Orphan Disease in Structure of Recurrent Lower Respiratory Tract Diseases in Children from One To Five

Abstract

Abstract The paper contains structure analysis of the lower respiratory tract recurrent disease in 180 children aged one to five years, residents of Odessa, who were treated in pulmonology department of Children’s Academician BJ Reznik City Clinical Hospital. The examinees are represented by two groups: 150 children with recurrent wheezing and 30 - with recurrent pneumonia. The selection criteria were: abnormality of central nervous system, abnormality, cystic fibrosis, congenital heart defects, tuberculosis, human immunodeficiency virus-infection. The analysis detected that in the survey sample with recurrent lower respiratory tract disease 3.88% of children had Orphan disease. Thus in the group of children with recurrent wheezing were diagnosed 2 cases of congenital lobar hyperexpansion (ORPHA 1928), one case of lung bronchogenic cyst (ORPHA 2357), one case of pulmonary hypoplasia in the lower lobe of the left lung (ORPHA 2257), and one case of primary ciliary dyskinesia (ORPHA 98,861). Regarding the entire structure of recurrent wheezing, most frequently was detected atopic asthma (49.33%). In 24.6% cases the cause of recurrent wheezing was associated with the otolaryngological pathology. Bronchopulmonary dysplasia was diagnosed in two children. Recurrent pneumonia cases were differentiated if the localization of process was at the same or different places. When one-sided localization of pneumonia was established, next congenital airway malformations were diagnosed - 3 cases of congenital cystic adenomatoid malformations (type I). Genetic pathology often occurred in cases with different lesions location. In all cases of uncertain diagnosis, predictors of recurrent lower respiratory tract diseases were revealed.