Alveolar Proteinosis

Alveolar proteinosis is a rare hereditary disease transmitted through the autosomal recessive type. Characterized by accumulation in the alveoli of the protein-lipoid substance and progressive respiratory insufficiency. The disease occurs mainly in young men aged 30-40 years. In children, the disease is extremely rare.

The causes and mechanisms of the development of this disease have not been studied enough.

For a long time, the disease can be asymptomatic and can be detected accidentally with a preventive X-ray examination. The first and main symptom of the pathology is progressive dyspnoea, often a cough with the spitting of a small amount of sputum, body temperature does not exceed 38 ° C, sweating, pain in the chest, hemoptysis. As the disease progresses, the degree of respiratory failure increases, a cyanotic skin color develops, and changes in the end phalanges of the fingers are formed. Such people often suffer from repeated viral, bacterial, fungal infections. The presence of a chronic inflammatory process in the bronchi leads to an increase in pressure in the pulmonary artery and the formation of the pulmonary heart.

Diagnosis of pulmonary alveolar proteinosis is difficult because of the nonspecific clinical manifestations, so correct diagnosis often takes several years. There are no typical changes on the part of blood, biochemical indicators of blood, immunological status. Clinical manifestations of alveolar proteinosis of adults and children differ. If adult patients have predominantly symptoms of respiratory tract infection, the initial manifestations of the disease in children may be abdominal pain, nausea, vomiting.

X-ray examination of chest organs reveals small-dot blackouts located in both the right and left lungs, which tend to merge. In this case, the middle and lower parts of the lungs are mainly affected. There is no clear connection between the manifestations of the disease and the data obtained by X-ray examination of the lungs.

An effective method of treatment of the disease is therapeutic bronchoalveolar lavage. Mechanical removal from the lungs of the protein-lipid complex leads to a reduction in dyspnea and a positive dynamics in lung function. A positive effect is observed when using trypsin and chymotrypsin in the form of aerosols, as well as when the enzymes are administered orally. When stratifying bronchopulmonary infection, antibiotic therapy is indicated.