Original Article from The New England Journal of Medicine — Bronchiectasis: Treatment and Prevention. Original Article from The New England Journal of Medicine — The Pathogenesis of Bronchiectasis. 4 days ago Read the latest NEJM Case Records of the Massachussetts General Bronchiectasis may develop in patients with a history of recurrent.

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Bronchiectasis is characterized by irreversible damage of the airways that results in dilatation.

A Woman with Recurrent Sinusitis, Cough, and Bronchiectasis

We will not share your email with anyone. Inthe brojchiectasis mutations for the administration of ivacaftor were expanded to include DH. This is the target of action of the drugs lumacaftor and tezacaftor. What are some of the causes of bronchiectasis? Describe features of primary ciliary dyskinesia.

The primary bronchiecctasis that is caused by the Phedel mutation is that the CFTR protein is synthesized but misfolded, which keeps it from reaching beonchiectasis cell surface. A hypersensitivity response, known as allergic bronchopulmonary aspergillosis, may ensue, leading to a cycle of bronchial inflammation, mucoid impaction, and bronchial obstruction that results in bronchiectasis.


Create your account Back to Social Login. The DH mutation is considered to be a partial-function mutation that results in diminished ion transport.

This type of mutation is the target of the drug ivacaftor, which restores partial ion transport.

Therefore, nodal ciliary dysfunction can produce dextrocardia, situs inversus totalis, and situs ambiguus. Password must be at least 8 characters. With the administration of these treatments at cystic fibrosis—specific treatment centers, median survival has increased from The CFTR protein forms a chloride channel that is critical to efficient mucus transport. Bronchiectasis may develop in patients with a history of recurrent pneumonia, particularly those with chronic aspiration.

There are close to recognized CFTR mutations, and each one confers a different degree of diminished chloride bronchievtasis transport. In some people, inhalation of Aspergillus fumigatus provokes a brisk allergic response that is characterized by eosinophilia and a high level of IgE antibodies.

Patients with immunodeficiency syndromes are at high risk for the development of bronchiectasis. Mutations in CFTR disrupt sodium absorption, chloride secretion, and water transport, leading to the development of viscous mucus that adheres to the airway and bronchiectaais bacterial clearance. Back to Social Login. Many lung infections can result in the development of bronchiectasis, including those caused by Mycobacterium tuberculosis and Bordetella pertussis.


A Woman with Recurrent Sinusitis, Cough, and Bronchiectasis | NEJM Resident

Show or Hide the password you are beonchiectasis. Log in via Email. Primary ciliary dyskinesia can also cause left—right asymmetry. In addition to impaired airway clearance, fertility problems can arise in males as a result of impaired spermatozoa motility and in females as a result of impaired ciliary function in the oviduct.

Cystic fibrosis is a recessive genetic disease that is caused by mutations in both alleles of the CFTR gene, which encodes the cystic fibrosis transmembrane conductance regulator CFTR. Primary ciliary dyskinesia is a congenital, autosomal recessive disorder that is characterized by immotile or dyskinetic cilia.

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