[A new genetic variant of the orofaciodigital syndrome].
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Biomedical subjects
Publications and source records attributed to T I Ternova.
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In 61 children with different patterns of ectopic arrhythmias (premature heart beat, paroxysmal and non-paroxysmal tachycardia), the peripheral blood leukocytes and myocardial tissues were examined for the content of calmodulin (CM), ionized calcium (Ca2+) and cyclic nucleotides (CN). The data obtained indicate that the CM-Ca2+ and CN system are involved in the genesis of arrhythmias in children and may be used as additional criteria in the diagnosis of cardiac rhythm abnormalities.
Examination of 200 children suffering from arrhythmias made it possible to define specific features of the phenotype, attesting to the pathogenetic relation to the condition of the body connective tissue. The authors describe combinations of the signs for the selection of persons at risk for arrhythmias and mitral valve prolapse.
The cyclic nucleotides (CN) system and Ca2+ content were examined in children with various heart rhythm disorders (HRD). Marked changes were demonstrated which correlated to clinical manifestations of the arrhythmias: their type, phase, duration and favorable changes in their course. Antiarrhythmic therapy was associated with a normalization of the altered levels of cyclase components and Ca2+. The results suggest that intracellular metabolism regulation may be disrupted in HRD cases and play an important role in the development of arrhythmias.
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Phentolamine has been shown to improve peripheral hemodynamics and have a favorable central hemodynamic effect, reflected in clinical improvement, in children with bradycardia (11 patients with second- or third-degree atrioventricular block) and signs of circulatory insufficiency.
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Forty-one children with cardiac arrhythmias were examined. It was established that cardiac rhythm disorders in children were accompanied by an increase in the cGMP levels as well as a decrease in the cAMP content, CPK activity and the cAMP/cGMP ratio varying in relation to the form of arrhythmia and the nature of heart damage.
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