Hospital pediatrics. Pathology of connective tissue. Alveolar microlithiasis, alveolar proteinosis (most important)

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Hospital pediatrics. Pathology of connective tissue. Alveolar microlithiasis, alveolar proteinosis (most important)

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50. Pathology of connective tissue. Alveolar microlithiasis, alveolar proteinosis

Pathology of connective tissue can accompany hereditary pathology of connective tissue (Marfan syndrome, Ehlers-Danlos syndrome, achantitrypsin deficiency).

clinical picture. Sudden sharp stabbing pain in the chest, aggravated by a deep breath, shortness of breath, percussion on the side of the lesion "box" sound, a sharp weakening of the breath sounds (auscultation), a shift in cardiac dullness in the opposite direction.

X-ray is determined by the presence of air in the pleural cavity, the collapse of the lung.

Treatment. Treatment for spontaneous pneumothorax is drainage of the pleural cavity with constant active aspiration (according to Belau).

Alveolar microlithiasis characterized by the formation in the pulmonary alveoli of the smallest calculi, which consist of calcium carbonate and thiophosphates with a small admixture of iron salts and traces of magnesium.

As a result of the deposition of calculi, an alveolar-capillary block occurs, ventilation-perfusion relations are disturbed. It is inherited in an autosomal recessive manner.

Pathogenesis. The formation of stones in the alveoli is associated with disturbances in the production of alveolar fluid, as well as a disorder of carbonic acid metabolism - alveolar microlithiasis. The disease occurs in all age groups.

Clinical manifestations. The clinical picture is varied.

A discrepancy between the poor clinical picture and radiological changes is characteristic.

Symptoms may be absent altogether; shortness of breath, cyanosis, decreased exercise tolerance may be disturbing.

As the process progresses, signs of chronic inflammation of the lungs join: cough, sputum, fever, "drum sticks", cor pulmonale appear.

Diagnostics. X-ray examination reveals small diffuse shadows of stony density, located mainly in the lower and middle parts of the lungs; compaction of the pleura is noted (differentiated with tuberculosis).

Treatment. Symptomatic.

Forecast. Adverse; death from pulmonary heart failure.

Alveolar proteinosis is caused by the accumulation of a protein-lipoid substance in the alveoli. The histological picture is characterized by the presence in the alveolar lumen of a granular exudate with a PAS-positive reaction. It is transmitted in an autosomal recessive manner.

Pathogenesis. A genetic defect leading to the synthesis of a defective surfactant that does not have surface-active properties; this lipoprotein is characterized by a strong CHIC-positive reaction; filling the alveoli with lipoprotein causes changes in lung function and corresponding clinical symptoms: progressive shortness of breath, cough, chest pain, hemoptysis; subsequently, a pulmonary heart is formed with corresponding symptoms.

Diagnostics. X-ray examination reveals bilateral small-focal (small-pointed) darkening, which tends to merge, and subsequently fibrous changes are revealed.

Treatment. Therapeutic bronchoalveolar lavage; administration of trypsin, chymotrypsin.

Author: Pavlova N.V.

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