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.

Impact of intrinsic and extrinsic risk factors on early-onset lung disease in cystic fibrosis.

Background: Although respiratory pathology is known to develop in young children with cystic fibrosis (CF), the determinants of early-onset lung disease have not been elucidated.

Objective: We aimed to determine the impact of potential intrinsic and extrinsic risk factors during the first 3 years of life, testing the hypothesis that both contribute significantly to early-onset CF lung disease.

Design: We studied 104 infants born during 2012-2017, diagnosed through newborn screening by age 3 months, and evaluated comprehensively to 36 months of age.

Consequences of an early catheter-based intervention on pulmonary artery growth and right ventricular myocardial function in a pig model of pulmonary artery stenosis.

Objective: To determine the consequences of an early catheter-based intervention on pulmonary artery (PA) growth and right ventricular (RV) myocardial function in an animal model of branch PA stenosis.

Background: Acute results and safety profiles of deliberate stent fracture within the pulmonary vasculature have been demonstrated. The long-term impact of early stent intervention and deliberate stent fracture on PA growth and myocardial function is not understood.

Treatment of Vitamin D Insufficiency in Postmenopausal Women: A Randomized Clinical Trial.

Importance: Experts debate optimal 25-hydroxyvitamin D (25[OH]D) levels for musculoskeletal health.

Objective: To compare the effects of placebo, low-dose cholecalciferol, and high-dose cholecalciferol on 1-year changes in total fractional calcium absorption, bone mineral density, Timed Up and Go and five sit-to-stand tests, and muscle mass in postmenopausal women with vitamin D insufficiency.

Design, Setting, And Participants: This randomized, double-blind, placebo-controlled clinical trial was conducted at a single center in Madison, Wisconsin, from May 1, 2010, through July 31, 2013, and the final visit was completed on August 8, 2014.

The Deinococcus radiodurans DR1245 protein, a DdrB partner homologous to YbjN proteins and reminiscent of type III secretion system chaperones.

The bacterium Deinococcus radiodurans exhibits an extreme resistance to ionizing radiation. A small subset of Deinococcus genus-specific genes were shown to be up-regulated upon exposure to ionizing radiation and to play a role in genome reconstitution. These genes include an SSB-like protein called DdrB.

Nutritional status is associated with health-related quality of life in children with cystic fibrosis aged 9-19 years.

Background: The impact of improved nutritional status on health-related quality of life (HRQOL) is unknown for children with cystic fibrosis (CF).

Methods: Associations between nutritional status and HRQOL were examined over 2 years in 95 children, aged 9-19 years, who were followed in the Wisconsin Newborn Screening Project. HRQOL was assessed using the Cystic Fibrosis Questionnaire (CFQ).

Early childhood weight status in relation to asthma development in high-risk children.

Background: Obesity has been proposed to be a risk factor for the development of childhood asthma.

Objective: We sought to examine weight status from birth to age 5 years in relation to the occurrence of asthma at ages 6 and 8 years.

Methods: Two hundred eighty-five full-term high-risk newborns with at least 1 asthmatic/atopic parent enrolled in the Childhood Origin of Asthma project were studied from birth to age 8 years.

Classification of malnutrition in cystic fibrosis: implications for evaluating and benchmarking clinical practice performance.

Background: In 2005, the Cystic Fibrosis Foundation (CFF) revised the nutrition classification guidelines to eliminate the use of percentage of ideal body weight (%IBW) to define "nutritional failure"; the CFF also recommended that children with cystic fibrosis maintain a body mass index percentile (BMIp) > or = 50th.

Objective: We assessed the effect of the 2005 CFF nutrition classification guidelines on evaluating the performance of nutritional care practices.

Design: Data from 14,702 children reported to the 2002 CFF Patient Registry were analyzed to compare malnutrition rates in 113 cystic fibrosis centers in the United States.

Association between initial disease presentation, lung disease outcomes, and survival in patients with cystic fibrosis.

This US study was conducted to determine whether mode of diagnosis and initial disease presentation influence lung disease and survival in patients with cystic fibrosis. The study population included 27,703 patients reported to the 1986-2000 Cystic Fibrosis Foundation Registry. Patients were segregated into four diagnostic categories: meconium ileus (MI), prenatal/neonatal screening (SCREEN), positive family history (FH), and symptoms other than meconium ileus (SYMPTOM).