Effects of long-term azithromycin therapy on auditory functions in children with chronic respiratory diseases.

BACKGROUND AND OBJECTIVES: In recent years, long-term azithromycin (AZT) use has been increased in pediatric patients with chronic respiratory tract problems. In this study, we aimed to assess auditory functions in children on long-term AZT therapy. METHOD: The study included 43 patients who received long-term AZT treatment and 27 age-matched, healthy controls. In the study and control groups, ear-nose-throat examination, pure tone audiometry, speech recognition threshold (SRT), speech discrimination score (SDS), uncomfortable loudness level (UCL), acoustic reflex tests, and otoacoustic emission tests were performed. The test results were statistically compared between groups. RESULTS: Mean age was 11.3 ± 0.56 years in the study group and 9.3 ± 3.07 years in the control group. The medain hearing examination was 8 months after end of AZT treatment (Ranged: 1-12 months). No significant difference was found in normal- and high-frequency audiogram tests at any frequency between study and control groups. Also, no significant difference was found in distortion product otoacoustic emissions (DPOAE) and transient evoked otoacoustic emissions (TEOAE) tests between groups. Also, there was no significant difference in acoustic reflex, SRT, SDS, and UCL test results between study and control groups. CONCLUSION: This is the first study assessing auditory functions comprehensively in pediatric patients who received long-term AZT therapy. In this study, it was shown that long-term AZT use had no deleterious effect on auditory function tests.

Relationships Between Bronchoscopy, Microbiology, and Radiology in Noncystic Fibrosis Bronchiectasis.

Background: Published data on the correlations of bronchoscopy findings with microbiological, radiological, and pulmonary function test results in children with noncystic fibrosis (CF) bronchiectasis (BE) are unavailable. The aims of this study were to evaluate relationships between Bronchoscopic appearance and secretion scoring, microbiological growth, radiological severity level, and pulmonary function tests in patients with non-CF BE. Methods: Children with non-CF BE were identified and collected over a 6-year period. Their medical charts and radiologic and bronchoscopic notes were retrospectively reviewed. Results: The study population consisted of 54 female and 49 male patients with a mean age of 11.7 ± 3.4 years. In the classification according to the bronchoscopic secretion score, Grade I was found in 2, Grade II in 4, Grade III in 9, Grade IV in 17, Grade V in 25, and Grade VI in 46 patients. When evaluated according to the Bhalla scoring system, 45 patients had mild BE, 37 had moderate BE, and 21 had severe BE. Microbial growth was detected in bronchoalveolar lavage fluid from 50 of the patients. Forced expiratory volume in 1 s (FEV1) and functional vital capacity decreased with increasing bronchoscopic secretion grade (P = 0.048 and P = 0.04), respectively. The degree of radiological severity increased in parallel with the bronchoscopic secretion score (P = 0.007). However, no relationship was detected between microbiological growth rate and radiological findings (P = 0.403). Conclusions: This study showed that bronchoscopic evaluation and especially scoring of secretions correlate with severe clinical condition, decrease in pulmonary function test, worsening in radiology scores, and increase in microbiological bacterial load in patients. Flexible endoscopic bronchoscopy should be kept in mind in the initial evaluation of non-CF BE patients.

Patients eligible for modulator drugs: Data from cystic fibrosis registry of Turkey.

BACKGROUND: A better understanding of cystic fibrosis transmembrane conductance regulator biology has led to the development of modulator drugs such as ivacaftor, lumacaftor-ivacaftor, tezacaftor-ivacaftor, and elexacaftor-tezacaftor-ivacaftor. This cross-sectional study evaluated cystic fibrosis (CF) patients eligible for modulator drugs. METHODS: Data for age and genetic mutations from the Cystic Fibrosis Registry of Turkey collected in 2018 were used to find out the number of patients who are eligible for modulator therapy. RESULTS: Of registered 1488 CF patients, genetic analysis was done for 1351. The numbers and percentages of patients and names of the drugs, that the patients are eligible for, are as follows: 122 (9.03%) for ivacaftor, 156 (11.54%) for lumacaftor-ivacaftor, 163 (11.23%) for tezacaftor-ivacaftor, and 57 (4.21%) for elexacaftor-tezacaftor-ivacaftor. Among 1351 genotyped patients total of 313 (23.16%) patients are eligible for currently licensed modulator therapies (55 patients were shared by ivacaftor and tezacaftor-ivacaftor, 108 patients were shared by lumacaftor-ivacaftor and tezacaftor-ivacaftor, and 22 patients were shared by tezacaftor-ivacaftor and elexacaftor-tezacaftor-ivacaftor groups). CONCLUSIONS: The present study shows that approximately one-fourth of the registered CF patients in Turkey are eligible for modulator drugs. As, frequent mutations that CF patients have in Turkey are different from North American and European CF patients, developing modulator drugs effective for those mutations is necessary. Furthermore, as modulator drugs are very expensive currently, financial support of the government in developing countries like Turkey is noteworthy.