Cystic fibrosis; a hereditary inflammatory process.

One out of 3600 new-born children in the Netherlands has Cystic Fibrosis (CF). It is an autosomal recessive disease and about 70% of the Dutch CF-patients are homozygous for the ∆F508 mutation. Although the genetic mutation is identical in this group of patients, the pulmonary disease is very diverse. Causative factors are environmental and also co-genetic ones. Morbidity is caused by chronic inflammation and infection of the lungs, which leads to irreversible lung damage. Neutrophils play a key role in the inflammatory cascade. It is assumed that parts of the acute inflammatory response of the neutrophil (chemotaxis/IL8 „± adhesion/selectines„± activation/TNFa „± production of e.g. superoxides or myeloperoxidase „±tissue destruction) play an important role in the inflammatory process in CF. There is a higher concentration of mediators (IL-8, sICAM1, sE-Selectin, TNFa) in patients with CF than in other patients with airway infections.
The CFTR protein acts not only as a Cl channel but also as a Na/H antiport and influences the intracellular pH. This might affect the functional activity of the neutrophil. Recently, new activation markers (MoPhabs A17 and A27) located on leukocytes were described that may be an early sign of pulmonary inflammation.
To be able to predict and intervene in the inflammatory process would improve the prognosis especially in young children before the process of irreversible lung damage.

The use of new and powerful inhaled corticosteroid medication enables us to give anti-inflammatory therapy to young children without the systemic side-effects of orally administered steroids.

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Inhaled HFA-Beclomethasone Diproprionate (Qvar®) 200 mcg twice daily by aerochamber or a placebo (also inhaled by aerochamber).