Interview with a guest editor of the Rossiyskiy Zhurnal Detskoy Gematologii i Onkologii (Russian Journal of Pediatric Hematology and Oncology), issue 1, 2015.

Academic workshop on the "Far Regions" program in the Republic of Northern Osetia – Alania: support point

Seminar within Far Regions program in the Chechen Republic: great opportunities.

The project of the scientific-educational seminars on Far Regions – new format.

Pediatric oncologist Kh. E. Khasmagomadova: “The father saved the books under fire”.

Start a contest for the best description of a clinical case “Case report – 2015”.

Opinion of Doctors of the Department of Clinical Oncology of the Federal Research Center of Pediatric Hematology, Oncology, and Immunology named after Dmitriy Rogachev at the 46 th Congress of the International Society of Paediatric Oncology (SIOP).

Ebola virus haemorrhagic fever is an acute infectious highly contagious diseases with high morbidity and mortality rate. Rapid development of clinical symptoms, severity of the disease and the mortality rate of up to 90 % require taking urgent medical measures already during the initial identification of the patient, of the quarantine measures and the development of preventive measures aimed at limiting spreading of the disease. Infectious agent is a virus of Ebolavirus of the Filoviridae family. First cases of the disease were revealed in 1976 in the west of Equatorial Province of Sudan with further in other countries of Africa. Bats and primates are natural reservoirs of the Ebola virus. The virus is transmitted from person to person from the first day of the onset of clinical symptoms. The patient remains contagious for several weeks after the onset of the disease. The virus has wide diversity of ways of transmission – through blood, nasopharyngeal mucus, urine, vomit, mucus of the genital tract of the patient. The incubation period is 3 to 21 days. The onset of the disease is acute: fever, chills, headache, muscle pain, maculopapular rash; in severe forms – the development of multiple organ failure. The death is caused with bleeding and / or shock. Diagnostics is based upon the analysis of epidemiological, clinical, and laboratory data. The combination of the analyses that determine the antigen or RNA and IgM or IgG antibodies is usually used. Polymerase chain reaction in real time or ELISA may be used to determint the antigen in blood, serum or homogenates of organs (presence of IgM antibodies indicates recent infection). No specific therapy has been developed as of the present moment. Therapeutic events boil down to pathogenetic and symptomatic treatment. No specific vaccine has been developed yet. The most important method of Ebola disease control is conducting control activities within the epidemic focus and beyond its borders to limit the spread of infection.

At present, endoprosthesis of bones and joints with the use of modular oncology prosthesis is the most common method of organ-preserving treatment. Since the moment of opening of the FCRC of PHOI named after Dmitriy Rogachev, over 20 surgical operations for endoprosthesis replacement have been performed in the department of surgery of children and adolescents. MSTS (Musculoskeletal Tumor Society Score) international scale was used for assessment of the functional result after the surgical therapy. The worst result in accordance with the scale comprised 50 %, the best result comprised 93 %. The average index was 76 %. The possibility of the intra-operational varying of the replacement volume of the postresection defect is a good feature of modular endoprosthesis that saves the surgeon from possible errors of the pre-operational planning and unpleasant findings in the course of the operation. This simplified performance of endoprosthesis replacement in conditions of a small medical center with little clinical experience in this field, particularly in the course of performance of operations for children. Endoprosthesis secures good onclological and functional results as well as favors the most adequate social adaptation of a child.

Introduction. Today, the problem of therapy of autoimmune diseases has acquired high relevance. The strategy of achievement of success is interaction of doctors of various specializations. One of the advanced approaches to treatment of this group of diseases is joining of efforts with hematologists and oncologists.

A variant of such interaction is an example of successful treatment of children with refractory forms of multiple sclerosis or cell therapy with the use of autologous transplantation of hematopoietic stem cells. The article represents a brief review of therapy of autoimmune diseases with the focus on observation of patients with multiple sclerosis.

Materials and methods. 13 patients with multiple sclerosis were included into the analysis. Distribution of sexes: 9 girls (69.2 %) and 4 boys (30.8 %). Age median: 16.7 ± 1.7 years. The median of duration of the disease prior to treatment is 15.5 ± 4.1 years. The age of onset of the disease is 4 to 14 years (12.3 ± 1.7 years). The average point in accordance with the Expanded Disability Status Scale (EDSS) is 6.16 ± 0.2 points. A severe refractory course of disease was registered with all patients. Until the moment of commencement of the therapy with the methods represented in the article, the patients received treatment with corticosteroids, interferon, plasmapheresis, and mitoxantrone with negative results. All patients in the group of autologous transplantation commenced the therapy with the signs of autoimmune inflammation. Transfusions of ex vivo cultivated T-regulatory cells were applied in the context of protocol regarding the cell therapy. The conditioning scheme with the use of the combination of cyclophosphamide (200 mg / kg) and atgam drug (160 mg / kg) was used in the case of performance of autologous transplantation.

Results. Remission without aggravation episodes was maintained with the patients that received therapy with autologous T-regulatory cells. Rapid improvement in the early post-transplantation period was registered with all children after auto-TGSC. The maximum improvement under the EDSS scale comprised 5.5 points. The article represented the experience of long-term observation of patients of this group (the maximum observation period is 60 months). No serious side effects were registered as a result of the therapy. Only 2 patients at late terms demonstrated repeated aggravation of the disease.

Conclusions. Thus, the presented methods of therapy demonstrated their efficiency in the context of refractory forms of multiple sclerosis and can be translated to other types of autoimmune diseases.

Despite the onset of neuroblastoma (NB) in most cases takes place at the age of 18 months, this solid tumor is one of the most common malignant neoplasms in the neonatal period. Epidural compression (EC) as a sign of distribution of the tumor process into the spinal channel is a very rare phenomenon among clinical manifestations of the congenital NB. Exactly rarity of this observed condition hardens differential diagnostics of the states manifested with availability of neurological disorders such as cuts (paralyses) during the neonatal period. A similar neurological situation can show the pathology of the nervous system most frequent in this age group, such as malformations, perinatal damage, ischemic and hemorrhagic changes in the spinal arteries, certain genetically determined diseases of the neuromuscular apparatus. In most cases, the main factor that unfavorably affects recovery of movement functions of a child us the EC duration until the moment of commencement of treatment. At the same time, children have favorable oncologic forecast with the congenital NB and EC: The 5‑year survival rate comprises up to 86.2 %. The main objective of this publication is to highlight a rare clinical case; problems of differential diagnostics of neurological status with malformations and perinatal lesions of the nervous system; as well as the problems associated with the conduct of this group of patients. Exactly correctly and timely EC diagnostics shortens the interval from the moment of setting the diagnosis to the commencement of the operation. Suspect availability of malignant neoplasms of the fetus, as well as the development of neurological disorders, possibly with the help of screening methods for diagnosis – ultrasonic examination. Starting from the III trimester of pregnancy, it is possible to visualize paravertebral hyperechoic masses penetrating into the spinal canal. Decreasing of movement activities in extremities in the course of this process is the sign of EC.

EC therapy caused with NB include poliochemotherapy, surgical intervention (laminotomy and laminectomy), and radiation therapy. The selection of therapeutic approach remains individual for each oncologic in-patient department due to certain factors. Surgical intervention as the preferred method of treatment aimed at rapid withdrawal of symptoms of spinal cord compression is associated with the risk of postoperative deformities of the spine, retarded growth, prolonged bed rest period, and, possibly, subsequent reconstructive surgery on the spinal column. Nevertheless, surgical intervention is required in the case of rapidly growing neurological deficiency, return of neurological symptoms or absence of tumor shrinkage on the background of chemotherapy.

In most cases, chemotherapy is performed in accordance with NB-2004 protocol, however, daily monitoring of the neurological status is required for therapy correction and, if necessary, settlement of the issue of operative intervention.

Radiation therapy is very rarely used for treatment of the NB that caused EC due to high risk of development of secondary malignant neoplasms. Irrespectively of selection of the therapeutic approach to treatment of the EC caused with NB, permanent motor deficiency was observed in 50 % of cases. Disorders of functions of pelvic organs were also observed. In order to achieve a favorable outcome in terms of improved neurological status, the attention should be primarily focused on timely diagnosis and the choice of therapy in order to reduce the duration of the EC, which, in turn, has a major influence on the motor deficiency.

This article represents a clinical case of development of congenital NB of a child during their first days of life accompanied with the clinic of the spine EC. The issues of differential diagnostics of pathological states of the early infancy period revealed with neurological symptoms were reviewed. On the reasons of arising of such issues may be NB. The issues of the clinic, diagnostics, treatment, and observation of such patients were reviewed separately.

Malignant neoplasms of the liver comprise 1.3 % in the structure of malignant neoplasms of children at the age of 0 to 14 y. o. The most frequently met malignant neoplasms of the liver of children are hepatoblastomas. The morbidity rate of hepatoblastomas comprises 0.1 per 100 thousand of children's population at the age of 0 to 14 years old. The basis of therapeutic strategy of patients with hepatoblastomas is the riskadapted approach that includes the assessment of such factors, such as the stage of disease under the PRETEXT (Pre-Treatment Extent of Disease) system and the level of alpha-fenoprotein (AFP). We use protocols of the International Childhood Liver Tumors Strategy Group (SIOPEL) in our practice. In accordance with recommendations of the SIOPEL group, patients with hepatoblastomas are stratified into the low risk group and high risk group. Patients in the low risk group receive therapy with the use of cisplatin (totally, 6 injections). Patients with hepatoblastomas in the high risk group receive therapy with the use of cisplatin, carboplatin, and doxorubicin. It must be noted that hepatoblastomas, unlike many types of malignant neoplasms of children, produce AFP and is attributed to “secreting” tumors. Increasing of the AFP level with hepatoblastomas during the onset of the disease is marked in 90 % of cases. AFP is both a diagnostics marker with hepatoblastomas and a marker of response to the therapy performed. Assessment of the AFP level is used for revealing of disease recurrences with dynamic observation of the patients that have completed the specific therapy. The possibility of using AFP level for early revealing of disease recurrences allows significantly decreasing of the number of imaging studies used in programs of follow-up observation, unlike other, "nonsecreting" types of malignant neoplasms of childhood. A whole number of performed international studies demonstrated increasing of the risk of development of malignant neoplasms associated with the radiation received in the course of X-ray diagnostics and computed tomography. Thus, in the course of making up the protocols of observation (imaging) of the patients that have completed the treatment associated with malignant neoplasms, it is necessary to take into consideration all possible risks that include both risks of development of tumor recurrences and the risk of development of severe late consequences including those caused with excessive imaging studies. In our article, we have provided the protocol of observation of patients with hepatoblastomas that have completed the specific therapy including the patients that have undergone transplantation of the liver. This protocol is based upon recommendations of the SIOPEL group. Determination of the AFP level in blood of patients with hepatoblastomas, X-ray diagnostics of the chest organs, and ultrasonic study of the abdominal organs are the major imaging methods used for controlling of disease recurrences. Besides, monitoring and early revealing of late effects of treatment are important in the course of clinical examination of children and adolescents after malignant neoplasms. The article reviews the problems of organ toxicity caused with chemotherapy included into the schemes of treatment of patients with hepatoblastomas. Taking into consideration the specter of these chemical drugs, an important factor is monitoring of such side effects as nephrotoxicity, ototoxicity, and cardiac toxicity that require long-term observation. The volume of examination and resolution of examination is represented in the form of tables for patients of groups of low and high risk, and separately for the patients after the liver transplantation.

This material submits information on Department of Oncologic Haematology of CRI “Children’s Clinical Hospital” as one of the Russian leading regional centers majoring in children’s haemotology and oncology.

The history of the Department began in 1988, when a detached department was opened within Children’s Hospital of Simferopol, managed by V.P. Usachenko. The Department contributed to implementation of various chemotherapy protocols within the cooperation between the international research groups. The history of Department development is remarkable by the names of western scientists – D. Pinkel, G. Shellong, А. Righter and others. In 2014 the Department was visited by the Prime-Minister of the Russian Federation D. Medvedev and Ministry of Health V.I. Skvortsova. The article sets out data on number of beds and medical staff of the Department, adult and children population in the Republic of Crimea, incidence of haemotological diseases for the recent years.

It sets out details of 5-year relapse free survival for: acute lymphoblastic leukemia – 78 %, acute myeloleukemia – 55 %, non-Hodgkins lymphoma – 78 %, Hodgkin's lymphoma – 95 % and gives an information on the charitable entities helping people.