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Idiopathic diffusion intersticial pneumosclerosis

This seldom meeting chronic, usually coming to an end with death, a disease of the unknown nature adults, but sometimes and children of chest and early age usually have. The data obtained by in vitro allow to assume an immunological basis of an illness. The clinical picture of an idiopathic diffusion intersticial pneumosclerosis is characterized by the progressing pulmonary insufficiency as a result of fibrosing of fabric and the alveolar and capillary block. The disease usually gradually begins, and very first there is short wind, in the beginning only at an exercise stress, and later and at rest. The patient is often disturbed by dry cough, it can sometimes be followed by department of a phlegm with blood impurity. Body temperature usually does not increase. In process of progressing of an illness appetite is lost, body weight decreases, fatigue increases and, at last, cyanosis, fingers in the form of drum sticks, a pulmonary heart and signs of insufficiency of the right heart join. Usually changes in lungs at auscultation are not revealed, but sometimes it is possible to listen to wet rattles. Most of children die of the insufficiency of breath which developed in time of one of often joining pulmonary infections. On serially made roentgenograms it is possible to see the increasing speckled centers of a granular or mesh look or small nodular consolidations. There can come the anoxemia amplifying at an exercise stress. Resistance of respiratory tracts does not increase, and the vital capacity of lungs, distensibility and diffusion capacity decrease.
Pathomorphologic changes at Hammen's syndrome — Rich in lungs are various. In an early stage fibrosis, as a rule, is absent, but walls of alveoluses, the alveolar courses and peribronchial fabric of an infiltrirovana lymphocytes, plasmocytes and sometimes eosinophils. These changes usually progress before extensive diffusion proliferation of fibrous fabric on all lung lobe that is connected with the organization of intra alveolar exudate.

Corticosteroids promote some symptomatic improvement of a state, but do not stop a course of a disease and do not increase function of lungs. Other types of treatment also are symptomatic. At some adult patients immunodepressive means were effective.

 
"Respiratory insufficiency   Infectious rhinitis"