ABSTRACT
Cystic Fibrosis (CF) is the most common autosomal-recessive genetic disease affecting approximately 8000 people in Germany. The disease is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene leading to dysfunction of CFTR, a transmembrane chloride channel. This defect causes insufficient hydration of the epithelial lining fluid which leads to chronic inflammation of the airways. Recurrent infections of the airways as well as pulmonary exacerbations aggravate chronic inflammation, lead to pulmonary fibrosis and tissue destruction up to global respiratory insufficiency, which is responsible for the mortality in over 90â% of patients. The main aim of pulmonary treatment in CF is to reduce pulmonary inflammation and chronic infection. Pseudomonas aeruginosa (Pa) is the most relevant pathogen in the course of CF lung disease. Colonization and chronic infection are leading to additional loss of pulmonary function. There are many possibilities to treat Pa-infection. This is a S3-clinical guideline which implements a definition for chronic Pa-infection and demonstrates evidence-based diagnostic methods and medical treatment for Pa-infection in order to give guidance for individual treatment options.
Subject(s)
Cystic Fibrosis Transmembrane Conductance Regulator/genetics , Cystic Fibrosis/diagnosis , Cystic Fibrosis/therapy , Practice Guidelines as Topic , Pseudomonas aeruginosa/isolation & purification , Cystic Fibrosis/complications , Cystic Fibrosis/microbiology , Cystic Fibrosis Transmembrane Conductance Regulator/metabolism , Germany , Humans , Pseudomonas Infections/diagnosisABSTRACT
Adolescents and young adults with cystic fibrosis (CF) are increasingly having difficulties to take over the complete responsibility for their daily treatment. Particularly inhalative therapy poses special problems. In order to overcome this unsatisfactory situation typically occurring during this vulnerable period, the German Airway League has now created a poster "Correct Inhalation Therapy For Patients With Cystic Fibrosis", according to the model presented by this League already in 2013 for correct inhalation under certain disease conditions. This give an opportunity to adolescents and young adults with CF to obtain anonymously, independently of time and location, autonomously and in a time-saving manner information on correct inhalative treatment. Adolescents and young adults with CF can thus be actively supported on their way to independence. Furthermore, the video clips and the poster offer support physicians, nurses and physiotherapists and can be employed in training of all involved persons in inhalation techniques.
Subject(s)
Computer-Assisted Instruction/methods , Cystic Fibrosis/rehabilitation , Oxygen Inhalation Therapy/methods , Patient Education as Topic/methods , Pulmonary Medicine/education , Video Recording/methods , Germany , Humans , Internet , Self Care/methodsABSTRACT
OBJECTIVE: Fibrosing colonopathy (FC) is a rare entity associated with cystic fibrosis (CF). Until now, patients with stricturing FC have usually been treated surgically. In this instance, we aimed at avoiding surgery by applying a new conservative approach. - METHODS: Case report on an adult with CF who developed persistent abdominal pain due to a non-passable stricture in the right transverse colon. Histology confirmed fibrosing colonopathy. - RESULTS: Initially we treated the patient with prednisolone pulse therapy and additive antibiotic therapy. For maintenance therapy we administered budesonide. The patient underwent clinical, laboratory and endoscopic follow-up over a three-year period. The stricture healed and was easy to pass. A relapse in the cecum at the ileocecal valve again improved under steroid and antibiotic therapy. - CONCLUSIONS: We present a novel therapeutic approach for advanced stricturing FC in an adult patient which successfully avoided surgery (right hemicolectomy) over a three year follow up.
Subject(s)
Colon/pathology , Colonic Diseases/drug therapy , Colonic Diseases/etiology , Cystic Fibrosis/complications , Fibrosis/drug therapy , Fibrosis/etiology , Adult , Anti-Bacterial Agents/therapeutic use , Follow-Up Studies , Humans , Male , Time Factors , Treatment OutcomeABSTRACT
Guidelines for prehospital emergency care within the chain of rescue are recommended. At the scene, information about the patient and therapeutical tools are limited. Accidents and emergencies happen outside of hospitals and doctor's offices, while environmental factors and logistic problems strongly determine the rescue process. The sequence of interventions or the choice of the right hospital with specific triage criterias are only examples for these problems. There is only limited scientific evidence for therapies performed prehospitally. However, scientific material for the preparation of guidelines should be evaluated by using standardized criteria. The rules of evidence based medicine according to Cook are a practical way to evaluate and classify scientific material and to establish practical guidelines. In this paper, an 8-step-program is introduced to develop practical guidelines for the Emergency Medical Services Cologne.
