[Cardiopathies in children].

Cardiomyopathies in children are rather rare, but extremely severe disorders that are little known in practical healthcare. Many of them are a cause of sudden death and they are familial. By using their findings and the data available in the literature, the authors describe the etiology, pathogenesis, morphology, and clinical presentation of dilated, hypertrophic, arhythmogenic right ventricular, histiocytoid cardiomyopathies, as well as the non-compact myocardium, and a type of cardiac connective tissue dysplasia. By keeping in mind the frequency of inheritance of cardiomyopathies, it is necessary to improve the diagnosis of these diseases not only for timely treatment, but also medical genetic counseling.

[Clinico-morphological comparison in children with infectious endocarditis]. / Kliniko-morfologicheskie sopostavlenii pri infektsionnom éndokardite u detei.

As many as 21 children suffering from infectious endocarditis (IE) were examined for the clinical and morphological picture of the disease. The patients' age ranged from 2 to 15 years. In 15 children, IE developed in association with different congenital diseases; in one patient, in the presence of rheumatic heart disease, and in 5 patients, in unaffected heart valves. The sectional material was examined in 19 cases whereas the operational material in 2 cases. Analysis of the data obtained has shown that early diagnosis of IE (primary and secondary) may not infrequently be fairly difficult, which is likely to result in the late administration of antibacterial therapy. In addition to the involvement of the valvular apparatus of the heart, the majority of the patients demonstrated the signs of focal myocarditis and postmyocarditic cardiosclerosis, which is one of the causes of the development of refractory heart insufficiency. IE children mostly die from heart insufficiency and thromboembolism.

[Various hereditary immune deficiencies linked to the X-chromosome]. / Nekotorye nasledstvennye immunodefitsity, stseplennye s X-khromosomoi.

The organs of immune system in hereditary immunodeficiencies linked to X-chromosome, particularly in Nezelof's syndrome and chronic granulomatosis (4 cases) were studied histologically. The alterations of thymus found in Nezelof's syndrome suggest the disturbance of its morphogenesis at the embryonal stage, those found in chronic granulomatosis suggest the postnatal disturbance of thymus morphogenesis. The children die of polytopic infections foci. Generalized BCG-itis is observed in children with chronic granulomatosis.