[Immunomorphology of the extracellular matrix of the uterus, placenta fetal and infant heart in maternal rheumatism]. / Immunomorfologiia ekstratselliuliarnogo matriksa matki, platsenty, serdtsa plodov i detei pri revmatizme materi.

20 uteri, 80 placentas and the hearts of 66 normal fetuses from rheumatic mothers have been studied. Exacerbations of rheumatism were observed in some mothers during pregnancy. Sclerosis with enhanced synthesis of collagen type I, III, IV, V and fibronectin was observed in ECM when rheumatism during the pregnancy was not active. Activation of rheumatism during the pregnancy was followed by disturbances of synthesis of interstitial and membrane collagen types, alteration of stromal-parenchymatous relations and development of dyschronosis in the placenta and fetal heart. The study of the heart from children aged 3 months-14 years showed a long-term effect of maternal rheumatism.

Enfermedades reumáticas pediátricas

Se presenta el caso de un niño de cinco años de edad que consulta por primera vez por edema y dolor en maléolo interno izquierdo presentando posteriormente hematuria en el que se estudia una enfermedad reumática de origen autoinmune.

Collagenopathic cardiopathies.

Collagenopathic cardiopathies are a subject of extreme etiologic, pathogenetic and clinical interest. These disorders are associated with congenital or acquired anomalies of the connective tissue and because of the diffusion and nearly total distribution of this tissue, have a higher frequency than what has been previously estimated. The collagenopathic cardiopathies, can be divided into two main groups: one deriving from hereditary connective tissue diseases, and the other from acquired connective tissue diseases. The first group has a Mendelian type of transmission whereas the other appears to be secondary to various kinds of stimuli (viral, immunologic etc.) although polygenic factors are present. Of the first group we considered Marfan's syndrome, the Ehlers-Danlos syndrome, osteogenesis imperfecta, pseudoxanthoma elasticum, cutis laxa and the diseases of the fundamental substance with particular reference to mucopolysaccharidosis type 1H (Hurler's syndrome). In all of these disorders a specific metabolic disturbance is responsible for the cardiovascular damage which is expressed, depending on the specific genetic component in a more or less serious form. Among the acquired diseases of the connective tissue, we examined rheumatoid arthritis, systemic lupus erythematosus, polydermatomyositis, scleroderma; of the reactive arthritis, rheumatic fever; of the seronegative forms, spondyloarthritis, ankylosing spondylitis and Reiter's syndrome, mixed connective tissue disease and Lyme's disease. It must be emphasized that all of these disorders share relatively common pathogenetic characteristics which point to the importance of the presence of various types of antigens, immune complexes and the significant role of some of the histocompatibility antigens, as well as possible disturbances of cell-mediated immunity.(ABSTRACT TRUNCATED AT 250 WORDS)