Ultrasound findings of pubertal development in girls with cystic fibrosis and their association with clinical outcomes and Tanner staging.

OBJECTIVE: Patients with cystic fibrosis (CF) have a high incidence of pubertal and growth delay. In girls with CF, pubertal delay has an important psychological impact. Still, only a few studies have explored the occurrence of pubertal delay in girls with CF. The aims of this study were to compare the pubertal development of girls with CF compared with healthy controls regarding Tanner staging and pelvic ultrasound and, in girls with CF, correlate the findings with those of spirometry, body mass index, Shwachman-Kulczycki score (SKS), and genotyping. METHODS: This was a cross-sectional, case-control study including 35 girls with CF aged 6-17 years and following up at the Pediatric Pulmonology Outpatient Clinic of a tertiary hospital. These patients were compared with 59 healthy controls who had undergone pelvic ultrasound as part of another study conducted by the same group. Girls with CF were consecutively enrolled in the study during their annual routine check-up visit. Data collected in the CF group included spirometry and anthropometric results, SKS values, bone age, occurrence of current cystic fibrosis-related diabetes (CFRD) and Pseudomonas aeruginosa colonization, history of meconium ileus, genotype, ultrasound parameters, and Tanner stage. RESULTS: Pelvic ultrasound findings and Tanner stage reflected less pubertal development in girls with CF compared with healthy controls. Pubertal stage in patients with CF who presented CFRD (3.17 ± 1.16), had chronic colonization by Pseudomonas aeruginosa (3.10 ± 1.10), or were homozygous for the F508del mutation (1.91 ± 1.30) was more delayed than in controls (3.41 ± 1.41). Tanner stage correlated with age at menarche, bone age, and anthropometric and ultrasound data. CONCLUSION: Girls with CF presented a delay in pubertal development evaluated by Tanner stage and ultrasound parameters, which was more evident in the presence of comorbidities.

Sinonasal computed tomography in pediatric cystic fibrosis: do we know the indications?

OBJECTIVES: To investigated the correlation between sinonasal computed tomography (SNCT) findings and sinonasal symptoms, genotype, chronic colonization by Pseudomonas aeruginosa, forced expiratory volume in the first second (FEV1), body mass index (BMI), Shwachman-Kulczycki (SK) score, and Bhalla score in patients with cystic fibrosis. METHODS: We retrospectively reviewed the medical records of 61 patients aged 2-16 years who received care at the Pediatric Cystic Fibrosis Center of our institution over a 10-year period. SNCT findings were graded using the Lund-Mackay scoring system. For patients who had SNCT scans performed at two different time points, the findings from both examinations were compared. RESULTS: Patients with chronic P. aeruginosa colonization and patients with atelectasis had higher Lund-Mackay scores (p = 0.04 and p = 0.01, respectively). There was no difference in Lund-Mackay scores between sinonasal symptomatic and asymptomatic patients (p = 0.45). Among patients who had two SNCT scans available (n = 11), those with no evidence of bronchiectasis on chest CT had decreased Lund-Mackay scores compared to those with evidence of bronchiectasis, who had an increase in their scores (p = 0.03). Variations in the Bhalla score were positively and variations in the SK score were negatively correlated with variations in the Lund-Mackay score (r = 0.74, p = 0.01; and r = -0.85, p < 0.01). CONCLUSIONS: Associations between SNCT findings, chronic P. aeruginosa colonization, SK score, and chest CT findings were demonstrated. Further studies with larger sample sizes are needed to evaluate patient follow-up and assess the benefits of the sinonasal treatment strategy adopted for patients who exhibit pulmonary deterioration despite controlling for other factors associated with exacerbation.