Announcement of the 2015 Rossiyskiy Zhurnal Detskoy Gematologii i Onkologii (Russian Journal of Pediatric Hematology and Oncology) issues 

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Announcement of the Sixth Interregional Meeting of the National Society of Pediatric Hematologists and Oncologists on Prospects for Pediatric Hematology/Oncology: A Multidisciplinary Approach

Diamond Blackfan anemia (DBA) is an inherited bone marrow failure syndrome. As with the other rare inherited bone marrow failure syndromes, the study of these disorders provides important insights into basic biology and, in the case of DBA, ribosome biology; the disruption of which characterizes the disorder. Thus DBA serves as a paradigm for translational medicine in which the efforts of clinicians to manage DBA have informed laboratory scientists who, in turn, have stimulated clinical researchers to utilize scientific discovery to provide improved care.

In this review we describe the clinical syndrome Diamond Blackfan anemia and, in particular, we demonstrate how the study of DBA has allowed scientific inquiry to create opportunities for progress in its understanding and treatment.

Question /Answer : An expert ’s opinion

We observed 567 patients with invasive aspergillosis in 19 hospitals of St.-Petersburg from 1998 to 2012 years. It is known that invasive aspergillosis is a severe opportunistic infection with high mortality. The most severe invasive aspergillosis we observed in children. We analyzed the risk factors, etiology, basic clinical signs and treatment of invasive aspergillosis in children.

This paper gives information on the structure of immunoglobulin molecules and the functions of each of them, as well as on the plasma levels of immunoglobulins. It provides basic requirements for the safety of intravenous immunoglobulins (IVVGs) and indications for their use in hematology/oncology and hematopoietic stem cell transplantation. Their doses, regimens, and possible adverse reactions are shown for a number of diseases. Moreover, the paper describes the experience with 10 % IVVGs in the federal center and gives brief clinical examples of the efficiency of using these agents.

Children with malignancies have a high risk of developing inanition for a variety of reasons associated with both a tumor and the toxicity of performed therapy. Enteral feeding via percutaneous endoscopic gastrostomy (PEG) is one of the procedures to correction the nutritional status. The authors show the feasibility and safety of PEG in children with solid malignancies, as well as the possibility of using PEG in patients receiving the cycles of intensive chemoradiation therapy. The paper analyzes the case histories of patients undergoing PEG to correct the nutritional status, as well as indications for its application, complications, and efficiency of this technique. Furthermore, a procedure for gastrostoma application and the specific features of management in patients with PEG are described in detail.

High-intensive treatment in pediatric hematology / oncology requires organization of active medical and psychosocial rehabilitation for the patients. Timely rehabilitation makes it possible to improve the results of therapy. In the article a concept of stepwise rehabilitation is discussed.

Retinoblastoma is the most common disease among pediatric ocular malignancies. The possibility of its successful treatment directly depends on the early initiation of treatment and on the extent of a tumor. The timely and accurate diagnosis of the illness is now an imperative factor for the early detection of the tumor. This review presents methods for the instrumental and laboratory diagnosis of retinoblastoma.

The clinical guidelines are a result of systematized knowledge and an analysis of practical experience in placing, using, and handling venous catheters. They reflect organizational issues and indications for catheter insertion, replacement, and removal. Main emphasis is laid on patent safety at all stages of work with the intravascular device. The major groups of complications to be prevented are identified. The strict observance of the sanitary standards and regulations, order and sequence of manual actions can assure the prevention of catheter-associated blood circulatory infection and intracatheter and intravascular thrombosis. The clinical guidelines contain the required minimum of information to make a hospital venous access protocol independently of the specific features of each therapeutic-and-prophylactic institution.

The paper describes the history and experience of the Saint Petersburg children’s hospice. It gives its main work areas, the standards and methods for palliative care to children.