RESUMEN
BACKGROUND: The article is devoted to the study of clinical and laboratory characteristics of the current of hemorrhagic vasculitis in children in the Republic of Buryatia. PATIENTS AND METHODS: The study included 27 patients aged 7.6 +/- 4.02 years, who conducted clinical and laboratory tests, immunological study of antiphospholipids of antibodies, genetic testing for thrombophilia markers of candidate genes. RESULTS: The results showed that hemorrhagic vasculitis often affects children of Buryat nationality. In 96% of cases there are mixed clinical forms of the disease. 63% of children of hemorrhagic vasculitis preceded by various factors, a higher percentage of infectious diseases. The first clinical symptom in 63% of patients is a typical purpura hemorrhagic rash. Results of clinical laboratory blood tests revealed no significant deviations. Circulation of lupus anticoagulant was detected in 37% of subjects. The alphaCL IgM detected in 3 children, alphabeta2-GP-I IgA--in 4, alphabeta2-GP-I IgM--in 1 patient. Carriers of thrombophilia polymorphisms were in 95% of children. Noted that homozygous variants of genes polymorphisms of methylenetetrahydrofolatered reductase and plasminogen activator inhibitor-1 correlate with the presence of urinary symptoms and recurrence of the rash. CONCLUSION: The study shows the risk of recurrent flow of hemorrhagic vasculitis and nefritis of Henoch-Schonlein in children with thrombophilia gene polymorphism.
Asunto(s)
Vasculitis por IgA , Metilenotetrahidrofolato Reductasa (NADPH2)/genética , Inhibidor 1 de Activador Plasminogénico/genética , Trombofilia , Niño , Preescolar , Exantema/fisiopatología , Femenino , Predisposición Genética a la Enfermedad/epidemiología , Humanos , Vasculitis por IgA/complicaciones , Vasculitis por IgA/epidemiología , Vasculitis por IgA/genética , Vasculitis por IgA/inmunología , Vasculitis por IgA/fisiopatología , Masculino , Monitorización Inmunológica , Polimorfismo Genético , Recurrencia , Federación de Rusia/epidemiología , Trombofilia/complicaciones , Trombofilia/epidemiología , Trombofilia/genética , Trombofilia/inmunología , Trombofilia/fisiopatología , Sistema Urinario/fisiopatologíaRESUMEN
Aim. To determine limits of interval QT in children and adolescents aged 0-17 years and to detect possible ethnic differences of its changes. Material and Method. Twelve lead ECGs were recorded in 1531 subjects without overt cardiovascular pathology (47.2% females, 52.8 males, 57.3% of Slavic and 42.7% of Buryat i.e. mongoloid ethnicity). Results. Corrected QT interval (QTc) exceeded 440, 460 and 480 ms in 2.3, 1.05, and 0.26% of children. Prolongation of QTc was found in 0.46% and 0.11% of Slavic and Buryat children, respectively (p=ns). Starting from the age of 8 years girls had longer QTc than boys (454.1+/-15.2 and 438.3+/-8.4 ms, respectively, p<0.05). QTc less than 350 ms was registered in 12 children (0.78%). Eight of these children with mean QTc 329.1+/-32.3 ms had family history of syncope or sudden death. Variability of absolute QT values was 8+/-14.3 ms (maximally up to 40 ms). Conclusions. QT interval is prolonged when QTc duration exceeds 440 ms in children younger than 8 years and in male adolescents or 460 ms in girls aged 8 years or older and in children during first year of life. QT interval is shortened when QTc is less than 350 ms (1st degree of shortening). In children with QTc below 330 ms (2nd degree of shortening) short QT syndrome should be excluded. Normal variability of absolute QT value during sinus arrhythm on ECG at rest does not exceed 40 ms.