Cystic fibrosis (CF) is a genetic disorder inherited in an autosomal recessive manner that impairs the normal clearance of mucus from the lungs, which facilitates the colonization and infection of the lungs by bacteria, notably Staphylococcus aureus. CF is a rare genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys, and intestine. The hallmark feature of CF is the accumulation of thick mucus in different organs. Long-term issues include difficulty breathing and coughing up mucus as a result of frequent lung infections. Other signs and symptoms may include sinus infections, poor growth, fatty stool, clubbing of the fingers and toes, and infertility in most males. Different people may have different degrees of symptoms.
A cough is a sudden expulsion of air through the large breathing passages which can help clear them of fluids, irritants, foreign particles and microbes. As a protective reflex, coughing can be repetitive with the cough reflex following three phases: an inhalation, a forced exhalation against a closed glottis, and a violent release of air from the lungs following opening of the glottis, usually accompanied by a distinctive sound.
Hemoptysis or haemoptysis is the discharge of blood or blood-stained mucus through the mouth coming from the bronchi, larynx, trachea, or lungs. It does not necessarily involve coughing. In other words, it is the airway bleeding. This can occur with lung cancer, infections such as tuberculosis, bronchitis, or pneumonia, and certain cardiovascular conditions. Hemoptysis is considered massive at 300 mL. In such cases, there are always severe injuries. The primary danger comes from choking, rather than blood loss.
Sputum is mucus that is coughed up from the lower airways. In medicine, sputum samples are usually used for a naked eye examination, microbiological investigation of respiratory infections and cytological investigations of respiratory systems. It is crucial that the specimen does not include any mucoid material from the nose or oral cavity.
Bronchiectasis is a disease in which there is permanent enlargement of parts of the airways of the lung. Symptoms typically include a chronic cough with mucus production. Other symptoms include shortness of breath, coughing up blood, and chest pain. Wheezing and nail clubbing may also occur. Those with the disease often get lung infections.
Primary ciliary dyskinesia (PCD) is a rare, autosomal recessive genetic ciliopathy, that causes defects in the action of cilia lining the upper and lower respiratory tract, sinuses, Eustachian tube, middle ear, fallopian tube, and flagella of sperm cells. The alternative name of "immotile ciliary syndrome" is no longer favored as the cilia do have movement, but are merely inefficient or unsynchronized. When accompanied by situs inversus the condition is known as Kartagener syndrome.
Atelectasis is the partial collapse or closure of a lung resulting in reduced or absent gas exchange. It is usually unilateral, affecting part or all of one lung. It is a condition where the alveoli are deflated down to little or no volume, as distinct from pulmonary consolidation, in which they are filled with liquid. It is often referred to informally as a collapsed lung, although more accurately it usually involves only a partial collapse, and that ambiguous term is also informally used for a fully collapsed lung caused by a pneumothorax.
Chest wall oscillation is when devices are used in airway clearance therapy to clear excess mucus from lung airways. It is principally used in the treatment of cystic fibrosis, but is gaining use in the treatment of other diseases, such as bronchiectasis, COPD, cerebral palsy and muscular dystrophy, in which excessive mucus can block airways due to excessive production or impaired clearance.
Benjamin Franklin Deford III was an American sportswriter and novelist. From 1980 until his death in 2017, he was a regular sports commentator on NPR's Morning Edition radio program.
Dorothy Hansine Andersen was the American physician and researcher who first identified and named cystic fibrosis. During her almost thirty year tenure at Babies Hospital of Columbia-Presbyterian Medical Center, Andersen not only identified CF and its inheritance through a recessive gene, she was also at the forefront of developing diagnostic tests and life-extending treatments for the disease. Andersen was also active in researching other diseases that are diagnosed in children. She was the first to describe Glycogen storage disease type IV, which, in recognition of her contributions, became known as Andersen's Disease. Her research on heart malformations informed the development of open heart surgery and the training of new surgeons.
An aspergilloma is a clump of mold which exists in a body cavity such as a paranasal sinus or an organ such as the lung. By definition, it is caused by fungi of the genus Aspergillus.
Allergic bronchopulmonary aspergillosis (ABPA) is a condition characterised by an exaggerated response of the immune system to the fungus Aspergillus. It occurs most often in people with asthma or cystic fibrosis. Aspergillus spores are ubiquitous in soil and are commonly found in the sputum of healthy individuals. A. fumigatus is responsible for a spectrum of lung diseases known as aspergilloses.
Peribronchial cuffing, also referred to as peribronchial thickening or bronchial wall thickening, is a radiologic sign which occurs when excess fluid or mucus buildup in the small airway passages of the lung causes localized patches of atelectasis. This causes the area around the bronchus to appear more prominent on an X-ray. It has also been described as donut sign, considering the edge is thicker, and the center contains air.
Mucociliary clearance (MCC), mucociliary transport, or the mucociliary escalator describes the self-clearing mechanism of the airways in the respiratory system. It is one of the two protective processes for the lungs in removing inhaled particles including pathogens before they can reach the delicate tissue of the lungs. The other clearance mechanism is provided by the cough reflex. Mucociliary clearance has a major role in pulmonary hygiene.
Diffuse panbronchiolitis (DPB) is an inflammatory lung disease of unknown cause. It is a severe, progressive form of bronchiolitis, an inflammatory condition of the bronchioles. The term diffuse signifies that lesions appear throughout both lungs, while panbronchiolitis refers to inflammation found in all layers of the respiratory bronchioles. DPB causes severe inflammation and nodule-like lesions of terminal bronchioles, chronic sinusitis, and intense coughing with large amounts of sputum production.
Pulmonary hygiene, also referred to as pulmonary toilet, is a set of methods used to clear mucus and secretions from the airways. The word pulmonary refers to the lungs. The word toilet, related to the French toilette, refers to body care and hygiene; this root is used in words such as toiletry that also relate to cleansing.
Frédéric Chopin's disease and the reason for his premature death at age 39 were frequently debated for over 150 years. Although he was diagnosed with and treated for tuberculosis throughout his lifetime, a number of alternative diagnoses have been suggested since his death in 1849. A comprehensive review of the possible causes of Chopin's illness was published in 2011. A visual examination of Chopin's heart, for which permission was finally given in 2014, indicated the likely cause of death as pericarditis, caused by tuberculosis. This has been disputed by pathologists who say that a visual examination alone cannot confirm such a disease.
Physiotherapists treating patients following uncomplicated coronary artery bypass surgery surgery continue to use interventions such as deep breathing exercises that are not supported by best available evidence. Standardised guidelines may be required to better match clinical practice with current literature.
Airway clearance therapy is treatment that uses a number of airway clearance techniques to clear the respiratory airways of mucus and other secretions. Several respiratory diseases cause the normal mucociliary clearance mechanism to become impaired resulting in a build-up of mucus which obstructs breathing, and also affects the cough reflex. Mucus build-up can also cause infection, and inflammation, and repeated infections can result in damage to the airways, and the lung tissue.
