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Growth failure in children with cystic fibrosis
Alessandra Scaparrotta1, Sabrina Di Pillo, Marina Attanasi
1Department of Pediatrics, University of Chieti, Italy. ale.scaparrotta@libero.it
Insights
Poor linear growth in children with cystic fibrosis (CF) is linked to malnutrition, inflammation, and lung disease. Early diagnosis and nutritional support are crucial for improving growth and overall prognosis in CF patients.
Area of Science:
- Pediatrics
- Genetics
- Pulmonology
Background:
- Poor linear growth and inadequate weight gain are prevalent issues in pediatric cystic fibrosis (CF).
- Key contributing factors include malnutrition, chronic inflammation, lung disease, and corticosteroid use.
- Despite catch-up growth post-diagnosis, deficits in height and weight persist into adulthood.
Purpose of the Study:
- To elucidate the etiological and pathogenetic mechanisms underlying growth failure in children with CF.
- To clarify the impact of these mechanisms on disease morbidity and clinical outcomes.
- To emphasize the relationship between growth failure and progressive pulmonary function decline.
Main Methods:
- Literature review of studies on cystic fibrosis growth failure.
- Analysis of etiological factors: nutrition, inflammation, lung disease, and medical treatments.
- Examination of the impact on morbidity, clinical outcomes, and pulmonary function.
Main Results:
- Identified undernutrition, chronic inflammation, lung disease, and corticosteroid treatment as primary drivers of growth failure.
- Highlighted the importance of nutritional support and recombinant human growth hormone therapy.
- Emphasized that early diagnosis is critical for better nutritional status, growth, and potentially improved respiratory outcomes.
Conclusions:
- Growth failure in CF is multifactorial, significantly impacting morbidity and clinical outcomes.
- Early diagnosis and intervention, including nutritional support, are essential for optimizing growth and prognosis in CF children.
- Addressing growth failure is integral to managing CF and mitigating progressive pulmonary decline.
Abstract:
Poor linear growth and inadequate weight gain are very common problems in cystic fibrosis (CF) children. The most important factors involved in growth failure are undernutrition or malnutrition, chronic inflammation, lung disease, and corticosteroid treatment. Nutritional support and pharmacological therapy with recombinant human growth hormone are essential for a good management of children with CF, although these children are shorter and lighter than healthy children, and despite the catch-up growth observed after diagnosis, deficit in length/height and weight continues to be seen until adulthood. Early diagnosis is essential to ensure better nutritional status and growth, potentially associated with better respiratory function and prognosis. The aims of this review are try to explain etiology and pathogenetic mechanisms of growth failure in CF children and clarify their role in the disease morbidity and in clinical outcome, especially in relation to progressive decline of pulmonary function.
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