Paediatric respiratory reviews
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Pulmonary surfactant reduces surface tension at the air-liquid interface in the alveolus, thereby maintaining lung volumes during the respiratory cycle. In premature newborn infants, the lack of surfactant causes atelectasis and respiratory failure, characteristic of respiratory of distress syndrome. ⋯ Mutations in the genes encoding these proteins cause lethal respiratory distress in newborn infants. This review discusses the clinical and pathological findings associated with these inherited disorders of alveolar homeostasis.
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Although aerosol therapy is most commonly used to treat asthma and COPD, there are a large number of aerosol medications now used or in development for other diseases. Mucoactive agents have long been available by aerosol, but now we have truly effective drugs to improve effective airway clearance including dornase alfa, hyperosmolar saline, and aerosol surfactant. ⋯ Insulin, recently approved in the US as aerosol therapy, and other peptides are systemically absorbed from the distal airway and alveolus. Aerosol gene transfer therapy to correct abnormalities associated with cystic fibrosis, primary ciliary dyskinesia and other airway diseases also holds great potential.
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Paediatr Respir Rev · Jan 2006
ReviewSpinal muscular atrophy type 1: what are the ethics and practicality of respiratory support?
Spinal Muscular Atrophy Type I (SMA I) is the most severe form of SMA. It presents in infancy and without treatment death occurs by 2 years. Treatments in use address respiratory and nutritional issues but even with aggressive treatment death is still likely in childhood. ⋯ However, pediatric respirologists must be willing and comfortable at presenting all treatment options, including the option of palliative care, to families and then supporting the family's choice. Whatever the chosen treatment regimen, decision making is difficult for families. Support and help must be provided from the time of presentation till death by a knowledgeable and compassionate team.