Alveolar is the rare disease of lungs which is characterized by formation of protein and mineral complexes in a gleam of alveoluses. It is clinically shown by the accruing short wind, the general weakness, cough, weight and pressure in a breast. In process of progressing of pathology symptoms of respiratory heart failure join. Diagnostic actions include beam methods of a research of bodies of a thorax, laboratory analyses, the ECG, spirometry, a lung biopsy. Etiotropny treatment of this pathology does not exist. Symptomatic therapy is appointed.
Alveolar (pulmonary) is an accumulation disease. Results from adjournment in alveoluses of protein which becomes impregnated with salts of metals subsequently, mainly compounds of calcium, to a lesser extent - magnesium. The disease meets seldom. Today in special literature only about 400 cases are described. Equally often persons men's and female of different age groups are ill. Quite often pathology has congenital character, is sometimes combined with other malformations. About 25% of all cases of a disease are diagnosed at children's age. About 50% of patients are blood relatives.
The causes of a pulmonary mikrolitiaz are up to the end not studied. The genetic etiology with inheritance of a disease on autosomno-recessive type is supposed. This theory is confirmed by family cases of a disease, and also detection of this pathology at children whose parents were close relatives. As an additional prerequisite to development and progressing of a mikrolitiaz serves long inhalation of the dust containing compounds of calcium, magnesium and some other metals. Such dust gets to an organism by production and use of cement, agricultural fertilizers, utilization became, the use of snuff tobacco.
Presumably, genetic defect leads to violation of exchange processes and decrease in synthesis of alveolar liquid, hyperproduction and accumulation of protein in air cells and bronchioles. This protein becomes impregnated with salts of calcium and forms microlitas (a concrement the size 0,3-2mm). The alveolar gleam is half and more filled with a microconcrement. Process gradually strikes all pulmonary parenchyma. Lungs become stony and dense and heavy. The alveolar blood-groove and gas exchange is broken. Respiratory insufficiency develops.
At a patomorfologichesky research increase in density of pulmonary fabric in basal and average departments comes to light. The weight of lungs can sometimes reach 4 kilograms. In gleams of alveoluses and bronchioles microlitas of a complex concentric structure are found. As a part of the smallest concrements carbonates and phosphates of calcium, and also magnesium, zinc, sodium, copper and other minerals come to light. In interstitsialny tissue of lungs symptoms of fibrosis are defined.
Alveolar it is characterized by the chronic, steadily progressing current. Symptoms of a disease appear and made heavier in process of accumulation of microlitas in air cells and exhaustion of compensatory opportunities of an organism of the patient. Pathological process proceeds step by step. Distinguish the following stages of a disease:
- I stage. There is a formation of microlitas. At this stage proceeds asymptomatically. Radiological signs can be found at a preventive x-ray research of lungs.
- II stage. There are respiratory violations which are partially compensated for the account of reserves of an organism.
- III stage. Pulmonary heart is formed, there comes the pulmonary and warm decompensation.
Symptoms of an alveolar mikrolitiaz
During the long period of time pulmonary proceeds it is hidden. At first symptoms of a disease are expressed poorly. One of the first clinical displays of pulmonary pathology is short wind. At the initial stages of a mikrolitiaz this symptom appears only at essential physical activity – run, bystry rise on a ladder. In the course of progressing of a disease short wind accrues, arises when walking, the slightest movement.
Cough at a pulmonary mikrolitiaz moderate, unproductive. A small amount of a light mucous phlegm separates. At accession of a bacterial infection the bronchial secret becomes flavovirent. The blood spitting is sometimes observed. The pain syndrome is not a specific symptom of a disease. The patient complains of discomfort, feeling of weight, pressure in a thorax. Pain can be localized from one or two parties, happens different degree of intensity.
Also the general condition of an organism suffers. The patient feels increasing sensations of weakness, fatigue, shows complaints to periodic temperature increase of a body to subfebrilny and febrilny values. Sometimes proceeds latentno up to emergence of the symptoms characteristic of chronic pulmonary heart. In a terminal stage of a disease arise breast pain and the right podreberye. The patient is disturbed by heartbeat increase, interruptions in work of heart. Hypostases of legs join, respiratory insufficiency sharply progresses. Short wind arises during the conversation and at rest.
Complications of a serious illness of alveolar fabric arise at the II-III stages of a disease. Presence of microlitas at bronchioles and larger bronchial tubes provokes emergence of inflammatory process in a wall of airways. At accession of a secondary infection bronchitis becomes purulent, bronchial pneumonia develops. The progressing hypoxia affects activity of all bodies and systems. Sick mikrolitiazy children lag behind in physical and mental development. The heaviest, the complication conducting by the death of the patient is pulmonary heart failure.
Alveolar treats one of the most hardly diagnosed diseases in pulmonology. At survey of the patient with the developed clinical symptomatology signs of chronic come to light, it is long the current pathology of respiratory organs. Skin cyanosis, deformation of disteel phalanxes of fingers as drum sticks, hypostases, increase in a liver is observed. Perkutorny and auskultativny data are also not specific. In the lower departments of lungs damp melkopuzyrchaty and krepitiruyushchy rattles can be listened. The following methods of a research help to confirm the diagnosis:
- Laboratory analyses. In clinical blood test the politsitemiya characteristic of pulmonary heart failure is defined. At a biochemical research the increased content of calcium and phosphates comes to light. In a phlegm and washing liquid of bronchial tubes concentric microlitas quite often are found.
- Radiodiagnosis methods. On roentgenograms of a thorax the small dissemination localized in the lower and average departments of lungs and reminding a sand scattering – a symptom of "sandstorm" is defined. On KT of lungs numerous small diffusion kaltsinata come to light. Accumulation of isotope when carrying out a perfuziony stsintigrafiya of lungs confirms a kaltsifikation of pulmonary fabric.
- Functional researches. The dynamic research of function of external breath reveals the progressing restrictive violations. Gradually decrease in indicators of ZhEL accrues. On the electrocardiogram signs of a hypertrophy of a myocardium of the right departments of heart, tachycardia, premature ventricular contraction are defined.
- Lung biopsy. Main method of verification of the diagnosis. The fence of a bioptat is carried out when carrying out a bronkhoskopiya or transtorakalno. In biopsiyny material the concentric microconcrements located in alveoluses, gleams of bronchioles and small bronchial tubes are found. In walls of bronchial tubes excess adjournment of granules of a glycogen is defined.
The pulmonologist needs to differentiate alveolar from pulmonary tuberculosis. Poor clinical manifestations against the background of considerable radiological changes are characteristic of an early stage of a disease. Quite often the disease is mistakenly taken for disseminirovanny tuberculosis of respiratory organs and unsuccessfully treat antitubercular medicines. For an exception of specific process consultation of the phthisiatrician and a research of a phlegm is appointed to Koch's bacilli.
treatment should be carried out only under the supervision of a doctor. Self-treatment is unacceptable!!!
The methods of treatment allowing to stop formation of microlitas in alveolar fabric do not exist yet. Experimental search is conducted. The symptomatic conservative treatment directed to reduction of expressiveness of clinical manifestations, improvement of quality of life, knocking over of infectious complications is appointed. Bronchial spasmolytics, expectorant means, antibacterial and kardiotropny medicines are applied. At the last stage of pathological process long oxygenotherapy is carried out.
Forecast and prevention
The forecast at a mikrolitiaza of alveolar fabric adverse. Respiratory heart failure gradually results in disability and death of the patient. However the disease usually progresses slowly. Treatment-and-prophylactic actions help to delay development of pulmonary heart several. The healthy lifestyle, refusal of smoking, rational employment, vaccination against respiratory infections is recommended to patients with the established diagnosis. The work connected with a zapyleniye, a gas contamination, heavy physical activities is forbidden.