FIBROSIS QUÍSTICA

	F
	FIBROSIS QUÍSTICA
	*FIBROSIS QUÍSTICA*

FIBROSIS QUÍSTICA

Quística Fibrosis

A hereditary disease (autosómica recesiva) of most frequent (1:2.500 been born alive) in the white populations, characterized by the excessive espesamiento of the bronchial and pancreáticas secrecciones.

Prognosis

Incurable, exquisite a clinical handling and the possibility of pulmonary transplant have increased of important form the life expectancy of these patients.

Complications

So frequent that it is almost an aspect more of the disease is the colonization of tracto respiratory by Pseudomonas Aeruginosa, bacterium that forms films in the bronchi of these patients who practically make their elimination impossible. In fact the analysis of the genome of this organism was financed by the Foundation of Fibrosis American Quística.

Factors of risk

It is a recesiva autosómica disease, reason why both parents must be carrying of the defective gene, and in that case the probability of having a son with quística Fibrosis is of 25 %.

The women with quística Fibrosis can have children, who can suffer it (if the father is carrying) in 50 % of the cases, and transmit it in the rest.

Prevention

It does not have prevention.

The genetic advice can be important to avoid new cases.

Diagnosis and treatment

DIAGNOSIS:

The diagnosis is made by means of the study of the sweat, that presents/displays an abnormal amount of chlorine (although in until 2 % of the cases this test it is normal) in a patient with respiratory problems.

Other tests that can be necessary are x-rays of thorax, bones and abdomen, and analysis of blood.

TREATMENT:

Intense studies with genetic therapies are being made to resolve this disease, at least in their respiratory facet.

At the moment it consists of respiratory fisioterapia and rehidratación. Also good results with the lung transplant have communicated.

General measures

Correct hidratación.

Medication

Pancreáticos enzymes by oral route.

Precocious detection and treatment of the respiratory reagudizaciones with antibiotics.

Vitamin injections To, D and K.

Activity

It does not have porqué to restrict itself outside the most active periods, although the dehydration will have to be avoided.

Diet

Diet with abundant water and proteins and fat poor man.

Warn its medico if

It presents/displays symptoms of reagudización or intestinal malabsorción

Causes

The quística Fibrosis is produced by a defect in the gene that codifies a secretory chlorine protein, reason why this does not work.

Signs and symptoms

In most of the cases the disease it appears in the first years of life. The affected organs more are the lungs and the páncreas, although all the secretory glands of the organism are affected. Respiratory symptoms:

The form of more frequent presentation is cough than he becomes constant, with secrección of greenish and very viscous snot.

The evolution is by buds, appearing episodes of important worsening that yield with the antibiotic treatment until the next time.

Little by little the pulmonary function gets worse, which leads the respiratory insufficiency.

Digestive symptoms:

Sometimes a snot retention takes place after the childbirth (meconial Ileo), that causes lee retention, abdominal distension and vomits.

In greater children a similar obstruction can take place.

The more typical digestive characteristic is the pancreática Insufficiency, which causes the bad digestion of fats and proteins:

Abundant lees

malolientes

frequent

Hipovitaminosis A (Xeroftálmia), D (raquitismo) and K (coagulation problems), when being absorbed these vitamins with fats.

The Mellitus Diabetes is not frequent in spite of the affectation of the páncreas exocrino.

The puberty is delayed, and the children present/display azoospermia (absence of spermatozoa in sémen). The women present/display sterility in near 20 % of the cases, although if they are pregnant later they give the chest normally.

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