BMI increase during early childhood in boys with cystic fibrosis and early adrenarche.

BACKGROUND: Increase in body mass index (BMI) in early childhood (1-6 years) was found to be a contributing factor for impaired final height in boys with Cystic Fibrosis (CF). Early adrenarche (before age 9 years in boys) may contribute to an impaired final height by triggering an early acceleration of bone age resulting in a compromised growth spurt during puberty. We aimed to analyze the timing of adrenarche in boys with CF and to associate BMI increase in early childhood to timing of adrenarche. METHODS: Boys with CF, aged 8-9 years, visiting the CF expertize center Utrecht were included. Since 2018, anthropomorphic, pubertal and endocrine data were collected. Early adrenarche in boys was defined as a dehydroepiandrosterone sulfate (DHEAS) ≥ 1 µmol/L before the age of 9 years. RESULTS: Thirteen boys (mean age 8.55 ± 0.27 years) were enrolled. The median (IQR) DHEAS-level was 1.3 µmol/L (0.71-2.40). Eight boys (61.5%) had an early rise in DHEAS-levels ≥ 1 µmol/L. Mean increase in BMI Z-score between 1 and 6 years of age (ΔBMI1-6) was -0.07 ± 0.86. A significant correlation was found between ΔBMI1-6 and DHEAS-levels at the age of 8-9 years (r = 0.624, p = 0.040). In five boys with early rise in DHEAS, accelerated bone age was found (average 1.55 ± 0.96 years). CONCLUSION: In this small cohort, 61.5% of boys with CF between 8 and 9 years had an early rise of DHEAS, which was correlated to ΔBMI1 -6 between 1 and 6 years. Early adrenarche may be caused by ΔBMI1 -6.

Infection, inflammation and exercise in cystic fibrosis.

Regular exercise is positively associated with health. It has also been suggested to exert anti-inflammatory effects. In healthy subjects, a single exercise session results in immune cell activation, which is characterized by production of immune modulatory peptides (e.g. IL-6, IL-8), a leukocytosis and enhanced immune cell functions. Upon cessation of exercise, immune activation is followed by a tolerizing phase, characterized by a reduced responsiveness of immune cells. Regular exercise of moderate intensity and duration has been shown to exert anti-inflammatory effects and is associated with a reduced disease incidence and viral infection susceptibility. Specific exercise programs may therefore be used to modify the course of chronic inflammatory and infectious diseases such as cystic fibrosis (CF).Patients with CF suffer from severe and chronic pulmonary infections and inflammation, leading to obstructive and restrictive pulmonary disease, exercise intolerance and muscle cachexia. Inflammation is characterized by a hyper-inflammatory phenotype. Patients are encouraged to engage in exercise programs to maintain physical fitness, quality of life, pulmonary function and health.In this review, we present an overview of available literature describing the association between regular exercise, inflammation and infection susceptibility and discuss the implications of these observations for prevention and treatment of inflammation and infection susceptibility in patients with CF.