Vol 16, No 3 (2017)
- Year: 2017
- Published: 10.08.2017
- Articles: 14
- URL: https://hemoncim.com/jour/issue/view/11
The results of pilot trial ALL-REZ-MВ-2014 for children with relapsed acute lymphoblastic leukemia
Abstract
The relapse of acute lymphoblastic leukemia (ALL) in children is still a difficult task. This is due both to the difficulties of achieving a second remission and to the problems of organizing allogeneic bone marrow transplantation (BMT) in those patients who need it. The standard approach is the use of second-line drugs in the regime of high-dosage chemotherapy blocks, but the response rate for this treatment in patients with early relapses remains low and the response time is short. Recently, new drugs with different mechanisms of action have appeared that can give an opportunity for a full remission. The organization of a multicentre study on anti-relapse therapy in the Russian Federation is an urgent task, the solution of which can help in studying new approaches to therapy and in the organization of BMT logistics. This article presents the results of a pilot multicentre study on the treatment of relapses of ALL in children in the Russian Federation. The effectiveness of standard therapy for a group with late relapses and the efficacy and toxicity of interventional blocks with the use of bortezomib, clofarabine, nelarabine are presented.



Minimally invasive surgery treatment of children with abdominal neuroblastoma
Abstract
Neuroblastoma is the most common extracranial solid tumor of pediatrics, with the most favorable prognosis in children under 1 year. In the Dmitry Rogachev National Research Centre of Pediatric Hematology, Oncology and Immunology radical endosurgical treatment of the neuroblastoma abdominal localization received 39 patients. Local relapses were not observed with the median follow-up time of 36 months. Due to the positive results of endosurgical procedures laparoscopic approach may be the procedure of choice for localized forms of abdominal neuroblastoma without image-defined risk factors.



MRI T2*-mapping for liver iron assessment in pediatric patients with secondary iron overload
Abstract
Iron accumulation assessment in the body is important for iron overload determination in parenchymal organs and also necessary for planning and monitoring the chelation therapy. In this article, we demonstrate non-invasive diagnostics of iron accumulation in the liver using magnetic resonance imaging. MRI investigation includes 55 patients (2–18 years, median 8 years) with secondary iron overload due to regular blood transfusions. Image analysis was conducted by special program of signal decay evaluation for automatically T2* values calculation in liver. We have received a linear regression equation between the biopsy data and T2* values and have obtained the grading system of T2* values based on siderosis grades. Non-invasive MRI diagnostics in patients with iron overload can introduce new approaches for quantitative estimation of body iron deposition.



The usage of superimposed high-frequency jet ventilation in the treatment of acute respiratory distress syndrome in children with oncohematological diseases
Abstract
The treatment of acute respiratory distress syndrome (ARDS) in children with oncohematological diseases remains one of the most difficult problem of the intensive care. The present study was performed to assess the potential of the usage of superimposed highfrequency jet ventilation (SHFJV) in the treatment of ARDS in children with oncohematological diseases. High-frequency ventilation was performed with TwinStream™ (Carl Reiner, Austria) apparatus in the superimposed high-frequency jet ventilation mode. 13 cases of ARDS treatment were analysed. High-frequency ventilation was indicated in cases of a confirmed ARDS or the inefficacy of conventional mechanical ventilation: peak airway pressure (Ppeak) > 30 mm Hg, FiO2 – 100%, SpO2 < 92%, pCO2 > 50 mm Hg. SHFJV was conducted for 2–12 days (5±3 days on average). 10 patients (76,9%) showed an improvement in lung X-ray, increased levels of РаО2 > 100 mm Hg, РаО2/FiO2 > 200, a reduced fraction of inspired oxygen (FiO2), the optimization of SHFJV parameters with a subsequent switch-over to traditional ALV techniques and extubation. Five out of 13 patients died. The first results of SHFJV application in oncohematological pediatric patients with severe ARDS showed the efficacy of this alternative method used when adequate gas exchange cannot be achieved with traditional methods of lung ventilation.



Implementation of the algorithm of empirical antibacterial therapy for febrile neutropenia in the Сenter of pediatric hematology/oncology
Abstract
Febrile neutropenia is a life-threatening condition that requires the immediate administration of broad-spectrum antibiotics. On the other hand, the most serious problem of medicine is the continuously growing resistance of microorganisms to antibiotics. It is known that the main reason for this phenomenon is the irrational and excessive use of antibacterial drugs in the hospital. To make treatment of infectious complications more effective, optimize use of antibiotics and reduce resistance of microorganisms in Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology (Center) the algorithm for empirical antibacterial therapy of febrile neutropenia was developed. Analysis of early results showed a reduction in the consumption of all classes antibiotics in the intensive care unit (ICU) a year after the start of the algorithm. After three years, it was possible to reduce the consumption of β-lactams, fluoroquinolones and linezolid throughout the Center. When analyzing the level of resistance to antibiotics, its decrease for such pathogens as Kl. pneumoniae, Ps. aeruginosae and Ent. Cloacae was revealed. To obtain other results of the algorithm, further detailed analysis is needed.



Dynamics of the stabilometric parameters in children with the opsoclonus myoclonus syndrome in carrying out various rehabilitation methods
Abstract
Optimization of the choice of rehabilitation methods in children with the oploskonus myoclonus syndrome on the basis of the analysis of stabilometric parameters. The study included 48 children of both sexes in the age range from 3 to 7 years with the diagnosis of «opsclonus-myoclonus syndrome». All patients were separated into 4 groups according to applied rehabilitation methods: in the first group (12 patients) patients received craniosacral therapy, in the II group - modified Vojta therapy (13 patients), in the third group patients underwented an isolated course of Galileo vibration therapy (10 patients), in the fourth group patients received modified Vojta therapy together with Galileo vibration therapy (13 patients). Patients underwented the course of craniosacral therapy demonstrated the increases rates of speed (V) in 1.48 times and statokinesiogram square in 1.94 times after fifth procedure as compared with data before. In the second group 1.32 times speed decrease (V) and 1.56 statokinesiogram square decrease (S) were marked. In the third group there were 1.73 times statokinesiogram square increase (S) and the displacement of pressure center midposition in frontal plane (X) to the left after implementation of 10 manipulation. In the fourth group 1.42 times speed decrease (V) and 2.7 times statokinesiogram square decrease (S) were marked after implementation of 10 manipulation, as well as displacement of pressure center midposition (X) in frontal plane to the right and tendency to displacement of pressure center in sagittal plane to the front after conducted manipulations. Modified Vojta therapy together with Galileo vibration therapy can be applied in the capacity of most effective rehabilitation methods for children with opsoclonus-myoclonus syndrome leading to useful increase of stabilometric parameteres.



The use of plerixafor and G-CSF during conditioning for hematopoietic stem cell transplantation in a patient with Wiscott–Aldrich syndrome
Abstract
High incidence of impaired graft function remains a significant issue in patients with Wiskott–Aldrich Syndrome after hematopoietic stem cell transplantation (HSCT). We report a clinical case of graft rejection and second HSCT with novel alternative approach to the conditioning regimen. We used of G-CSF and plerixafor as additional agents in conditioning regimen for stem cell release and opening of bone marrow niches to improve stem cell engraftment.



The Peitz–Eggers' syndrome as the cause of iron deficiency anemia in a 5-year-old child: the diagnostic value of capsule enteroscopy
Abstract
This clinical case demonstrates the diagnostic value of capsule endoscopy for the detection of polyposis of gastro-intestinal tract (Peutz–Jeghers syndrome). We report the case of a 5-year-old male presenting with a chronic iron refractory iron deficiency anemia. Multiple polyps of the stomach and small intestine with an erosive and hemorrhagic component were identified by the method of capsule endoscopy.



Hyperostosis of facial bones as a sign of beta-thalassemia in pediatric patients
Abstract
One of the frequent manifestations of beta-thalassemia is the defeat of the bones of the skeleton in the form of local or diffuse hyperostosis, which may lead to a number of neurological and osteoarticular complications. The authors demonstrate pathognomonic changes in the bones of the facial skeleton in beta-thalassemia, and discuss the features of the radiation pattern and the differential diagnosis of this pathology.



Dynamics of platelet functional activity and hemostatic status in a child with chronic immune thrombocytopenia on romiplostim treatment
Abstract
Immune thrombocytopenia (ITP) is characterized by changes in both quantity and quality of platelets. As a result, severity of bleeding does not always correllate with the degree of thrombocytopenia, The quality of platelets on treatment becomes difficult to evaluate. The most promising method to investigate quality of platelets in ITP is flow cytometry. Here we describe a clinical case of monitoring of a pediatric ITP patient status on romiplostim treatment for 50 weeks using flow cytometry for platelet functional activity control.



Congenital immature teratoma of head and neck: case report
Abstract
The clinical case of a giant congenital germ cell tumor (immature teratoma, 2 grade) of soft tissues of the head and neck with destruction of the large wing of the sphenoid bone, with spreading to the oral cavity with the transition beyond the middle line, infiltration of the tongue, complete obstruction of the nasopharyngeal the child 15 days of life. The article describes Specific features of performing surgical intervention are described taking into account the prevalence of the tumor process. The discussed issues of teratoma classification, methods of diagnosis, provision of modern principles of therapy for patients with this disease.



Синдром задней обратимой энцефалопатии у пациента с острым миелобластным лейкозом



Inherited disorders of iron metabolism
Abstract
The presented review provides information about genetic diseases associated with impaired iron metabolism, includes the main aspects of normal iron metabolism, the mechanisms of its disruption for each described pathology. The review also presents information about major clinical manifestation, changes in laboratory parameters and the principles of therapy.



Dabigatran etexilate: a new oral anticoagulant
Abstract
The article presents a review of the data about a new oral anticoagulant dabigatran etexilate (DE), which is the pro-drug, but in the body converts after oral administration in the active compound, a direct low-molecular weight thrombin inhibitor dabigatran. The major properties of dabigatran and DE, pharmacokinetic parameters of the preparation and its interactions with the other drugs are examined. Efficiency and safety of this preparation's clinical use in different pathological states are discussed also. The review of pharmacodynamics and the methods for measurement of the DE anticoagulant activity are presented. The possible advantages of DE compare to other anticoagulants are discussed, also as the questions of clinical trials of DE for using in pediatric population.


