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Cystic fibrosis: symptoms and treatment in children

When the disease cystic fibrosis symptoms in children are manifested in stagnant process in the stomach, poor assimilation of food, constant cough and bad patency of sputum, the growth and development happen slowly. This disease has another name – testaferros pancreas. This disease is hereditary and is manifested in the secretion of glands in the digestive and airway viscous secretion, resulting in blockage of the ducts. With age, mucus becomes larger, it begins to affect other organs.

problema mukoviscidoza u detej

Disease, as practice shows, is rare. Per 1,000 children may be one case of cystic fibrosis. This pathology is considered incurable condition which worsens over time. People with this diagnosis live until 35-40 years. Modern medicine does not stand still, new methods of treatment allow the patient to extend life by slowing the process of mucus formation.

Causes of cystic fibrosis

The main reason for testaferrata pancreatic cancer is a mutation of the gene transmembrane regulator cystic fibrosis. It is due to him through the ducts of the membrane which lines the cells of the exocrine glands, are electrolytes, especially chlorine. When mutations during the disease violated the integrity and structure of the transmembrane protein. In the end, the secret, which is secreted by glands becomes overly thick and viscous.

Symptoms of cystic fibrosis in newborns

apatiya i nepodvizhnost - simptomy mukoviscidoza

If after birth you find the following symptoms, it is possible that this cystic fibrosis:

  1. Intestinal obstruction, ileus or mechanically. The intestines cannot absorb water, sodium and chloride properly, and the obstruction of meconium. The stomach swells, starts vomiting bile, the skin is dry and pale. The baby is motionless and apathetic, there are signs of self-poisoning stool.
  2. Yellowness of the skin, it appears during makanalina ileus or as a separate symptom of the disease. This occurs from the fact that the bile becomes viscous and thick and bad passes through the bile duct.
  3. On the face, underarms and other areas of the skin appear salt crystals, from which it becomes salty taste.

Symptoms of cystic fibrosis older children

In this disease, there are three basic forms of flow:

  1. Combined form – pulmonary-intestinal, which is found in 80% of patients.
  2. Bronchopulmonary form, the percentage of affected children is 15-20%.
  3. The intestinal formis rarer and was observed only in 5% of patients with cystic fibrosis.

At the mixed form of the child after a year having trouble breathing. In the bronchi, small and medium-sized departments, the accumulation of thick secretions, which is with difficulty separated.

A cough Intrusive and painful nature, when the sputum is very difficult to separate. At this point, the accession of a bacterial infection causing prolonged chronic bronchitis and pneumonia. The body temperature rises to 38-39 degrees, with shortness of breath and coughing spells become stronger.

kashel pri mukoviscidoze

Intestinal form of cystic fibrosis symptoms of the disease begin to manifest especially after the baby was transferred to an additional lure or changed breastfeeding for artificial mixtures. Food is broken down with labour, the observed intestinal putrefactive processes, the accumulation of gases. Often the child defecates. Pain in the abdomen cramping in nature – this is due to increased gases.

A newborn child appetite is not suffering or is often upgraded, but over time, in disorders of the digestive tract of growing malnutrition and polyhypovitaminosis. In any form of cystic fibrosis children have the syndrome of "salty baby" when the salt crystals are on the skin.

The screening of newborns for cystic fibrosis

To accurate diagnosis, should undergo some laboratory tests. Currently, early diagnosis of cystic fibrosis modern medicine has implemented newborn screening for detection of congenital and hereditary pathologies.

Still in the hospital, the child takes a blood sample that is sent to the study. This is done on the 4th day of birth or on day 7 in preterm infants. If you suspect testaferros doctors notify the parents and recommend the delivery of additional analyzes.

The treatment of the disease

The child's parents should remember that the child's treatment should be initiated as early as possible - then disappear the threat to his life.

osmotr rebenka vrachom

Therapy is aimed at facilitating the work of the respiratory and digestive system. An integrated approach provides life-long treatment for cystic fibrosis.

The pulmonary form involves performing procedures aimed at restoring bronchial conductivity, liquefaction of thick sputum and anti-inflammation. Your doctor may recommend the admission of mucolytics in the form of inhalations and aerosols, which are taken throughout life.Intestinal obstruction appointed by enzymes.

A good result at the time of exacerbations give physiotherapy – postural drainage and vibratory massage of the chest. If there is a joining of a bacterial infection, can be antibacterial therapy. Since suffering and heart system, the therapy of metabolic drugs, and glycosides glucocorticoids.

Intestinal form of the disease is recommended a special diet, rich in protein. It is found mostly in meat, cheese, fish, eggs. Fats and carbohydrates are used in limited quantities. Do not use rough fiber, and if the child has a lactase deficiency, do not receiving cow's milk.

It is very important to a child with this diagnosis to drink plenty of fluids, foods you need to constantly add some salt, take vitamins.

If the child has lost sensation of pain, normal stools and digestion, as well as in the stool is not observed impurities of bile, it speaks to the effectiveness of the treatment.

In severe cases, can be appointed surgery. The indication for surgery may be suspected peritonitis, a severe intestinal obstruction, bleeding from the lungs.

Children with this diagnosis are registered in the local clinic, constantly observed by a pediatrician and pulmonologist. Parents interviewed about the proper care of such children, they teach techniques of vibratory massage. The issue of vaccination is handled individually.

The child with this diagnosis do not visit kindergartens. The expediency of attending school depends on the state of the little patient. If the parents together with the doctor decided that the child can attend school in the week allocated an extra day for rest and a full exemption from examination. If the disease occurs in mild form, it is recommended that a trip to specialized sanatoria. Children with cf are registered up to 15 years, after which their card is transferred to the therapist.