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Possible effects of N-acetylcysteine in autism spectrum disorders: major clinical aspects, eating behaviors, and sleeping habits. N-乙酰半胱氨酸对自闭症谱系障碍的可能影响:主要临床方面、饮食行为和睡眠习惯。
Pub Date : 2023-01-01 DOI: 10.24953/turkjped.2022.573
Kevser Nalbant, Semih Erden

Background: N-acetylcysteine (NAC) is a promising agent for reducing irritability and hyperactivity and enhancing social responsiveness in children with autism spectrum disorders (ASD). This study aims to examine the effects of NAC on cardinal symptoms, eating, and sleeping habits in preschool children with autism.

Methods: The medical records of ASD patients were investigated retrospectively. 37 children with ASD who regularly received oral NAC in two divided doses per day (400-600 mg/day) for 8 weeks were included as the study group. The control group consisted of 21 children with ASD who were recommended NAC but never used it. The initial and second assessment scores after 8 weeks of regular use of the NAC group and control group on the Childhood Autism Rating Scale (CARS), Aberrant Behavior Checklist (ABC), Children Eating Behavior Questionnaire (CEBQ), and the Sleep Habits Questionnaire (CSHQ) were compared.

Results: Our findings suggested that oral NAC alleviated the intensity of cardinal autistic symptoms in areas of social withdrawal, interpersonal relationships, body use, listening response, and verbal communication. Corresponding problem behaviors such as irritability, stereotypic behavior, and hyperactivity were reduced. It was determined that there was no difference between the two groups in terms of eating behaviors and sleeping habits.

Conclusions: According to the results, NAC alleviated the severity of cardinal symptoms and reduced problem behaviors in autism. Additional trials with more systematic planning, controlling for confounding effects, and long-term follow-up should be provided in future studies.

背景:N-乙酰半胱氨酸(NAC)是一种很有前途的药物,可减少自闭症谱系障碍(ASD)儿童的易怒和多动,增强社会反应能力。本研究旨在探讨NAC对学龄前自闭症儿童主要症状、饮食和睡眠习惯的影响。方法:对ASD患者的病历资料进行回顾性分析。37名ASD儿童被纳入研究组,他们定期口服NAC,每天两次(400-600mg/天),持续8周。对照组包括21名被推荐但从未使用NAC的ASD儿童,比较NAC组和对照组在儿童自闭症评定量表(CARS)、异常行为量表(ABC)、儿童饮食行为问卷(CEBQ)和睡眠习惯问卷(CSHQ)上常规使用NAC 8周后的初次和第二次评估得分。结果:我们的研究结果表明,口服NAC在社交退缩、人际关系、身体使用、听力反应和言语交流等方面减轻了主要自闭症症状的强度。相应的问题行为,如易怒、刻板行为和多动都减少了。确定两组在饮食行为和睡眠习惯方面没有差异。结论:根据研究结果,NAC减轻了自闭症主要症状的严重程度,减少了问题行为。在未来的研究中,应提供具有更系统计划、控制混杂效应和长期随访的额外试验。
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引用次数: 0
Attitudes of parents with children aged 12-18 to COVID-19 vaccines for themselves and their children: vaccine hesitancy in Türkiye. 有 12-18 岁子女的父母对自己及其子女接种 COVID-19 疫苗的态度:图尔基耶的疫苗犹豫不决。
Pub Date : 2023-01-01 DOI: 10.24953/turkjped.2023.563
Ayça Kömürlüoğlu, Sıddika Songül Yalçın
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引用次数: 0
Evaluation of the relationship between neonatal serum asprosin levels and anthropometric measurements in newborns of mothers with and without gestational diabetes mellitus. 妊娠期糖尿病和非妊娠期糖尿病母亲新生儿血清asp松香水平与人体测量之间关系的评估。
Pub Date : 2023-01-01 DOI: 10.24953/turkjped.2023.275
Emine Esin Yalınbaş, Raziye Akcılar

Background: Asprosin is a newly identified adipokine that is expressed in the placenta. Its production is increased in women with gestational diabetes mellitus (GDM), and it is a factor related to insulin resistance. This study aimed to determine whether neonatal serum asprosin levels are associated with anthropometric characteristics of newborns born to mothers with and without GDM.

Methods: This study included 51 newborns of mothers with GDM (insulin-treated or diet-treated) and 55 control newborns with their mothers. In newborns, anthropometric parameters were measured, and the concentrations of asprosin were detected by ELISA. Maternal blood glucose levels, body weight, and length were measured and body mass index (BMI) was calculated.

Results: Serum asprosin levels were significantly higher and linked to a higher risk in the newborns of mothers with GDM compared with those of the control newborns (170.3 [132.6] vs. 91.4 [68.7] ng/mL, p < 0.001). Serum asprosin levels were negatively correlated with blood glucose concentrations (r = -0.282, p = 0.045) in the newborns of mothers with GDM and significantly positively correlated with birth weight (r = 0.315, p = 0.019) in the control newborns. Newborn serum asprosin levels were positively correlated with the glucose levels (r = 0.264, p = 0.006) of all mothers. In addition, newborns born to an insulin-treated mother with GDM had significantly higher birth weight and length than newborns born to a diet-treated mother with GDM (3262.9 vs. 3137 g, p = 0.032, and 49.7 vs. 49.2 cm, p = 0.05). Although asprosin levels were higher in newborns of mothers treated with insulin, these differences were not statistically significant. Mothers with GDM had high blood glucose levels (p = 0.032).

Conclusions: Serum levels of asprosin are increased and negatively correlated with glucose concentrations in newborns of mothers with GDM. Asprosin could be used as an early biomarker in newborns of GDM mothers.

背景:天冬氨酸是一种新发现的在胎盘中表达的脂肪因子。妊娠期糖尿病(GDM)妇女的胰岛素生成增加,是与胰岛素抵抗有关的因素。本研究旨在确定新生儿血清asprosin水平是否与患有和不患有GDM的母亲所生新生儿的人体测量特征有关。方法:本研究包括51例GDM(胰岛素治疗或饮食治疗)母亲的新生儿和55例对照新生儿及其母亲。在新生儿中,测量人体测量参数,并通过ELISA检测asp松香的浓度。测量母亲的血糖水平、体重和身长,并计算体重指数(BMI)。结果:与对照新生儿相比,患有GDM的母亲的新生儿血清asprosin水平显著更高,并与更高的风险相关(170.3[132.6]vs.91.4[68.7]ng/mL,p<0.001)对照组新生儿出生体重(r=0.315,p=0.019)。新生儿血清asprosin水平与所有母亲的血糖水平呈正相关(r=0.264,p=0.006)。此外,接受胰岛素治疗的GDM母亲所生的新生儿的出生体重和身长明显高于接受饮食治疗的GDM.(3262.9 vs.3137 g,p=0.032,49.7 vs.49.2 cm,p=0.05)。尽管接受胰岛素治疗母亲的新生儿asprosin水平较高,但这些差异无统计学意义。GDM母亲的血糖水平较高(p=0.032)。结论:GDM母亲新生儿的血清asprosin水平升高,且与血糖浓度呈负相关。天冬氨酸可以作为GDM母亲新生儿的早期生物标志物。
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引用次数: 0
A scoping review of the management of acute mastoiditis in children: What is the best approach? 儿童急性乳突炎治疗范围综述:最佳方法是什么?
Pub Date : 2023-01-01 DOI: 10.24953/turkjped.2023.320
Lorenzo Di Sarno, Ignazio Cammisa, Antonietta Curatola, Valeria Pansini, Gemma Eftimiadi, Antonio Gatto, Antonio Chiaretti

Background: Acute mastoiditis (AM) is a severe infection of the mastoid air cells that occurs in cases of acute, sub-acute, or chronic middle ear infections. No definitive consensus regarding the management of AM has been identified. The current guidelines include a conservative approach (parenteral antibiotics alone, antibiotics plus minor surgical procedures such as myringotomy with a ventilation tube inserted or drainage of the subperiosteal abscess through retro-auricolar incision or needle aspiration) or surgical treatment (mastoidectomy). The main aim of this review was to evaluate and summarize the current knowledge about the management of pediatric AM by analyzing the current evidence in the literature.

Methods: We examined the following bibliographic electronic databases: Pubmed and the Cochrane Library, from the inception date until February 2023. The search was guided by the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISM). The key words used for the search across electronic databases were: `mastoiditis` and `management`; `mastoiditis` and `surgery`; `mastoiditis` and `conservative`; `mastoiditis` and `antibiotics`; `mastoiditis` and `myringotomy`; `mastoiditis` and `grommet`; `mastoiditis` and `drainage`; and `mastoiditis` and `mastoidectomy`.

Results: We selected 12 articles involving 1124 episodes of mastoiditis. Some of these studies considered medical therapy alone as a valid first step, whereas others considered a minor surgical intervention as an initial approach along with antibiotic therapy. Considering the studies that evaluated medical therapy as the initial sole treatment option, the success rate of antibiotics alone was 24.6%. Overall, the success rate of minor surgical procedures, excluding mastoidectomy, was 87.7%, whereas the mastoidectomy success rate was 97%.

Conclusions: Overall, there is no shared consensus on the diagnostic or therapeutic approach to mastoiditis. Conservative therapy has gained considerable ground in recent times, quite limiting the predominant role of mastoidectomy. Further studies will be necessary to definitely develop standardized protocols shared in the scientific community.

背景:急性乳突炎(AM)是乳突气室的一种严重感染,可发生在急性、亚急性或慢性中耳炎病例中。目前尚未就急性乳突炎的治疗达成明确共识。目前的指导方针包括保守治疗(单纯肠外抗生素、抗生素加小手术,如插入通气管的耳廓切开术或通过耳后切口或针吸引流骨膜下脓肿)或手术治疗(乳突切除术)。本综述的主要目的是通过分析文献中的现有证据,评估和总结有关小儿乳突炎治疗的现有知识:我们研究了以下文献电子数据库:方法:我们检索了以下文献电子数据库:Pubmed 和 Cochrane 图书馆,检索时间从开始日期起至 2023 年 2 月。检索以《系统综述和元分析首选报告项目》(Preferred Reporting Items for Systematic Reviews and Meta-Analysis,PRISM)为指导。在电子数据库中搜索的关键词为类风湿性关节炎 "和 "管理";"类风湿性关节炎 "和 "手术";"类风湿性关节炎 "和 "保守";"类风湿性关节炎 "和 "抗生素";"类风湿性关节炎 "和 "耳环切开术";"类风湿性关节炎 "和 "胃镜";"类风湿性关节炎 "和 "引流";以及 "类风湿性关节炎 "和 "类风湿性关节炎切除术":我们选择了12篇文章,涉及1124次乳突炎发作。其中一些研究认为单纯药物治疗是有效的第一步,而另一些研究则认为小手术干预是与抗生素治疗一起使用的初步方法。考虑到将药物治疗作为最初唯一治疗方案的研究,单纯抗生素治疗的成功率为 24.6%。总体而言,除乳突切除术外,小手术的成功率为87.7%,而乳突切除术的成功率为97%:总体而言,乳突炎的诊断或治疗方法尚未达成共识。近来,保守疗法取得了相当大的进展,从而限制了乳突切除术的主导作用。有必要开展进一步研究,以确定科学界共享的标准化方案。
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引用次数: 0
Choice and switch of biologic drugs in juvenile idiopathic arthritis. 幼年特发性关节炎生物药物的选择和转换。
Pub Date : 2023-01-01 DOI: 10.24953/turkjped.2023.22
Seher Şener, Özge Başaran, Ezgi Deniz Batu, Müşerref Kasap Cüceoğlu, Zeynep Balık, Emil Aliyev, Yağmur Bayındır, Yelda Bilginer, Seza Özen

Background: In this study, we aimed to evaluate choices and changes of biologic drugs in juvenile idiopathic arthritis (JIA) patients according to disease subtypes.

Methods: We retrospectively analyzed JIA patients who received biologic treatment between January 2004 and July 2022.

Results: Of 294 JIA patients, 80 (27.2%) had systemic JIA, 68 (23.1%) had oligoarticular JIA, 61 (20.7%) had polyarticular JIA, 79 (26.9%) had enthesitis-associated arthritis (ERA), and six (2.1%) had psoriatic arthritis (PsA). Anakinra (n=66, 82.5%) was the most commonly preferred first line biologic in systemic JIA. Etanercept was the most frequently used biologic drug in patients with ERA (n=69, 87.3%), oligoarticular (n=37, 54.4%) and polyarticular JIA (n=43, 70.5%). Adalimumab was used as a first-line biologic drug in all PsA patients (n=6, 100%). One hundred-fourteen patients (38.8%) were switched to second-line and 29 (9.9%) to third-line biologic drugs. While the most common reason for switching to a second-line biologic was difficulty in usage of daily injections (n=37, 60.6%) in systemic JIA patients, it was an inadequate response to first biologics in non-systemic JIA patients (n=42, 79.2%). Side effects were detected in only seven patients (2.4%) during the follow-up.

Conclusion: In this study, we revealed the biologic drug usage and switch strategies in our JIA patients. Good responses were obtained in most of our patients with a reliable profile. However, studies on larger patient groups are needed to clarify these results.

背景本研究旨在根据疾病亚型评估幼年特发性关节炎(JIA)患者对生物制剂药物的选择和变化:我们对2004年1月至2022年7月期间接受生物制剂治疗的JIA患者进行了回顾性分析:在294例JIA患者中,80例(27.2%)为全身性JIA,68例(23.1%)为少关节型JIA,61例(20.7%)为多关节型JIA,79例(26.9%)为关节炎相关性关节炎(ERA),6例(2.1%)为银屑病关节炎(PsA)。Anakinra(66人,82.5%)是全身性JIA最常用的一线生物制剂。Etanercept是ERA(69人,87.3%)、少关节型(37人,54.4%)和多关节型JIA(43人,70.5%)患者最常使用的生物制剂药物。阿达木单抗是所有PsA患者(6人,100%)的一线生物药物。有14名患者(38.8%)转用二线生物制剂药物,29名患者(9.9%)转用三线生物制剂药物。全身性JIA患者转用二线生物制剂的最常见原因是难以使用每日注射(37人,60.6%),而非全身性JIA患者转用二线生物制剂的最常见原因是对一线生物制剂的反应不足(42人,79.2%)。在随访期间,仅有7名患者(2.4%)出现副作用:在这项研究中,我们揭示了JIA患者的生物制剂使用和转换策略。大多数患者的反应良好,情况可靠。然而,要澄清这些结果,还需要对更大的患者群体进行研究。
{"title":"Choice and switch of biologic drugs in juvenile idiopathic arthritis.","authors":"Seher Şener, Özge Başaran, Ezgi Deniz Batu, Müşerref Kasap Cüceoğlu, Zeynep Balık, Emil Aliyev, Yağmur Bayındır, Yelda Bilginer, Seza Özen","doi":"10.24953/turkjped.2023.22","DOIUrl":"10.24953/turkjped.2023.22","url":null,"abstract":"<p><strong>Background: </strong>In this study, we aimed to evaluate choices and changes of biologic drugs in juvenile idiopathic arthritis (JIA) patients according to disease subtypes.</p><p><strong>Methods: </strong>We retrospectively analyzed JIA patients who received biologic treatment between January 2004 and July 2022.</p><p><strong>Results: </strong>Of 294 JIA patients, 80 (27.2%) had systemic JIA, 68 (23.1%) had oligoarticular JIA, 61 (20.7%) had polyarticular JIA, 79 (26.9%) had enthesitis-associated arthritis (ERA), and six (2.1%) had psoriatic arthritis (PsA). Anakinra (n=66, 82.5%) was the most commonly preferred first line biologic in systemic JIA. Etanercept was the most frequently used biologic drug in patients with ERA (n=69, 87.3%), oligoarticular (n=37, 54.4%) and polyarticular JIA (n=43, 70.5%). Adalimumab was used as a first-line biologic drug in all PsA patients (n=6, 100%). One hundred-fourteen patients (38.8%) were switched to second-line and 29 (9.9%) to third-line biologic drugs. While the most common reason for switching to a second-line biologic was difficulty in usage of daily injections (n=37, 60.6%) in systemic JIA patients, it was an inadequate response to first biologics in non-systemic JIA patients (n=42, 79.2%). Side effects were detected in only seven patients (2.4%) during the follow-up.</p><p><strong>Conclusion: </strong>In this study, we revealed the biologic drug usage and switch strategies in our JIA patients. Good responses were obtained in most of our patients with a reliable profile. However, studies on larger patient groups are needed to clarify these results.</p>","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":"65 6","pages":"980-989"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139418922","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}
引用次数: 0
Systemic treatments in atopic dermatitis in children. 儿童特应性皮炎的系统治疗。
Pub Date : 2023-01-01 DOI: 10.24953/turkjped.2023.203
Deniz İlgün Gürel, Özge Soyer, Ümit Murat Şahiner

Atopic dermatitis (AD) is a very common skin disease caused by inflammatory reactions, in which the main symptoms of severe itching and recurrent eczema diminish quality of life. As epidermal barrier function and the immune system play a critical role in atopic dermatitis, promoting IgE-mediated sensitization can be the main targets of AD treatment. The goal of AD treatment should be to eliminate the symptoms and obtain longterm eczema control with a multi-step approach adapted to the severity of the disease. Basic management for all patients comprises the use of moisturisers and avoiding triggers. While topical therapy is effective for most children diagnosed with AD, there may also be children who require systemic therapy. The aim of this paper was to present an extensive review of the systemic agents commonly used in childhood atopic dermatitis which mainly target cutaneous inflammation.

特应性皮炎(AD)是一种由炎症反应引起的非常常见的皮肤病,其主要症状是剧烈瘙痒和反复发作的湿疹,会降低患者的生活质量。由于表皮屏障功能和免疫系统在特应性皮炎中起着至关重要的作用,因此促进 IgE 介导的致敏作用可作为特应性皮炎治疗的主要目标。特应性皮炎的治疗目标应该是消除症状,并根据疾病的严重程度采取多步骤方法长期控制湿疹。对所有患者的基本治疗包括使用润肤霜和避免诱发因素。虽然局部治疗对大多数确诊为 AD 的儿童都有效,但也有一些儿童可能需要系统治疗。本文旨在广泛综述儿童特应性皮炎常用的主要针对皮肤炎症的系统性药物。
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引用次数: 0
The role of red blood cell distribution width (RDW) in the diagnosis of pediatric sepsis. 红细胞分布宽度(RDW)在诊断小儿败血症中的作用。
Pub Date : 2023-01-01 DOI: 10.24953/turkjped.2023.182
Lingjun Xu, Lingfeng Yuan, Keqiang Chi, Dan Zhong, Liang Huang

Background: Early diagnosis of pediatric sepsis is difficult, so it is necessary to find a reliable auxiliary diagnostic method. The purpose of the study was to assess the role of RDW in the diagnosis of pediatric sepsis.

Methods: We did a case control study reviewing pediatric inpatients (≥28 days, < 18 years old) who were diagnosed with sepsis between April 2020 and November 2022. According to the sepsis-3 and Pediatric Sequential Organ Failure Assessment (pSOFA) scoring standards, 66 septic inpatients of the pediatric intensive care unit (PICU) were included in the sepsis group and 66 non-septic inpatients of the PICU were included by using the random sampling method during the same period as the control group.

Results: RDW values in the sepsis group were higher than those in the control group (P < 0.001). The cut-off value, sensitivity, specificity and area under curve of RDW for sepsis were 39.15, 0.955, 0.758 and 0.943,respectively.

Conclusions: Our study confirms that RDW may have a good value on the early diagnosis of pediatric sepsis.

背景:小儿败血症的早期诊断非常困难,因此有必要找到一种可靠的辅助诊断方法。本研究旨在评估 RDW 在诊断小儿败血症中的作用:我们对 2020 年 4 月至 2022 年 11 月期间被诊断为败血症的儿科住院患者(≥28 天,年龄小于 18 岁)进行了病例对照研究。根据脓毒症-3和儿科序贯器官功能衰竭评估(pSOFA)评分标准,将儿科重症监护室(PICU)的66名脓毒症住院患者纳入脓毒症组,并采用随机抽样方法将同期儿科重症监护室的66名非脓毒症住院患者纳入对照组:败血症组的 RDW 值高于对照组(P<0.001)。脓毒症组 RDW 的临界值、敏感性、特异性和曲线下面积分别为 39.15、0.955、0.758 和 0.943:我们的研究证实,RDW 对小儿败血症的早期诊断有很好的价值。
{"title":"The role of red blood cell distribution width (RDW) in the diagnosis of pediatric sepsis.","authors":"Lingjun Xu, Lingfeng Yuan, Keqiang Chi, Dan Zhong, Liang Huang","doi":"10.24953/turkjped.2023.182","DOIUrl":"10.24953/turkjped.2023.182","url":null,"abstract":"<p><strong>Background: </strong>Early diagnosis of pediatric sepsis is difficult, so it is necessary to find a reliable auxiliary diagnostic method. The purpose of the study was to assess the role of RDW in the diagnosis of pediatric sepsis.</p><p><strong>Methods: </strong>We did a case control study reviewing pediatric inpatients (≥28 days, < 18 years old) who were diagnosed with sepsis between April 2020 and November 2022. According to the sepsis-3 and Pediatric Sequential Organ Failure Assessment (pSOFA) scoring standards, 66 septic inpatients of the pediatric intensive care unit (PICU) were included in the sepsis group and 66 non-septic inpatients of the PICU were included by using the random sampling method during the same period as the control group.</p><p><strong>Results: </strong>RDW values in the sepsis group were higher than those in the control group (P < 0.001). The cut-off value, sensitivity, specificity and area under curve of RDW for sepsis were 39.15, 0.955, 0.758 and 0.943,respectively.</p><p><strong>Conclusions: </strong>Our study confirms that RDW may have a good value on the early diagnosis of pediatric sepsis.</p>","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":"65 6","pages":"964-972"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139418932","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}
引用次数: 0
The etiologies and management of spinal cord compression in childhood cancers: Are we aware of the emergency of cord compression? 儿童癌症脊髓压迫的病因和处理:我们知道脊髓压迫的紧急情况吗?
Pub Date : 2023-01-01 DOI: 10.24953/turkjped.2023.240
Arzu Okur, Özge Vural, Faruk Güçlü Pınarlı

Background: The spinal cord compression causes irreversible long-term permanent neurological sequelae. This study aims to increase awareness of childhood cancers that cause cord compression by comparing histopathological diagnosis, treatments, and survival rates to the literature.

Methods: Seventy-three patients (38 male, 35 female) with spinal cord compression, among 1085 patients diagnosed with solid tumors at Gazi University Department of Pediatric Oncology between 1991 and 2021 were retrospectively evaluated.

Results: The mean time between the onset of complaints and diagnosis was 27.5± 24.9 (2-150) days. The first three most common tumors that caused cord compression; were central nervous system tumors in 22 (30%), neuroblastoma in 17 (23%), and malignant germ cell tumors in 8 (10%) cases. Of the patients, 46 (63%) had compression due to extradural masses, and 27 (37%) patients had an intradural compression. The most common symptoms were pain in 60 (82%), weakness in 57 (78%), and pins and needles in 28 (38%) patients, respectively. The clinical physical neurological examination findings were motor deficit in 62 (84%), and deep tendon reflex changes in 54 patients (73.9%). Compression findings were detected in 58 (79.5%) patients at diagnosis, and in 15 (20.5%) of them during follow-up. The most common level of compression was seen in the thoracolumbar region in 19 (26%) cases. In 65 (89%) patients with cord compression, corticosteroids were given as anti-edema treatment. Surgical excision was performed in 39 (53%) patients. Spinal radiotherapy was given to 35 patients (48%) with radiosensitive tumors. Chemotherapy protocols were started in 52 (71.2%) cases according to their diagnoses. Complete neurological recovery was achieved in 33 (45%) patients. The 5-year overall survival rates for solid tumors with extradural compression and intradural compression were 62% and 22%, respectively (p=0.002).

Conclusions: Neurological sequela-free recovery is possible with early diagnosis and urgent treatment. Spinal compression must be detected by detailed systemic and neurological examination and imaging methods. Patients should be rapidly transferred to pediatric oncology units after starting anti-edema treatment.

背景:脊髓压迫会导致不可逆转的长期永久性神经后遗症。本研究旨在通过将组织病理学诊断、治疗和存活率与文献进行比较,提高人们对导致脊髓压迫的儿童癌症的认识。方法:回顾性评估1991年至2021年间在加孜大学儿科肿瘤系诊断为实体瘤的1085名患者中的73名脊髓压迫患者(38名男性,35名女性)。结果:从发病到确诊的平均时间为27.5±24.9(2-150)天。导致脊髓压迫的前三种最常见的肿瘤;中枢神经系统肿瘤22例(30%),神经母细胞瘤17例(23%),恶性生殖细胞肿瘤8例(10%)。在这些患者中,46名(63%)患者因硬膜外肿块而受到压迫,27名(37%)患者受到硬膜内压迫。最常见的症状分别是疼痛60例(82%)、虚弱57例(78%)和针扎28例(38%)。临床物理神经检查结果为62例(84%)运动功能障碍,54例(73.9%)深肌腱反射改变。58例(79.5%)患者在诊断时发现压迫,15例(20.5%)患者在随访中发现压迫。19例(26%)胸腰椎受压最常见。在65例(89%)脊髓压迫患者中,给予皮质类固醇作为抗水肿治疗。手术切除39例(53%)患者。35例(48%)放射敏感肿瘤患者接受了脊髓放射治疗。52例(71.2%)患者根据诊断开始了化疗方案。33名(45%)患者的神经系统完全恢复。硬膜外压迫和硬膜内压迫的实体瘤的5年总生存率分别为62%和22%(p=0.002)。脊椎压迫必须通过详细的系统和神经检查以及成像方法进行检测。在开始抗水肿治疗后,患者应迅速转移到儿科肿瘤科。
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引用次数: 0
Evaluation of toll-like receptors 2 and 4 polymorphism and intestinal microbiota in children with food allergies. toll样受体2和4多态性与食物过敏儿童肠道微生物群的评估。
Pub Date : 2023-01-01 DOI: 10.24953/turkjped.2023.389
Mehmet Kılıç, Elif Beyazıt, Ebru Etem Önalan, Tuğçe Kaymaz, Erdal Taşkın

Background: Mutual regulation between immune system and gut microbiota is achieved through several mechanisms including the engagement of toll-like receptors (TLRs) which is expressed on numerous cell types. In this study we aimed to explore the association between food allergies and TLR gene polymorphisms in association with gut microbiota.

Methods: Toll-like receptors polymorphism frequencies and some bacteria in the gut microbiota in 130 infants aged 1-24 months with egg and/or milk allergy in a prospective cohort were compared with 110 non-food allergic controls. Four candidate polymorphisms (TLR2 rs1898830/rs5743708 and TLR4 rs4986790/rs4986791) were genotyped by allelic discrimination polymerase chain rection (PCR) method. Gut microbiota analysis was achieved by using high-throughput sequencing.

Results: The TLR4 rs4986790 (Asp299Gly) single nucleotide polymorphism (SNP) major/minor allele frequency was 0.788/0.212 in food allergy patients and 0.719/0.280 in controls (p=0.017). There was a statistically significant difference between groups in terms of genotype frequencies (AA, AG, GG). Gut microbiota analysis revealed increased Firmicutes phylum in stool of the patients with food allergy. Except for TLR4 rs4986791 (Thr399lle) allele, the other TLR polymorphisms were not associated with food allergies in children. When the bacteria in the intestinal microbiota and TLR2 and TLR4 gene polymorphisms were compared; we determined a statistically significant increase in Bifidobacterium concentration in the intestinal microbiota in TLR4 rs4986791 CT heterozygous genotype (p=0.004).

Conclusions: This study demonstrated a partial role of TLR4 gene polymorphism and gut microbiota in the development of food allergies. Future work in this area will be required to clarify the roles of different microbial strains that modulate gut microbiota composition and function in conjunction with TLR transcription pathways.

背景:免疫系统和肠道微生物群之间的相互调节是通过几种机制实现的,包括在多种细胞类型上表达的toll样受体(TLRs)的参与。在这项研究中,我们旨在探索食物过敏和TLR基因多态性与肠道微生物群之间的关系。方法:在一个前瞻性队列中,将130名1-24个月大的鸡蛋和/或牛奶过敏婴儿的Toll样受体多态性频率和肠道微生物群中的一些细菌与110名非食物过敏对照进行比较。采用等位基因鉴别聚合酶链反应(PCR)方法对四种候选多态性(TLR2 rs189883/rs5743708和TLR4 rs4986790/rs4986791)进行基因分型。肠道微生物群分析是通过高通量测序实现的。结果:食物过敏患者TLR4 rs4986790(Asp299Gly)单核苷酸多态性(SNP)大小等位基因频率为0.788/0.212,对照组为0.719/0.280(p=0.017),各组基因型频率(AA、AG、GG)差异有统计学意义。肠道微生物群分析显示,食物过敏患者粪便中厚壁菌门增加。除了TLR4 rs4986791(Thr399lle)等位基因外,其他TLR多态性与儿童食物过敏无关。当肠道微生物群中的细菌与TLR2和TLR4基因多态性进行比较时;我们测定了TLR4 rs4986791 CT杂合基因型肠道微生物群中双歧杆菌浓度的统计学显著增加(p=0.004)。结论:本研究证明了TLR4基因多态性和肠道微生物群在食物过敏的发展中的部分作用。未来需要在这一领域开展工作,以阐明不同微生物菌株在调节肠道微生物群组成和功能以及TLR转录途径方面的作用。
{"title":"Evaluation of toll-like receptors 2 and 4 polymorphism and intestinal microbiota in children with food allergies.","authors":"Mehmet Kılıç,&nbsp;Elif Beyazıt,&nbsp;Ebru Etem Önalan,&nbsp;Tuğçe Kaymaz,&nbsp;Erdal Taşkın","doi":"10.24953/turkjped.2023.389","DOIUrl":"10.24953/turkjped.2023.389","url":null,"abstract":"<p><strong>Background: </strong>Mutual regulation between immune system and gut microbiota is achieved through several mechanisms including the engagement of toll-like receptors (TLRs) which is expressed on numerous cell types. In this study we aimed to explore the association between food allergies and TLR gene polymorphisms in association with gut microbiota.</p><p><strong>Methods: </strong>Toll-like receptors polymorphism frequencies and some bacteria in the gut microbiota in 130 infants aged 1-24 months with egg and/or milk allergy in a prospective cohort were compared with 110 non-food allergic controls. Four candidate polymorphisms (TLR2 rs1898830/rs5743708 and TLR4 rs4986790/rs4986791) were genotyped by allelic discrimination polymerase chain rection (PCR) method. Gut microbiota analysis was achieved by using high-throughput sequencing.</p><p><strong>Results: </strong>The TLR4 rs4986790 (Asp299Gly) single nucleotide polymorphism (SNP) major/minor allele frequency was 0.788/0.212 in food allergy patients and 0.719/0.280 in controls (p=0.017). There was a statistically significant difference between groups in terms of genotype frequencies (AA, AG, GG). Gut microbiota analysis revealed increased Firmicutes phylum in stool of the patients with food allergy. Except for TLR4 rs4986791 (Thr399lle) allele, the other TLR polymorphisms were not associated with food allergies in children. When the bacteria in the intestinal microbiota and TLR2 and TLR4 gene polymorphisms were compared; we determined a statistically significant increase in Bifidobacterium concentration in the intestinal microbiota in TLR4 rs4986791 CT heterozygous genotype (p=0.004).</p><p><strong>Conclusions: </strong>This study demonstrated a partial role of TLR4 gene polymorphism and gut microbiota in the development of food allergies. Future work in this area will be required to clarify the roles of different microbial strains that modulate gut microbiota composition and function in conjunction with TLR transcription pathways.</p>","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":"65 5","pages":"758-768"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49687318","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}
引用次数: 0
Late-presenting congenital diaphragmatic hernia in a child with gastric perforation and acute pancreatitis. 儿童胃穿孔并发急性胰腺炎的晚期先天性膈疝。
Pub Date : 2023-01-01 DOI: 10.24953/turkjped.2022.512
Qing Li, Caixia Liu

Background: Late-presenting congenital diaphragmatic hernia occurs beyond the neonatal period, and is relatively rare, presenting with nonspecific respiratory and gastrointestinal symptoms.

Case: We report a rare case of late-presenting congenital diaphragmatic hernia in a 7-year-old girl, who presented with abdominal pain, shortness of breath and fever on admission. Work-up revealed intrathoracic gastric perforation, acute pancreatitis and septic shock with a diaphragmatic defect. Due to the high content of amylase in pleural effusion, we suspected the presence of a pancreaticopleural fistula, and we were also puzzled whether the gastric perforation was caused by a pleural indwelling catheterization, but this was ruled out. We about performed a laparotomy to reposition the herniated organs, repair the hernia and the gastric perforation, and undergo the gastrostomy. The girl had an uneventful post-operative recovery.

Conclusions: Late-presenting congenital diaphragmatic hernias are often misdiagnosed. Clinicians should combine multiple imaging modalities to make a definite diagnosis and perform surgery as soon as possible to avoid severe complications.

背景:晚期先天性膈疝发生在新生儿期之后,相对罕见,表现为非特异性呼吸道和胃肠道症状。病例:我们报告了一例罕见的晚期先天性膈疝病例,患者为一名7岁女孩,入院时表现为腹痛、呼吸急促和发烧。检查显示胸腔内胃穿孔、急性胰腺炎和感染性休克伴膈肌缺损。由于胸腔积液中淀粉酶含量高,我们怀疑存在胰胸膜瘘,我们也对胃穿孔是否是由胸膜留置导管插入术引起感到困惑,但这被排除了。我们大约进行了剖腹手术,以重新定位疝出的器官,修复疝和胃穿孔,并进行胃造口术。这个女孩术后恢复得很顺利。结论:迟发性先天性膈疝常被误诊。临床医生应结合多种成像方式进行明确诊断,并尽快进行手术,避免出现严重并发症。
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The Turkish journal of pediatrics
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