Department of Pediatrics, Nicolae Testemitanu State Medical and Pharmaceutical University, Congresul III al Medicilor de Familie din Republica Moldova, 17–18 mai, 2012, Chişinău, Republica Moldova, Conferinţa Naţională „Maladii bronhoobstructive la copii”, consacrată profesorului universitar, doctor habilitat Victor Gheţeul, 27 aprilie, Chişinău, Republica MoldovaCystic fibrosis (CF) is a monogenic autosomal recessive disorder with a chronic progressive evolution, which determines an
abnormal production of viscous secretions from the glands of
exogenous excretion, and characterized by chronic obstructive
pneumopathy, chronic diarrhea, malnutrition and malabsorption
syndromes.
Respiratory symptoms onset in CF patients usually starts early
– 80% in the first year of life with recurrent bronchitis, mostly
with severe obstructive syndrome, latent persistent pneumonias,
pulmonary and non-respiratory complications development. CF
is also characterized by the installation of chronic obstructive
pulmonary disease, which manifests itself by wheezing, prolonged
expiration, persistent cough during respiratory infectious episodes
which has latent evolution, nocturnal exacerbations, paroxysmal and exhausting evolution. Bronhoobstructive syndrome is
develops al the level of the small bronchi and is conditioned by
the viscous, sticky secretions and infective bacterial component.
Expectorated secretions are abundant, purulent, in cases with
progressive evolution haemoptysis may develop. In long-term
evolution children develop progressive respiratory failure. The
progressive evolution of the disease is conditioned also by resistant
bacterial agents (S.aureus, Ps aerugenosae, H. influenzae), which
accelerates destructive processes of the lung parenchyma and
contribute to the expansion of the pulmonary fibrosis phenomena,
and development of complications in the lungs (pneumothorax,
atelectasis, bronchiectasis, bullous-distrophy, lung abscess, haemoptysis, asphyxia, calcinates in lungs, pulmonary hypertension
and pulmonary heart disease).
Chest deformity is a clinical expression of the severe pulmonary pathological process: thoracic cage expansion, dorsal kyphosis, hypertrophic pulmonary osteoarthropathy (in schoolage
children) which causes chest pain, bone brittleness (fragility),
swelling, and hydrarthrosis. Chronic persistent severe hypoxia
determines the presence in CF children of fingers hippocratism.
ENT disorders at children with CF are presented by the nasal
polyposis, sinusitis and chronic rhinitis, transmission deafness.
The prognosis is reserved, with high risks of death in cases
with severe neonatal onset. Currently the disease may have a stable
evolution, if favorable circumstances are present: early diagnosis,
efficient treatment with digestive enzymes, control of pulmonary
infections, respiratory kinesiotherapy
