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Cystic Fibrosis: A Cure or A Treatment?

Journal of Cystic Fibrosis

❶From this search, we selected randomised controlled trials, high quality journal reviews and meta-analyses. Each child of parents who have faulty copies of the gene has a one in four chances inheriting the disease.

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What are the clinical features and when should the diagnosis be considered?
What is the cause of cystic fibrosis?

What is the cause of cystic fibrosis? Open in a separate window. Organ Manifestation of cystic fibrosis Treatment Comments Upper airway Polyps 18 Topical steroids; antibiotics; surgery if medical management fails Surgery may have medium term benefit, but recurrence common Sinusitis Topical steroids; antibiotics; surgery if medical management fails Most cases are asymptomatic changes seen on x ray films or computed tomograms almost universal: What are the clinical features and when should the diagnosis be considered?

How is cystic fibrosis diagnosed? Management Most patients in the UK and Europe receive care coordinated by a tertiary cystic fibrosis centre, which improves outcomes. Psychological issues Cystic fibrosis clearly poses a huge burden to patients and families in terms of the life shortening nature of the disease, the time consuming treatments prescribed, and the ongoing morbidity. Ongoing research Research is being conducted into gene therapy that aims at introducing a normal copy of CFTR into lung epithelial cells.

Because of this, the UK Cystic Fibrosis Gene Therapy Consortium, which has been formed in recent years to develop cystic fibrosis gene therapy for clinical benefit is focusing current efforts on a non-viral approach. New drugs to improve ion transport 22 and osmotic agents 23 to increase airway surface liquid are currently in phase II clinical trials, as are anti-inflammatory agents, mucolytics, and pseudomonas vaccines.

New methods for administering current agents, such as the development of dry powder formulations ease of administration and of liposomal preparations enhanced activity of antibiotics are being developed.

Standards of care for patients with cystic fibrosis: J Cyst Fibros ;4: Cystic fibrosis in the 21st century progress in respiratory research. Cystic Fibrosis Foundation www. Genetics Home Reference http: Association of Clinical Biochemistry. Guidelines for the performance of the sweat test for the investigation of cystic fibrosis in the UK. Cystic Fibrosis Mutation Database www.

Information on the UK newborn screening programme www. Cystic fibrosis mortality and survival in the UK: Eur Respir J ; Identification of the cystic fibrosis gene: Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease.

Pathophysiology and management of pulmonary infections in cystic fibrosis. Cystic fibrosis since The diagnosis of cystic fibrosis: J Pediatr ; Protocols for in vivo measurement of the ion transport defects in cystic fibrosis nasal epithelium. Eur Respir J ; 7: A survey of newborn screening for cystic fibrosis in Europe.

J Cyst Fibros ; 6: Recombinant human deoxyribonuclease for cystic fibrosis. Cochrane Database Syst Rev ; 3: A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med ; Smyth A, Walters S. Prophylactic anti-staphylococcal antibiotics for cystic fibrosis. Antibiotic strategies for eradicating Pseudomonas aeruginosa in people with cystic fibrosis. Cochrane Database Syst Rev ; 1: Macrolide antibiotics for cystic fibrosis. Cochrane Database Syst Rev ; 2: Oral non-steroidal anti-inflammatory drug therapy for cystic fibrosis.

International guidelines for the selection of lung transplant candidates: J Heart Lung Transplant ; Liver disease in cystic fibrosis. J Pediatr Gastroenterol Nutr ; 43 suppl 1: Nasal polyposis in children with cystic fibrosis: Ann Otol Rhinol Laryngol ; Onady GM, Stolfi A.

Insulin and oral agents for managing cystic fibrosis-related diabetes. Psychological interventions for cystic fibrosis. Adv Genet ; If you do not already have an account you will need to register here. Due to migration of article submission systems, please check the status of your submitted manuscript in the relevant system below:. Once production of your article has started, you can track the status of your article via Track Your Accepted Article.

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The most downloaded articles from Journal of Cystic Fibrosis in the last 90 days. CF can also lead to many digestive complications such as: Victims of Cystic Fibrosis are at a higher risk of having Osteoporosis because they cannot absorb vitamin D. They can also have an electrolyte imbalance because they have saltier sweat which upsets the balance of minerals in their blood. Cystic Fibrosis can cause problems in the reproductive system.

Cystic Fibrosis patients have many more sexual reproduction problems than a healthy human. In males with CF, they are likely to be infertile because of the tube connecting the testes and the vas deferens is missing or blocked with mucus. In women with CF, pregnancy can be a lot more difficult. Pregnancy can worsen the symptoms of Cystic Fibrosis. Women with CF may also be less fertile than women without CF.

Women with CF should always consult with a doctor to know all of the possible risks. In order to get CF, both partners must be a carrier because the CF gene is recessive. If someone is a carrier, they could pass it on to their own child. Cystic Fibrosis tends to run in families, since it is an inherited disease. It is also most common in Caucasians. Many tests may be performed before a person is ever diagnosed with Cystic Fibrosis. Many other tests must be completed before diagnosis.

A sweat test is necessary for confirmation of CF. If a sweat test is not completely accurate, genetic testing must be performed. The damage to the lungs and intestines can be monitored by imaging tests including: Lung function tests may also have to be performed. These function to measure the size of the lungs, the speed and capacity of air they can breathe in and out, and how well the lungs are delivering oxygen to the blood.

Sadly, there is no cure for Cystic Fibrosis. Antibiotics are used to treat and prevent lung infections. Mucus thinning drugs help to reduce the stickiness of mucus, improving lung function. A bronchodilator helps to relax the muscles and keep airways open.


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In a group of parents founded the Cystic Fibrosis Foundation, which later became the National CF research Foundation in as well. In ,b The foundation created the Research Development Program, which is a network of research centers at top universities.

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Cystic Fibrosis- Option 2 Justin Jang and Junior Suwannapeng Jasper High School Cystic fibrosis is an autosomal (not sex linked), hereditary disease caused by a mutation in the gene called the cystic fibrosis transmembrane conductance regulator gene, or the CFTR gene. This CFTR gene belongs to a group of genes called ABC (ATP-binding cassette). These are transport molecules for molecules such. The most downloaded articles from Journal of Cystic Fibrosis in the last 90 days.

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Cystic Fibrosis Research Paper. Cystic Fibrosis, a very serious inherited genetic disease, is also known as CF and sixty-five roses. This disease affects one in every 3, live births. It may first appear in a newborn, but can appear all the way up until a young adult. Diet Health & Disease Cystic Fibrosis Essay Background on cystic fibrosis: Cystic fibrosis (CF) is a chronic disease that affects organs such as the liver, lungs, pancreas, and intestines. It disrupts the body's salt balance, leaving too little salt and water on the outside of cells and causing the.