Cystic fibrosis (CF) is the most common monogenic autosomal recessive disorder with progressive chronic evolution which is potentially lethal. Poor growth is a characteristic of children suffering from
cystic fibrosis. A poor nutritional status is an independent risk factor for inadequate survival in
cystic fibrosis and is associated with disease complications. The appropriate nutritional management is an important part of the treatment so that the patient with
cystic fibrosis can achieve normal growth and development and maintain the best possible health status. A balanced diet supplemented with snacks high in fat and calories is necessary to increase the caloric intake in children with
cystic fibrosis. Children with
cystic fibrosis have higher caloric needs than healthy children of the same age and sex.
Malnutrition in CF is multifactorial.
Cystic fibrosis is a complex multisystem disorder affecting mainly the gastrointestinal tract and respiratory system. In the past,
malnutrition was an inevitable consequence of
disease progression, leading to poor growth, impaired respiratory muscle function, decreased exercise tolerance and immunological impairment. A positive association between
body weight and height and survival has been widely reported. The energy requirements of patients with CF vary widely and generally increase with age and disease severity.
Cystic fibrosis remains a paediatric disorder which is often underdiagnosed but which, if therapeutically managed properly (by means of
drug therapy as well as by appropriate
physiotherapy techniques), can lead to improved quality of life and, thus, to a bigger life expectancy.