Related Experiment Videos
Nutritional challenges of infants with cystic fibrosis
1Kinderklinik und Kinderpoliklinik im Dr. v. Haunerschen Kinderspital, Ludwig-Maximilians-Universität, Pettenkoferstr. 8a, D-80336, München, Germany. koletzko@pk-i.med.uni-muenchen.de
Insights
Early nutritional assessment and management are crucial for infants with cystic fibrosis (CF). Prompt intervention, including pancreatic enzyme replacement, can address malnutrition common at diagnosis.
Area of Science:
- Pediatrics
- Gastroenterology
- Nutritional Science
Background:
- Nutrition is vital for managing cystic fibrosis (CF), especially in infants with high growth-related energy needs.
- Malnutrition is prevalent in CF infants at diagnosis due to energy imbalance, increased losses, and insufficient intake.
- Nutritional deficits are common even in asymptomatic infants identified via neonatal screening, with many born with pancreatic insufficiency.
Purpose of the Study:
- To provide recommendations for assessing nutritional status in infants at CF diagnosis.
- To outline nutritional management strategies for the first year of life in CF infants.
- To emphasize the importance of pancreatic enzyme supplementation.
Main Methods:
- This review synthesizes current evidence and clinical guidelines.
- It focuses on assessment tools for nutritional status in infants.
- It details management protocols, including enzyme therapy.
Main Results:
- Most CF infants present with malnutrition at diagnosis, often linked to pancreatic insufficiency present from birth.
- Early nutritional deficits are detectable even in screened, asymptomatic infants.
- Timely assessment and intervention are critical for optimal growth and health outcomes.
Conclusions:
- Comprehensive nutritional assessment at diagnosis is essential for all CF infants.
- Proactive nutritional management, including pancreatic enzyme replacement therapy, is key during the first year of life.
- Addressing energy deficits early can mitigate long-term complications associated with CF.
Abstract:
Nutrition plays an essential role in the management of cystic fibrosis (CF), particularly in infants with their high energy requirement due to rapid growth. Most infants are already malnourished at the time of clinical diagnosis due to an energy imbalance with increased losses and needs not compensated by energy intake. Even in very young asymptomatic CF patients diagnosed by neonatal screening programs, nutritional deficits can be detected in a high proportion of infants, since about half of all CF patients have evidence of pancreatic insufficiency at the time of birth. This review provides recommendations for assessment of the nutritional status at diagnosis and nutritional management including supplementation with pancreatic enzymes during the first year of life.