Mucoviscidosis of lungs

Description:

Mucoviscidosis of lungs - the hereditary disease which is characterized by systemic lesion of muciparous excretory glands of a respiratory appart.

Symptoms of the Mucoviscidosis of lungs:

Characteristic feature of a mucoviscidosis is increase in viscosity of a secret of muciparous glands and as a result causes sharp disturbance of cleaning function of bronchial tubes and bronchial passability. Changes in a respiratory organs can meet almost separately or be combined with disturbances of functions of digestive organs (preferential a pancreas and intestines). Defeat of not muciparous glands plays considerably a smaller role in pathology and is expressed in significant increase in secretion by them of ions of sodium and chlorine.

Signs of damage of lungs at a mucoviscidosis most often arise during a first or second the child's lives (at 90%) and at 10% - at teenage age. The early beginning of pulmonary manifestations along with existence in the anamnesis of data on mekonialny Ilheus in the period of a neonatality, the slowed-down physical development, a frequent diarrhea, intolerance of greasy food it is necessary to consider a characteristic sign of a mucoviscidosis.

Complaints: on painful pristupoobrazny cough with knitting, it is difficult the expectorated phlegm (is the typical complaint), an asthma at moderate exercise stresses, and sometimes even at rest. The pneumorrhagia is sometimes noted.

Physical inspection: pallor of integuments, sometimes puffiness of the person, the moderated or expressed cyanosis of visible mucous membranes. The thorax sometimes has the increased sagittal size (a barrel-shaped form).

At percussion symptoms of emphysema (bandbox tone, narrowing of percussion borders of heart, low standing and small mobility of a diaphragm) can be defined. At auscultation quite often weakened breath, scattered dry rattles, wet average and small-bubbling rattles, especially pronounced during the periods of aggravations are listened rigid, and in upper parts.

X-ray pattern: widespread strengthening of the pulmonary drawing due to intersticial changes. The pulmonary drawing is submitted tyazhisty in case of dominance of peribronchial changes or mesh when involving in process of periatsinarny and perilobulyarny elements of connecting fabric. Roots of lungs are expanded. At emphysematous changes are found increase in transparency of pulmonary fields, it is preferential in upper parts. During the periods of aggravations intersticial changes accrue, segmented or polysegmented pneumonic infiltration appears.

At a bronchography the changes connected with hypersecretion of a viscous phlegm ("breaks" of bronchial tubes, the fragmented their filling, roughness of contours, reduction of number of lateral branches) come to light. More than a bronchietasia in lower parts of lungs is found in a third of patients cylindrical, more rare mixed.

Bronkhoskopiya: a diffusion purulent endobronchitis with the characteristic mucopurulent tyazha which are located on longitudinal folds of a mucous membrane, sometimes extensive fibrinous ("diphtheroid") films. The secret is viscous, it is hardly aspirated.

Research of function of external respiration: ventilation is broken on obstructive restrictively type, residual lung volume increases already at early stages of a disease, the ratio residual capacity the easy/vital capacity of lungs decreases, decrease Tiffno's index (the forced exhalation for 1 sec. / the vital capacity of lungs) and other high-speed indicators.

Crucial importance in diagnosis and differential diagnosis of a mucoviscidosis has a research of electrolytes of sweat (stalemate test) which the International association on cystous fibrosis recommends to make by Gibson and Cook's method.

Reasons of the Mucoviscidosis of lungs:

Infectious process in a bronchial tree plays an important role in a mucoviscidosis pathogeny. The main role in an etiology of infectious process in bronchial tubes is played by golden staphylococcus, a hemophilic stick and a pyocyanic stick, and the two first a microorganism come to light mainly at early children's age whereas further major importance is got by a pyocyanic stick, fight against which is very complex problem of treatment in connection with its resistance to phagocytosis by polynuclear leukocytes and to the majority of antibacterial agents.

Treatment of the Mucoviscidosis of lungs:

About three main directions are carried out: 1) recovery of air-conducting and cleaning function of a bronchial tree; 2) suppression of infectious process in bronchial tubes; 3) consists in a dietotherapy and correction of enzymatic insufficiency of a pancreas at patients with the mixed disease form.

Traditional expectorants practically do not give effect. Mucolyticums have favorable therapeutic effect.

Apply 5 ml of 10% of solution of N-Acetylcysteinum in inhalations (fluimucil), etc. At purpose of Mucolyticums intramusculary or inside they make impact and on mucous glands of digestive organs.

Medical (sanatsionny) bronkhoskopiya which during the periods of aggravations it is necessary to carry out 1-3 times a week with introduction to a bronchial tree of mucolytic drugs are of great importance.  In improvement of bronchial passability play physiotherapeutic procedures, for example vibromassage, and also LFK.

Antibacterial therapy is appointed  during the periods of aggravations. Antibacterial agents choose according to features of the microflora sowed from respiratory tracts.

The dietotherapy at the mixed form of a disease provides restriction of fats with co-administration of fat-soluble vitamins (A, D, E) and increase in consumption of protein up to 3-5 g at 1 kg of body weight in days. Enzymatic insufficiency of vneshnesekretorny function of a pancreas is the indication to replacement therapy (Pancreatinum, panzinorm-forte, festal, etc.).