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Without treatment, some individuals may survive through late childhood or early adolescence. People with milder forms of the disorder usually live into adulthood, although they may have reduced life expectancy. Heart disease and airway obstruction are major causes of death in people with Maroteaux–Lamy syndrome. [2]
Medical treatment for restrictive lung disease is normally limited to supportive care since both the intrinsic and extrinsic causes can have irreversible effects on lung compliance. [10] The supportive therapies focus on maximizing pulmonary function and preserving activity tolerance through oxygen therapy, bronchodilators, inhaled beta ...
The disease is more common in males, [25] with the male to female ratio at 1.4–2:1 (or about 5 men to 3 women). [4] The average onset of the disease is around age 40, and two-thirds of those affected are non-smokers, although smoking is not believed to be a cause. [7] The presence of HLA-Bw54 increases the risk of diffuse panbronchiolitis 13. ...
Bronchiolitis obliterans (BO), also known as obliterative bronchiolitis, constrictive bronchiolitis and popcorn lung, is a disease that results in obstruction of the smallest airways of the lungs (bronchioles) due to inflammation. [1] [6] Symptoms include a dry cough, shortness of breath, wheezing and feeling tired. [1]
The disease newly occurs in about 12 per 100,000 people per year. [4] Those in their 60s and 70s are most commonly affected. [4] Males are affected more often than females. [4] Average life expectancy following diagnosis is about four years. [1]
Chronic obstructive pulmonary disease (COPD) is a type of progressive lung disease characterized by chronic respiratory symptoms and airflow limitation. [8] GOLD 2024 defined COPD as a heterogeneous lung condition characterized by chronic respiratory symptoms (dyspnea or shortness of breath, cough, sputum production or exacerbations) due to abnormalities of the airways (bronchitis ...
In a cohort of patients in the United Kingdom, 10 years after symptom onset, 55% of 77 patients were breathless walking on flat ground and 10% were housebound. [127] The average annual rate of decline in FEV1 and DLCO in 275 patients studied in a single pulmonary function laboratory at the NHLBI was 75 ± 9 mL, and 0.69 ± 0.07 mL/min/mm Hg ...
Variable: Disease can range from asymptomatic to life-threatening Tracheobronchomalacia ( TBM ) is a condition characterized by flaccidity of the tracheal support cartilage which leads to tracheal collapse. [ 1 ]