ABSTRACT
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.
Subject(s)
Cardiomyopathies/diagnosis , Cardiomyopathies/pathology , Heart Defects, Congenital/diagnosis , Heart Defects, Congenital/pathology , Myocardium/pathology , Cardiomyopathies/etiology , Cardiomyopathies/physiopathology , Female , Heart/physiopathology , Heart Defects, Congenital/etiology , Heart Defects, Congenital/physiopathology , Humans , MaleSubject(s)
Fetus/metabolism , Lipoproteins, HDL/metabolism , Liver/metabolism , Receptors, Cell Surface/metabolism , Cells, Cultured , Electrophoresis, Polyacrylamide Gel , Humans , In Vitro Techniques , Ligands , Liver/cytology , Microscopy, Phase-Contrast , Molecular Weight , Receptors, Cell Surface/chemistrySubject(s)
Cholesterol/metabolism , Duodenum/metabolism , Enzyme Inhibitors/pharmacology , Intestinal Mucosa/metabolism , Sterol O-Acyltransferase/antagonists & inhibitors , Adult , Cholesterol Esters/metabolism , Duodenum/drug effects , Epithelium/drug effects , Epithelium/metabolism , Female , Humans , Intestinal Mucosa/drug effects , Male , Micelles , Middle Aged , Organ Culture TechniquesSubject(s)
Bacterial Infections/pathology , Sepsis/pathology , Shock, Septic/pathology , Virus Diseases/pathology , Child , HumansABSTRACT
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.
Subject(s)
Endocarditis, Bacterial/pathology , Adolescent , Child , Child, Preschool , Endocarditis, Bacterial/diagnosis , Endocarditis, Bacterial/mortality , Female , Humans , MaleABSTRACT
The paper provides strong evidence for further postmortem investigations in the ante- and perinatal periods, in the combination of those of the afterbirth. The very early antenatal abnormalities (gametopathy and blastopathy) have been less studied. There are predominant congenital malformations of the organs concurrent with placental incompetence among embryopathies. In the fetal period, the authors identify an abnormality in the early fetal phase when placental insufficiency, fetal hypoxia and intrauterine death prevail; that in the mid fetal phase concomitant with infectious fetal injuries and acute and chronic placental failures due to maternal diseases; and that in the late fetal phase that covers perinatal death. The time course of reasons for stillbirth and neonatal death was analyzed in Moscow in 1986-1989. Etiological causes of early and late abortions have been identified, which is a real reserve of reduction in ante- and perinatal mortality rates in this country.
