Chilean consensus for the integral care of children and adults with cystic fibrosisCystic Fibrosis (CF) is the most frequent hereditary disease in whites, with a reserved prognosis. Since 2003, Chile began a comprehensive National Cystic Fibrosis Program, directed by the Respiratory Health Unit of the Ministry of Health. To date, the main results of the Program record a significantly longer survival (average 27 years) and a significant reduction in the age of diagnosis of patients admitted from 2006 onwards. Access to Chilean Explicit Health Guarantees, the implementation of neonatal screening in some regions of the country, the organization and setting up of CF-trained teams of various specialties, has contributed to improving results. Although the main manifestations are of the respiratory and digestive system, the multisystemic nature of CF makes it necessary to know the different aspects involved in its management, in order to optimize the results of the treatment and the resources invested, both in the public and private sectors. This document is a review and an update on the main aspects of the diagnosis, monitoring and treatment of the respiratory and non-respiratory manifestations of CF.

No abstract

Clinical and tomographic characterization of patients hospitalized with CoVid-19Objective: To perform a clinical and imaging characterization in patients hospitalized for and to analyze whether there are risk predictors associated with greater severity of the condition. Method: Observational, retrospective study. Patients hospitalized with COVID-19 were included between April and July 2020. Demographic data, comorbidities, laboratory tests, tomographic pattern in thorax tomography (TC), therapies received, and type of respiratory support were recorded. In the statistical analysis to identify risk factors, we used Pearson's χ 2 test or Fisher's test to compare categorical variables and Mann-Whitney test to compare continuous variables. Results: 164 patients were analyzed. Median age was 57 years (21 to 89). 111 patients (68%) were male and a median of 7 days of symptoms prior to admission (1 to 23). 68 patients (41%) have obesity (significantly higher in patients < 60 years, p = 0.026), 56 (34%) arterial hypertension (HT) and 43 (26%) with diabetes mellitus. The predominant pattern in the admission CT scan was ground glass opacity (GGO) with "crazy paving" (35%) and then pure GGO (28%). Type of ventilatory support required was considered as an indicator of severity. 51 patients (31%) require non-invasive ventilatory support (high-flow nasal cannula or NIMV) and 19 (11%) invasive ventilation (IMV). The statistically significant predictor variables of severity were HT (p = 0.001), Diabetes Mellitus (p = 0.001) and Obesity. (p = 0.002). Conclusions: Patients hospitalized for COVID 19 with the highest risk of respiratory torpid evolution were obese, hypertensive and diabetic patients

RESUMENLa Fibrosis Quística (FQ) es una enfermedad hereditaria autosómica recesiva. La detección precoz sumado a medidas de intervención temprana han modificado el curso de esta enfermedad con mejorías en su sobrevida, lo que ha llevado a una población creciente de pacientes de 18 años. La mutación genética determina una alteración en una Proteína Reguladora de Conductancia Transmembrana (CFTR) que afecta a numerosos órganos y sistemas, pero el compromiso pulmonar es el que causa mayor morbimortalidad. El germen más frecuente que infecta a adultos es Pseudomonas aeruginosa y si bien hay una serie de medidas para el manejo de la infección crónica por Pseudomonas las terapia dirigida a la restauración de la función de la proteína CFTR ha tomado relevancia. Cuando la falla respiratoria progresa, la única alternativa disponible es el trasplante pulmonar que mejora la sobrevida y la calidad de vida en estos pacientes.