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Computational design of soluble functional analogues of integral membrane proteins. 整体膜蛋白可溶性功能类似物的计算设计。
Q3 Medicine Pub Date : 2024-03-07 DOI: 10.1101/2023.05.09.540044
Casper A Goverde, Martin Pacesa, Nicolas Goldbach, Lars J Dornfeld, Petra E M Balbi, Sandrine Georgeon, Stéphane Rosset, Srajan Kapoor, Jagrity Choudhury, Justas Dauparas, Christian Schellhaas, Simon Kozlov, David Baker, Sergey Ovchinnikov, Alex J Vecchio, Bruno E Correia

De novo design of complex protein folds using solely computational means remains a significant challenge. Here, we use a robust deep learning pipeline to design complex folds and soluble analogues of integral membrane proteins. Unique membrane topologies, such as those from GPCRs, are not found in the soluble proteome and we demonstrate that their structural features can be recapitulated in solution. Biophysical analyses reveal high thermal stability of the designs and experimental structures show remarkable design accuracy. The soluble analogues were functionalized with native structural motifs, standing as a proof-of-concept for bringing membrane protein functions to the soluble proteome, potentially enabling new approaches in drug discovery. In summary, we designed complex protein topologies and enriched them with functionalities from membrane proteins, with high experimental success rates, leading to a de facto expansion of the functional soluble fold space.

仅利用计算手段重新设计复杂的蛋白质折叠仍然是一项重大挑战。在这里,我们使用强大的深度学习管道来设计复杂的折叠和整体膜蛋白的可溶性类似物。独特的膜拓扑结构(如来自 GPCR 的拓扑结构)在可溶性蛋白质组中找不到,我们证明了它们的结构特征可以在溶液中重现。生物物理分析表明,这些设计具有很高的热稳定性,而实验结构则显示出显著的设计准确性。可溶性类似物具有本地结构基团的功能,是将膜蛋白功能引入可溶性蛋白质组的概念验证,有可能为药物发现提供新方法。总之,我们设计了复杂的蛋白质拓扑结构,并用膜蛋白的功能丰富了这些拓扑结构,实验成功率很高,从而事实上扩大了功能性可溶性折叠空间。
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引用次数: 0
Role of functional nutrition in reducing the incidence of acute respiratory infections in children 功能性营养在降低儿童急性呼吸道感染发病率中的作用
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.20953/1727-5784-2023-1-14-25
T. G. Malanicheva, E. Agafonova, O. Kuznetsova
Objective. To study the role of adapted formulas and drinks in reducing the incidence of acute respiratory infections (ARIs) in infants and toddlers. Patients and methods. This study was conducted in two stages: stage 1 included infants (0–1 year) (n = 105), and stage 2 included the same children aged 1 to 3 years (n = 96). In the control group (n = 35, 0–1 year; n = 32, 1–3 years), children were breastfed during the first year of life. In the study group (n = 35, 0–1 year; n = 32, 1–3 years), children received adapted formulas and drinks based on New Zealand goat’s whole milk: between 1 and 5 months – NANNI 1; from 6 months – NANNI 2; between 1 and 3 years – NANNI 3 in the amount of 200 mL/day. In the comparison group (n = 35, 0–1 year; n = 32, 1–3 years), children received an adapted milk formula based on whole cow’s milk protein: formula 1 in the first 6 months of life, formula 2 in the second 6 months of life, and formula 3 between the ages of 1 and 3 years in the amount of 200 mL/day. At the first and second stages, we evaluated the incidence of ARI episodes during the year and mucosal immunity parameters: cellular composition of smears, cytomorphological features and functional activity of neutrophils (phagocytosis, intracellular biocide concentration, intraleukocytic enzyme systems: myeloperoxidase, cationic proteins, acid phosphatase). Results. The mean number of ARI episodes during the first and second stages in the study group (2.3) did not differ from the control group (1.8, p > 0.05). The incidence of ARIs was higher in the comparison group (3.6; p < 0.05). The content of the main cell populations in the rhinocytogram (neutrophils, lymphocytes, eosinophils, epithelial cells) at the first and second stages was comparable in children in the study and control groups; in these groups, fewer cells with pronounced damage and higher phagocytic activity of neutrophils were recorded. The maximum levels of enzyme systems were registered in children in the control group (myeloperoxidase: 0.41 OD × 10-3; acid phosphatase: 0.39 OD × 10-3; cationic proteins: 0.29 OD × 10-3). The levels of enzyme systems in the study group at the first and second stages were comparable. Conclusion. A lower incidence of ARIs, optimal body resistance both in infants and toddlers develop against the background of breastfeeding. Children who received formulas based on New Zealand goat’s milk had higher resistance compared to those who received adapted milk formulas based on whole cow’s milk protein, and their mucosal immunity parameters approached the values of these parameters in breastfed infants. Key words: children, infants and toddlers, functional nutrition, adapted formulas, acute respiratory infections
目标。研究适应配方和饮料在降低婴幼儿急性呼吸道感染发生率方面的作用。患者和方法。本研究分为两个阶段进行:第一阶段包括0-1岁的婴儿(n = 105),第二阶段包括1 - 3岁的儿童(n = 96)。对照组(n = 35, 0 ~ 1岁;N = 32, 1-3岁),儿童在出生后第一年接受母乳喂养。研究组(n = 35, 0-1岁;n = 32, 1 - 3岁),儿童接受基于新西兰山羊全脂牛奶的改编配方和饮料:1至5个月- NANNI 1;6个月起-南尼2;1至3岁之间- NANNI 3的量为200毫升/天。对照组(n = 35, 0-1年;n = 32, 1 - 3岁),儿童接受以全脂牛奶蛋白为基础的改编配方奶粉:前6个月服用配方奶粉1,后6个月服用配方奶粉2,1 - 3岁服用配方奶粉3,剂量为200 mL/天。在第一和第二阶段,我们评估了一年中ARI发作的发生率和粘膜免疫参数:涂片的细胞组成、细胞形态学特征和中性粒细胞的功能活性(吞噬作用、细胞内杀菌剂浓度、白细胞内酶系统:髓过氧化物酶、阳离子蛋白、酸性磷酸酶)。结果。研究组第一和第二阶段ARI发作的平均次数(2.3次)与对照组(1.8次,p < 0.05)没有差异。ARIs的发生率高于对照组(3.6;P < 0.05)。第一和第二阶段鼻细胞图中主要细胞群(中性粒细胞、淋巴细胞、嗜酸性粒细胞、上皮细胞)的含量在研究组和对照组的儿童中是相当的;在这些组中,有明显损伤的细胞较少,中性粒细胞的吞噬活性较高。对照组儿童的酶系统最高水平(髓过氧化物酶:0.41 OD × 10-3;酸性磷酸酶:0.39 OD × 10-3;阳离子蛋白:0.29 OD × 10-3)。研究组在第一和第二阶段的酶系统水平具有可比性。结论。在母乳喂养的背景下,婴儿和学步儿童的ARIs发生率较低,最佳的身体抵抗力。食用新西兰羊奶配方奶粉的儿童比食用全牛奶蛋白配方奶粉的儿童具有更高的抵抗力,其粘膜免疫参数接近母乳喂养婴儿的这些参数值。关键词:儿童,婴幼儿,功能性营养,适应配方,急性呼吸道感染
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引用次数: 0
Intramural esophageal dissection, a rare complication of eosinophilic esophagitis 壁内食管夹层,嗜酸性食管炎的一种罕见并发症
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.20953/1727-5784-2023-2-63-69
U.M.K. Babaeva, N.K. Pavelets, E. Kornienko
Eosinophilic esophagitis (EoE) is characterized clinically by typical endoscopic features and the presence of more than 15 eosinophils visible per high-power field in esophageal mucosal biopsy samples. Due to deep chronic eosinophilic inflammation, it can lead to complications, such as esophageal stenosis and rarely perforation. Intramural esophageal dissection (IED) is a previously undescribed complication of EoE in children. This article presents a clinical case of IED in a 15-year-old boy with clinical debut as sudden epigastric pain and nausea against the background of acute respiratory viral infection. Endoscopy, computed tomography, and X-ray examination revealed a double-barrel esophagus with two cavities, the esophagus itself and a blind diverticulum (pouch or “pocket”), in which granulation tissue and active inflammation with many neutrophils were detected. Initial histological assessment showed no significant eosinophilic infiltration in the esophagus. After 6 months, endoscopy revealed characteristic signs of EoE: longitudinal grooves and erosions, transverse rings, narrowing of the esophageal lumen. In the middle third, the picture of double-barrel esophagus remained unchanged, but without signs of acute detachment and with a dense septum between two esophageal cavities. Histologically, the infiltrate contained more than 50 eosinophils per high-power field. The diagnosis of EoE complicated by IED was established. A strict hypoallergenic diet and an oral gel budesonide were prescribed. Endoscopic dissection of the esophageal septum was performed, its patency was restored. In 2 months, during a re-examination after a course of pharmacotherapy and diet, there were no complaints, radiological evaluation of esophageal patency showed no abnormalities. Endoscopically, the esophagus was freely passable, there was a small fold at the surgical site, longitudinal grooves and small erosions in the lower third maintained. Histologically, the number of eosinophils decreased significantly. The therapy was continued. This clinical case demonstrates the possibility of IED development as a complication of EoE. The course of EoE can be asymptomatic, while IED can serve as the first manifestation of the disease. The endoscopic presentation of double-barrel esophagus is a consequence of dissection. The combination of a hypoallergenic diet, topical steroids, and endoscopic septectomy is effective in the treatment of EoE with IED. Key words: eosinophilic esophagitis, intramural esophageal dissection, double-barrel esophagus
嗜酸性粒细胞性食管炎(EoE)的临床特征是典型的内镜特征,食管粘膜活检样本在高倍视野下可见超过15个嗜酸性粒细胞。由于深慢性嗜酸性粒细胞炎症,它可导致并发症,如食管狭窄,很少穿孔。壁内食管夹层(IED)是一种以前未被描述的儿童EoE并发症。本文报告一个15岁男孩的IED临床病例,临床表现为急性呼吸道病毒感染背景下的突发性上腹痛和恶心。内窥镜、计算机断层扫描及x线检查显示双桶状食管,有两个空腔,食管本身和盲区憩室(袋或“口袋”),其中可见肉芽组织和活跃的炎症,有许多中性粒细胞。初步组织学检查显示食管无明显嗜酸性粒细胞浸润。6个月后,内镜检查发现EoE的特征性征象:纵沟和糜烂,横环,食管管腔狭窄。在中间三分之一,双桶食管图像保持不变,但没有急性脱离的迹象,两个食管腔之间有致密的间隔。组织学上,每高倍视场浸润含有50多个嗜酸性粒细胞。建立了EoE合并IED的诊断。严格的低过敏性饮食和口服布地奈德凝胶。内镜下切开食管隔,恢复其通畅。2个月后,在一个疗程的药物治疗和饮食后复查,无主诉,食道通畅影像学检查未见异常。内镜下,食管通畅,手术部位有小褶皱,食管下三分之一处有纵沟和小糜烂。组织学上,嗜酸性粒细胞数量明显减少。治疗继续进行。本临床病例证明了IED发展为EoE并发症的可能性。EoE病程可无症状,而IED可作为该病的首发表现。双桶食管的内镜表现是剥离的结果。低过敏性饮食、局部类固醇和内窥镜下鼻中隔切除术联合治疗EoE合并IED是有效的。关键词:嗜酸性粒细胞性食管炎,壁内食管夹层,双桶食管
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引用次数: 0
Current principles of nutritional management of late preterm infants 晚期早产儿营养管理的现行原则
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.20953/1727-5784-2023-3-53-65
H. Sarkisyan, I. V. Zhuravleva, P. Shumilov, A. Volkova, O. V. Ryazanova, O. Khandamirova
Neonates born between 34 0/7 and 36 6/7 weeks of gestation are considered late preterm (LPT) and constitute the largest group of all preterm infants. The rate of morbidity and mortality, as well as the frequency of repeated hospitalizations among LPT infants are significantly higher compared to full-term infants, which is explained by immaturity due to the lack of a critical period of their intrauterine growth and development. Fairly common they face nutritional problems leading to adverse immediate and long-term outcomes. Until recently, the lack of clear feeding recommendations for LPT infants determined the use of individual approaches to optimize their nutrition. The annual increase in the number of LPT infants has prompted the accumulation of new scientific information concerning both the anatomical and physiological characteristics and principles of development of these children in general and specific problems, including those related to nutrition. Comprehensive support and appropriate breastfeeding are the cornerstone of adequate nutrition for LPT infants. Depending on factors such as gestational age, birth weight, and comorbidities, it is often necessary to increase nutritional support, including the use of breast milk fortifiers, nutrientenriched formulas, and parenteral nutrition prescription. The process of achieving normal nutritional status in LPT infants can be complicated by episodes of hypoglycemia and prolonged jaundice, delay in postnatal growth and development, which requires a special approach. This review highlights the most common problems of nutritional management of LPT infants and provides current recommendations for optimal feeding of this group of preterm infants. Key words: late preterm infant, nutritional requirements, breastfeeding, donor milk, specialized formula, breast milk fortifiers, hypoglycemia, hyperbilirubinemia
在妊娠34 0/7周至36 6/7周之间出生的新生儿被认为是晚期早产儿(LPT),构成了所有早产儿中最大的群体。LPT婴儿的发病率和死亡率以及反复住院的频率明显高于足月婴儿,这是由于缺乏宫内生长发育的关键时期而导致的不成熟。相当普遍的是,他们面临营养问题,导致不良的即时和长期后果。直到最近,对LPT婴儿缺乏明确的喂养建议决定了使用个别方法来优化他们的营养。LPT婴儿数量的逐年增加促使人们积累了关于这些儿童一般和具体问题(包括与营养有关的问题)的解剖和生理特征以及发展原则的新的科学信息。全面的支持和适当的母乳喂养是LPT婴儿获得充足营养的基石。根据胎龄、出生体重和合并症等因素,通常有必要增加营养支持,包括使用母乳强化剂、营养丰富的配方奶和肠外营养处方。LPT患儿在实现正常营养状态的过程中,会出现低血糖、黄疸延长、出生后生长发育迟缓等并发症,需要特殊的治疗方法。这篇综述强调了LPT婴儿营养管理中最常见的问题,并提供了当前对这组早产儿的最佳喂养建议。关键词:晚期早产儿,营养需求,母乳喂养,供乳,专用配方奶,母乳强化剂,低血糖,高胆红素血症
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引用次数: 0
Impact of nutrients on gut microbiota 营养物质对肠道微生物群的影响
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.20953/1727-5784-2023-1-66-75
A. Khavkin, A. Zavyalova, V. Novikova
The influence of nutrition on the gut microbiome formation is undeniable. From the moment a childr is born, the duration and exclusivity of breastfeeding, the timing of introduction and choice of complementary foods shape his or her microbiome. Subsequently, acute and chronic diseases, drug exposure, and physical activity maintain the “health” of the luminal microbiome. Nutritional adequacy and balance in accordance with age standards, as well as eating habits are important for the formation of the gut microbiome. Various foods with prebiotic properties contribute to the microbiome biodiversity. This review describes the effects of foods, nutrients, and prebiotics on the microbiome of children, athletes, and the elderly. The system’s fluidity leads to metabolic changes that affect the whole body. The time span of this impact matters: it either changes the developmental vector with early influence or corrects the constants already in place. At the same time, the directions of microbiome modifications are diverse: from improving athletic performance to preventing sarcopenia and cachexia in older people. Key words: microbiota, prebiotics, children, athletes, sarcopenia
营养对肠道微生物群形成的影响是不可否认的。从婴儿出生的那一刻起,母乳喂养的持续时间和排他性、引入辅食的时间和选择就会塑造他或她的微生物群。随后,急性和慢性疾病、药物暴露和身体活动维持了肠道微生物群的“健康”。符合年龄标准的营养充足和平衡,以及饮食习惯对肠道微生物群的形成很重要。具有益生元特性的各种食物有助于微生物群的生物多样性。本文综述了食物、营养物质和益生元对儿童、运动员和老年人微生物组的影响。该系统的流动性导致影响整个身体的代谢变化。这种影响的时间跨度很重要:它要么改变具有早期影响的发展矢量,要么纠正已经存在的常数。与此同时,微生物组修饰的方向是多样的:从提高运动成绩到预防老年人的肌肉减少症和恶病质。关键词:微生物群,益生元,儿童,运动员,肌肉减少症
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引用次数: 0
Clinical case of acute food-protein induced enterocolitis syndrome (FPIES) as a manifestation of multiple food allergies 以多种食物过敏为表现的急性食物蛋白性小肠结肠炎综合征1例
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.20953/1727-5784-2023-1-94-100
E. A. Filippova, N. Esakova, E. Varlamov, A. Pampura
Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated food allergy manifested by delayed onset of gastrointestinal symptoms (1–4 hours after allergen consumption), which can lead to severe life-threatening conditions. The absence of specific laboratory markers of FPIES allowing to determine the trigger and confirm the diagnosis, unclear clinical features often masked by acute intestinal infections and other gastrointestinal diseases create significant difficulties in identifying patients with FPIES, choosing the right tactics for their management, and prescribing an adequate elimination diet. This article presents a clinical case of a patient who had multiple episodes of severe clinical manifestations of FPIES since the age of 6 months, requiring emergency hospitalization. The diagnosis of FPIES was first suspected at the age of 1.5 years; subsequently, the diagnosis was clinically confirmed, and a plan for further management of the child was developed. Key words: food protein-induced enterocolitis syndrome (FPIES), children, diagnosis, food allergy, trigger, oral food challenge test, cow’s milk protein allergy, meat allergy
食物蛋白诱导的小肠结肠炎综合征(FPIES)是一种非ige介导的食物过敏,表现为胃肠道症状延迟发作(食用过敏原后1-4小时),可导致严重危及生命的疾病。由于缺乏特异性的实验室标记物来确定诱发因素并确认诊断,不明确的临床特征常常被急性肠道感染和其他胃肠道疾病所掩盖,因此在识别FPIES患者、选择正确的治疗策略和制定适当的消除饮食方面存在重大困难。本文报告一例患者自6个月大以来多次出现严重的临床表现,需要紧急住院治疗。在1.5岁时首次怀疑诊断为FPIES;随后,诊断得到临床证实,并制定了儿童进一步治疗的计划。关键词:食物蛋白性小肠结肠炎综合征(FPIES),儿童,诊断,食物过敏,诱发因素,口服食物激发试验,牛奶蛋白过敏,肉类过敏
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引用次数: 0
Malnutrition in children with cystic fibrosis and its correction with a specialized formula 囊性纤维化儿童营养不良及专用配方矫正
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.20953/1727-5784-2023-1-5-13
T. Bushueva, T. Borovik, E. Roslavtseva, O. Simonova, I. Sokolov, I. Guseva, N. V. Lyabina, A. Sokolova, A. Fisenko
Objective. To determine the safety, tolerability, and clinical efficacy of the specialized dietary product “Clinutren Junior” in children with cystic fibrosis at the age of 1 to 10 years. Patients and methods. This study included 20 patients with cystic fibrosis (CF) aged 1 to 10 years who underwent treatment and rehabilitation at the Pulmonology Department of the National Medical Research Center for Children’s Health. According to the Shwachman index (SI), a severe course of CF (SI = 34.24 ± 1.97) was observed in 4 (20%) patients, a moderate course of CF (SI = 49.53 ± 1.76) in 12 (60%) patients, and a mild course of CF (SI = 61.5 ± 2.05) in 4 (20%) patients. All patients had malnutrition of varying severity. During dynamic observation, the children’s general health condition, nutritional status, including anthropometric and biochemical parameters, biological and nutritional value of the diet were evaluated. Results. Beneficial organoleptic properties of the formula and its good tolerability were noted. The analysis of the actual nutrition showed that the study product increased the protein quota and energy value of the age-differentiated standard sparing diet by 23-24%. Against the background of using the specialized formula, there was a tendency to improve the physical development of children, as evidenced by the complete elimination of mild malnutrition in 3 (3.8%) patients and a decrease in the number of patients with severe malnutrition. Conclusion. Inclusion of the specialized dietary product “Clinutren Junior” in the therapeutic diet for CF patients enhances and maintains their nutritional status at a satisfactory level, which in general provides a positive prognosis of the disease and improves the quality of life of patients and their families. Key words: cystic fibrosis, children, malnutrition, specialized food products
目标。确定专用膳食产品“Clinutren Junior”在1至10岁囊性纤维化儿童中的安全性、耐受性和临床疗效。患者和方法。本研究包括20例1至10岁囊性纤维化(CF)患者,他们在国家儿童健康医学研究中心肺病科接受治疗和康复。根据Shwachman指数(SI), 4例(20%)患者为重度CF (SI = 34.24±1.97),12例(60%)患者为中度CF (SI = 49.53±1.76),4例(20%)患者为轻度CF (SI = 61.5±2.05)。所有患者都有不同程度的营养不良。在动态观察中,评估儿童的一般健康状况、营养状况,包括人体测量和生化参数、饮食的生物学和营养价值。结果。注意到该配方有益的感官特性及其良好的耐受性。实际营养分析表明,研究产品可使分龄标准节约日粮的蛋白质配额和能量值提高23-24%。在使用专业配方奶粉的背景下,儿童的身体发育有改善的趋势,3例(3.8%)患者轻度营养不良完全消除,严重营养不良患者数量减少。结论。在CF患者的治疗性饮食中加入专门的膳食产品“Clinutren Junior”,可以提高并维持CF患者的营养状况在一个令人满意的水平,总体上提供了疾病的积极预后,并改善了患者及其家庭的生活质量。关键词:囊性纤维化;儿童;营养不良
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引用次数: 0
Population-based study of the prevalence of celiac disease among schoolchildren in Moscow 莫斯科学龄儿童乳糜泻患病率的人群基础研究
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.20953/1727-5784-2023-2-13-23
A. Antishin, A.R. Polishchuk, M. A. Manina, A. S. Tertychnyy, Ju.A. Drozdova, S. I. Erdes
Objective. Examination of schoolchildren in Moscow using a specially designed questionnaire to identify children at risk of developing celiac disease. Patients and methods. The study enrolled 3070 Moscow schoolchildren aged 7–18 years old. Serological and genetic examinations were performed in 42 children at risk for celiac disease, including determination of IgA and IgE to wheat, IgA and IgG to tissue transglutaminase, and IgA to endomysium. Molecular HLA testing for alleles that predispose to celiac disease was conducted. Endoscopic and morphological diagnostics were performed when indicated. Results. The risk group for celiac disease amounted to 10.2% of all schoolchildren surveyed. According to the results of the questionnaire, children in the risk group significantly more often had abdominal pain, excess gas, bloating, flatulence, dyspeptic complaints (diarrhea, constipation, vomiting), asthma attacks or other forms of allergies, headache, joint or body pain, fatigue and weakness, mood swings, depression, anxiety, or episodes of emotional arousal. Changes in height, weight, delayed physical or sexual development, dental enamel defects, and skin rashes were also noted. Of the 42 children in the risk group, the DQ2 heterodimer was detected in 13 (31%) children, the DQ8 heterodimer – in 2 (4.8%), and a combination of DQ2 and DQ8 heterodimers – in 1 (2.4%). Thus, characteristic HLA haplotypes were identified in 38% of the examined children. Three children had IgA antibodies to tissue transglutaminase (at >10 norm) and IgA anti-endomysium antibody titers. Conclusion. The method of questioning by means of a specially designed questionnaire for school-age children allows to identify individuals at risk of developing celiac disease. According to serological, genetic, endoscopic, and morphological investigations, the incidence of celiac disease among children at risk was 7.1%. The prevalence of celiac disease among school-age children in Moscow corresponds to global trends, amounting to 0.7%. Key words: questionnaire, gluten-free diet, cereals, gluten intolerance, celiac disease, children
目标。使用特别设计的问卷对莫斯科学童进行检查,以确定有患乳糜泻风险的儿童。患者和方法。这项研究招募了3070名7-18岁的莫斯科小学生。对42名有乳糜泻风险的儿童进行血清学和遗传学检查,包括测定小麦的IgA和IgE,组织转谷氨酰胺酶的IgA和IgG,以及肌内膜的IgA。对易患乳糜泻的等位基因进行HLA分子检测。内镜和形态学诊断时进行指示。结果。患乳糜泻的风险群体占所有受访学童的10.2%。根据问卷调查的结果,风险组的儿童更经常出现腹痛、胀气、胀气、消化不良症状(腹泻、便秘、呕吐)、哮喘发作或其他形式的过敏、头痛、关节或身体疼痛、疲劳和虚弱、情绪波动、抑郁、焦虑或情绪觉醒的发作。身高、体重、身体或性发育迟缓、牙釉质缺陷和皮疹的变化也被注意到。在危险组的42名儿童中,13名(31%)儿童检测到DQ2异源二聚体,2名(4.8%)儿童检测到DQ8异源二聚体,1名(2.4%)儿童检测到DQ2和DQ8异源二聚体。因此,在38%的被检查儿童中发现了特征性HLA单倍型。3例患儿有组织转谷氨酰胺酶IgA抗体(bbb10标准)和IgA抗肌内膜抗体滴度。结论。通过为学龄儿童特别设计的问卷的提问方法,可以确定有患乳糜泻风险的个体。根据血清学、遗传学、内窥镜和形态学调查,乳糜泻在高危儿童中的发病率为7.1%。莫斯科学龄儿童的乳糜泻患病率与全球趋势相符,为0.7%。关键词:问卷,无麸质饮食,谷物,麸质不耐症,乳糜泻,儿童
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引用次数: 0
Algorithm of formula selection for nutritional support and enteral nutrition in children with cerebral palsy 脑瘫患儿营养支持及肠内营养配方选择算法
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.20953/1727-5784-2023-3-28-40
O. Titova, N. Taran, A. V. Keleinikova, M. Shavkina, E. Pavlovskaya, T. Strokova
Objective. To develop an algorithm for selecting a nutritional support / enteral nutrition product in children with cerebral palsy based on nutritional status indicators and the nature of concomitant pathology. Patients and methods. This study included 261 children aged 2 to 17 years (159 (61%) boys) diagnosed with spastic cerebral palsy. All children were divided into different groups according to the Gross Motor Function Measure (GMFM) level and feeding method (per os / gastrostomy). Nutritional status and characteristics of gastrointestinal symptoms were investigated, and actual nutrition was assessed. Body composition was evaluated by measuring triceps skinfold thickness and mid-upper arm circumference. Dietary adjustments were made considering the identified disorders of nutritional status and gastrointestinal function. Follow-up examination was performed after 6–9 months to assess the efficacy of diet therapy. Results. An underweight condition was detected in 40–79% of cases and was most frequently observed in groups IV, V, VTF of GMFCS levels: 14 (21.9%), 33 (40.7%) and 18 (39.1%) children, respectively (pVTF–V–IV < 0,001). Stunting was recorded in groups IV, V, and VTF. The most common gastrointestinal disorder was constipation (47.1%). Energy deficiency was registered in 213 (81.6%) children and amounted to 12.3–61.8% from the norm. The rate of macronutrient deficiency: protein – 11.4–60.0%, fats – 10.9–61.6%, carbohydrates – 20.3–72.6% from the individual norm / recommended dietary intake. In the follow-up period, statistically significant positive dynamics of weight and height indicators, an increase in fat mass and skeletal muscle mass, and relief of gastrointestinal manifestations were observed against the background of diet therapy. Analysis of findings allowed us to develop an algorithm for selecting a nutritional support / enteral nutrition product for children with cerebral palsy. Conclusion. Evaluation of nutritional status and correction of its disorders requires a comprehensive approach in patients with cerebral palsy. In the absence of positive dynamics after 1–3 months, it is necessary to revise a dietary regimen. Key words: diet therapy algorithm, cerebral palsy, motor activity level, underweight, nutritional support
目标。研究一种基于营养状况指标和伴随病理性质的脑瘫患儿营养支持/肠内营养产品选择算法。患者和方法。本研究包括261名2至17岁的儿童(159名(61%)男孩)诊断为痉挛性脑瘫。根据大运动功能测量(GMFM)水平和喂养方式(每os /胃造口)将所有儿童分为不同的组。调查营养状况和胃肠道症状特征,并评估实际营养状况。通过测量肱三头肌皮褶厚度和中上臂围来评估身体组成。考虑到确定的营养状况和胃肠功能紊乱,对饮食进行调整。随访6-9个月,评估饮食治疗的效果。结果。在40-79%的病例中检测到体重不足,最常见于GMFCS水平的IV、V、VTF组:分别有14例(21.9%)、33例(40.7%)和18例(39.1%)儿童(pVTF-V-IV < 0.001)。IV组、V组和VTF组记录发育迟缓。最常见的胃肠道疾病是便秘(47.1%)。213名(81.6%)儿童登记为能量缺乏,比正常水平高出12.3-61.8%。宏量营养素缺乏率:蛋白质- 11.4-60.0%,脂肪- 10.9-61.6%,碳水化合物- 20.3-72.6%,低于个人标准/推荐膳食摄入量。在随访期间,在饮食治疗的背景下,观察到体重和身高指标有统计学意义的积极动态,脂肪量和骨骼肌量增加,胃肠道症状缓解。对研究结果的分析使我们能够开发出一种为脑瘫儿童选择营养支持/肠内营养产品的算法。结论。对脑瘫患者营养状况的评估和营养失调的纠正需要综合的方法。在1-3个月后没有积极的动力,有必要修改饮食方案。关键词:饮食治疗算法,脑瘫,运动水平,体重不足,营养支持
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引用次数: 0
Aerophagia in children with autism spectrum disorder 自闭症谱系障碍儿童的嗜气症
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.20953/1727-5784-2023-3-66-71
S. Polyakova, G.V. Khazykova, M. Dubrovskaya, O. V. Ryazanova, A. Botkina
Aerophagia is a functional disorder associated with air swallowing, which is manifested by belching, abdominal distension and bloating and can carry a pathological character with possible surgical complications. The prevalence of aerophagia among children ranges from 1% to 5%. Children with cognitive deficits and neurological and psychiatric diseases are 4 times more likely to suffer from aerophagia than neurotypical children. In these patients, aerophagia should be considered not as an independent functional disorder, but as a manifestation of neurological and psychiatric pathology (in particular, stereotypic behavior and sensory deficits). Uncontrolled aerophagia in children with autism spectrum disorders requires minimum examination and therapy correction for the underlying disease. This article presents a clinical case of aerophagia in two children with autism spectrum disorder. Key words: aerophagia, bloating, children, cognitive deficits, swallowing disorders, belching, autism spectrum disorder
食气症是一种与空气吞咽相关的功能障碍,表现为打嗝、腹胀和腹胀,具有病理特征,可能伴有手术并发症。儿童嗜气症的患病率在1%到5%之间。患有认知缺陷、神经和精神疾病的儿童患嗜气症的可能性是正常儿童的4倍。在这些患者中,食气症不应被视为一种独立的功能障碍,而应被视为神经和精神病理的一种表现(特别是刻板行为和感觉缺陷)。患有自闭症谱系障碍的儿童不受控制的嗜气症需要最少的检查和对潜在疾病的治疗纠正。本文报告两例自闭症谱系障碍患儿的嗜气症。关键词:食气症,腹胀,儿童,认知缺陷,吞咽障碍,打嗝,自闭症谱系障碍
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引用次数: 0
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Voprosy Detskoi Dietologii
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