Therapy goals Improvement of the symptomatology Stabilization of lung function Therapy recommendations Note: Cystic fibrosis therapy is based on the three pillars of nutritional medicine (see below. , sports medicine, and physiotherapy (see “Other Therapy” below), as well as pharmacotherapy. Pharmacotherapy Pharmacotherapy of cystic fibrosis (CF). Secretolytic therapy (liquefaction of secretions: Use of oral expectorants … Cystic Fibrosis: Drug Therapy
Optional medical device diagnostics – depending on the results of the history, physical examination, and obligatory laboratory parameters – for differential diagnostic clarification. Nasal transepithelial potential difference (nPD) – if the sweat test (determination of chloride ion concentration; gold standard) was unremarkable or borderline, but suspicion persists (because of inconclusive mutation analysis). Abdominal ultrasonography (ultrasound … Cystic Fibrosis: Diagnostic Tests
1st order In the case of large pneumothorax* , the installation of a chest drain is required Bronchial artery embolization (= artificial occlusion of a pulmonary artery by a vascular plug) is used in cases of hemoptysis (coughing up blood) In severe respiratory insufficiency (respiratory weakness), the only curative measure is lung transplantation (LUTX) * … Cystic Fibrosis: Surgical Therapy
The following symptoms and complaints may indicate cystic fibrosis (CF): Leading symptoms Chronic bronchitis with productive cough (sputum). Chronic sinusitis (sinusitis) and nasal polyps (mucosal protrusions). Recurrent (reoccurring) infections of the respiratory tract. Failure to thrive Meconium ileus (intestinal obstruction in newborns) or lack of meconium clearance within the first 48 hours of life – … Cystic Fibrosis: Symptoms, Complaints, Signs
Pathogenesis (development of disease) Cystic fibrosis – cystic fibrosis (CF) – is an autosomal recessive inherited disease caused by the mutation (permanent genetic change) of the cystic fibrosis transmembrane conductance regulator (CFTR) gene (“cystic fibrosis transmembrane regulator”), a regulator protein of chloride transport, on the chromosome. In most cases, there is a deletion (loss) of … Cystic Fibrosis: Causes
General measures Inhalation of hypertonic saline solutions is a useful and effective measure To maintain stable lung function, respiratory training with the flutter valve should be performed several times a day. Observance of the general hygiene measures! Nicotine restriction (refrain from tobacco use). Aim for normal weight!Determination of BMI (body mass index, body mass index) … Cystic Fibrosis: Therapy
The following are the major diseases or complications that may be contributed to by cystic fibrosis (CF): Respiratory system (J00-J99) Progressive respiratory insufficiency (respiratory failure) with chronic cor pulmonale (pressure-loaded right heart). Allergic bronchopulmonary aspergillosis/Aspergillus bronchitis (mold infection) – about 30% of patients with cystic fibrosis have colonization of the lungs with the mold Aspergillus … Cystic Fibrosis: Complications
A comprehensive clinical examination is the basis for selecting further diagnostic steps: General physical examination – including blood pressure, pulse, body temperature, body weight, body height [failure to thrive]; further: Inspection (viewing). Skin, mucous membranes, and sclerae (white part of the eye) [Icterus prolongatus (= jaundice (jaundice) > 14 days persisting)] Auscultation (listening) of the … Cystic Fibrosis: Examination
For the diagnosis of cystic fibrosis according to), at least one diagnostic clue must be present and CFTR dysfunction must be demonstrated: Screening for cystic fibrosis in Germany is performed in three stages as a serial combination of two biochemical tests for immunoreactive trypsin (IRT) and pancreatitis-associated protein (PAP) and DNA mutation analysis. Beginning September … Cystic Fibrosis: Test and Diagnosis
Medical history (history of illness) represents an important component in the diagnosis of cystic fibrosis (CF). Family history Is there a frequent history of lung disease in your family? Are there any hereditary diseases in your family? Social anamnesis Current medical history/systemic medical history (somatic and psychological complaints). What symptoms have you noticed? Do you … Cystic Fibrosis: Medical History
Respiratory System (J00-J99) Pleural effusion – accumulation of fluid in the pulmonary fissure between the lung and pleura. Bronchiectasis (synonym: bronchiectasis) – persistent irreversible saccular or cylindrical dilatation of the bronchi (medium-sized airways) that may be congenital or acquired; symptoms: chronic cough with “mouthful expectoration” (large-volume three-layered sputum: foam, mucus, and pus), fatigue, weight loss, … Cystic Fibrosis: Or something else? Differential Diagnosis
