Клинико-лабораторные характеристики, лечение и прогноз синдрома Гийена-Барре у детей

A retrospective study of 42 cases of acute flaccid paralysis (AFP) in children aged between 7 months and 15 years, registered at the Municipal Clinical Hospital №1 throughout a 7 year period (2007—2014), was performed to investigate the features of pediatric Guillian-Barre Syndrome (GBS). GBS has shown to be the most common cause of AFP in children, with prevalence of 74% of all 31 cases. Clinical manifestations, functional status, laboratory and electrodiagnostic data were evaluated in group of 31 children in order to highlight particular features of childhood GBS in Russia. The highest frequency of GBS was observed in children aged between 1 to 3 with the median 6 [3; 11] years. Boys with GBS outnumbered girls by a 2,1:1 ratio. No seasonal dependence has been observed, with children equally suffering from this disease without a seasonal pattern throughout the year. According to the electrophysiological and clinical data, 24 children were diagnosed with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) (77%), 5 with acute motor axonal neuropathy (AMAN) (16%) and 2 with аcute motor-sensory axonal neuropathy (AMSAN) in a total of cases (7%). Several exclusive features of GBS in children for Russia were discovered. The most common initial symptom was limb pain, with the impartial sensory disturbance found only in 13% of the patients observed, 10% of which were paresthesias and the remaining 3% belonging to hypostesias. Children reached the nadir state rapidly, the median time from onset to nadir was 9.5 [6,25; 12,5] days. Cranial nerve dysfunction at nadir was observed in a greater percentage of patients (51%) compared to that of 23% cases at the onset, with the facial palsy increasing from 10 to 32% and the bulbar palsy from 12 to 19%. The patients were given intravenous immunoglobulin in various doses: from 0.2 to 1.75 mg/kg per course (0.5 [0.5; 0.8] g/kg) and/or plasmapheresis with a median volume of 93 [81; 100] ml/kg per course. The treatment has shown to be effective for the majority of patients, but three children was resistant to the intravenous immunoglobulin. An important feature of pediatric GBS is a nonthreatening prognosis at the point of discharge, with the length of hospitalization numbering in with a median of 28 [20,5; 38] days.В работе были проанализированы 42 случая острых вялых параличей (ОВП) у детей в возрасте от 7 месяцев до 15 лет, зарегистрированных в ИКБ №1 г. Москвы в период с 2007 по 2014 гг. Согласно ретроспективному анализу, ведущей причиной ОВП среди детей является Синдром Гийена-Барре (СГБ), доля которого составила 74% (31 пациент). Изучена клиническая картина и данные лабораторных и электрофизиологических исследований для выявления особенностей течения и диагностики СГБ у детей. СГБ чаще всего встречается в возрастной группе от 1 до 3 лет, медиана 6 [3;11] лет. Мальчики болеют чаще (2,1:1). Небыло выявлено сезонной зависимости СГБ: дети переносят заболевание в разные месяцы на протяжении всего года. Острая воспалительная демиелинизирующая полирадикулонейропатия (ОВДП) диагностирована у 24 детей (77%), острая моторная аксональная невропатия (ОМАН) — в 5 случаях (16%) и острая моторно-сенсорная аксональная невропатия (ОМСАН) — в 2 случаях (7%). Выявлен ряд особенностей клинической картины СГБ у детей. Ведущим симптомом в дебюте заболевания является интенсивная боль в конечностях, при этом объективные сенсорные нарушения выявляются редко (13%): в виде парестезий (10%) или гипостезий (3%). Темп нарастания параличей достаточно высокий: медиана достижения пика заболевания 9,5 [6,25; 12,5] суток от начала заболевания. На пике заболевания доля поражения ЧМН увеличивается с 23 до 51%: частота парезов VII пары возрастает с 10 до 32%, а частота бульбарного синдрома с 12 до 19% случаев. Проведено лечение внутривенным иммуноглобулином в дозе от 0,2 до 1,75 г/кг за курс (медиана 0,5 [0,5; 0,8] г/кг) и/или плазмаферезом в объёме 93 [81; 100] мл/кг за курс. В подавляющем большинстве случаев был достигнут положительный результат, но три ребёнка оказались рефрактерны к внутривенной иммунотерапии. Важной особенностью СГБ у детей является благоприятный прогноз исхода к моменту выписки из стационара, при сроках госпитализации 28 [20,5; 38] суток

Clinical and Laboratory Features, Treatment and Prognosis in Children with Guillian-Barre Syndrome

A retrospective study of 42 cases of acute flaccid paralysis (AFP) in children aged between 7 months and 15 years, registered at the Municipal Clinical Hospital №1 throughout a 7 year period (2007—2014), was performed to investigate the features of pediatric Guillian-Barre Syndrome (GBS). GBS has shown to be the most common cause of AFP in children, with prevalence of 74% of all 31 cases. Clinical manifestations, functional status, laboratory and electrodiagnostic data were evaluated in group of 31 children in order to highlight particular features of childhood GBS in Russia. The highest frequency of GBS was observed in children aged between 1 to 3 with the median 6 [3; 11] years. Boys with GBS outnumbered girls by a 2,1:1 ratio. No seasonal dependence has been observed, with children equally suffering from this disease without a seasonal pattern throughout the year. According to the electrophysiological and clinical data, 24 children were diagnosed with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) (77%), 5 with acute motor axonal neuropathy (AMAN) (16%) and 2 with аcute motor-sensory axonal neuropathy (AMSAN) in a total of cases (7%). Several exclusive features of GBS in children for Russia were discovered. The most common initial symptom was limb pain, with the impartial sensory disturbance found only in 13% of the patients observed, 10% of which were paresthesias and the remaining 3% belonging to hypostesias. Children reached the nadir state rapidly, the median time from onset to nadir was 9.5 [6,25; 12,5] days. Cranial nerve dysfunction at nadir was observed in a greater percentage of patients (51%) compared to that of 23% cases at the onset, with the facial palsy increasing from 10 to 32% and the bulbar palsy from 12 to 19%. The patients were given intravenous immunoglobulin in various doses: from 0.2 to 1.75 mg/kg per course (0.5 [0.5; 0.8] g/kg) and/or plasmapheresis with a median volume of 93 [81; 100] ml/kg per course. The treatment has shown to be effective for the majority of patients, but three children was resistant to the intravenous immunoglobulin. An important feature of pediatric GBS is a nonthreatening prognosis at the point of discharge, with the length of hospitalization numbering in with a median of 28 [20,5; 38] days

Новые фармакотерапевтические подходы к лечению периферической Т-клеточной лимфомы

Today, it is difficult to overestimate the new directions in the pharmacotherapy of peripheral T-cell lymphomas (PTCL): immunotherapy, including adoptive, targeted therapy and chemotherapy. However, there are few biomarkers that predict response to therapy. A big problem is patients with refractory and recurrent PTCL who do not respond to such therapy or demonstrate adverse events, which makes it important to personalize therapy and search for predictive markers, followed by thorough analytical and clinical validation. The literature highlights the importance of using biomarkers obtained from whole exome sequencing and tumor transcriptome sequencing. Thе review discusses the T cell ontogenesis, as well as the possibilities of personalization of anticancer drugs such as azacitidine, duvelisib, romidepsin, and bortezomib for the treatment of refractory or recurrent PTCL.На сегодняшний день сложно переоценить новые направления в фармакотерапии периферических Т-клеточных лимфом (ПТКЛ): иммунотерапии, в т.ч. адоптивной, таргетной и химиотерапии. Тем не менее биомаркеров, которые бы предсказывали ответ на лечение, крайне мало. Большую проблему составляют пациенты с рефрактерной и рецидивирующей ПТКЛ, которые не отвечают на подобную терапию или у них развиваются нежелательные явления, что делает актуальным вопрос персонификации лечения и поиска новых предиктивных маркеров с последующей тщательной аналитической и клинической валидацией в реальной врачебной практике. В литературе подчеркивается важность применения биомаркеров, полученных в результате полноэкзомного секвенирования и секвенирования транскриптома опухолей. В обзоре рассмотрен Т-клеточный онтогенез, а также возможности персонализации таких противоопухолевых препаратов, как азацитидин, дувелисиб, ромидепсин и бортезомиб, для терапии рефрактерной или рецидивирующей ПТКЛ

EFFECTIVENESS OF CORRECTION OF HYPOVITAMINOSIS D IN ADOLESCENTS LIVING IN MOSCOW CITY

Schoolchildren and their state of health, in particular, deficiencies, deserve no less attention than children of early age, while the recognition of the existence of inadequate vitamin D sufficiency in children of all age groups has not yet led to the introduction of mass prophylaxis for hypovitaminosis D. There are a number of factors determining the concentration of 25(OH)D-calcidiol in the blood. However, it is obvious that among many factors one should focus on several most significant and develop a scheme for correcting deficiency in each region.Purpose: to assess vitamin D sufficiency in adolescents living in Moscow, to correct calcidiol status in adolescents on the basis of baseline concentration of the metabolite transported in the blood. Material and methods: 769 adolescents aged 11-18 years for whom 25(OH)D concentration in the blood was evaluated, then 218 patients were randomized into 2 groups: the study group received tableted dietary supplement Minisun® vitamin D3, the controlgroup received placebo. The dosage of cholecalciferol was defined based on the initial level of calcidiol in patients. The study continued for 6 months, then blood was re-sampled to determine the level of calcidiol.Results: the study found low sufficiency with calcidiol among the schoolchildren: 25(OH)D median was 16.3 [11.4-20.8] ng/ml, only 5.2% of patients had normal concentration of calcidiol in the blood. At the second examination, positively significant differences in the status of vitamin D were observed in the study compared to the control group, while patients in the study group,against a background of cholecalciferol donation, demonstrated 25(OH)D median increase from 16.2 [12.25-19.3] ng/ml to 24.2 [21.05-26.4] ng/ml (p <0.001) .Conclusions: a large proportion of the child population (70.6%) have a concentration of 25(OH)D <20 ng/ml. The doses of cholecalciferol used in the study make it possible to eliminate the deficiency of calcidiol and to overcome the threshold of 20 ng/ml, but in order to normalize vitamin D status in the blood of adolescents living in Moscow, it is necessary to use higher dosages that need to be confirmed by further research

Seasonal variations in vitamin and micronutrient sufficiency in a cohort of children aged 3-14 years

Vitamin sufficiency in children can be estimated by the different values of their consumption in autumn-winter and spring-summer seasons. The paper presents the results of a study assessing the prevalence of vitamin deficiency among children of various ages depending on the season. The study included children and adolescents aged from 3 to 14 years (n = 2587, 51% boys) in various regions of Russia. As a result, significant seasonal differences were found in vitamin sufficiency in children aged 3–14 years. Analysis of clusters of parameter interactions demonstrated a relationship between seasonal differences in sufficiency of vitamins B6, E, A, B2 and the risk of various pathologies in all age groups of children. Finally, the presence of seasonal variations in vitamin sufficiency in children was confirmed. This should be taken into account when prescribing vitamin and mineral complexes to children

Клинико-лабораторные характеристики, лечение и прогноз синдрома Гийена-Барре у детей

A retrospective study of 42 cases of acute flaccid paralysis (AFP) in children aged between 7 months and 15 years, registered at the Municipal Clinical Hospital №1 throughout a 7 year period (2007—2014), was performed to investigate the features of pediatric Guillian-Barre Syndrome (GBS). GBS has shown to be the most common cause of AFP in children, with prevalence of 74% of all 31 cases. Clinical manifestations, functional status, laboratory and electrodiagnostic data were evaluated in group of 31 children in order to highlight particular features of childhood GBS in Russia. The highest frequency of GBS was observed in children aged between 1 to 3 with the median 6 [3; 11] years. Boys with GBS outnumbered girls by a 2,1:1 ratio. No seasonal dependence has been observed, with children equally suffering from this disease without a seasonal pattern throughout the year. According to the electrophysiological and clinical data, 24 children were diagnosed with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) (77%), 5 with acute motor axonal neuropathy (AMAN) (16%) and 2 with аcute motor-sensory axonal neuropathy (AMSAN) in a total of cases (7%). Several exclusive features of GBS in children for Russia were discovered. The most common initial symptom was limb pain, with the impartial sensory disturbance found only in 13% of the patients observed, 10% of which were paresthesias and the remaining 3% belonging to hypostesias. Children reached the nadir state rapidly, the median time from onset to nadir was 9.5 [6,25; 12,5] days. Cranial nerve dysfunction at nadir was observed in a greater percentage of patients (51%) compared to that of 23% cases at the onset, with the facial palsy increasing from 10 to 32% and the bulbar palsy from 12 to 19%. The patients were given intravenous immunoglobulin in various doses: from 0.2 to 1.75 mg/kg per course (0.5 [0.5; 0.8] g/kg) and/or plasmapheresis with a median volume of 93 [81; 100] ml/kg per course. The treatment has shown to be effective for the majority of patients, but three children was resistant to the intravenous immunoglobulin. An important feature of pediatric GBS is a nonthreatening prognosis at the point of discharge, with the length of hospitalization numbering in with a median of 28 [20,5; 38] days.В работе были проанализированы 42 случая острых вялых параличей (ОВП) у детей в возрасте от 7 месяцев до 15 лет, зарегистрированных в ИКБ №1 г. Москвы в период с 2007 по 2014 гг. Согласно ретроспективному анализу, ведущей причиной ОВП среди детей является Синдром Гийена-Барре (СГБ), доля которого составила 74% (31 пациент). Изучена клиническая картина и данные лабораторных и электрофизиологических исследований для выявления особенностей течения и диагностики СГБ у детей. СГБ чаще всего встречается в возрастной группе от 1 до 3 лет, медиана 6 [3;11] лет. Мальчики болеют чаще (2,1:1). Небыло выявлено сезонной зависимости СГБ: дети переносят заболевание в разные месяцы на протяжении всего года. Острая воспалительная демиелинизирующая полирадикулонейропатия (ОВДП) диагностирована у 24 детей (77%), острая моторная аксональная невропатия (ОМАН) — в 5 случаях (16%) и острая моторно-сенсорная аксональная невропатия (ОМСАН) — в 2 случаях (7%). Выявлен ряд особенностей клинической картины СГБ у детей. Ведущим симптомом в дебюте заболевания является интенсивная боль в конечностях, при этом объективные сенсорные нарушения выявляются редко (13%): в виде парестезий (10%) или гипостезий (3%). Темп нарастания параличей достаточно высокий: медиана достижения пика заболевания 9,5 [6,25; 12,5] суток от начала заболевания. На пике заболевания доля поражения ЧМН увеличивается с 23 до 51%: частота парезов VII пары возрастает с 10 до 32%, а частота бульбарного синдрома с 12 до 19% случаев. Проведено лечение внутривенным иммуноглобулином в дозе от 0,2 до 1,75 г/кг за курс (медиана 0,5 [0,5; 0,8] г/кг) и/или плазмаферезом в объёме 93 [81; 100] мл/кг за курс. В подавляющем большинстве случаев был достигнут положительный результат, но три ребёнка оказались рефрактерны к внутривенной иммунотерапии. Важной особенностью СГБ у детей является благоприятный прогноз исхода к моменту выписки из стационара, при сроках госпитализации 28 [20,5; 38] суток.</p