ; WILSCHANSKI, 2021). Aproximadamente 10% a 20% dos recém-nascidos com FC podem apresentar íleo meconial (IM), uma condição grave em que o mecônio espesso e viscoso obstrui o lúmen no terminal local do íleo (MAILHOT et al, 2023). A SOID, por sua vez, é causada pela secreção deficiente de fluido no lúmen intestinal, associada à baixa reabsorção de bile e ao aumento da gordura viscosa no lúmen intestinal, que retarda o trânsito e gera acúmulo de material fecal desidratado, levando à obstrução parcial ou completa do intestino (MAVILIA, 2019).…”

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