Roman V. Epishev, Alexander I. Materikin, Leonid A. Opryatin, Roman A. Ivanov, Maria A. Leonova, Alena A. Savelova
Atopic dermatitis (AD) is a chronic inflammatory skin disease with high risk of skin and systemic infections. Preventive AD therapy is based on skin barrier improvement and anti-inflammatory treatment, whereas, severe skin and systemic infections require systemic therapy. This review describes pathophysiology and possible treatment and prevention methods for AD infectious complications.
{"title":"Management and Prevention of Atopic Dermatitis Infectious Complications in Children","authors":"Roman V. Epishev, Alexander I. Materikin, Leonid A. Opryatin, Roman A. Ivanov, Maria A. Leonova, Alena A. Savelova","doi":"10.15690/vsp.v22i5.2638","DOIUrl":"https://doi.org/10.15690/vsp.v22i5.2638","url":null,"abstract":"Atopic dermatitis (AD) is a chronic inflammatory skin disease with high risk of skin and systemic infections. Preventive AD therapy is based on skin barrier improvement and anti-inflammatory treatment, whereas, severe skin and systemic infections require systemic therapy. This review describes pathophysiology and possible treatment and prevention methods for AD infectious complications.","PeriodicalId":37561,"journal":{"name":"Voprosy Sovremennoi Pediatrii - Current Pediatrics","volume":"12 6","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135725629","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Leyla S. Namazova-Baranova, Eduard T. Ambarchyan, Vladislav V. Ivanchikov, Anastasia D. Kuzminova, Anna G. Shandra, Elena A. Vishneva, Grigorii V. Revunenkov, Kirill A. Valyalov
Background. Psoriasis is an independent risk factor for cardiovascular diseases (CVD). One of the markers associated with the CVD course is epicardial fatty tissue (EFT) that is thicker in psoriasis patients. EFT assessment can be used as a useful indicator of CVD in psoriasis patients. The data about the effect of genetically engineered biological therapy (GEBT), used for psoriasis management, on the EFT thickness is limited. Examination of GEBT effects on EFT may improve our understanding of CVD prevention in psoriasis patients. Objective. The aim of the study is to study the changes in EFT thickness on GEBT. Methods. A prospective cohort study included 56 children with severe and moderate psoriasis. Patients underwent transthoracic two-dimensional echocardiography (M-mode) with EFT thickness assessment and PASI (Psoriasis Area and Severity Index) scoring before the GEBT initiation. All the parameters were re-evaluated after 16 weeks. All patients were divided into three groups according to the initiated therapy: adalimumab, secukinumab and ustekinumab. When dividing the therapy received into groups, the age of the patients was taken into account: inclusion in the adalimumab group was carried out from 4 years, in the secukinumab and ustekinumab groups — from 6 years. Otherwise, the process of group assignment was random. The study results were processed using descriptive statistics methods: the changes in EFT thickness in individual groups were compared via the Wilcoxon test, and results were considered statistically significant at p 0.05. Results. Before the start of therapy, in 56 patients the mean of EFT thickness was 2.11 mm, the mean PASI — 18.32. The adalimumab group had the following indicators: the mean EFT thickness before the therapy was 2.1 mm, and it has decreased to 1.77 mm after 16 weeks of therapy. The mean change in EFT thickness was 0.33 mm, and the median — 0.17 mm [CI 0.33 ± 0.25]. The ustekinumab group: the mean EFT thickness before the therapy was 2.13 mm, 16 weeks after — 1.69 mm. The mean change in EFT thickness was 0.44 mm, and the median — 0.38 [CI 0.44 ± 0.13]. The secukinumab group: the mean EFT thickness before the therapy was 2.08 mm, 16 weeks after — 1.82 mm. The mean change in EFT thickness was 0.27 mm, and the median — 0.27 [CI 0.27 ± 0.07]. Evaluation of indicators via Wilcoxon test has shown statistically significant decrease in the EFT after therapy in all groups (p 0.05). 73% of patients achieved PASI 50, and 6% — PASI 75 in the adalimumab group. 21% of patients did not achieve PASI 50. The mean PASI score before therapy was 16.73 points, and after 16 ± 4 weeks — 6.4 points, the mean dynamics was 10.33 points, the median dynamics was 7 points [CI 10.33 ± 4]. All patients achieved PASI 50, 75.3% — PASI 75, 8% — PASI 90, and 16.7% — PASI 100 in the ustekinumab group. The mean PASI score before therapy was 22.17 points, and after 16 weeks — 3.67 points, the mean dynamics was 19.28 points, the median dynamics was
背景。牛皮癣是心血管疾病(CVD)的独立危险因素。与CVD病程相关的标志物之一是牛皮癣患者较厚的心外膜脂肪组织(EFT)。EFT评价可作为银屑病患者CVD的有效指标。用于牛皮癣治疗的基因工程生物疗法(GEBT)对EFT厚度的影响数据有限。检查GEBT对EFT的影响可能会提高我们对银屑病患者CVD预防的认识。目标。本研究的目的是研究GEBT上EFT厚度的变化。方法。一项前瞻性队列研究包括56名患有重度和中度牛皮癣的儿童。患者在GEBT开始前接受经胸二维超声心动图(m模式)，EFT厚度评估和PASI(银屑病面积和严重程度指数)评分。16周后重新评估所有参数。所有患者根据起始治疗分为三组:阿达木单抗、secukinumab和ustekinumab。在将接受的治疗分组时，考虑了患者的年龄:阿达木单抗组从4岁开始纳入，secukinumab组和ustekinumab组从6岁开始纳入。否则，分组分配过程是随机的。研究结果采用描述性统计方法处理，各组EFT厚度变化比较采用Wilcoxon检验，p < 0.05认为结果有统计学意义。结果。治疗前56例患者EFT平均厚度2.11 mm，平均PASI - 18.32。阿达木单抗组EFT平均厚度治疗前为2.1 mm，治疗16周后降至1.77 mm。EFT厚度的平均变化为0.33 mm，中位变化为- 0.17 mm [CI 0.33±0.25]。ustekinumab组:治疗前平均EFT厚度为2.13 mm，治疗后16周- 1.69 mm。EFT厚度的平均变化为0.44 mm，中位变化为- 0.38 [CI 0.44±0.13]。secukinumab组:治疗前平均EFT厚度为2.08 mm，治疗后16周- 1.82 mm。EFT厚度的平均变化为0.27 mm，中位数变化为- 0.27 [CI 0.27±0.07]。经Wilcoxon检验评价指标显示，治疗后各组EFT下降有统计学意义(p < 0.05)。在阿达木单抗组中，73%的患者达到PASI 50, 6%达到PASI 75。21%的患者未达到PASI 50。治疗前PASI平均为16.73分，治疗后16±4周- 6.4分，平均动力学为10.33分，中位动力学为7分[CI 10.33±4]。在ustekinumab组中，所有患者均达到PASI 50、75.3% - PASI 75、8% - PASI 90和16.7% - PASI 100。治疗前PASI平均评分为22.17分，治疗16周后为3.67分，平均动力学为19.28分，中位动力学为17分[CI 18.5±3.03]。在secukinumab组中，所有患者均达到PASI 50, 47% - PASI 75和11% - PASI 90。治疗前PASI中位数为14.29分，治疗后16±4周- 3.71分，治疗前PASI平均为14.29分，治疗后16周- 3.7分，平均动力学为10.59分，动力学中位数为10分[CI 10.59±2.27]。经Wilcoxon检验评价指标显示，各组治疗后PASI下降有统计学意义(p < 0.05)。随访期间无不良事件导致停止治疗。结论。各组EFT厚度和PASI评分均有所下降。在ustekinumab组观察到最显著的动态。研究的局限性是患者样本小，缺乏对照组(无牛皮癣的参与者)。
{"title":"Research Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery","authors":"Leyla S. Namazova-Baranova, Eduard T. Ambarchyan, Vladislav V. Ivanchikov, Anastasia D. Kuzminova, Anna G. Shandra, Elena A. Vishneva, Grigorii V. Revunenkov, Kirill A. Valyalov","doi":"10.15690/vsp.v22i5.2641","DOIUrl":"https://doi.org/10.15690/vsp.v22i5.2641","url":null,"abstract":"Background. Psoriasis is an independent risk factor for cardiovascular diseases (CVD). One of the markers associated with the CVD course is epicardial fatty tissue (EFT) that is thicker in psoriasis patients. EFT assessment can be used as a useful indicator of CVD in psoriasis patients. The data about the effect of genetically engineered biological therapy (GEBT), used for psoriasis management, on the EFT thickness is limited. Examination of GEBT effects on EFT may improve our understanding of CVD prevention in psoriasis patients. Objective. The aim of the study is to study the changes in EFT thickness on GEBT. Methods. A prospective cohort study included 56 children with severe and moderate psoriasis. Patients underwent transthoracic two-dimensional echocardiography (M-mode) with EFT thickness assessment and PASI (Psoriasis Area and Severity Index) scoring before the GEBT initiation. All the parameters were re-evaluated after 16 weeks. All patients were divided into three groups according to the initiated therapy: adalimumab, secukinumab and ustekinumab. When dividing the therapy received into groups, the age of the patients was taken into account: inclusion in the adalimumab group was carried out from 4 years, in the secukinumab and ustekinumab groups — from 6 years. Otherwise, the process of group assignment was random. The study results were processed using descriptive statistics methods: the changes in EFT thickness in individual groups were compared via the Wilcoxon test, and results were considered statistically significant at p 0.05. Results. Before the start of therapy, in 56 patients the mean of EFT thickness was 2.11 mm, the mean PASI — 18.32. The adalimumab group had the following indicators: the mean EFT thickness before the therapy was 2.1 mm, and it has decreased to 1.77 mm after 16 weeks of therapy. The mean change in EFT thickness was 0.33 mm, and the median — 0.17 mm [CI 0.33 ± 0.25]. The ustekinumab group: the mean EFT thickness before the therapy was 2.13 mm, 16 weeks after — 1.69 mm. The mean change in EFT thickness was 0.44 mm, and the median — 0.38 [CI 0.44 ± 0.13]. The secukinumab group: the mean EFT thickness before the therapy was 2.08 mm, 16 weeks after — 1.82 mm. The mean change in EFT thickness was 0.27 mm, and the median — 0.27 [CI 0.27 ± 0.07]. Evaluation of indicators via Wilcoxon test has shown statistically significant decrease in the EFT after therapy in all groups (p 0.05). 73% of patients achieved PASI 50, and 6% — PASI 75 in the adalimumab group. 21% of patients did not achieve PASI 50. The mean PASI score before therapy was 16.73 points, and after 16 ± 4 weeks — 6.4 points, the mean dynamics was 10.33 points, the median dynamics was 7 points [CI 10.33 ± 4]. All patients achieved PASI 50, 75.3% — PASI 75, 8% — PASI 90, and 16.7% — PASI 100 in the ustekinumab group. The mean PASI score before therapy was 22.17 points, and after 16 weeks — 3.67 points, the mean dynamics was 19.28 points, the median dynamics was","PeriodicalId":37561,"journal":{"name":"Voprosy Sovremennoi Pediatrii - Current Pediatrics","volume":"8 3","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135726332","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nikolay N. Murashkin, Ekaterina S. Pavlova, Roman V. Epishev, Alexander I. Materikin, Roman A. Ivanov, Alena A. Savelova, Dmitriy V. Fedorov, Maria A. Leonova
The skin is the largest organ of the human body, it creates protective barrier between the internal and external environment. Skin barrier damage may result in homeostasis imbalance, inflammation, or bacterial infection. The microbiome plays a crucial role in maintaining normal skin functioning: control of pathogenic diversity of microorganisms, stimulate immune cells, and modulate chronic dermatoses development. There are various mechanisms for restoring skin barrier function. They are associated with the microorganisms’ activity. Thus, skin restoration is an important task included in the general concept of atopic dermatitis management. One of such methods is the skin surface colonization with commensals, so significant role is assigned to the new dermatological drugs. The normalization of the microbiome in affected skin areas with cosmetic care products can significantly affect the result of skin barrier restoration.
{"title":"Skin Microbiome Composition and Key Factors of its Barrier Function","authors":"Nikolay N. Murashkin, Ekaterina S. Pavlova, Roman V. Epishev, Alexander I. Materikin, Roman A. Ivanov, Alena A. Savelova, Dmitriy V. Fedorov, Maria A. Leonova","doi":"10.15690/vsp.v22i5.2624","DOIUrl":"https://doi.org/10.15690/vsp.v22i5.2624","url":null,"abstract":"The skin is the largest organ of the human body, it creates protective barrier between the internal and external environment. Skin barrier damage may result in homeostasis imbalance, inflammation, or bacterial infection. The microbiome plays a crucial role in maintaining normal skin functioning: control of pathogenic diversity of microorganisms, stimulate immune cells, and modulate chronic dermatoses development. There are various mechanisms for restoring skin barrier function. They are associated with the microorganisms’ activity. Thus, skin restoration is an important task included in the general concept of atopic dermatitis management. One of such methods is the skin surface colonization with commensals, so significant role is assigned to the new dermatological drugs. The normalization of the microbiome in affected skin areas with cosmetic care products can significantly affect the result of skin barrier restoration.","PeriodicalId":37561,"journal":{"name":"Voprosy Sovremennoi Pediatrii - Current Pediatrics","volume":"12 9","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135725626","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nikolay N. Murashkin, Maria A. Leonova, Leonid A. Opryatin, Alexander I. Materikin, Roman V. Epishev, Dmitri V. Fedorov
Acne is chronic inflammatory skin disease. Its examination and management tactics depend on the patient's age and disease clinical form. This article presents treatment algorithms for acne vulgaris in adolescent and pre-adolescent children and describes clinical cases.
{"title":"Papulopustular Acne: Differential Diagnosis and Therapy Algorithms in Prepubertal and Pubertal Age","authors":"Nikolay N. Murashkin, Maria A. Leonova, Leonid A. Opryatin, Alexander I. Materikin, Roman V. Epishev, Dmitri V. Fedorov","doi":"10.15690/vsp.v22i5.2639","DOIUrl":"https://doi.org/10.15690/vsp.v22i5.2639","url":null,"abstract":"Acne is chronic inflammatory skin disease. Its examination and management tactics depend on the patient's age and disease clinical form. This article presents treatment algorithms for acne vulgaris in adolescent and pre-adolescent children and describes clinical cases.","PeriodicalId":37561,"journal":{"name":"Voprosy Sovremennoi Pediatrii - Current Pediatrics","volume":"8 1-2","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135726333","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nikolay N. Murashkin, Roman V. Epishev, Olesya D. Dubenko, Alexander I. Materikin, Leonid A. Opryatin, Roman A. Ivanov, Alena A. Savelova, Maria A. Leonova
Background. Hypotrichosis is a heritable form of alopecia that causes almost complete scalp hair loss in childhood. The diagnosis is typically established according to medical history and clinical picture. Genetic testing is an additional diagnostic method that allows to establish “hypotrichosis” in cases of ambiguous or subtle clinical signs of the disease, as well as to perform differential diagnosis of this condition with others commonly associated with atopic dermatitis (focal and diffuse alopecia). Clinical diagnosis of hypotrichosis in patients with severe dermatosis is challenging. Clinical case description. The girl, aged 2 years 4 months, was hospitalized with complaints on extended rash on the body with severe itching, and changes in hair thickness, quality of hair shaft, and hair loss. Severe atopic dermatitis and hypotrichosis were diagnosed. The diagnosis of atopic dermatitis was established clinically (SCORAD — 65), the diagnosis of hypotrichosis (type 6, monilethrix-like hypotrichosis) was confirmed via molecular genetic testing (nucleotide variant c.699C>T in exone 13 of the DSG4 gene (OMIM # 607892)). Patient's mother, brother, and two sisters were diagnosed with the nucleotide variant chr18:31409487C>T in heterozygous state in the DSG4 gene. Mother and brother had no clinical manifestations, while both sisters had mild scalp hair damage, as well as eyebrows and eyelashes, since birth. There were no clinical manifestations of atopic dermatitis in parents and other children in the family. Conclusion . Hypotrichosis clinical signs can be subtled, smoothed, or imitate the symptoms of other comorbid conditions, especially in patients with comorbidities or severe dermatosis. Also worth noting is that changes in certain genes can aggravate the course of atopic dermatitis. In this clinical case, mutation in the DSG4 gene leads to epidermal barrier failure by disrupting the synthesis of desmosomes transmembrane components. Thus, geneticist consultation and genetic testing (search for changes in certain genes) are crucial in such cases.
{"title":"<i>DSG4</i> Gene Variants as a Cause of Hypotrichosis in the Child with Severe Atopic Dermatitis: Clinical Case","authors":"Nikolay N. Murashkin, Roman V. Epishev, Olesya D. Dubenko, Alexander I. Materikin, Leonid A. Opryatin, Roman A. Ivanov, Alena A. Savelova, Maria A. Leonova","doi":"10.15690/vsp.v22i5.2620","DOIUrl":"https://doi.org/10.15690/vsp.v22i5.2620","url":null,"abstract":"Background. Hypotrichosis is a heritable form of alopecia that causes almost complete scalp hair loss in childhood. The diagnosis is typically established according to medical history and clinical picture. Genetic testing is an additional diagnostic method that allows to establish “hypotrichosis” in cases of ambiguous or subtle clinical signs of the disease, as well as to perform differential diagnosis of this condition with others commonly associated with atopic dermatitis (focal and diffuse alopecia). Clinical diagnosis of hypotrichosis in patients with severe dermatosis is challenging. Clinical case description. The girl, aged 2 years 4 months, was hospitalized with complaints on extended rash on the body with severe itching, and changes in hair thickness, quality of hair shaft, and hair loss. Severe atopic dermatitis and hypotrichosis were diagnosed. The diagnosis of atopic dermatitis was established clinically (SCORAD — 65), the diagnosis of hypotrichosis (type 6, monilethrix-like hypotrichosis) was confirmed via molecular genetic testing (nucleotide variant c.699C>T in exone 13 of the DSG4 gene (OMIM # 607892)). Patient's mother, brother, and two sisters were diagnosed with the nucleotide variant chr18:31409487C>T in heterozygous state in the DSG4 gene. Mother and brother had no clinical manifestations, while both sisters had mild scalp hair damage, as well as eyebrows and eyelashes, since birth. There were no clinical manifestations of atopic dermatitis in parents and other children in the family. Conclusion . Hypotrichosis clinical signs can be subtled, smoothed, or imitate the symptoms of other comorbid conditions, especially in patients with comorbidities or severe dermatosis. Also worth noting is that changes in certain genes can aggravate the course of atopic dermatitis. In this clinical case, mutation in the DSG4 gene leads to epidermal barrier failure by disrupting the synthesis of desmosomes transmembrane components. Thus, geneticist consultation and genetic testing (search for changes in certain genes) are crucial in such cases.","PeriodicalId":37561,"journal":{"name":"Voprosy Sovremennoi Pediatrii - Current Pediatrics","volume":"12 8","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135725627","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Background . Congenital central hypoventilation syndrome (CCHS), or Ondine’s Curse, is rare, incurable and life-threatening disease characterized by autonomic nervous system disorders, it manifests with disability to maintain ventilation function during sleep. Sensitivity to hypoxia and hypercapnia is reduced in case of CCHS, thus, it leads to recurrent episodes of deep apnea. The world literature describes just over 1000 cases of this disease. Clinical case description . An infant born at 37th week of gestation, weight of 3330 g, had episodes of apnea and hypercapnia from the first day of life. CCHS was suspected by the 28th day of life after excluding other causes of respiratory disorders, and it was genetically confirmed by the 43rd day of life — pathogenic variant of PHOX2B gene was revealed. Mechanical ventilation has been initiated by the age of 1 month after disease worsening. Analysis of CCHS cases published in Russian-language medical literature was performed. Typical symptoms and timing of their manifestation, as well as the time before correct diagnosis were mentioned. Conclusion . Symptoms that can be suggestive of CCHS presence early after birth and can urge to perform all the necessary genetic testing that are crucial for timely treatment onset and for minimizing the negative effect of hypoxemia and hypercapnia on the child are described.
背景。先天性中枢性低通气综合征(Congenital central hypoventilation syndrome, CCHS)，又称Ondine 's Curse，是一种罕见的、无法治愈的、危及生命的疾病，以自主神经系统功能紊乱为特征，表现为睡眠时无法维持通气功能。CCHS对缺氧和高碳酸血症的敏感性降低，因此导致深呼吸暂停反复发作。世界文献描述了1000多例这种疾病。临床病例描述。妊娠第37周出生的婴儿，体重3330 g，从出生第一天起就出现呼吸暂停和高碳酸血症发作。在排除其他呼吸系统疾病原因后，于出生第28天怀疑为CCHS，在出生第43天遗传学上确诊为PHOX2B基因致病性变异。病情恶化后1个月开始机械通气。对俄文医学文献中发表的CCHS病例进行分析。并介绍了典型症状、表现时间及正确诊断前的时间。结论。出生后早期可能提示CCHS存在的症状，并可能敦促进行所有必要的基因检测，这对于及时治疗和减少低氧血症和高碳酸血症对儿童的负面影响至关重要。
{"title":"Congenital Central Hypoventilation Syndrome (Ondine’s Curse): Clinical Case","authors":"Teimur S. Adylov, Evgenii V. Shestak","doi":"10.15690/vsp.v22i4.2592","DOIUrl":"https://doi.org/10.15690/vsp.v22i4.2592","url":null,"abstract":"Background . Congenital central hypoventilation syndrome (CCHS), or Ondine’s Curse, is rare, incurable and life-threatening disease characterized by autonomic nervous system disorders, it manifests with disability to maintain ventilation function during sleep. Sensitivity to hypoxia and hypercapnia is reduced in case of CCHS, thus, it leads to recurrent episodes of deep apnea. The world literature describes just over 1000 cases of this disease. Clinical case description . An infant born at 37th week of gestation, weight of 3330 g, had episodes of apnea and hypercapnia from the first day of life. CCHS was suspected by the 28th day of life after excluding other causes of respiratory disorders, and it was genetically confirmed by the 43rd day of life — pathogenic variant of PHOX2B gene was revealed. Mechanical ventilation has been initiated by the age of 1 month after disease worsening. Analysis of CCHS cases published in Russian-language medical literature was performed. Typical symptoms and timing of their manifestation, as well as the time before correct diagnosis were mentioned. Conclusion . Symptoms that can be suggestive of CCHS presence early after birth and can urge to perform all the necessary genetic testing that are crucial for timely treatment onset and for minimizing the negative effect of hypoxemia and hypercapnia on the child are described.","PeriodicalId":37561,"journal":{"name":"Voprosy Sovremennoi Pediatrii - Current Pediatrics","volume":"33 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134930585","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
XXIV Конгресс педиатров России, в котором приняли участие около 10 тыс. участников (из них свыше 3 тыс. очных делегатов) из различных регионов Российской Федерации, среди которых — педиатры, детские специалисты, организаторы детского здравоохранения, а также ученые, преподаватели высшей школы, студенты, ординаторы и аспиранты, обсудил актуальные проблемы охраны здоровья детей и принял следующую резолюцию.
{"title":"Resolution of the ХХIV Congress of Pediatricians of Russia with International Participation “Actual Problems of Pediatrics”","authors":"Article Editorial","doi":"10.15690/vsp.v22i4.2596","DOIUrl":"https://doi.org/10.15690/vsp.v22i4.2596","url":null,"abstract":"XXIV Конгресс педиатров России, в котором приняли участие около 10 тыс. участников (из них свыше 3 тыс. очных делегатов) из различных регионов Российской Федерации, среди которых — педиатры, детские специалисты, организаторы детского здравоохранения, а также ученые, преподаватели высшей школы, студенты, ординаторы и аспиранты, обсудил актуальные проблемы охраны здоровья детей и принял следующую резолюцию.","PeriodicalId":37561,"journal":{"name":"Voprosy Sovremennoi Pediatrii - Current Pediatrics","volume":"28 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134930588","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Профилактика инфекционных заболеваний является одним из приоритетов в сфере охраны здоровья (Федеральный закон от 21 ноября 2011 г. № 323-ФЗ «Об основах охраны здоровья граждан в Российской Федерации» (действующая редакция, 2016 г.).
{"title":"Resolution of the Independent Expert Council of The Union of Experts in the Field of Immunoprophylaxis","authors":"Article Editorial","doi":"10.15690/vsp.v22i4.2598","DOIUrl":"https://doi.org/10.15690/vsp.v22i4.2598","url":null,"abstract":"Профилактика инфекционных заболеваний является одним из приоритетов в сфере охраны здоровья (Федеральный закон от 21 ноября 2011 г. № 323-ФЗ «Об основах охраны здоровья граждан в Российской Федерации» (действующая редакция, 2016 г.).","PeriodicalId":37561,"journal":{"name":"Voprosy Sovremennoi Pediatrii - Current Pediatrics","volume":"18 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134930584","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
28 июня 2023 г. исполняется 100 лет со дня рождения выдающегося ученого и организатора здравоохранения, лидера отечественной детской хирургии второй половины ХХ столетия, академика, заслуженного деятеля науки Юрия Федоровича Исакова.
{"title":"Outstanding pediatric surgeon Yuri Fedorovich Isakov (on the 100th anniversary of his birth)","authors":"Article Editorial","doi":"10.15690/vsp.v22i4.2589","DOIUrl":"https://doi.org/10.15690/vsp.v22i4.2589","url":null,"abstract":"28 июня 2023 г. исполняется 100 лет со дня рождения выдающегося ученого и организатора здравоохранения, лидера отечественной детской хирургии второй половины ХХ столетия, академика, заслуженного деятеля науки Юрия Федоровича Исакова.","PeriodicalId":37561,"journal":{"name":"Voprosy Sovremennoi Pediatrii - Current Pediatrics","volume":"20 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-08-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135466013","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Leonid M. Roshal is 90 Years old","authors":"Article Editorial","doi":"10.15690/vsp.v22i2.2561","DOIUrl":"https://doi.org/10.15690/vsp.v22i2.2561","url":null,"abstract":".","PeriodicalId":37561,"journal":{"name":"Voprosy Sovremennoi Pediatrii - Current Pediatrics","volume":"39 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-05-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135962096","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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