Vitamin D deficiency is widespread in patients with cystic fibrosis (CF). From 2016 to 2018, prophylactic doses of vitamin D were prescribed for the patients of the Russian cystic fibrosis Center in accordance with the National cystic fibrosis consensus and they were informed about the role of vitamin D deficiency. of the study was to conduct a comparative analysis of the frequency of deficiency and insufficiency of vitamin D in children with CF of different ages in Moscow region in 2016 and 2018. . The study involved 115 patients with CF at the age of 0-18 in 2016 and 211 children of the same age in 2018. All children underwent determination of 25(OH)D in blood serum by ELISA. . The frequency of vitamin D deficiency and insufficiency [25(OH)D level <30 ng/ml] in CF patients in 2016 was 64.3%, and in 2018 - 48.7%. Among children of 0-3 years normal serum 25(OH)D levels (>30 ng/ml) were registered in 62.5% in 2016 and in 62.2% in 2018, in children of 4-11 years - 28.8% in 2016 and 58.1% in 2018, among adolescents (11-18 years) - 11.8 and 30.2%, respectively. . Comparative analysis showed a positive dynamics in reducing the proportion of CF patients with vitamin D deficiency and insufficiency against the background of continuous use of prophylactic doses of cholecalciferol and educational work for the period from 2016 to 2018. However, in a significant proportion of patients (48.7%), vitamin D level did not reach the norm in 2018 that requires the correction of preventive doses and increase in patient compliance.
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http://dx.doi.org/10.24411/0042-8833-2020-10019 | DOI Listing |
Exp Clin Endocrinol Diabetes
January 2025
Department of Molecular Medicine, University of Pavia, Pavia, Italy.
The issue of a possible association between Shwachman-Diamond Syndrome and diabetes has been debated for many years. This review updates the Italian Shwachman-Diamond registry, confirming our previous findings that suggest that these patients might be at higher risk of developing diabetes, particularly type 1. These data are of relevance in the clinical follow-up of patients in everyday life, emphasizing the need for early diagnosis and timely intervention.
View Article and Find Full Text PDFJ Cyst Fibros
January 2025
Leeds Institute of Medical Research, University of Leeds, School of Medicine, Leeds, United Kingdom; The Leeds Adult CF Unit, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom. Electronic address:
Background: Whether improvements in gastrointestinal (GI) symptoms observed with Elexacaftor/Tezacaftor/Ivacaftor (ETI) treatment are sustained in the longer-term requires exploration. This study investigated how GI-symptoms change with longer-term ETI use in pancreatic insufficient adults with cystic fibrosis (awCF).
Methods: Participants completed up to three abdominal symptom questionnaires, employing the validated CFAbd-Score.
Microbiol Res
January 2025
State Key Laboratory for Crop Stress Resistance and High-Efficiency Production, Shaanxi Key Laboratory of Agricultural and Environmental Microbiology, College of Life Sciences, Northwest A&F University, Yangling, Shaanxi 712100, China. Electronic address:
Pseudomonas aeruginosa is a prominent respiratory pathogen in cystic fibrosis (CF) patients, thriving in the hypoxic airway mucus. Previous studies have established the role of the oxygen-binding hemerythrin, Mhr, in enhancing P. aeruginosa's fitness under microaerobic conditions.
View Article and Find Full Text PDFJ Bras Pneumol
January 2025
. Departamento de Cirurgia Torácica, Instituto do Coracao, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, BR.
Objective: Surgical resection remains the gold standard treatment for bronchiectasis in patients who present with hemoptysis or suppuration, as well as in those who do not respond to clinical treatment. We sought to investigate the efficacy of sublobar resection (segmentectomy) and compare it with that of lobar resection (lobectomy) in patients with non-cystic fibrosis bronchiectasis.
Methods: Patients undergoing lobectomy or segmentectomy between 2019 and 2023 were included in the study.
PLoS One
January 2025
Asthma and Air Quality Branch, Division of Environmental Health Science and Practice, National Center for Environmental Health, Centers for Disease Control and Prevention, Atlanta, Georgia, United States of America.
The epidemiology of allergic bronchopulmonary aspergillosis (ABPA) in the United States is not well-described. To estimate national ABPA prevalence among patients with asthma or cystic fibrosis, characterize ABPA testing practices, and describe ABPA clinical features, treatment, and 6-month outcomes. We used the 2016-2022 Merative™ MarketScan® Commercial/Medicare and Multi-State Medicaid Databases to identify cohorts of patients with 1) asthma, 2) cystic fibrosis (CF), and 3) ABPA.
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