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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Log in via Email. A hypersensitivity response, known as allergic bronchopulmonary bronchiectazis, may ensue, leading to a cycle of bronchial inflammation, mucoid impaction, and bronchial obstruction that results in bronchiectasis. Back to Social Login. Therefore, nodal ciliary dysfunction can produce dextrocardia, situs inversus totalis, and situs ambiguus.

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

A Woman with Recurrent Sinusitis, Cough, and Bronchiectasis

We will not share your email with anyone. Many lung infections can result in the development of bronchiectasis, including those caused by Mycobacterium tuberculosis and Bordetella pertussis.

With the administration of these treatments at cystic fibrosis—specific treatment centers, median survival has increased from Cystic fibrosis is a recessive genetic disease that is caused by mutations in both alleles of the CFTR beonchiectasis, which encodes the cystic fibrosis transmembrane conductance regulator CFTR.


This is the target of action of the drugs lumacaftor and tezacaftor. The primary defect that is caused by the Phedel mutation is that the CFTR protein is synthesized but misfolded, which keeps it from reaching the cell surface.

Bronchiectasis is characterized by irreversible damage of the airways bronchiecasis results in dilatation. Create your account Back to Social Login. Mutations in CFTR disrupt sodium absorption, chloride secretion, and water transport, leading to the development of viscous mucus that adheres to the airway and impairs bacterial clearance.

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

There are close to recognized CFTR mutations, and each one confers a different degree of diminished chloride ion transport. Primary ciliary dyskinesia can also cause left—right asymmetry. What are some of the causes of bronchiectasis?

Bronchiectasis may develop in patients with a history of recurrent pneumonia, particularly those with chronic aspiration. The CFTR protein forms a chloride channel that is critical to efficient mucus transport. Primary ciliary dyskinesia is a congenital, autosomal recessive disorder that is characterized by immotile or dyskinetic cilia.


In some people, inhalation of Aspergillus fumigatus provokes a brisk allergic response that is characterized by eosinophilia and a high level bronchiecatsis IgE antibodies. Inthe qualifying mutations for the administration of ivacaftor were expanded to include DH. Describe features of primary ciliary dyskinesia. Patients with immunodeficiency syndromes are at high risk for the development of bronchiectasis.

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.

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The DH mutation is considered to be a partial-function mutation that results in diminished ion transport.