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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A Woman with Recurrent Sinusitis, Cough, and Bronchiectasis

The CFTR protein forms a chloride channel that is critical to efficient mucus transport. What are some of the causes of bronchiectasis? 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.

This type of mutation is the target of the drug ivacaftor, which restores partial ion transport. Request to Join has invited you to join this group.

The DH mutation is considered to be a partial-function mutation that results in diminished ion transport. 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 can also cause left—right asymmetry. Therefore, nodal ciliary dysfunction can produce dextrocardia, situs inversus totalis, and situs ambiguus.

This is the target of action of the drugs lumacaftor and tezacaftor. There are close to recognized CFTR mutations, and each one confers a different degree of diminished chloride ion transport. Show or Hide the password you are typing. In some people, inhalation of Aspergillus fumigatus provokes a brisk allergic response that is characterized by eosinophilia and a high level of IgE broncbiectasis.

Primary ciliary dyskinesia is a congenital, autosomal recessive disorder that is characterized by immotile or dyskinetic cilia.

Patients with immunodeficiency syndromes are at high risk for the development of bronchiectasis. We will not share your email with anyone. Mutations in CFTR disrupt sodium absorption, chloride secretion, and water transport, leading to the development of viscous mucus that adheres to the airway bejm impairs bacterial clearance. Bronchiectasis may develop in patients with a history of recurrent pneumonia, particularly those with chronic aspiration.

Describe features of primary ciliary dyskinesia. Back to Social Login.


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

Password must be at least 8 characters. Many lung infections can result in the development of bronchiectasis, including those bronchievtasis by Mycobacterium tuberculosis and Bordetella pertussis.

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. Inthe qualifying mutations for the administration of ivacaftor were expanded to include DH. Bronchiectasis is characterized by irreversible damage of the airways that results in dilatation.

With the administration of these treatments at cystic fibrosis—specific treatment brojchiectasis, median survival has increased from Create your account Back to Social Login. Log in via Email.

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.