Diagnosis and classification of mitral valve prolapse in children and adolescents

We examined 1734 children and adolescents with admittance diagnosis mitral valve prolapse (MVP) and confirmed its presence using accepted Framingham criteria in 32.3% of cases. Reason for overdiagnosis in other cases was incorrect interpretation of auscultation or echocardiography data. Most often MVP was familial with recessive or dominant inheritance according to genealogical analysis. Contrary to MVP in adults children and adolescents commonly had no myxomatous transformation of valves. Taking into account constitutional and physiological peculiarities of the body in children and adolescents we distinguished two forms of MVP characteristic for this age: MVP as a variant of asthenic constitution and physiological MVP. Both forms are widely spread and represent normal states for juvenile population.     

First experience with the modified program NHL-BFM-90 application in adult patients with primary diffuse large B-cell gastric lymphosarcoma with unfavourable prognosis

AIM: To assess efficacy of a modified program NHL-BFM-90 in adult patients with primary diffuse large B-cell gastric lymphosarcoms (PDLBGL) with unfavourable prognosis. MATERIAL AND METHODS: Modified courses of NHL-BFM-90 were conducted in 5 patients aged 27-67 years from January 2004 to September 2005. Four patients received chemotherapy of the first line, in one patient block therapy followed monotherapy with chlorambucil and a CHOEP course. All the patients were in a severe clinical condition and had several initial factors of unfavourable prognosis: size of the tumor more than 10 cm; stage IE and more advanced; B-symptoms; proliferative activity above 70%. The program NHL-BFM-90 was modified because of the patients' age. Chemotherapy was conducted according to the middle arm of the original program NHL-BFM-90, but methotrexate was introduced in a dose 1 g/m2 for 12 hours, while leukovorin was given 18 hours after the start of methotrexate injection. In two cases the blocks were enhanced with rituximab, 2 patients had doxorubicin in block A, in one case block C was enhanced with methotrexate. A total of 23 modified blocks NHL-BFM-90 were performed: one patient was given 6 blocks, two patients--5, one patient--4 blocks and one patient--3 blocks. RESULTS: Four patients after block 2 and one patient after block 3 of polychemotherapy NHL-BFM-90 achieved remission of the disease of 6 to 22 months duration which still continues. Infectious complications related to hematological toxicity arose more frequently at the latest courses of chemotherapy. CONCLUSION: Treatment according to the modified program NHL-BFM-90 in adult patients with PDLBGL and unfavourable prognosis is highly effective. For a mean follow-up of 10.2 months no recurrences occurred. The number of courses can be reduced to decrease accumulated hematological toxicity and in case of rapid achievement of remission.     

International prognostic index in diffuse B large cell lymphosarcoma

AIM: To evaluate efficacy of the International Prognostic Index (IPI) in relation to diffuse B-large cell lymphosarcoma (BLCLS), to identify significant prognostic factors. MATERIAL AND METHODS: The trial enrolled 121 patients (67 males and 54 females, mean age 54 years) suffering from BLCLS with involvement of the lymph nodes, spleen, tonsils. The diagnosis was made according to WHO criteria. 83 patients (29 with local lesion of lymph nodes, 10 with primary lesion of the tonsils, 9 with primary lesion of the spleen and 35 with generalized lesion) received polychemotherapy (PCT): CHOP-21, R-CHOP-21. Patients with primary splenic lesion have undergone splenectomy followed by CHOP-21 PCT, radiotherapy on the splenic region and that of regional lymph nodes. Radiation therapy was also given to patients with stage I-II, 38 patients received NHL-BFM-90 PCT. RESULTS: In distribution of patients into prognosis groups according to IPI 5-year event-free survival on CHOP-21 and R-CHOP-21 therapy was 83% in the group of low risk, 79% in the group of low/intermediate risk, 52% in the group of high/intermediate risk, 34% in the group of a high risk. However, there were poor outcomes in the group of low risk (recurrence, resistance) and long-term persistent remissions in high risk groups. Frequency and duration of complete remissions depended on location of the primary lesion. Five-year event-free survival in patients with solitary lesion of the peripheral lymph nodes on CHOP-21, R-CHOP-21 in spite of their size (bulky) and high concentration of lactate dehydrogenase) was 97% while in tonsillar lesion (according to IPI all the patients entered groups of low and low/intermediate risk) - 50%. All the patients with primary splenic lesion according to IPI had high risk but after splenectomy and PCT (CHOP-21, R-CHOP-21) followed by radiotherapy they demonstrated 100% 5-year event-free survival (follow-up since 1998). CONCLUSION: Determination of prognosis groups in BLCLS is not valid as such distribution gives no grounds for choice of adequate therapy. A decisive prognostic factor is now site of the lesion.     

Specific features of the incidence of ischemic heart disease in Dagestan

The authors analyze the culture, socio-economic and communal characteristics of life style in Daghestan, determining the population health in this republic. Recommendations on developing the strategies for prevention of coronary disease in the Republic of Daghestan are offered.