246 Wilschanski^
Adolescence
This stage is associated with increased growth,
puberty and increased physical activity. This adds
up to markedly increased nutritional require-
ments which are often difficult to attain. Pulmo-
nary infections are more common, as is the onset
of CF-related diabetes and, in a small minority of
cases, CF-related liver disease. Female patients
are at a greater risk of nutritional failure at this
age [11]. This may be partly due to dissatisfaction
with weight and body shape in healthy adolescent
females. Growth retardation and pubertal delay
occur with increased social pressure and psycho-
social stress. These factors must be considered
when nutritional advice is provided to teenagers.
Ideally, dietary advice should be passed on before
conception as a low prepregnancy BMI is associ-
ated with reduced birth weight. Nutrition should
be optimized throughout pregnancy and vitamin
levels should be monitored [12].
Bone Health
A decrease in bone mineral density (BMD) in pa-
tients with CF may begin at a young age [13].
There are many factors that influence bone health
both in healthy individuals and in patients with
CF; these include nutritional status, calcium, vita-
mins D and K, pulmonary infection, exercise, glu-
cocorticoids and the class of CFTR mutation. The
causes of poor BMD, a reflection of bone health,
are thus multifactorial. Evidence for the efficacy
of treatments for maintaining and improving
bone health is lacking in CF; however, consensus
Cellular
defect? Psychogenic
Biliary Gastrointestinal
Vomiting
Energy deficit
Needs Losses Intake
Weight loss Pulmonary
infections
Immune
dysfunction
Deteriorating lung function
Pancreatic
Intestinal
Iatrogenic
Respiratory
muscles
Lung
parenchyma
++
Anorexia
Fig. 1. The pathogenesis of energy
imbalance in CF.
Koletzko B, et al. (eds): Pediatric Nutrition in Practice. World Rev Nutr Diet. Basel, Karger, 2015, vol 113, pp 244–249
DOI: 10.1159/000367876