Cystic fibrosis is a multi-organ disease best managed in a multidisciplinary setting in conjunction with a specialist centre for Cystic Fibrosis, with treatment tailored to the individual. Of the 22,301 patients with CF in the 2000 Cystic Fibrosis Foundation Patient Registry Annual Data Report, 8,637 (38.7%) were ≥ 18 years of age.This represents a dramatic increase in the number of adults over the past 3 decades, up from about 700 (10% of all CF patients) in 1970. We think that although this dissease has no cure it can be controlled with the specialist centre for Cystic Fibrosis for a good quality of life.
KEY WORDS
· Cystic fibrosis: autosomal recessive genetic disorder affecting most critically the lungs, and also the pancreas, liver, and intestine.
· Autosome: is a chromosome that is not an allosome.
· Genetic disorder: is an illness caused by abnormalities in genes or chromosomes, especially a condition that is present from before birth. Most genetic disorders are quite rare and affect one person in every several thousands or millions.
· Allosome is a sex chromosome that differs from an ordinary autosome in form, size, or behavior.
INTRODUTION
Cystic fibrosis is a life-threatening lung disorder That you cause severe damage and nutritional deficiencies. An inherited condition, cystic fibrosis That Affects the cells produce mucus, sweat and digestive juices. Normally, These are thin and slippery Secretions, But in cystic fibrosis, a defective gene you cause the Secretions to become sticky and thick. Instead of acting as a lubricant, the Secretions plug up tubes, ducts and passageways you, especially in the pancreas and lungs.Cystic fibrosis Often happens in MOST white people of northern European ancestry, Occurring in about 1 out of 3.000 live births. In the past, MOST People with cystic fibrosis died in Their teens. Improved screening and treatments now allow Many People with cystic fibrosis to live Into Their 50s or longer events.
OBJETIVES:
In order to understand this disease will get a diagnosis based on symptoms and consequences involved to suffer for so get early treatment and thus assess the quality of life of patients who have it.In cystic fibrosis, a defective gene alters a protein normally That Regulates the movement of salt (sodium chloride) in and out of cells. This results in thick, sticky Secretions in the respiratory and digestive tracts, as well as in the reproductive systemThe Affected Gene Which is inherited from a child's parents, is a recessive gene. With recessive genes, children need to inherit two copies of the gene, one from Each parent, in order to Have the disease
METHODOLOGY:
Cystic Fibrosis Symptoms and signs can Vary from child to child, Depending on the severity of the disease. Even in the Same child, or Improve Symptoms may worsen as time passes. In Some children, Symptoms begin DURING infancy. Other people may not begin experiencing adolescence or adulthood Until Symptoms.Respiratory Symptoms and signs The thick and sticky mucus clogs Associated with cystic fibrosis That the tubes carry air in and out of your lungs. This can cause:· Persistent cough· Wheezing· Repeated lung Infections· Repeated Sinus Infections
RESULTS:
According to the testimony of a patient with cystic fibrosis respiratory tract. We observe that leads a stable life under treatment, but these are neverthelessessential instability in the disease is because it needs much more careful not only of the treatments.
DISCUSSION:We conclude that it is an incurable disease that based on the case is not easy to treat and is difficult to lead a normal life because you become dependent on drugsin addition to other factors that influence health.
BIBLIOGRAPHY:
- What is cystic fibrosis? National Heart Lung and Blood Institute. http://www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_all.html. Accessed April 17 2012
- Simon RH. Cystic fibrosis: Overview of the treatment of lung disease. http://www.uptodate.com/home/index.html. Accessed April 17 2012
- Mayo Clinic; Cystic Fibrosis http://www.mayoclinic.com/health/cystic-fibrosis/DS00287 Accessed April 17 2012
- Patient, trusted medical information and support; Cystic Fibrosis http://www.patient.co.uk/doctor/Cystic-Fibrosis.htm Accessed April 17 2012
- James R. Yankaskas, MD†, Bruce C. Marshall, MD†, Beth Sufian, JD, Richard H. Simon, MD, and David Rodman, MD; Cystic Fibrosis Adult Care http://chestjournal.chestpubs.org/content/125/1_suppl/1S.long Accessed April 17 2012
- World Health Organization; Genes and human disease http://www.who.int/genomics/public/geneticdiseases/en/index2.html#CF Accessed April 17 2012
