С реди острых лейкозов у детей отмечается преобладание острого лимфоидного лейкоза, доля которого составляет 75-85% [1-3]. О поражение костей при лейкозах сообщалось еще в начале XX века. Полиморфность клинических проявлений может считаться диагностической трудностью, в то время как и аномальные рентгенологические результаты не являются традиционными характеристиками больных лейкемией [4, 5]. По данным отдельных авторов, костно-суставной синдром наблюдается у каждого третьего больного с острым лейкозом [6]. Чаще всего изменения в костях обнаруживаются при остром лимфоидном лейкозе из В-клеточных предшественников с нормальным/сниженным количеством лейкоцитов в отсутствие бластных клеток в периферической крови в дебюте заболевания. В некоторых случаях костные проявления могут быть первым и единственным симптомом острого лимфоидного лейкоза, что затрудняет и служит причиной запоздалой постановки правильного диагноза [7]. Поражения костной системы при дебюте острого лимфоидного лейкоза разнообразны и включают © Коллектив авторов, 2020 Адрес для корреспонденции: Уланова Анна Сергеевна-асс. кафедры детской хирургии Северного государственного медицинского университета, врач-детский онколог отделения химиотерапии опухолей Архангельской областной детской клинической больницы им. П.Г. Выжлецова, ORCID: 0000-0002-0326-6774 Григорьева Наталья Александровна-асс. кафедры детской хирургии Северного государственного медицинского университета, врач-детский онколог отделения химиотерапии опухолей Архангельской областной детской клинической больницы им. П.Г. Выжлецова Турабов Иван Александрович-д.м.н., доц., зав. кафедрой детской хирур-гии Северного государственного медицинского университета, гл. внештатный детский специалист-онколог Минздрава Архангельской области, ORCID: 0000-0003-4047-406x 163000 Архангельск, пр. Троицкий, д. 51 Рыков Максим Юрьевич-д.м.н., доц., доц. кафедры онкологии Института клинической медицины им. Н.В. Склифосовского Первого МГМУ им. И.М. Сеченова (Сеченовский университет),
Background: St. Petersburg is a city of federal importance with a large number of primary patients, identified annually. Objective: analysis of the main indicators characterizing medical care for children with cancer in St. Petersburg and the Leningrad region. Methods: The operative reports for 2013-2017 of the Health Committee of the Government of St. Petersburg and the Health Committee of the Leningrad Region were analyzed. Results. In 2013-2017 in the Russian Federation, 18 090 primary patients were identified, 927 (5.1%) of them in the analyzed subjects: in St. Petersburg - 697 (75,2%), in the Leningrad Region - 230 (24,8%). For 5 years, the number of primary patients increased in St. Petersburg - by 36%, in the Leningrad Region - by 2,5%. The incidence increased in St. Petersburg by 18,1% (from 14,9 in 2013 to 17,6 in 2017 per 100 000 of children aged 0-17). The incidence in the Leningrad Region fell by 4.9% (from 14.4 in 2013 to 13.7 in 2017). Mortality in 2016-2017 in St. Petersburg increased by 50% (from 2 to 3), in the Leningrad Region - by 12,5% (from 2,4 to 2,7). The one-year mortality rate in St. Petersburg increased by 3,9% (from 2,5 to 6,4%). In the Leningrad Region, the one-year mortality rate decreased from 6,5% in 2016 to 0 in 2017. The number of pediatric oncological beds did not change in St. Petersburg (0,9 per 10,000 children aged 0-17 years) and the Leningrad Region (0). In St. Petersburg patients were not identified actively in 2016-2017; in the Leningrad Region their percentage decreased from 8,7 to 0. The number of oncologists increased in St. Petersburg from 0,09 to 0.12 (+33,3%), in the Leningrad Region - from 0 to 0,03. Conclusion: Morbidity in St. Petersburg and the Leningrad region is significantly different, which indicates obvious defects in statistical data. Patients were not identified during routine preventive examinations which indicate a low oncologic alertness of district pediatric physicians. Delivery of medical care for children with cancer and the statistical data accumulation procedures should be improved.
АКТУАЛЬНОСТЬГерминальноклеточная опухоль -достаточно редкая разновидность неоплазий; ежегодная забо-леваемость составляет не более 0,6 случаев на 100 000 детей [1]. Прогенитором (от англ. progenitor -предшественник) таких опухолей является промор-диальная полипотентная клетка. Опухоль возникает при нарушении развития или миграции таких клеток в процессе эмбриогенеза [1, 2].
Background. Treatment tactics for the intussusception (IS) in children is mainly defined the formation cause. Non operative IS treatment is most preferable. The surgery is often unreasonably performed in children over three years as the cause for IS occurrence can be a malignant tumor. Objective. Our aim was to define the features of clinical picture and diagnostics for IS associated with malignant tumors in children. Methods. We conducted the retrospective comparative assessment of reasons, registered clinical manifestations, and applied diagnostic methods in IS cases (n=373) at Arkhangelsk children's clinical hospital in 1981–2016. Two groups were analyzed: the first group (n=7) enrolled patients with IS caused by malignant tumor, the second one (n=366) — patients with IS caused by other reasons (idiopathic, mesenteric nodes hyperplasia, diverticulum, postoperative). Results. The typical combination was detected in the first group: symptoms of gastrointestinal tract (GIT) diseases and significant (8–12%) weight loss. In the first group, IS associated with tumor was observed in a few cases (1.9%), proceeded chronically (1–3 months), relapses did not occur, acute intestinal obstructions (р<0.001) were not registered; all the patients over five years were diagnosed with non-Hodgkin lymphomas if compare with the second group. The most informative diagnostic method was ultrasound imaging including hydrocolonic sonography which allowed IS diagnosing and tumor identification. In the second group, the clinical picture changed eventually: «triad» of symptoms typical for IS (abdominal colic pain, blood from rectum, abdominal mass) was registered in 1/3 of cases, significantly more often (p=0.001) the disease was demonstrated by «dyad» of symptoms (vomiting and abdominal colic pain) but always progressed to the acute form. Conclusion. The IS clinical course in children following its usual pattern significantly differs from the clinical picture associated with the tumor (though it occurs rarely, commonly in children over five years): the disease proceeds chronically with symptoms of GIT diseases and significant weight loss, relapses did not occur, no symptoms of acute intestinal obstruction are registered.
Background. The study of oncological morbidity in children, as well as the characteristics of its structure and the analysis of temporal trends within the subject of the Russian Federation is aimed at improving the organization of medical care: increasing early diagnosis, reliability of static data, and optimizing patient routing. Objective. Our aim was to analyze the incidence and its structure in children (0–17 years old) who lived in the Arkhangelsk Region and the Nenets Autonomous District in 2007–2016. Methods. The analysis of the incidence of malignant neoplasms of the child population in the Arkhangelsk region and the Nenets Autonomous District was carried out. Results. During the study period, the diagnosis of cancer was first established in 329 (including 6 patients from other subjects of the Russian Federation children (boys — 177, girls — 146) at the age of 0–17 years, of which 260 children at the age of 0–14 years and 63 — at the age of 15–17 years. The average annual incidence of children over 10 years was 14.3: for the age group 0–14 years old — 13.8, for the age group 15–17 years old — 16.6. During the analyzed period, there were significant fluctuations in annual incidence rates (minimum — 9.8 in 2010; maximum — 16.8 in 2009). The average annual incidence in the age group 15–17 years (16.6) was 18% higher than in the age group 0–14 years (14). The morbidity structure was characterized by some predominance of solid tumors (51.7%) over hemoblastosis (48.3%), and in the group of 0–14 years this ratio was 54.6/45.4%, in the group of 15–17 years — 50, 9/49.1%. Conclusion. In the analyzed subjects in recent years there has been practically no increase in the incidence in children. The incidence in the age group 15–17 years is slightly higher than in the age group 0–14 years. Changes in the morbidity structure are noted, mainly due to a decrease in the proportion of lymphomas.
.Objective. To study the opinion of parents (legal representatives) on the problems of medical care for children with oncological diseases.Methods. The study was based on the Questionnaire for parents (legal representatives) on medical care children with cancer. The questionnaire consisted of 27 questions, the respondents were asked to choose one or several answers or to enter their own option.Results. This medical and social study demonstrated that 81.1±1.9% of respondents are not satisfied with the attitude of doctors and nurses towards them and their children, 15.9±1.8% of respondents are partially satisfied and only 3±0.8% of respondents are fully satisfied. The reasons for dissatisfaction were as follows: rude communication (35.8±2.4%), inadequate attention of medical personnel (21.3±2.0%) and lack of interest in the treatment success (19.7±2.0%). The combination of these options was noted by 23.2±2.0% of the respondents. 63.7±2.4% of respondents would prefer to treat their children at a medical organization of federal subordination, 33.9±2.4% – at medical organization outside the territory of the Russian Federation, of which 57.4±2.4% would like to receive a patient-oriented service and simplified routing between medical organizations during treatment. 12.7±1.6% mistrust in the qualifications of medical personnel in the Russian Federation, 11.8±1.6% of respondents indicated the lack of comfortable conditions for examination and treatment, 5.3±1.1% of respondents indicated the lack of necessary diagnostic and treatment methods and 12.8 1.6% of respondents indicated a combination of the above options.Conclusion. The results obtained indicate the need to implement patient-oriented approaches and to improve the routing of children with cancer.
Malignant neoplasms prevail in the structure of mortality in children all over the world, while most patients are diagnosed at the common stages; the second place is taken by external causes. It determines a particular relevance of developing new approaches to strengthen the role of district pediatricians in the early diagnosis of malignant neoplasms in children.Material and methods. The Algorithm for determining the appropriateness of referring a patient to a pediatric oncologist was introduced in the medical organizations of the Arkhangelsk region in 2016. To evalute the effectiveness of the Algorithm, we compared two groups of patients with histologically verified solid malignant neoplasms: Group 1: 49 patients who received medical care in the Arkhangelsk region in 2011–2015 (prior to the experiment); Group 2: 51 patients who received medical care in the Department of Pediatric Oncology of the Arkhangelsk region in 2016–2018. Results. The average survival time was significantly increased in Group 2016–2018 (30.3 ± 1.57 months) as compared with the survival time of patients receiving treatment in 2011–2015 (25.04 ± 2.05 months) (p=0.045). The following time parameters were reduced: from the moment of contacting the pediatric oncologist to the verification of the diagnosis – from 9.0 to 7.0 days; from verification of the diagnosis to the start of specialized treatment – from 12.0 to 8.0 days; from the moment of contacting the local pediatrician to the referral to the pediatric oncologist – from 11.0 to 2.0 days; from the moment of contacting the local pediatrician to the start of specialized treatment – from 23.0 to 9.0 days. Conclusion: The organizational experiment confirmed the effectiveness of the Algorithm and the expediency of its implementation in the medical organizations.
АннотацияАктуальность. Для диагностики и прогнозирования острого лейкоза (ОЛ) применяется комплекс клинических и лабораторных методов. Важную роль среди них играет хромосомный анализ. В клинической практике постоянно используются цитогенетические маркеры лейкозного клона. Современная классификационная система ВОЗ опухолей кроветворных и лимфоидных тканей создана на основе объединенного анализа клинических, цитоморфологических, иммунофенотипических и цитогенетических данных. Иммунофенотипирование бластных клеток с использованием моноклональных антител позволило значительно улучшить диагностику лейкозов за счет выявления степени дифференцировки опухолевых клеток. Цель исследования -описать структуру ОЛ на основании результатов иммунофенотипического и цитогенетического исследований и изучить влияние цитогенетических нарушений на прогноз при ОЛ в детской популяции Архангельской области. Материал и методы. Архивные данные были использованы для анализа результатов цитогенетического и иммунофенотипического обследований всех детей в возрасте от 0 до 17 лет в Архангельской области, у которых в период с 01.01.2004 по 31.12.2018 был диагностирован ОЛ. Результаты. В результате иммунофенотипического исследования бластных клеток костного мозга больных ОЛ выявлено, что преобладал В-линейный лимфобластный лейкоз (74,8 %). Среди количественных аномалий были выявлены гиперплоидии, наблюдались дополнительные хромосомы 4,10,17. Среди структурных хромосомных аномалий больше всего случаев пришлось на t(12;21). Филадельфийская хромосома (ph) (9;22) встретилась в 6,5 % случаев. Риск смертельного исхода у пациентов с ОЛ был наиболее высоким при остром миелобластном лейкозе (p<0,001). Общая выживаемость пациентов с ОЛ значительно увеличилась за период исследования (p<0,001). Заключение. По данным проведенной работы, в Архангельской области структура ОЛ детской популяции по иммунофенотипической характеристике не противоречит общероссийским тенденциям. В структуре установленных хромосомных аномалий в ходе исследования специфических отличий не выявлено. Все хромосомные изменения, имеющие отрицательное влияние на течение заболевания, также проявляли свое негативное влияние на прогноз. Представлено увеличение числа хромосомных
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