What is cystic fibrosis

Cystic fibrosis - an inherited disease caused by a gene mutation and characterized by lesions of exocrine glands (pancreas, obstructive lung, intestinal, sweat, saliva, and others) with severe functional disorders of the digestive system and the respiratory system.

In cystic fibrosis because of changes (mutations) of a particular gene is disrupted structure and properties of the protein, known as transmembrane regulator cystic fibrosis, which does not perform its function, which is why the secret of exocrine glands dramatically thickens, becomes viscous secrets glands hardly stand, they linger in a place of education. Stagnation leads to shrinkage (atrophy) gland tissue and its gradual replacement by connective tissue - a process called fibrosis. At the same time the expanded cells of the connective tissue (fibroblasts) violated the metabolic processes.

Changes affecting mainly the pancreas, lungs and intestine. The pancreas is sealed because overgrown connective tissue, gland ducts take the form of small cysts. In the intestine atrophy (shrink) the mucous layer, the submucosal tissue swells, cancer of the intestine become impassable because of thick secretions, meconium (stool newborns) is mixed with thick mucus and can clog the intestinal lumen. Changes in the lungs characterized by expansion and viscous mucus blockage of the lumen of the small bronchi (the formation of the so-called bronchiectasis), and proliferation of connective tissue in the lungs.

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Symptoms of the disease are very diverse and depend on the degree of involvement in individual organs. Cystic fibrosis can occur with a primary lesion of various organs, as well as a mixed form - the most severe.

The defeat of the respiratory system appears, usually in the first weeks and months after birth persistent paroxysmal cough, shortness of breath, blue lips and fingers. When connecting infections there is inflammation of the bronchi and lungs.

Pancreatic insufficiency Diabetes and pancreas - the things you need to know   and intestines manifested in the fact that with good and even increased appetite child behind in weight and height. Kal such a child has a putrid smell. Breastfeeding helps digestion lipase, which is contained in mother's milk. After the transition to a normal diet feces becomes mushy, greasy, more abundant, going gray. There are unreasonable vomiting, sometimes - constipation Constipation - Watch out for food . Children are far behind in weight.

Early manifestation of cystic fibrosis can be expressed as meconium ileus, when the swelling increases Prevention and treatment of edema - it is important to understand the root cause   the intestinal wall and the intestine becomes blocked with mucus and meconium. The child inflates the abdomen, vomiting, and dehydration occurs. The condition may worsen perforation of the intestinal wall and inflammation of the peritoneum (peritonitis) - the inner membrane lining the abdominal cavity.

The most severe form of cystic fibrosis - mixed, which combines both lung (cough, shortness of breath, bronchial and lung infection) and gastrointestinal (eating disorder) symptoms.

The severity of cystic fibrosis depend on when identified early signs of the disease (the younger the child was, the harder it is for disease).

Diagnostics

Timely diagnosis of cystic fibrosis is important in terms of prognosis for a child's life. Diagnosis can be made in the presence of characteristic changes in the lungs, the backlog weight and height with a good appetite, the cases of similar diseases in close relatives and the positive results of tests for cystic fibrosis.

Most rezultativen cystic fibrosis sweat test, in which the research is conducted in the pot the amount of sodium and chlorine. The effectiveness of this test is 99%. The concentration of sodium and chloride ions in sweat healthy child should not exceed 45 mmol / l, but in cystic fibrosis it is much higher.

To confirm the diagnosis of cystic fibrosis is investigated baby feces, which revealed a large amount of neutral fat and undigested food.

Dietary food

The earlier the correct adequate treatment, the more likely that child survive. Treatment is aimed at the restoration of disorders of the respiratory and digestive systems, it must be carried out over a lifetime. In case of violation of breath trying to restore patency of the bronchi using thinning phlegm (mucolytics) funds in the form of aerosols (eg mukosolvina). Simultaneously, the treatment of broncho-pulmonary infections.

Treatment of changes in the gastrointestinal tract begins with the appointment of the diet increased caloric enough salt (child loses salt through sweat), with a lot of protein (meat, fish, eggs, dairy products) and reduced fat. But by limiting the absorption of some fat breaks down vitamins (A, E, D), so they must be administered by injection. Water-soluble (B, C) vitamins may be given in the form of tablets.

Be sure to be appointed as replacement therapy - drugs that contain digestive enzymes (mezim, Festal Festal - help for problems with stomach   and so on), the dose of which is chosen for the study of feces.

Cystic fibrosis - an inherited disease, it can not be avoided, but you can quickly identify and maintain the health of the child in a normal state.

  Galina Romanenko

Treatment

Some symptoms of hyperthyroidism (such as tremor of the limbs and heart palpitations) can be alleviated in a few hours with the help of medicines from the group of beta-blockers (these include, for example, propranolol and propranolol). These preparations limit exposure of thyroid hormones on some systems of the body, but do not reduce their secretion. Their method is prescribed for patients with temporary forms of hyperthyroidism (caused by thyroiditis or taking certain medications). In other cases it is necessary to take antithyroid drugs, or even resort to surgery.

Antithyroid drug

Preparations of this type are assigned to patients who have hyperthyroidism caused by disorders such as Graves' disease or toxic nodular goiter. The purpose of such drug therapy is to reduce the secretion of thyroid hormones.

The most frequently used are antithyroid drugs as propylthiouracil and methimazole - they both limit the production of T3 and T4. If the patient takes the drugs correctly, they are already in a few weeks will reduce the level of thyroid hormones in the blood, causing the symptoms of hyperthyroidism Hyperthyroidism: symptoms - how to determine what happens to you?   will become less pronounced.

Antithyroid drugs can cause side effects such as rash, itching and fever, but they are rare. In very rare cases, patients taking these drugs develop serious complications - inflammation of the liver or deficiency of white blood cells. You should immediately stop taking the drug and call your doctor if the following symptoms: A very high body temperature, the yellowish color of the skin or severe sore throat.

The main disadvantage of drug treatment is that hyperthyroidism resumed soon after the patient stops taking the drug. Therefore, in most cases it is more preferred are treatments allowing permanently restrict secretion of thyroid hormones Thyroid hormones: mechanism of action and physiological effects .

Treatment with radioactive iodine

This method of treatment of hyperthyroidism doctors recommend most. The basis of this method is the fact that thyroid cells - these are the only cells in the body, capable of absorbing iodine. When the body gets the radioactive iodine, the thyroid cells absorb him, too, and it hurts them or completely destroy. In other parts of the body when it has minimal impact.

Currently, the radioactive iodine may be administered orally, and the patient after administration should not stay in the hospital. The cells of the thyroid gland may continue to die within one or two months, but the radioactive material is completely eliminated from the body within a few days. The main part of the patients sufficient to successfully treat a single dose of radioactive iodine.

The only, but common side effect of treatment with radioactive iodine - a low level of thyroid hormones, or hypothyroidism. The problem is that that the dose of radioactive iodine, which is usually given to patients kills too many cells of the thyroid gland, and the remaining cells can not produce sufficient hormones.

The rest of this therapy is considered completely safe. There is no evidence that it can provoke the development of cancer, or to reduce a woman's chances of getting pregnant and having a healthy baby in the future.

Surgical intervention

Another method for treating hyperthyroidism - complete or partial removal of the thyroid gland. Surgery is rarely used, as most of the other treatments are more effective and safe, but sometimes surgery is the only option. Depending on the cause of hyperthyroidism, the surgeon may remove part of the thyroid gland, which is a node that produces excessive amounts of hormones, or the entire gland.

Before carrying out such operations require prolonged hospitalization, but now the patient is sent home just one day, and in certain cases - a few hours later (with partial removal of the thyroid gland). Length of stay in the hospital, of course, can vary depending on the patient's overall health.

Overall thyroidectomy - a safe procedure, but it may have unwanted effects. Associated with it a small risk of damage to structures located in the vicinity of the thyroid gland - for example, the larynx, or nerve fibers. This occurs in approximately 1% of cases. In addition, as treatment with radioactive iodine, thyroidectomy (and full and partial) can lead to hypothyroidism. In such a case is assigned to patients receiving levothyroxine, which fully compensates for the deficit of thyroid hormones Deficiency of thyroid hormone - revealed a previously unknown reason . If the dose is chosen correctly, it can be taken during the whole life without any side effects and complications.

A new way to treat hyperthyroidism

Patients with hyperthyroidism often suffer from severe tremors, irritability Irritability - you try to control my temper , Irritability and impaired concentration. All this hinders not only to work efficiently, but also to simply enjoy life. Fortunately, there are several ways to treat the disease, and recently opened another, and can be reasonably considered as a significant breakthrough in the treatment of hyperthyroidism to the effects of 50-60 years.

One of the most common causes of hyperthyroidism is Graves' disease - it occurs in about one in a thousand women of childbearing age. If untreated, Graves disease can lead to osteoporosis; moreover, it greatly increases the risk of developing serious stroke and heart problems.

A new way to treat hyperthyroidism is the arterial embolization of the thyroid gland. Experiments with this procedure began in 2002, in the course of the study conducted in China. Earlier hyperthyroidism treated with medicines, which did not give permanent results, radiotherapy, or - using the surgical removal of the entire thyroid gland, or part of it. The new method is much less invasive than surgery. During the procedure, a small incision in the groin area, through which the artery by means of X-rays are administered thin hollow tube. Therethrough introduced artificial emboli (typically spherical or cylindrical) which block the artery, leading to thyroid, restricting the flow of blood thereto.

The doctor who developed this type of treatment, specialized in embolization has for many years - he used it to treat tumors formed in the brain, neck and other parts of the body. However, only recently it emerged the idea of ​​using arterial embolization for the treatment of hyperthyroidism. The first such operation was successful, but it is not known when the embolization will also be available to patients with hyperthyroidism, as well as other treatments. The effectiveness of a new type of therapy has yet to be thoroughly explored and evaluated.

Treatment of hyperthyroidism may be associated with overweight and obesity

New research shows that successful treatment of hyperthyroidism may be associated with a significant risk of being overweight and even obesity. Physicians should keep this in mind and given to patients who are undergoing treatment for hyperthyroidism, detailed information on the risks and how to prevent unwanted weight gain. To understand how effective to prevent excess weight is a lifestyle change, more research is needed.

Hyperthyroidism - a very common endocrine disorder that sick, on average, two out of 100 women (all age groups), and two men from 1000. The study, whose task was to study the treatment of hyperthyroidism due and weight gain, took part in 1074 patients It diagnosed with hyperthyroidism.

In 727 patients (69.4% of subjects), weight increased by more than 5% over the 22 months of treatment. In 44.2% of patients who have pre-treatment had a normal body mass index (BMI) appeared overweight or obese, and 44.6% of subjects who have previously been overweight after treatment there was some degree of obesity. Most often gain weight, patients who have had severe hypothyroidism; those with pre-treatment showed a significant reduction in weight, and those with a course of treatment lasted much longer than average. Moreover, in men the probability of overweight and obesity was higher than in women. Apparently, the type of treatment a significant risk of body weight gain was not dependent.

At the same time, experts noted that the patients in any case can not delay treatment because of the fear of being overweight - the consequences of running hyperthyroidism can be much more serious.