Introduction. Atypical teratoid/rhabdoid tumor (ATRT) of the central nervous system (CNS) belongs to the embryonic group, occurs mainly in children under 3 years of age and is characterized by an extremely aggressive clinical course and unfavorable outcome. However, there is not enough data about the heterogeneity of the clinical course of CNS ATRT and the role of clinical and therapeutic prognostic factors in patients under 1 year of age and 1–3 years of age.The aim of this study was to conduct a comparative evaluation of treatment results in children with CNS ATRT in the indicated age groups.Materials and methods. From 2008 till 2021 years 106 patients were included in this study. Each patient underwent a therapeutic program according to determined protocol (ATRT-2006, MUV-ATRT, EU-RHAB and individual variant). All patients were divided into 2 groups according to age: from 1 to 12 months – 41 patients, from 13 to 36 months – 65. Median of the age was 16 (9–23) months. These cohorts of patients (1–12 months and 13–36 months) were comparable in clinical and therapeutic characteristics depending on gender, localization of the primary tumor site, stage of the disease, extent of surgical resection, therapeutic protocol, number of patients, who underwent radiation therapy (RT), high-dose chemotherapy (HDCT) with autologous hematopoietic stem cell transplantation (auto-HSCT), intrathecal/ intraventricular chemotherapy.Results. At the time of analysis 48 (45.3 %) patients were alive, 58 (54.7 %) patients died, of whom 52 (90 %) from disease progression and 6 (10 %) from therapeutic complications. One-year progression-free survival (PFS) in the group from 1 to 12 months was 32 %, 2-year – 18 %, 5-year – 18 %; one-year overall survival (OS) – 53 %, 2-year – 29 %, 5-year – 25 % with a median follow-up 8 and 13 months respectively. One-year PFS in the group from 13 to 36 months was 61 %, 2-year – 36 %, 5-year – 33 %; one-year OS – 86 %, 2-year – 67 %, 5-year – 49 % with a median follow-up 19 and 38 months respectively. In our study we identified the main predictors of the PFS and OS improvement in patients with ATRT CNS of each age group. In the 1–12 month group, these factors were: absence of metastatic involvement, MUV-ATRT therapeutic protocol, RT and HDCT with auto-HSCT; in the 13–36 months group: gross total/near gross total resection of the primary tumor cite, ATRT-2006 therapeutic protocol and RT.Conclusion. Despite the fact, that the presence of a diagnosis of ATRT CNS in children under 3 years of age is usually associated with a high risk of disease progression and recurrence, the results of our study demonstrate the heterogeneity of the clinical and therapeutic profile in this age group of patients.
Relevance. Infectious septic complications caused by polyresistant gram-negative micro-organisms are a pressing issue in the treatment of patients after polychemotherapy (PCT) and hematopoietic stem cell transplantation at the high risk of the fulminant current and high lethality against the background of hematopoesis aplasia. One of the therapeutic strategies of antimicrobial treatment is the systematic use of 0.5 % hydroxymethylquinoxaline dioxide (dioxidine) solution in the complex antibacterial therapy of patients with severe infectious-septic complications. The preparation has a bactericidal type of action, a wide spectrum of antibacterial activity. Experience in adult clinical practice has demonstrated the effectiveness of dioxidine in the treatment of the most severe forms of aerobic and anaerobic infection. Strict dose enforcement and injection technique to avoid the appearance of side effects. Data on the intravenous use of dioxin in children are presented in a limited number of scientific literature.The aim of the study was to demonstrate the efficacy of systemic use of hydroxymethylquinoxaline dioxide (0.5 % dioxidine solution) in children with infectious complications progressing against the background of aplasia of hematopoiesis caused by multidrug-resistant pathogens Pseudomonas aeruginosa, Klebsiella pneumoniae, Enterobacter cloacae, Stenotrophomonas maltophilia.Materials and methods. 16 patients with a verified gram-negative infection were prescribed 0.5 % hydroxymethylquinoxaline dioxide solution as part of a combination antimicrobial therapy were included in the retrospective study. The median age of patients was 5 years (6 months – 16 years), 11 (69 %) were boys and 5 (31 %) girls.All children included in the study has infectious-septic complications at the PCT-induced hematopoietic aplasia, obtained according to the protocols of the main disease: severe combined immune deficiency (n = 2), idiopathic aplastic anaemia (n = 3), solid tumor (n = 2), acute myeloblastic leukemia (n = 7), acute lymphoblastic leukemia (n = 2). The main criterion for adding to the study was the existence at the least one site with a verified gram-negative infection (Pseudomonas aeruginosa, Klebsiella pneumoniae, Enterobacter cloacae, Stenotrophomonas maltophilia): bacteriemia (n = 11), oral mucosa (n = 6), ulcerative necrotic damage of perineum (n = 6), enterocolite (n = 6), infectionseptic compartments in the subcutaneous fat (n = 4), pleuropneumonia (n = 4), abscesses and inflammatory infiltration of the liver, spleen, pancreas, kidneys, lymph nodes (n = 1), infection of soft tissues in the area of the ventricular bypass with inflammatory changes of the brain membranes (n = 1).All patients received 0.5 % of the solution of dioxin by injection according of vital importance, as they had pathogens with confirmed laboratory resistance or clinical progression of the infectious process against the background of combined antibacterial therapy.Discussion. There is a complete control of fulminant developing infectious-septic processes caused by polyresistant micro-organisms against the background of hydroxymethylquinoxaline dioxide therapy in all 16 patients. The eradication of the pathogen, according to the microbiological study, has been confirmed in almost all observed patients, the efficacy of the drug has been preserved throughout the period of treatment, and the resistance of micro-organisms has not been observed. Strict adherence to the dosing and infusion technique of hydroxymethylquinoxaline dioxide has helped to achieve the full resolution of the infection process in all children without side-effects.Conclusion. On the basis of the experience presented, in immunocomputed patients of young age, 0.5 % dioxidine solution can be used as a necessary reserve preparation for the treatment of the most severe forms of infections of different localization, caused by polyresistant strains of gram-negative micro-organisms.
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