الفهرس | Only 14 pages are availabe for public view |
Abstract Background: Cystic fibrosis (CF) is the most common lethal autosomal recessive respiratory disease in the western world with an estimated incidence of 1 per 300 live births. Most patients with CF succumb to respiratory failure from chronic pulmonary failure infections. Objectives: To assess vitamin D effect on decreasing pulmonary exacerbations in cystic fibrosis and noncystic fibrosis bronchiectasis. Patients and Methods: Cross sectional study. Chest department & chest clinic in Eldemerdash children hospital. This study will include 20 cystic fibrosis patients, 20 non cystic fibrosis bronchiectasis patients, 20 controls (all should be above 6 months old). Patients will be recruited from the pediatric emergency room, inpatient wards and specialized chest & GIT clinics of the children’s Hospital, Ain Shams university hospitals. Vitamin D serum level will be measured in CF & non CF bronchiectasis patients and controls & pulmonary function tests will be done to all the patients prior starting vitamin D supplementation then vitamin D serum level will be followed up in the patients after treatment & also pulmonary function tests will be repeated. Controls will be assessed by basal serum vitamin D level. Results: We found that 67.5% of our patients were from consanguineous parents. 75% of CF patients had consanginous parents (15 cases),while 60% of non CF bronchiectasis cases had consanginous parents (12 cases). A large number of patients (18 patients) had positive family history of same condition (45% of cases). 6o% of CF patients (12 cases) had history of sib death with same condition, while only 30% of non CF bronchiectasis patients had positive family history of same condition. As regarding of respiratory symptoms, 100% of our patients suffered from productive cough, dyspnea reported in 60% of cases, 82% of our patients suffered from wheezy chest and were on inhaled bronchodilators & inhaled steroids. 80% of our patients had frequent attacks of respiratory distress, 37% of them gave history of cyanosis due to exacerbation of their chest condition. 30% of our 40 patients complained from osteopenia, as regard our study. Conclusion: There is an association between vitamin D deficiency and bronchiectasis (both CF & non-CF). It was found that bronchiectasis patients are more vitamin D deficient than normal population. The more deficiency in Vitamin D, the more sever the lung disease. Vitamin D deficiency is also associated with more risk for pulmonary exacerbations (IV antibiotics need, hospital stay, ICU admission and missed school days). Improving vitamin D status in bronchiectasis patients leads to improvement of pulmonary functions, less frequent & less sever exacerbations & hospital stay. |