[Neurinoma of the small intestine].
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Biomedical subjects
Publications and source records attributed to S A Iampol'skaia.
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A fatal case of generalzyed hemochromatosis in a man of 30 was studied.The disease ran a course with the predominant involvement of the myocardium accompanied by circulatory insufficiency. At the end of the disease a large thrombus formed in the left ventrical cavity. No correlation between the biological tests pathognomonic for the disease (the normal iron and sugar content in the blood) and significant morphological changes in the visceral organs was observed. The familial predisposition to hemochromatosis was revealed.
Clinical and morphological observations of choriocarcinoma in infants of 6 and 18 months localized in the right lateral ventricle of the brain and in the sacralcoccygeal region are presented. The grounds for verification of the heterotopic tumor include the presence of one large degenerating node in the sacral-coccygeal region and the absence of neoplasia in the central nervous system, its typical histological characteristics corresponding to choriocarcinoma as well as extensive metastasizing along the lymph tracts. The dysontogenetic nature of the tumor is suggested. The difficulties of the clinical and histological diagnosis are due to the rarity of choriocarcinoma and heterotopic localization.
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A severe visceral form of Niemann-Pick disease from the first days of life was manifested in the progressive retardation of psychomotor development and hepatosplenomegaly. The disease was complicated by anemia and cachexia which led to a fatal outcome in a girl of 7 months.
Clinico-morphological and histochemical studies of changes in the lungs were carried out in 15 fatal cases of Wegner's granulomatosis. Morphological manifestations of the lesions consisted in bilateral extensive destructive-productive panangiitis in the system of bronchial, was well as pulmonary arteries and veins. As a rule, there developed an extremely polymorphic granular tissue which subsequently underwent necrosis with resulting cavern formation. Deep and sometimes irreversible changes observed in the vessels of the microcirculatory bed led to alveolar-capillary blockade and hypoxia, the latter culminating with circulatory crisis. Drastic dyscirculatory disorders were complicated with auto- and superinfection: development of destructive-suppurative bronchitis, peribronchitis, interstitial and also confluent pneumonia with "motley" exudate in alveoli, not infrequently with an abscess formation, sometimes with formation of hyaline membranes. The whole complex of these changes play a significant part in pathogenesis of pulmonary-cardiac insufficiency occurring in the majority of patients with Wegener's granulomatosis and often is responsible for fatal outcomes.
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