Breastfeeding, growth, and lung disease in the first 3 years of life in children with cystic fibrosis.

Background: The 2009 cystic fibrosis (CF) infant care guidelines recommend breastmilk as the initial feeding but do not address if/when it should be fortified or supplemented with formula to promote optimal growth and pulmonary health.

Methods: We conducted a prospective multi-center cohort study in breastfed and formula-fed infants that included 172 infants with CF who were born during 2012-17, enrolled after newborn screening at age 1.9 ± 1.

Early-Life Height Attainment in Cystic Fibrosis Is Associated with Pulmonary Function at Age 6 Years.

In contrast to the well-described association between early-life weight for age, body mass index (BMI), and later lung disease in people with cystic fibrosis (CF), the relationship between height-for-age (HFA) percentiles and respiratory morbidity is not as well-studied. We hypothesized that changes in HFA in children with CF in the first 6 years of life would be associated with pulmonary function at the age of 6-7 years. To determine if an association exists between changes in HFA in early life and pulmonary function in school-aged children with CF.

Nutrition in cystic fibrosis: From the past to the present and into the future.

Nutritional management is an integral part of multidisciplinary care for persons with cystic fibrosis. This review will look at how nutrition care has evolved over time. In addition, we will look at how some newer therapies impact nutrition care.

Clinical Practice Guidelines From the Cystic Fibrosis Foundation for Preschoolers With Cystic Fibrosis.

Cystic fibrosis (CF) clinical care guidelines exist for the care of infants up to age 2 years and for individuals ≥6 years of age. An important gap exists for preschool children between the ages of 2 and 5 years. This period marks a time of growth and development that is critical to achieve optimal nutritional status and maintain lung health.

Maximal daily dose of pancreatic enzyme replacement therapy in infants with cystic fibrosis: a reconsideration.

The current recommendations for dosing of pancreatic enzyme replacement therapy (PERT) in infants with cystic fibrosis (CF) were made using a limited evidence base. The per meal recommended dose was extrapolated from dosing guidelines for older patients into a maximal daily dose for infants. We discuss why this maximal daily dose recommendation may be insufficient for young infants with CF, although the optimal dose of PERT for infants with CF remains unknown.

Hypoalbuminemia in a cystic fibrosis patient with severe erosive esophagitis.

An 8-year-old patient with cystic fibrosis presented with hypoalbuminemia as the main symptom of severe erosive esophagitis. Extensive evaluation failed to reveal a nutrition, pancreatic, intestinal, or renal explanation of his hypoalbuminemia. Identification and treatment of the esophagitis led to resolution of the hypoalbuminemia.

Efficacy and tolerability of a new formulation of pancrelipase delayed-release capsules in children aged 7 to 11 years with exocrine pancreatic insufficiency and cystic fibrosis: a multicenter, randomized, double-blind, placebo-controlled, two-period crossover, superiority study.

Background: Pancreatic enzyme replacement therapy (PERT) is essential for maintaining adequate nutrition in children with exocrine pancreatic insufficiency (EPI) due to cystic fibrosis (CF). The US Food and Drug Administration regulations now require all PERT products to undergo clinical efficacy and safety studies before they can be considered for marketing approval.

Objective: This study was conducted to compare the efficacy of a new formulation of pancrelipase (pancreatin) delayed-release 12,000-lipase unit capsules with placebo in children with EPI due to CF.

Efficacy and safety of Creon 24,000 in subjects with exocrine pancreatic insufficiency due to cystic fibrosis.

Background: Pancreatic enzyme replacement therapy is critical for adequate nutrition in cystic fibrosis (CF) patients with exocrine pancreatic insufficiency (EPI).

Methods: This was a double-blind, randomised, placebo-controlled, two-period crossover study assessing efficacy and safety of Creon 24,000-unit capsules in CF subjects > or =12 years with EPI. Patients were randomised to one of two 5-day sequences, Creon/placebo or placebo/Creon (target dose, 4000 lipase units/g fat).