Search

Aim. To evaluate the efficiency of the original ALL-MB-2002 protocol within the multicenter study of treatment of acute lymphoblastic leukemia (ALL) in children. Subjects and methods. A total of 1873 primary patients with ALL aged 1 to 18 years, of whom 1544 patients were enrolled in this study, were notified at 36 clinics of Russia and Belarus from April 15, 2002, to January 1, 2008. Results. With the median observation of 4.12 years, 7-year event-free survival (EFS) was 73±13%; overall survival (OS) 78±2%; relapse-free survival 82±1%. The rates of EFS and OS were equal and amounted to 76±2 and 80±2% in the standard-risk group (SRG) and intermediate-risk group (ImRG), respectively. In the high-risk group (HRG) patients, EFS and OS were as high as 30±6 and 37±6%, respectively. The frequency of relapses with central nervous system lesion was as much as 4.7% in all the patients, 6-year cumulative risk for isolated neurorecurrences being 2.5% in the SRG patients. Adolescents, patients with the baseline leukocytosis (more than 100109/l), and those with a splenic size of over 4 cm or more from the costal arch margin had substantially worse survival rates. A poor early response to therapy (on induction days 8 and 15) was also associated with its lower efficiency. Conclusion. Despite a considerable rise in the number of centers and a slight increase in the intensity of therapy, the results of the new ALL-MB-2002 protocol are as minimum equivalents obtained in the use of the previous ALL-MB-91 protocol. A significant improvement in the overall results of therapy and a reduction in the cumulative risk for isolated neurorecurrences were noted in the ImRG patients.

Болезнь Гоше (БГ) - аутосомно-репеесивное заболевание, обусловленное мутациями в структурном гене p-D-глюкоцереброзидазы, фермента, присутствующего в лизосомах всех типов тканей. Изменение структуры фермента в результате генных мутаций ведет к нарушению расщепления глюкоцереброзида, который накапливается в клетках всех тканей. При БГ выделяют три типа в зависимости от наличия неврологических признаков: ненейронопатический; острый нейронопатический; хронический нейронопатический. В исследовании, диагностике и лечении БГ последовательно реализовались все методические подходы, присущие наследственным болезням обмена. Они включают анализ патологических метаболитов, дефектного фермента, мутантного гена, методы ферментзаместительной терапии, субстратподавляющей и генотерапии. Gaucher disease (GD) is an autosomal recessive condition caused by mutations in the B-D-glucoeerebrosidase structural gene; this enzyme is present in the lysosomes of all tissue types. Modification of the enzyme structure as a result of gene mutations leads to disorders in the glucocerebroside cleavage, and it is accumulated in the cells of all tissues. Three types of BG are distinguished:non-neuronopathic; acute neuronopathic; and chronic neuronopathic. All methodological approaches used in hereditary metabolic diseases were realized in the studies, diagnosis, and treatment of GD. These approaches include analysis of pathological metabolites, defective enzyme, mutant gene, and enzyme substitution, substrate suppressive, and gene therapy methods. Зуб Н.В., врач-гематолог Челябинского детского онкогематологического центра (Челябинск);тел. 8-9097456190; E-mail: nanita_zub@bk.ru Zub N.V., the doctor-hematologist. Chelyabinsk children's oncohematology centre (Chelyabinsk); ph.8-9097456190; E-mail: nanita_zub@bk.ru. Жуковская Е.В., доктор медицинских наук, профессор кафедры детских болезней № 2 Челябинской государственной медицинской академии (Челябинск); тел. 8-351-2607424*1128.Zhukovskaya E.V., the Professor, the Doctor of Medical sciences The Chelyabinsk state medical academy, faculty of children's illnesses Ш 2 (Chelyabinsk);ph. 8-351-2607424*1128.

Для оценки мы использовали адаптационный потенциал по P.M. Баевскому, тест Спилберга-Ханина, пробу Мартинэ-Кушелевского. В результате исследования выявили тенденцию к снижению адаптационных механизмов сердечно-сосудистой системы у детей с хроническим пиелонефритом. For assessment we used adaptative potential by Baievsky R.M., Spielburg-Hanin test, Martine-Kushelevsky test. As a result we revealed a tendency to adaptation mechanisms decrease in cardiovascular system of children with long-- lasting pyelonephritis. Сабирова А.В., кандидат медицинских наук, ассистент кафедры детских болезней № 2 Челябинской государственной медицинской академии (г. Челябинск); тел. 89080953916; doctor_sabirova@mail.ru Sabirova A.V., the candidate of medical sciences, the assistant to chair of children's illnesses № 2 Chelyabinsk state medical academies (Chelyabinsk); ph. 89080953916; doctor_sabirova@mail.ru. Маценко А.А., врач-интерн Челябинской государственной медицинской академии (г. Челябинск);тел. 89124747815. Matsenko A.A., the doctor-intern of the Chelyabinsk state medical academy (Chelyabinsk); ph. 89124747815. Волосников Д.К., доктор медицинских наук, профессор, заведующий кафедрой детских болезней № 2 Челябинской государственной медицинской академии (г. Челябинск); тел. 89226357674. Volosnikov D.K., the doctor of medical sciences, the professor managing chair of children's illnesses № of 2 Chelyabinsk state medical academies (Chelyabinsk); ph. 89226357674. Жуковская E.B., доктор медицинских наук, профессор кафедры детских болезней № 2 Челябинской государственной медицинской академии (г. Челябинск); тел.8-9517772655. Zhukovskaja E.V., the doctor of medical sciences, the professor of chair of children's illnesses № 2 Chelyabinsk state medical academies (Chelyabinsk); ph. 8-9517772655.