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Cystic Fibrosis (Mucoviscidosis)

An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION.
Also Known As:
Mucoviscidosis; Cystic Fibrosis of Pancreas; Fibrocystic Disease of Pancreas; Pancreatic Cystic Fibrosis; Pulmonary Cystic Fibrosis; Cystic Fibrosis, Pancreatic; Cystic Fibrosis, Pulmonary; Fibrosis, Cystic; Pancreas Fibrocystic Disease; Pancreas Fibrocystic Diseases
Networked: 21553 relevant articles (1049 outcomes, 2239 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Infections
2. Lung Diseases (Lung Disease)
3. Bronchiectasis
4. Asthma (Bronchial Asthma)
5. Chronic Obstructive Pulmonary Disease (COPD)

Experts

1. Ratjen, Felix: 135 articles (12/2022 - 02/2002)
2. Rowe, Steven M: 104 articles (01/2022 - 12/2006)
3. Elborn, J Stuart: 98 articles (04/2022 - 09/2003)
4. Konstan, Michael W: 97 articles (01/2022 - 02/2002)
5. Mall, Marcus A: 76 articles (12/2022 - 02/2009)
6. Amaral, Margarida D: 76 articles (03/2022 - 09/2002)
7. Colombo, Carla: 63 articles (07/2022 - 03/2003)
8. Sermet-Gaudelus, Isabelle: 62 articles (12/2022 - 01/2002)
9. Davies, Jane C: 60 articles (12/2022 - 09/2005)
10. Hayes, Don: 60 articles (01/2022 - 05/2008)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Cystic Fibrosis:
1. Anti-Bacterial Agents (Antibiotics)IBA
2. Tobramycin (Nebcin)FDA LinkGeneric
3. ivacaftorIBA
4. ivacaftor drug combination tezacaftorIBA
5. Cystic Fibrosis Transmembrane Conductance Regulator (Protein, CFTR)IBA
6. elexacaftorIBA
7. dornase alfa (Pulmozyme)FDA Link
8. ChloridesIBA
9. Azithromycin (Zithromax)FDA LinkGeneric
10. Proteins (Proteins, Gene)FDA Link

Therapies and Procedures

1. Therapeutics
2. Lung Transplantation
3. Enzyme Replacement Therapy
4. Nebulizers and Vaporizers (Inhaler)
5. Microspheres (Microsphere)