Immunogenicity of the COVID-19 BNT162b2 vaccine in adolescents and young adults with cystic fibrosis.

Data regarding immunogenicity of SARS-CoV-2 BNT162b2 vaccine in cystic fibrosis (CF) patients are limited. We prospectively measured total (TAbs-RBD; U/ml) and neutralizing (NAbs-RBD; %) antibodies of SARS-CoV-2 spike-receptor binding domain (RBD) protein in 33 CF patients and 66 healthy controls with median age (IQR): 19.6 (17.6-24.3) years and 31 (29-36) years, respectively and investigated possible associations with epidemiological and clinical parameters. Compared to healthy controls, CF patients had higher levels of TAbs-RBD and NAbs-RBD after both doses (P-value < 0.001). One month after the second dose, CF patients and controls had TAbs-RBD: median (IQR): 3396 (2443) and 1452 (1231) U/ml, respectively. Similarly, the NAbs-RBD (%) were: 97.30 (1.00) and 95.70 (3.71) %, respectively. CF patients also had fewer local and systemic adverse events (AEs) (P-value < 0.001). Among CF patients, no significant differences in immunogenicity were detected regarding the phenotype, genotype, medications, or severity of the disease. BNT162b2 vaccine was immunogenic with limited reactogenicity in CF patients regardless of the phenotype or severity of disease.

Nebulizer Care and Inhalation Technique in Children with Cystic Fibrosis.

Nebulizers are used by the great majority of cystic fibrosis patients for delivery of cornerstone treatments. Inhalation technique and adequate disinfection and maintenance are important for optimizing medication delivery. In this study, inhalation technique and nebulizer disinfection/maintenance were assessed in cystic fibrosis patients by direct observation in clinic and completion of a scoring sheet. A total of 108 patients were recruited. The maximum inhalation technique score was attained by 30.5% and adequate inhalation technique score by 74.08% of patients. The inhalation technique score was best with the vibrating mesh nebulizer (p = 0.038), while patient age and number of nebulized medications did not affect ITS significantly (p > 0.05). Nebulizer disinfection/maintenance score was excellent in only 31.48%. Most families kept the nebulizer clean and used appropriate disinfection method, but only half of them replaced the nebulizer and nebulizer cup at the recommended time intervals. Nebulizer disinfection/maintenance score was positively affected by a number of nebulized medications and negatively by years of equipment use (p = 0.009 and p = 0.001, respectively). Even though inhalation technique and disinfection/maintenance practices were found to be adequate in a large proportion of cases, there is still a need for regular review and education. The type of nebulizer was associated with improved inhalation technique, but more data are required before making specific recommendations.

Comparison of International Growth Standards for Assessing Nutritional Status in Cystic Fibrosis: The GreeCF Study.

OBJECTIVE: The aim of the study was to compare 3 international growth references and explore their differences in assessing growth in Greek school-aged patients with cystic fibrosis (CF). METHODS: Sample included 114 patients (50 boys, age 11.5 ±â€Š3.9 years), provided care at Aghia Sofia Children's Hospital, Greece. Anthropometrics and predicted forced expiratory volume in 1 second (FEV1%) were measured. Body mass index (BMI) and height z scores were computed according to the Centers for Disease Control and Prevention (CDC), World Health Organization (WHO), and International Obesity Task Force (IOTF) references. Agreement between methods was analyzed with kappa statistics, repeated-measures analysis of variance, and Bland-Altman analysis. The relationship between FEV1% and BMI was explored with linear regression. RESULTS: Mean CDC BMI z score was the lowest (0.06 ±â€Š1.08), followed by WHO (0.17 ±â€Š1.14) and IOTF (0.35 ±â€Š1.05) (P ≤ 0.001 for all). The CDC and WHO growth references highly agreed for most weight status strata and stunting; all other comparisons produced lower agreements. Except for CDC and IOTF BMI z scores, all other comparisons produced wide levels of agreement and proportional bias. CDC reference classified more children as attaining low or normal weight, against WHO or IOTF (P ≤ 0.001 for all). Lowest prevalence of ideal and excess weight was recorded by CDC, compared to all other standards (P ≤ 0.001 for all). All BMI z scores provided moderate associations with FEV1%. CONCLUSION: Large variations across weight status classification were present when employing 3 growth standards in school-aged patients . Given than BMI z-scores from all references provided comparable associations with pulmonary function, our data indicate that no studied reference is better than others in assessing growth in CF.