Overweight and cystic fibrosis: An unexpected challenge.

Achieving a healthy weight balance has been a central focus of care for people who have cystic fibrosis (CF). Over the years, the emphasis has primarily been on promoting weight gain to optimize pulmonary outcomes. With continued improvements in CF care, including highly effective CF modulators available for many people, the CF community is now experiencing a new challenge: addressing the concern that some people are gaining weight excessively.

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.

Relationship of Initial Pancreatic Enzyme Replacement Therapy Dose With Weight Gain in Infants With Cystic Fibrosis.

Objective: The aim of the study is to test the hypothesis of a positive relationship between initial dose of pancreatic enzyme replacement therapy (PERT) in infants with cystic fibrosis (CF) and optimal weight gain over the first 2 years of life.

Methods: Using the CF Foundation Patient Registry, we identified 502 children born in 2010 and used multivariable models to compare as our primary analysis their 2-year changes in weight-for-age z score (WAZ) and as our secondary analysis weight-for-length percentile (W/L%) by initial PERT dose. We focused on initial dose without reference to subsequent changes in treatment to avoid confounding by indication (severity).

Nutrition Management of Cystic Fibrosis in the 21st Century.

Despite significant advancements made in life expectancy over the past century, cystic fibrosis remains a life-threatening genetic disease that affects the gastrointestinal tract, and it has significant impact on the nutrition status of those with the disease. Nutrition management includes a high-calorie/high-fat diet, pancreatic enzyme replacement therapy, vitamin and mineral replacement, and enteral support as needed. As patients are living longer, clinicians may encounter patients with cystic fibrosis in obstetrician offices, endocrine clinics, or hospital settings, owing to lung transplantation or for treatment for distal intestinal obstruction syndrome.

Vitamin D deficiency is associated with pulmonary dysfunction in cystic fibrosis.

Background: Vitamin D deficiency is common in CF. Whether vitamin D affects pulmonary function in CF is unknown.

Methods: Data were abstracted from clinically stable CF patients who had pulmonary function studies and serum 25-hydroxyvitamin D [25(OH)D, ng/ml] levels drawn within 2 months of each other.

Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis.

Newborn screening for cystic fibrosis (CF) offers the opportunity for early medical and nutritional intervention that can lead to improved outcomes. Management of the asymptomatic infant diagnosed with CF through newborn screening, prenatal diagnosis, or sibling screening is different from treatment of the symptomatically diagnosed individual. The focus of management is on maintaining health by preventing nutritional and respiratory complications.

Nutrition management of pediatric patients who have cystic fibrosis.

Since the identification of cystic fibrosis (CF) in the 1940s, nutrition care of patients who have CF has been a challenge. Through optimal caloric intake and careful management of malabsorption, patients are expected to meet genetic potential for growth. Yet factors beyond malabsorption, including nutrient activity at the cellular level, may influence growth and health.

Overweight among young children in the Philadelphia health care centers: incidence and prevalence.

Objectives: To estimate the prevalence and incidence of overweight among low-income, inner-city children aged 3 to 7 years and to determine predictors of changes in body mass index (BMI) (calculated as weight in kilograms divided by height in meters squared) percentile.

Design: Retrospective cohort study using administrative and medical records.

Setting: The Philadelphia Health Care Centers, 1996 through 2003.