Introduction. Immune thrombocytopenia (ITP) is a benign hematological disease characterized by an isolated decrease in platelet count, with different course options requiring both an urgent therapeutic decision and clinical observation of patients. At the present stage in the treatment of children with immune thrombocytopenia, especially with the chronic form, significant success has been achieved. Therapeutic options are determined by an individual approach to the patient and are based on the experience of a hematologist. Materials and methods. For the period from 01.01.2013 to 12.31.2017, in the Oncohematological Department of Chemotherapy of the Voronezh Regional Children’s Clinical Hospital № 1, 153 children with various forms of ITP, aged between 1 and 6 months to 17 years, received treatment. Diagnosis and stage of the disease were carried out on the basis of modern clinical guidelines and ITP classification. The analysis of the severity of hemorrhagic syndrome in the diagnosis of ITP was carried out. The principles of patient therapy were based on the pathogenetic aspects of ITP, the stage of the disease. Patients with newly diagnosed ITP (n = 106; 69.4 %), persistent form (n = 26; 16.9 %) received different lines of therapy, provided for by clinical guidelines, some patients were only under the supervision of a hematologist. The chronic form of the course of the disease was formed in 21 (14 %) children out of 153, regardless of the type of treatment. Children with the chronic form received various lines of therapy, including thrombopoietin agonists. The decision to manage such patients was based on a joint discussion with specialists from the largest federal centers. Splenectomy for the above period was performed for 1 child with a pronounced and chronic “wet” component of the disease. Discussion. Treatment of ITP in children, despite the emergence of a new class of drugs with a targeted effect, in some cases is a difficult task for a hematologist. The adoption of a specific therapeutic decision is based on the severity of the clinical and hematological data of the patient, the search for a possible cause of the thrombocytopenic condition, as well as the individual experience of the doctor. Prevention and prevention of bleeding in patients with different ITP, in particular with chronic ITP, with the absence or minimal manifestation of hemorrhagic syndrome according to the principle “0 bleeding per year” is the ultimate therapeutic task. Conclusion. ITP in children, in particular its chronic form, belongs to orphan diseases, therefore, the experience of managing such patients may be useful in determining the overall tactics of treating patients.
Diamond–Blackfen Anemia (DBA) is a rare, clinically and genetically heterogeneous disorder from the group of congenital syndromes of bone marrow failure. The purpose of this work is to identify the main medical-frequency characteristics of DBA (incidence, prevalence, mortality, cartographic analysis) in children in the Russian Federation during the observation period 2011–2016. The study was approved by the Independent Ethics Committee of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology. The Russian register of DBA patients, which had been developed and maintained since 2012 year by NMITS DGOI, was used to obtained epidemiological data and their analysis. At the end of 2018 year 141 patients from 137 families were included in the register. The average annual incidence rate of children with DBA for the Russian Federation was 0.63 ± 0.034, the average annual prevalence rate – 5.75 ± 0.87 per 100 thousand newborns born alive; mortality rate – 2.12%; the cartographic method of research showed that the largest number of patients was registered in the Central Federal District of the country, which is explained by the high population living in this district.
Воронежская областная детская клиническая больница № 1, Воронеж, Российская Федерация
named after N.N. Burdenko, 2 regional children's clinical hospital № 1, Voronezh, Russia РЕФЕРАТ ЦЕЛЬ. Оценка частоты и течения острого повреждения почек (ОПП) у детей на различных этапах терапии лейкозов. ПАЦИЕНТЫ И МЕТОДЫ. В исследование было включено 143 ребенка с различными вариантами лейкоза, получавшими лечение на базе онкогематологического отделения химиотерапии Воронежской областной детской клинической больницы №1 за период с 01.2008г. по 01.2014г. Наблюдение проводилось на 4-х этапах: до назначения полихимиотерапии (ПХТ), на этапе интенсивной ПХТ, в период поддерживающей ПХТ и на этапе после окончания лечения в периоде стойкой ремиссии. ОПП диагностировали с учетом уровня скорости клубочковой фильтрации (СКФ) и креатинина сыворотки крови, превышающих нормальные значения как минимум в 1,5 раза (на 25%). РЕЗУЛЬТАТЫ. ОПП диагностировано у 109 детей (76,2%) из 143 обследованных, причем стадия риска ОПП (Risk) выявлена у 44 детей (30,8%), стадия повреждения (Injury)-у 46 детей (32,2%), стадия недостаточности (Failure)-у 19 детей (13,3%). ОПП отмечалось на всех этапах терапии, но максимально часто (у 77 из 89 детей, 86,5%)-на этапе интенсивного лечения. У 16 из 30 детей (53,3%), наблюдавшихся на всех этапах терапии, ОПП различных стадий регистрировалось неоднократно, максимально до 4 раз. За период наблюдения летальный исход наблюдался у 20 пациентов, в основном в периоде интенсивной ПХТ, у всех было ОПП средней и тяжелой степени тяжести: 8-стадия Injury, 12-стадия Failure. ЗАКЛЮ-ЧЕНИЕ. ОПП достаточно часто регистрируется у детей с лейкозами, максимально часто на этапе интенсивной ПХТ. Развитие ОПП ухудшает прогноз лейкоза у детей в отношении выживаемости. У одного и того же пациента возможно неоднократное развитие ОПП на разных этапах лечения. Ключевые слова: острое повреждение почек, лейкозы, дети. ABSTRACT THE AIM. Evaluation of frequency and course of acute kidney injury (AKI) in children with leukemia at the different stages of therapy. PATIENTS AND METHODS. In our study we included 143 children with different variants of leukemia receiving polychemiotherapy (PCHT) at oncohematolological unit of VRCH №1 for the period from 01.2008 to 01.2014 year. The observation was conducted at 4 stages: before polychemiotherapy (PCHT), during intensive PCHT, during maintaining PCHT, after the end of the treatment during stable remission. AKI was diagnosed with regard to the level of glomerular filtration rate (GFR) and serum creatinine which were decreased and increased respectively minimum in 1.5 times (25%). RESULTS: We diagnosed AKI in 109 children (76,2 %) from 143 observed patients, Risk stage was found in 44 children (30,8 %), the stage of Injury-in 46 children (32,2 %), the stage of Failure-in 19 children (13,3%). AKI was noted at all phases of chemotherapy, maximum at the stage of intensive PCHT (in 77 from 89 children, 86,5 %). In 16 from 30 children (53,3 %) observed during all phases of treatment AKI registered several times (maximum 4 times). During the period of observation the fatal outcome was registrate...
The article describes a clinical case of early diagnosis of hereditary thrombophilia in a child in the neonatal period, based on clinical, laboratory and genetic examination. Clinical suspicion to hereditary hemostatic disorders, careful analysis of hereditary and family history allowed to identify and confirm with the help of PCR polymorphism of genes associated with hemostatic system in a child of two weeks of age. Early diagnosis allows to make an individual plan of observation and dynamic examination to prevent increased thrombosis and possible vascular catastrophe in the child, to prevent metabolic and hemostatic disorders in both the mother and her relatives, and in the probable future siblings of the patient, and in the long term and in his descendants.
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