Cystic fibrosis is one of the most frequent genetic hereditary diseases in our environment, also called mucoviscidosis. It is a chronic and sometimes fatal disease. It is transmitted from father to son, by mutation of a gene from the long arm of chromosome 7 that encodes the regulatory protein of the transmembrane conductance of cystic fibrosis It is an autosomal recessive disease. For each gene, we have two copies of alleles, one of our inherited father and one inherited from our mother. Then, if the mutation is in a single allele, it will be a carrier individual; If the variation exists in the two alleles, it will be a sick individual. It is thought that in our environment one out of every five people is a carrier of the mutation.symptomIn carrier individuals, cystic fibrosis does not produce any alteration, but if this individual had offspring with a spouse also carrying the genetic variation, it could lead to sick individuals.In individuals who are affected, it produces the accumulation of mucus especially in the lungs, and to a lesser extent to the pancreas, liver, and intestine. As the most critical affectation is in the lungs, they are usually patients with severe respiratory problems, with multiple pulmonary infections from infancy.Cystic fibrosis can also produce sterility in men due to the bilateral congenital absence of the vas deferens, the necessary conduit for sperm transport. Thus, these males will have azoospermia (absence of sperm in the ejaculate) and will need the appropriate genetic counseling before having offspring.How is it diagnosedCystic fibrosis is diagnosed by the sweat test or a blood test that demonstrates the mutation. Multiple mutations have been identified, of which the most important one is delta F508.Treatment of cystic fibrosisNo medicine cures this disease. There are multiple treatments to treat the symptoms, and ultimately it is possible even to have to resort to a lung transplant.Why is this mutation looked at the semen/ovum donors?In the clinics of assisted reproduction, we have the possibility of doing sterility treatments with donors to patients who can not use their eggs or sperm for various reasons.Therefore, it is essential before doing a five with the donation of gametes, confirm that these are free of this genetic mutation so prevalent in our environment.Clinical practice guide – Pulmonary complications in cystic fibrosisCystic fibrosis is an autosomal recessive disease, due to mutations in chromosome 7, involving multiple organs. One of the most frequent complications is the infection of the respiratory tract by Pseudomonas aeruginosa. Objectives: to describe the recommendations on the management of pulmonary complications reported in the guidelines of good quality clinical practice according to AGREE on II, with the aim of reducing the development of morbidities and improving the quality of life of patients. Methodology: the clinical practice guidelines for cystic fibrosis in English and Spanish published between 2005 and 2013 were reviewed, using the AGREE II instrument to evaluate its quality. Results: the search yielded a total of 126 guides, six were assessed, and 4 of them obtained a score in the domains of rigor and independence 60% according to AGREE on II. Of these, recommendations were made for the management of pulmonary exacerbations due to Pseudomonas aeruginosa, the airway, pneumothorax, hemoptysis, and allergic bronchopulmonary aspergillosis. Conclusions: the best inhaled antibiotic for chronic use is tobramycin. The dornase alpha is the best mucolytic. The method of corticosteroids is controversial, but it is useful in patients with asthma and allergic bronchopulmonary aspergillosis. The best quality clinical practice guide to date is that of Ecuador.About Us:To present the recommendations on the management of the pulmonary complications, reported in clinical practice guidelines, of good condition according to AGREE on II, with the aim of reducing morbidity and improving the quality of life of the patients.