Cystic fibrosis – what is it? Symptoms, treatmentand prevention in adults

Content:

  • What is cystic fibrosis?
  • Forms of cystic fibrosis disease
  • Pulmonary
  • Intestinal form
  • Mixed type
  • Atypical form
  • Stages of the disease cystic fibrosis
  • Possible complications
  • Diagnosis of cystic fibrosis
  • Cystic fibrosis treatment
  • Drug treatment
  • Cystic fibrosis diet
  • Massage and exercise therapy
  • Lung transplant
  • Video
  • Forecast

Severe genetic disease – cystic fibrosis. In adults
symptoms appear on the part of the respiratory system and often
taken by the patient for signs of pneumonia or bronchitis. Through
a few years, the patient affects almost all internal organs,
it is impossible to stop the process, the patient dies from a heart or
pulmonary insufficiency.

What is cystic fibrosis?

Cystic fibrosis refers to systemic hereditary diseases.
What it is? With this pathology, a gene mutates,
which is called the “transmembrane regulator of cystic fibrosis”. This
a gene is a protein responsible for transporting
chlorine ions across the cell membrane. Protein is present in the epithelium.
lungs, intestines, pancreas. If the gene mutates, then
human cystic fibrosis develops.

This disease is common (1 case per 10,000
newborns), so doctors pay great attention to the search
effective medication against her. Pathology has hereditary
nature If one parent is the carrier of the mutated gene,
then the baby will be born healthy. The disease manifests itself in the case of
carriers of broken genes by both parents.


Due to the impaired structure and functions of the protein, the patient is slow
evacuation of mucous secretions, as a result, the secret thickens,
changes its composition. This leads to mucostasis – the stagnation of mucus in
respiratory, digestive and endocrine glands. The result
it becomes an atrophy of glandular tissue that causes the clinical
picture of the disease.

Источник: https://ru.wikipedia.org/wiki/Муковисцидоз

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Forms of cystic fibrosis disease

In medical practice, adults have the following forms
cystic fibrosis:

  1. With damage to the respiratory system (respiratory, or
    pulmonary).
  2. With intestinal lesions (intestinal).
  3. Mixed
  4. Atypical course.

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Pulmonary

Due to thickening of the secretion mucostase develops in the lungs. it
causes bilateral severe pneumonia with abscesses. With
joining a bacterial infection begins purulent process.
Through несколько лет течения болезни у пациента возникает
chronic sinusitis, tonsillitis, adenoids, nasal polyps.


Withзнаки легочного поражения:

  1. Pale skin with a bluish tinge.
  2. Chest augmentation (rounded sternum).
  3. Thickening of the phalanges of the fingers.
  4. Long agonizing cough with sputum.

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Подробная пошаговая инструкцияto content ↑

Intestinal form

Patient’s impaired secretory failure
intestinal peristalsis, which leads to the stagnation of feces and
development of the putrefactive process. As a result, the patient’s body
does not absorb proteins and vitamins, so it starts
hypovitaminosis.

The main symptoms of intestinal forms:

  1. Frequent defecation, increase in daily fecal volume due to
    digestibility of food.
  2. Flatulence, abdominal pain.
  3. Dry mouth.
  4. Bad appetite.

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Mixed type

it наиболее тяжелое и прогностически неблагоприятное течение
diseases. It includes signs of damage to the lungs and organs.
digestion.


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Atypical form

With атипичном течении на первый план выходят изменения состава
blood and liver damage. Against the background of pancreatic dysfunction
the gland replaces the glandular tissue with connective tissue. also in
liver dystrophy forms and develops cirrhosis.

Symptoms of pathology:

  • enlarged liver;
  • pruritus;
  • jaundice;
  • pressure surges;
  • ascites (accumulation of fluid in the abdominal cavity).

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Stages of the disease cystic fibrosis

The respiratory form goes through 4 stages.
development:

  1. Intermittent functional changes. The patient appears
    shortness of breath and cough after exercise. This stage lasts up to 10
    years old.
  2. Chronical bronchitis. Differs in constant cough with sputum,
    deformation of the fingers. With прослушивании четко слышны хрипы, жесткое
    breath. Данный этап может продолжаться до 15 years old.
  3. The development of complications. In the lungs and bronchi, fibrous and
    sclerotic lesions, cysts. Pulmonary insufficiency develops,
    heart failure. In this condition, the patient can live
    2-4 years.
  4. The last stage is characterized by the development of acute cardiopulmonary
    failure from which the patient dies within a couple
    months.

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Possible complications

The course of the disease and possible complications depend on the form and
stage pathology. The most common complications that
eventually become the causes of death:

  1. Abscesses in the lungs.
  2. Pulmonary bleeding.
  3. Cardiopulmonary insufficiency.
  4. Sluggish diabetes.
  5. Intestinal obstruction.
  6. Cirrhosis of the liver.


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Diagnosis of cystic fibrosis

Diagnosis of cystic fibrosis входит в программу surveys
newborns, which allows you to identify the disease at an early age.
Currently used molecular genetic research,
however, its accuracy is 75%, so it is supplemented
other methods.

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treatment

For differentiation of cystic fibrosis from whooping cough, true
bronchitis, asthma use the following laboratory tests:

  • Sweat test
  • Coprogram.
  • Measurement of sodium concentration in saliva.
  • Microbiological examination of sputum.

To assess the degree of damage to internal organs, conduct
instrumental study:

  • Bronchoscopy.
  • Cardiogram.
  • Ultrasound of the internal organs.
  • X-ray of the chest.

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Cystic fibrosis treatment

Given the incurability of the disease, therapy for cystic fibrosis
is symptomatic. Врачи назначают treatment, которое
facilitate the patient’s condition and prolong his life.

Therapy includes:

  1. Withменение медикаментов.
  2. Dieting.
  3. Physiotherapy and massage.

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Drug treatment

A patient with cystic fibrosis must support his work.
pancreas and ensuring the outflow of mucus from the lungs and bronchi
(mucosal therapy). To do this, use the following
drugs:

  • Ферменты поджелудочной железы (Панкреатин,
    Мезим
    ).
Панкреатин
Мезим
  • Муколтические, бронхолитические средства
    (Ацетилцистеин, Пульмозим). They are used for
    carrying out inhalations.
Пульмозим
Ацетилцистеин

Данная терапия проводится каждый день до конца жизни. With
exacerbation or adherence of bacterial infection are required
antibacterial agents (penicillin, cephalosporin,
Aminoglycoside). Injections are combined with aerosol preparations.
(Tobramycin, Gentamicin). The course of antibiotics lasts at least
of the month.

If the patient’s condition is severe, along with antibiotics
Glucocorticosteroids (Prednisolone) are administered intravenously. In particular
severe cases an emergency measure is bronchoscopy with
washing the bronchi with sodium chloride. Also hold
симптоматическое treatment гипоксии, нарушения сердечного ритма,
hypertension.

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Cystic fibrosis diet

Nutrition of the patient is of great importance in the treatment of cystic fibrosis.
In the diet increase the amount of protein and limit fats. Also
in violation of pancreatic function exclude simple
carbohydrates.

Be sure to comply with the drinking regime (at least 2 liters of water).
Given the large loss of salt from the sweat, should food and water
add some salt. In the patient’s menu include juices, fruits, vegetables, to
prevent vitamin and mineral deficiencies.

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Massage and exercise therapy

Massage and physiotherapy help remove sputum from
lungs. Postural drainage the patient can conduct as
independently and with the help of relatives. Postural drainage
necessary daily, and vibrating massage – three times a day. Also
facilitate the patient’s state of physical education.

Once every three months, the patient must take a coprogram. Also
strict control of the patient’s chair and weight is necessary so as not to
допустить обострения diseases.

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Lung transplant

Donor lung transplantation is a relatively new way.
treatment of cystic fibrosis. The difficulty is that effective
is the only complete organ transplant. With частичной
transplantation pathological process moves from the patient part
lung to healthy.

Also пересадка возможна, если болезнью еще не затронуты другие
organs and systems. This method is effective only in relation to
pulmonary form of the disease.

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