Cystic Fibrosis

Disease information

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Cystic Fibrosis
Cystic Fibrosis.

An inherited disease (autosomal recessive) of the most frequent (1: 2. 500 live births) in white populations, characterized by excessive thickening of the bronchial and pancreatic secrecciones.


Incurable, a clinical management exquisite and the possibility of lung transplants have increased significantly the life expectancy of these patients.


So frequent that it is almost an aspect of the disease is the respiratory tract colonization by Pseudomonas aeruginosa, a bacterium that form films on the bronchi of these patients that make it almost impossible to eliminate. In fact the analysis of the genome of this organism was funded by the Cystic Fibrosis Foundation of America.

Risk Factors.

It is an autosomal recessive, so that both parents must be carriers of the defective gene, and in this case the probability of having a child with cystic fibrosis is 25%.

Women with cystic fibrosis can have children, who may suffer (if the parent is a carrier) in 50% of cases, and pass it on the rest.


It has no prevention.

Genetic counseling may be important to prevent new cases.

Diagnosis and Treatment.


The diagnosis is made through the study of sweat, which presents an abnormal amount of chlorine (although up to 2% of the cases this test is normal) in a patient with respiratory problems.

Other evidence that may be needed are chest X - rays, bone and abdomen, and blood tests.


Intensive studies are being conducted with genetic therapies to overcome this disease, at least in its facet of breath.

Today is chest physiotherapy and rehydration. Have also reported good results with lung transplantation.

General measures.

Proper hydration.


Pancreatic enzymes orally.

Detection and treatment of respiratory flare - ups with antibiotics.

Injections of vitamins A, D and K.


Need not be restricted outside the busiest periods, while carefully avoiding dehydration.


Diet with plenty of water and protein and low in fat.

Advise your doctor if.

Or symptoms of acute intestinal malabsorption.


Cystic Fibrosis is caused by a defect in the gene that encodes a protein secretory chlorine, so this does not work.

Signs and symptoms.

In most cases, the disease appears early in life. The organs most affected are the lungs and pancreas, although it affects all secretory glands of the body. Respiratory symptoms:

The manner of presentation is more frequent cough that becomes constant, with secrección of green and very viscous mucus.

The trend is for outbreaks, appearing episodes of significant deterioration to cede to the antibiotic treatment until the next time.

Little by little the worse lung function, which leads to respiratory failure.

Digestive symptoms:

Sometimes it occurs after giving birth to a retention of mucus (Ileo meconium) which causes retention of stool, abdominal distension and vomiting.

In older children may be a similar obstruction.

The feature is the most typical digestive pancreatic insufficiency, which leads to poor digestion of fats and proteins:

Abundant feces.



Vitamin A deficiency (xerophthalmia) D (rickets) and K (problems with Coagulation) these vitamins to be absorbed with fat.

It is not often Diabetes Mellitus despite the involvement of the exocrine pancreas.

Puberty is delayed, and the children had azoospermia (no sperm in the semen) Infertility in women present about 20% of cases, even if they become pregnant after breast normally.

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