Difference between revisions of "Cystic fibrosis"

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'''Cystic fibrosis''' (CF) is a hereditary [[disease]] whose symptoms usually appear shortly after birth. They include faulty digestion, breathing difficulties and respiratory infections due to mucus accumulation, and excessive loss of [[salt]] in sweat. These symptoms arise due to a genetic mutation in a membrane bound chloride ion channel. In the past, cystic fibrosis was almost always fatal in childhood, but treatment is now so improved that patients commonly live to their 20s and beyond.
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'''Cystic fibrosis''' (CF) is a hereditary [[disease]] whose symptoms usually appear shortly after birth. They include faulty digestion, breathing difficulties, respiratory infections due to mucus accumulation, and excessive loss of [[salt]] in sweat. These symptoms arise due to a genetic mutation in a membrane bound chloride ion channel. In the past, cystic fibrosis was almost always fatal in childhood, but treatment is now so improved that patients commonly live to their 20s and beyond. The average person with Cystic fibrosis lives to age 37.<ref>http://www.cff.org/AboutCF/</ref>
  
Cystic fibrosis is an inherited disease of your mucus and sweat glands. It affects mostly your lungs, pancreas, liver, intestines, sinuses, and sex organs.
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==Effects and Symptoms==
  
Normally, mucus is watery. It keeps the linings of certain organs moist and prevents them from drying out or getting infected. But in CF, an abnormal gene causes mucus to become thick and sticky.
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Normally, mucus is watery. It keeps the linings of certain organs moist and prevents them from drying out or getting infected. But in CF, an abnormal gene causes mucus to become thick and sticky.  The abnormal mucus builds up in the patient's lungs and blocks the airways. This makes it easy for bacteria to grow and leads to repeated life-threatening lung infections. Over time, these infections can cause serious damage to the lungs.
  
The mucus builds up in your lungs and blocks the airways. This makes it easy for bacteria to grow and leads to repeated serious lung infections. Over time, these infections can cause serious damage to your lungs.
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The mucus can also block tubes, or ducts, in the pancreas. As a result, the digestive enzymes produced by the pancreas cannot reach the small intestine. These enzymes help break down food, allowing this body to absorb it.   Without these enzymes, the intestines cannot fully absorb fats and proteins.
 
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The thick, sticky mucus can also block tubes, or ducts, in your pancreas. As a result, digestive enzymes that are produced by your pancreas cannot reach your small intestine. These enzymes help break down the food that you eat. Without them, your intestines cannot absorb fats and proteins fully.
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As a result:
 
As a result:
* Nutrients leave your body unused, and you can become malnourished.
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* Nutrients leave the body unused, leading to malnourishment.
* Your stools become bulky.
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* The stools become bulky.
* You may not get enough vitamins A, D, E, and K.
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* The body may lack vitamins A, D, E, and K.  
* You may have intestinal gas, a swollen belly, and pain or discomfort.
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* The patient may suffer from intestinal gas, a swollen belly, and pain or discomfort.
  
The abnormal gene also causes your sweat to become extremely salty. As a result, when you perspire, your body loses large amounts of salt. This can upset the balance of minerals in your blood. The imbalance may cause you to have a heat emergency.
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Due to the body's inability to fully digest food, a key symptom of cystic fibrosis is difficulty growing and/or gaining weight despite a healthy appetite.
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The abnormal gene which causes cystic fibrosis also causes the sweat to become extremely salty. As a result, when a person with cystic fibrosis perspires, his or her body loses large amounts of salt. This can upset the balance of minerals in the blood. The imbalance may cause a heat emergency.
  
 
CF can also cause infertility (mostly in men).
 
CF can also cause infertility (mostly in men).
  
The symptoms and severity of CF vary from person to person. Some people with CF have serious lung and digestive problems. Other people have more mild disease that doesn't show up until they are adolescents or young adults.
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The symptoms and severity of CF vary from person to person. Some people with CF have serious lung and digestive problems. Other people have more mild versions of the disease that doesn't show up until they are adolescents or young adults.
  
 
Respiratory failure is the most common cause of death in people with CF.
 
Respiratory failure is the most common cause of death in people with CF.
  
Until the 1980s, most deaths from CF occurred in children and teenagers. Today, with improved treatments, people with CF live, on average, to be more than 35 years old. Research continues to look for:
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==Diagnosis and Treatment==
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Until the 1980s, most deaths from CF occurred in children and teenagers. Today, with improved treatments, people with CF live, on average, to be more than 35 years old. 40% of people with CF are over age 18.<ref>http://www.cff.org/AboutCF/</ref>
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Around 1,000 new cases of CF are diagnosed each year, and 70% of those diagnosed are age two or younger.<ref>http://www.cff.org/AboutCF/</ref>
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Research continues to look for:
  
 
* Better treatments
 
* Better treatments
 
* A cure
 
* A cure
  
== Sources ==
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== References ==
 +
<references/>
  
 
* [http://www.nhlbi.nih.gov/health/dci/Index/c.html NIH Glossary]
 
* [http://www.nhlbi.nih.gov/health/dci/Index/c.html NIH Glossary]
 
[[Category:Medical Terms]]
 
[[Category:Medical Terms]]
 
[[Category:Diseases]]
 
[[Category:Diseases]]

Revision as of 09:12, 20 July 2009

Cystic fibrosis (CF) is a hereditary disease whose symptoms usually appear shortly after birth. They include faulty digestion, breathing difficulties, respiratory infections due to mucus accumulation, and excessive loss of salt in sweat. These symptoms arise due to a genetic mutation in a membrane bound chloride ion channel. In the past, cystic fibrosis was almost always fatal in childhood, but treatment is now so improved that patients commonly live to their 20s and beyond. The average person with Cystic fibrosis lives to age 37.[1]

Effects and Symptoms

Normally, mucus is watery. It keeps the linings of certain organs moist and prevents them from drying out or getting infected. But in CF, an abnormal gene causes mucus to become thick and sticky. The abnormal mucus builds up in the patient's lungs and blocks the airways. This makes it easy for bacteria to grow and leads to repeated life-threatening lung infections. Over time, these infections can cause serious damage to the lungs.

The mucus can also block tubes, or ducts, in the pancreas. As a result, the digestive enzymes produced by the pancreas cannot reach the small intestine. These enzymes help break down food, allowing this body to absorb it. Without these enzymes, the intestines cannot fully absorb fats and proteins.

As a result:

  • Nutrients leave the body unused, leading to malnourishment.
  • The stools become bulky.
  • The body may lack vitamins A, D, E, and K.
  • The patient may suffer from intestinal gas, a swollen belly, and pain or discomfort.

Due to the body's inability to fully digest food, a key symptom of cystic fibrosis is difficulty growing and/or gaining weight despite a healthy appetite.

The abnormal gene which causes cystic fibrosis also causes the sweat to become extremely salty. As a result, when a person with cystic fibrosis perspires, his or her body loses large amounts of salt. This can upset the balance of minerals in the blood. The imbalance may cause a heat emergency.

CF can also cause infertility (mostly in men).

The symptoms and severity of CF vary from person to person. Some people with CF have serious lung and digestive problems. Other people have more mild versions of the disease that doesn't show up until they are adolescents or young adults.

Respiratory failure is the most common cause of death in people with CF.

Diagnosis and Treatment

Until the 1980s, most deaths from CF occurred in children and teenagers. Today, with improved treatments, people with CF live, on average, to be more than 35 years old. 40% of people with CF are over age 18.[2]

Around 1,000 new cases of CF are diagnosed each year, and 70% of those diagnosed are age two or younger.[3]

Research continues to look for:

  • Better treatments
  • A cure

References

  1. http://www.cff.org/AboutCF/
  2. http://www.cff.org/AboutCF/
  3. http://www.cff.org/AboutCF/