[The analysis of the association of the polymorphic variants of the TPMT, COMT, and ABCC3 genes with the development of hearing disorders induced by the cisplatin treatment]. / Analiz assotsiatsii polimorfnykh variantov genov TPMT, COMT i ABCC3 c razvitiem narusheniia slukha, indutsirovannogo priemom tsisplatina.

Cisplatin and its derivatives are widely used chemotherapeutic agents for the treatment of many cancers, including hepatoblastoma, brain tumors, and germ-cell tumors. This therapy contributed to the dramatic increase in the survival rate. However, its use is restricted by the high incidence of irreversible ototoxicity associated with cisplatin application (in more than 60% of the children receiving it). Some studies have reported that genetic variants of TPMT (rs 12201199), COMT (rs4646316), and ABCC3 (rs 1051640) are conferring increased risk of developing cisplatin-induced hearing loss. However, in other studies the results were not replicated. In the present study, we replicated the previous studies based on an independent cohort of Russian patients. SNP genotypes for rs 12201199, rs4646316 and rs 1051640 were determined in DNA samples obtained from 16 patients who developed hearing loss and a group of 34 patients whose hearing was retained. The association between TPMT (rs 12201199), COMT (rs4646316), and ABCC3 (rs 1051640) variants and the hearing loss was not observed in our cohort.

[Invasive mucormycosis in patients with hemoblastosis in St.-Petersburg].

Thirty four patients with mucormycosis in 10 hospitals of St. Petersburg were observed in 2004-2013. The most frequent underlying diseases of mucormycosis were acute leukoses (64%). In 100% of the patients mucormycosis developed as a nosocomial infection. The risk factors, etiology, basic clinical signs and strategy in the treatment of mucormycosis were analyzed.

[Immunohistochemical study of small cell sarcomas and factors(nm23, CD44) predicting metastatic spreading in children]. / Immunogistokhimicheskoe issledovanie melkokletochnykh sarkom i factorov (nm23, CD44), prognoziruiushchikh ikh metastazirovanie u detei.

An immunohistochemical study was made of 43 tumors from children, which were classified as small blue round cell tumors (13 neuroblastomas, 13 rhabdomyosarcomas, 14 Ewing sarcomas/PNET, 2 undifferentiated sarcoma, 1 rhabdoid tumor). The use of a wide panel of antibodies confirmed (in 74% of the cases) and corrected (in 26%) the diagnosis of a diversity of small cell sarcomas established by routine methods. In neuroblastoma, the occurrence of distal metastases was associated with the loss of nm 23 protein. Increase in CD44 expression was more frequently observed in neuroblastomas with favorable prognostic signs and embryonic rhabdosarcomas that are not accompanied by metastases. This needs further study.

[Treatment results in children with B-cell lymphomas using a modified BFM protocol]. / Rezul'taty lecheniia detei s B-kletochnymi limfomami po modifitsirovannomu protokolu BFM.

AIM: Trial of the modified protocol BFM in the treatment of children with B-cell lymphomas. MATERIALS AND METHODS: 26 children with B-cell lymphoma were treated. Of them 2 children were treated according to the program for risk 2 group and 24 children were treated as risk 3 group. RESULTS: Complete remission was achieved in 23 patients. Two children were resistant. There were 2 cases of early recurrence, 1 case of early death, 1 case of death in remission. 20 patients are in complete remission. The 4-year survival is 78%. CONCLUSION: The modified protocol BFM proved to be highly effective against B-cell lymphoma in children. Its drawback is high toxicity.

[A trial of using allogeneic bone marrow transplantation in children with different hematologic neoplastic diseases]. / Opyt primeneniia allogennoi transplantatsii kostnogo mozga u detei s razlichnymi onkogematologicheskimi zabolevaniiami.

AIM: To define optimal time for transplantation of bone marrow (TBM) in children with hematological malignancies. MATERIALS AND METHODS: 20 allogenic TBMs were performed in children with acute myeloblastic leukemia (6 patients, 2 of them in recurrence), acute lymphoblastic leukemia (7 patients, 4 of them in recurrence), chronic myeloid leukemia (CML) in a chronic stage (3 patients), severe aplastic anemia (3 patients), generalized neuroblastoma (1 patient). Pretransplantation preparation included cyclophosphamide and busulphane or cyclophosphamide, busulphane and vepezide. The graft-versus-host reaction (GVHR) was prevented with cyclosporin A plus methotrexate or cyclosporin A plus urbazone. Engrafting was recognized by change of karyotype and blood group. RESULTS: From 13 children with acute leukemia subjected to TBM in a complete remission 4(33%) are alive, 5 died within 100 days after TBM (TBM was made in recurrence in 4 children), 3 patients died of recurrence 12 months after TBM. One patient with CML and one with severe aplastic anemia remain in remission. The main complications and causes of death in early posttransplantation period were hemorrhagic syndrome, infectious complications, GVHR. According to a one-year follow-up, the recurrent disease caused death most frequently. CONCLUSION: Positive result of TBM is related to the disease stage at transplantation.

[Neurogenic tumors of the mediastinum in children]. / Neirogennye opukholi sredosteniia u detei.

The age peak of neurogenic tumors of the mediastinum in children is from 2 to 7 years, malignant tumors being more frequently observed at the age before 2 years, benign tumors after 2 years of age. The method of choice in treatment of benign neurogenic tumors is operation. The selection of the method for treatment of malignant tumors depends on the stage of the disease: operative treatment is necessary for the I-II stages, the III-IV stages are better treated by radiation therapy and polychemotherapy. The survival of patients with mediastinum neuroblastomas made up 61.1%.