CYSTIC FIBROSIS IN TURKISH CHILDREN
Gocmen A, Anadol D, Ozcelik U, Kiper N
Hacettepe
University, Ihsan Dogramaci ChildrenĄ¯s Hospital, Ankara, Turkey
Objective: The aim of this study
is to evaluate the clinical and laboratory findings of cystic fibrosis (CF)
patients who were diagnosed and followed at Ihsan Dogramacı ChildrenĄ¯s
Hospital, Ankara, Turkey.
Methods: Patients diagnosed with CF between January 1981 and December 2000
were retrospectively evaluated.
Results: There were 286 patients; 168 of them were boys and 118 were girls.
Their ages at time of diagnosis ranged between 1 month and 21 years (mean
26 months). There were parental consanguinity in 121 patients and siblings
of 80 patients had symptoms of CF. Patients were diagnosed with high sweat
chloride values (>60 meq/lt) except in 13 patients whose diagnosis were
established clinically. Recurrent respiratory tract infection was the most
common presenting symptom (in 179 patients), followed by diarrhea (102
patients) and failure to thrive (45 patients). Hypoelectrolytemia (49
patients), metabolic alkalosis (41 patients) and anemia (39 patients) were
the most prominent laboratory findings. Chest X-rays revelaed bilateral
hyperaeration in most (107 ) patients, whereas they were normal in 51 of them.
P. aeruginosa grew in sputum culture of most (45) patients, followed by K.
pneumonia in 40 patients and S. aureus in 39 patients. Mutations of most
cases could not be determined; 28 patients were found to be delta F508
homozygous. Pseudobartter syndrome and bronchiectasis were the main
complications. Among all patients, 64 of them died who were aged between 1
month and 16 years(mean age 16 months) mainly because of respiratory
diseases, 48 patients were lost to follow up and 174 patients are still being
followed in our clinic.
Conclusion: CF is of growing importance in our country and survival rate of
our patients is increasing with earlier diagnosis and treatment.