Vol 14, No 4 (2015)

Cover Page

ONCOLOGY

Ommaya reservoir for intraventricular chemotherapy of children with neuro-oncological and oncohematological diseases: Design history, implantation technology, and experience gained at the Federal Research Center of Pediatric Hematology, Oncology, and Immunology named after Dmitry Rogachev

Ozerov S.S., Melnikov A.V., Samarin A.E., Tereshchenko G.V., Kumirova E.V., Myakova N.V., Grachev N.S., Zheludkova O.G.

Abstract

Ommaya reservoir is a convenient and reliable port for intraventricular injection of chemotherapeutic drugs in patients with oncohematological and neuro-oncological diseases. A total of 45 Ommaya reservoirs were implanted in 45 patients at the Federal Research Center of Pediatric Hematology, Oncology, and Immunology named after Dmitry Rogachev (Moscow) in 2012-2015. Because of small ventricles in most cases Ommaya reservoirs were implanted by stereotactic procedure. Intraventricular injection of methotrexate supported a high and stable concentration of the drug in the cerebrospinal fluid and proved to be a good alternative to intrathecal administration. In hydrocephalus Ommaya reservoir could be combined with pressure-regulated ventriculoperitoneal shunt, this providing a high concentration of methotrexate in the cerebrospinal fluid, safe for the patient.
Pediatric Hematology/Oncology and Immunopathology. 2015;14(4):5-9
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PALLIATIVE CARE

Management of chronic (persistent) pain syndrome in children

Kumirova E.V.

Abstract

Based on the international practice and recommendations, the author discusses the problems in management of chronic (persistent) pain syndrome and describes the use of analgesics - opioid and non-opioid - in children. Analgesics and their forms registered in Russian Federation, available for children, are listed.
Pediatric Hematology/Oncology and Immunopathology. 2015;14(4):10-16
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CLINICAL OBSERVATIONS

Diagnosis of Glanzmann’s thrombasthenia by assessment of plasma and platelet hemostatic parameters

Kumskova M.A., Demina I.A., Podoplelova N.A., Balandina A.N., Seregina E.A., Bondar E.V., Poletaev A.V., Konyashina N.I., Panteleev M.A.

Abstract

Glanzmann's thrombasthenia (GT) is a hereditary disorder referred to the group of thrombocytopathies. It develops as a result of abnormality in the platelet structural protein, leading to deficiency or dysfunction of membrane protein integrin aIIbß3. Clinically the disease is characterized by spontaneous and posttraumatic bleedings. A typical pattern of platelet aggregation in GT is the absence of aggregation with all agonists and normal response in the presence of ristocetin. A clinical case of GT is described: a boy aged 3 years 10 months with a characteristic clinical manifestation of the hemorrhagic syndrome. Stages in the disease diagnosis and results of laboratory assays of the hemostatic system are described in detail. In addition to evaluation of the platelet aggregation activity, the functional activity of these cells was assessed by flow cytometry, which showed reduction of integrin aIIbß3 - quantitative (76.4% lower expression of CD61 in assessment of non-activated platelets and 88% lower in assessment of activated platelets) and qualitative (34% lower expression of PAC1 in non-activated platelets and 79.3% lower in activated platelets) in comparison with healthy donors.
Pediatric Hematology/Oncology and Immunopathology. 2015;14(4):17-24
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Clinical manifestations and laboratory features of acute parvovirus infection in children with oncohematological diseases (Review of literature and clinical case reports)

Roppelt A.A., Vasilyeva A.P., Kalinina I.I., Baidildina D.D., Suntsova E.V., Salimova T.Y., Petrova U.N., Fisyun I.V., Raikina E.V., Goronkova O.V., Maschan M.A., Maschan A.A.

Abstract

Parvovirus B19 (РВ19) is an ubiquitous single strand DNA-virus, which in healthy persons causes the so-called fifth disease -benign infectious exanthema. However, in patients with disturbed erythropoiesis or immune deficiencies PB19 infection can lead to potentially life-threatening syndromes. Three pediatric patients (females) with primary PB19 infection are described. One patient with hereditary spherocytosis developed a classical transitory aplastic crisis which required several blood transfusions. In a second patient with acquired aplastic anemia and high titers of PB19 in peripheral blood, long-lasting fewer, and rash hematopoietic stem cell transplantation had to be postponed for 2 weeks because of the risk of graft function suppression. In a third patient high titers of PB19 in bone marrow resulted in long-lasting aplasia and delay of scheduled intensive chemotherapy and hence, in relapse of underlying acute myeloid leukemia. Importantly that 2 of 3 patients (the second and the third one) received platelet transfusions from the same donor. So far, the problem of diagnosis, treatment and prevention of PB19 infection in patients with hematological/oncological diseases remains unresolved and needs further discussion.
Pediatric Hematology/Oncology and Immunopathology. 2015;14(4):25-31
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Methemoglobinemias in children (Review of literature and a clinical case report of a child with hemoglobin M Saskatoon)

Usryugov A.Y., Kazanets E.G., Zakharova G.S., Plyasunova S.A., Sadelov I.O.

Abstract

A rare clinical case is discussed: congenital structural methemoglobinemia caused by hemoglobin M Saskatoon. The main clinical and laboratory markers of methemoglobinemia are described in detail. Photographs, hemolysate electrophoregram, and hemoglobin chain chromatogram are presented.
Pediatric Hematology/Oncology and Immunopathology. 2015;14(4):32-36
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Bilateral diffuse hyperplastic perilobar nephroblastomatosis: A case report

Chililova A.M., Kachanov D.Y., Mitrofanova A.M., Konovalov D.M., Sukhov M.N., Tereshchenko G.V., Shamanskaya T.V., Varfolomeeva S.R.

Abstract

Nephroblastomatosis is a rare congenital preneoplastic lesion associated with high risk of nephroblastoma (Wilms' tumor). A case report is presented: bilateral diffuse hyperplastic perilobar nephroblastomatosis in a 10-month-old infant. The classification of nephroblastomatosis, differential diagnosis of nephroblastomatosis and nephroblastoma, and current therapeutic strategies in patients with nephroblastomatosis are discussed.
Pediatric Hematology/Oncology and Immunopathology. 2015;14(4):37-43
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Development of neuroblastoma in a child with Shereshevsky-Turner’s syndrome

Shevtsov D.V., Kachanov D.Y., Shamanskaya T.V., Muftakhova G.M., Olshanskaya Y.V., Kazakova A.N., Tereshchenko G.V., Roshchin V.Y., Semenova N.S., Novichkova G.A., Varfolomeeva S.R.

Abstract

Neuroblastoma (NB) is the most incident extracranial solid tumor of children aged 0-14 years. The tumor is associated with many genetic syndromes predisposing to its development. The authors present a clinical observation of NB development in a child with Shereshevsky-Turner's syndrome.
Pediatric Hematology/Oncology and Immunopathology. 2015;14(4):44-48
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IMAGES

Extrapontine myelinolysis in a patient with rhabdomyosarcoma

Tereschenko G.V., Andrianov M.M., Moiseenko P.A., Politova E.A., Khomyakova S.P., Balashov D.N., Kachanov D.Y.
Pediatric Hematology/Oncology and Immunopathology. 2015;14(4):49-49
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INFORMATION

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DISSERTATION STUDIES

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Instructions for Authors

Article E.
Pediatric Hematology/Oncology and Immunopathology. 2015;14(4):59-60
pages 59-60 views