Today's issues, which have a delicate interest and specific consequences for society, are either hidden or discovered. Technology has been a form of spur, for its not only giant but accelerated pace, which encourages the discussion of what is possible and what is correct. Clinical and epidemiological characteristics of pediatric patients with cystic fibrosis in Panama, from January 2008 to December 2017

Abstract

Abstract
Introduction .The main objective of the present investigation is to describe the clinical and epidemiological characteristics of cystic fibrosis (CF) in Panama, which allow us to make a nearly diagnosis and make known the biochemical, phenotypic and associated morbidities in order to improve the needs of the patients with this disease. Materials and methodsA retrospective study of patients diagnosed with cystic fibrosis was conducted between January 2008 and December 2017, in hospitals in Panama City: Hospital del Niño Dr. José Renán Esquivel, Hospital de Especialidades Pediátricas Omar Torrijos Herrera and Hospital José Domingo de Obaldía, upon approval of the corresponding authorities. We analyzed the demographic, phenotypic characteristics, biochemical results, complications and treatment of patients diagnosed with Cystic Fibrosis. Through a descriptive design, the information of them was analyzed. Results. The average age for the diagnosis of cystic fibrosis was of 2.8 years, 52% corresponded tomale and 48% female. The average number of hospitalizations prior tothe diagnosis of cystic fibrosis was 3.8. In 72% of the patients the first symptoms appeared before the first year of life. In the majority of patients, the clinical manifestations were a combination of gastrointestinal and / or nutritional and respiratory symptoms (96 and 92% respectively). Molecular genetics examination was performed in 44% of the patients. According to the classification of the genetic defect, 20% of the patients were from the delta F 508 group. The average age of survival is 8.2 years. During the period 2008-2017 there were 6 deaths of patients with cystic fibrosis, which corresponds to a case-fatality rate of 22%.Conclusions.The results of our study showed conditions such as failure to thrive, recurrent respiratory infections, steatorrhea as the most frequent form of clinical presentation. We found correlation with the literature with the presence of respiratory manifestation associated with nutritional compromise. The genotype is not made to all patients, recognizing the importance of assessing the availability and costs of genetictesting. Despite early diagnosis, our patients have an important nutritional commitment. In recenty ears there is evidence of a lower age of diagnosis earlier compared with previous years.It is necessary to standardize diagnostic tests and follow-up, since not all patients fully meet the diagnostic criteria. At present, a high index of clinical suspicion is mandatory for early detection and immediate intervention of the treatment until updating of the diagnostic facilities.