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.

Author: Faezil Golkis
Country: Antigua & Barbuda
Language: English (Spanish)
Genre: Science
Published (Last): 26 March 2009
Pages: 144
PDF File Size: 13.17 Mb
ePub File Size: 6.91 Mb
ISBN: 420-7-66756-744-2
Downloads: 5611
Price: Free* [*Free Regsitration Required]
Uploader: Meztizshura

Bronchiectasis may develop in patients with a history of recurrent pneumonia, particularly those with chronic aspiration.

Primary ciliary dyskinesia can also cause left—right asymmetry. Patients with immunodeficiency syndromes are at high risk for the development of bronchiectasis.

A Woman with Recurrent Sinusitis, Cough, and Bronchiectasis

Many lung infections can result in the development of bronchiectasis, including those caused by Mycobacterium tuberculosis and Bordetella pertussis. Inthe qualifying mutations for the administration of ivacaftor were expanded to include DH. Back to Social Login.

The primary defect that is caused by the Phedel mutation bronchjectasis 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. Bronchiectasis is characterized by irreversible damage of the airways that results in dilatation.


Log in via Email.

Therefore, nodal ciliary dysfunction can produce dextrocardia, situs inversus totalis, and situs ambiguus. There are close to recognized CFTR mutations, and each one confers a different degree broncyiectasis diminished chloride ion transport.

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. Password must be at least 8 characters.

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

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. We will not share your email with anyone.

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

Cystic fibrosis is a recessive genetic disease that is caused by mutations in bronchiextasis alleles of the CFTR gene, which encodes the cystic fibrosis transmembrane conductance regulator CFTR. In some people, inhalation of Aspergillus fumigatus provokes a brisk allergic response that is characterized by eosinophilia and a high level of IgE antibodies.


With the administration of these treatments at cystic fibrosis—specific treatment centers, median survival has increased from Show or Hide the password you are typing. This is the target of action of the drugs lumacaftor and tezacaftor.

Describe features of primary ciliary dyskinesia.

What are some of the causes of bronchiectasis? 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. Bronchiectaasis CFTR protein forms a chloride channel that is critical to efficient mucus transport. Create your account Back to Social Login.