The prevalence of aberrations in body composition in pediatric cystic fibrosis patients and relationships with pulmonary function, bone mineral density, and hospitalizations.

Background: Body mass index (BMI) correlates with clinical outcomes in cystic fibrosis but has limitations.  Body composition aberrations in CF are multifactorial. We sought to evaluate body composition and relationships with pulmonary function, bone health, and hospital admissions.

Practitioner Due Diligence: Real-World Lumacaftor/Ivacaftor Use.

Objective: Previous trials evaluated the efficacy of lumacaftor/ivacaftor in Phe508del homozygotes. These trials are limited by manufacturer sponsorship and were conducted under strict protocol. Additionally, this therapy is costly and does not allow for reduction in daily cystic fibrosis therapies.

Factors associated with low bone mineral density in patients with cystic fibrosis.

Osteoporosis associated with cystic fibrosis is becoming more important as the life expectancy of patients continues to improve. This study was done to determine the prevalence of osteoporosis and osteopenia in CF patients and to identify risk factors. We reviewed the medical charts of 103 adults and adolescents with Cystic Fibrosis who had undergone a dual energy X-ray absorptiometry (DEXA) scan to measure the bone mineral content of the lumbar spine (L1-L4).

Copper enzyme activities in cystic fibrosis before and after copper supplementation plus or minus zinc.

One laboratory reports low activities for 2 blood copper enzymes in subjects with cystic fibrosis (CF), which suggests that moderate copper deficiency is common in this state. The present study attempted to confirm this proposition in 3 ways: repeat the measures for 1 of the 2 copper enzymes (superoxide dismutase) in a new group of CF patients (males and females, N = 38), add another copper enzyme measure (plasma diamine oxidase) that has high sensitivity to copper status, and test if copper enzyme activities in CF patients rise by copper supplementation. The last test was performed plus or minus zinc supplementation since poor zinc status may contribute to poor copper status.