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A retrospective analysis of clinical characteristics and outcomes of pediatric fulminant myocarditis. 对小儿暴发性心肌炎临床特征和预后的回顾性分析
IF 2 3区 医学 Q2 PEDIATRICS Pub Date : 2024-08-29 DOI: 10.1186/s12887-024-05022-4
Yuhang Zhao, Min Da, Xun Yang, Yang Xu, Jirong Qi

Background: The study aimed to explore clinical indicators that can predict the prognosis of children with acute fulminant myocarditis (AFM) through a retrospective analysis.

Methods: A retrospective analysis was conducted on the clinical indices of 79 children diagnosed with AFM and hospitalized from March 2013 to March 2023. Relevant demographic and clinical data, including symptoms at admission, laboratory results, and outcomes were extracted to identify factors associated with in-hospital mortality.

Results: A total of 79 children with AFM were analyzed. The survival group (n = 61) had a longer median hospital stay and higher medical expenses compared to the death group (n = 18). Significant differences in the levels of left ventricular ejection fraction (LVEF)(P < 0.001), myoglobin (MYO)(P < 0.001), aspartate aminotransferase (AST)(P < 0.001), lactate dehydrogenase (LDH)(P = 0.004), B-type natriuretic peptide (BNP)(P = 0.005), arterial potential hydrogen (PH)(P < 0.001), bicarbonate (HCO3-)(P = 0.003), serum lactate (Lac)(P = 0.001), peripheral oxygen saturation (SpO2)(P = 0.008), and white blood cell count (WBC)(P = 0.007) were observed between the two groups. Additionally, there were significant differences in the incidences of multi-organ failure (P = 0.003) and respiratory failure (P = 0.001) between the two groups.

Conclusions: Severe myocardial injury (AST > 194.00 U/L, LDH > 637.50 U/L, MYO > 265.75 µg/L, BNP > 1738.50 ng/L), acidosis (PH < 7.29, HCO3- <18.45 mmol/L, Lac > 12.30 mmol/L), hypoxia (SpO2 < 97.50%), inflammatory response (WBC > 9.69*109/L), left ventricular systolic dysfunction (LVEF < 28.25%), multi-organ failure, and respiratory failure are significantly associated with higher mortality rates. These factors can accurately identify AFM children at an increased risk of death.

背景:本研究旨在通过回顾性分析,探讨可预测急性暴发性心肌炎(AFM)患儿预后的临床指标:该研究旨在通过回顾性分析,探讨可预测急性暴发性心肌炎(AFM)患儿预后的临床指标:方法:对2013年3月至2023年3月期间住院的79名确诊为急性暴发性心肌炎患儿的临床指标进行回顾性分析。提取了相关的人口统计学和临床数据,包括入院时的症状、实验室结果和预后,以确定与院内死亡率相关的因素:结果:共分析了79名AFM患儿。与死亡组(18 人)相比,存活组(61 人)的中位住院时间更长,医疗费用更高。两组患儿的左心室射血分数(LVEF)(P 3-)(P = 0.003)、血清乳酸(Lac)(P = 0.001)、外周血氧饱和度(SpO2)(P = 0.008)和白细胞计数(WBC)(P = 0.007)水平存在显著差异。此外,多器官功能衰竭(P = 0.003)和呼吸衰竭(P = 0.001)的发生率在两组之间存在显著差异:严重心肌损伤(AST > 194.00 U/L、LDH > 637.50 U/L、MYO > 265.75 µg/L、BNP > 1738.50 ng/L)、酸中毒(PH 3- 12.30 mmol/L)、缺氧(SpO2 9.69*109/L)、左室收缩功能障碍(LVEF
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引用次数: 0
Body weight and eating attitudes influence improvement of depressive symptoms in children and pre-adolescents with eating disorders: a prospective multicenter cohort study. 体重和饮食态度影响进食障碍儿童和青少年前期抑郁症状的改善:一项前瞻性多中心队列研究。
IF 2 3区 医学 Q2 PEDIATRICS Pub Date : 2024-08-27 DOI: 10.1186/s12887-024-05024-2
Yuichi Suzuki, Shinichiro Nagamitsu, Nobuoki Eshima, Takeshi Inoue, Ryoko Otani, Ryoichi Sakuta, Toshiyuki Iguchi, Ryuta Ishii, Soh Uchida, Ayumi Okada, Shinji Kitayama, Kenshi Koyanagi, Yuki Suzuki, Yoshino Sumi, Shizuo Takamiya, Chikako Fujii, Yoshimitsu Fukai

Background: Pediatric patients with eating disorders in a multicenter joint study on 11 facilities were enrolled and prospectively investigated to determine whether improvement in body weight, eating attitudes, and psychosocial factors in children with eating disorders would also improve depressive symptoms.

Methods: In this study, 91 patients were enrolled between April 2014 and March 2016. The severity of underweight was assessed using the body mass index-standard deviation score (BMI-SDS), eating behavior was assessed using the children's eating attitude test (ChEAT26), the outcome of childhood eating disorders was assessed using the childhood eating disorder outcome scale, and depressive symptoms were assessed using the Children's Depression Inventory (CDI) score.

Results: After 12 months of treatment, depressive symptoms were evaluated in 62 of the 91 cases where it was evaluated at the initial phase. There was no difference in background characteristics between the included patients and the 29 patients who dropped out. A paired-sample t-test revealed a significant decrease in CDI scores after 12 months of treatment (p < 0.001, 95% CI: 2.401-7.373) and a significant increase in the BMI-SDS (p < 0.001, 95% CI: - 2.41973-1.45321). Multiple regression analysis revealed that BMI-SDS and ChEAT26 scores at the initial phase were beneficial in CDI recovery. In addition, BMI-SDS at the initial phase was useful for predicting BMI-SDS recovery after 12 months of treatment.

Conclusions: Depressive symptoms in children with eating disorders improved with therapeutic intervention on body weight and eating attitudes.

Trial registration: The Clinical Trial Number for this study is UMIN000055004.

背景:一项多中心联合研究招募了11家机构的儿童进食障碍患者,并对其进行了前瞻性调查,以确定改善进食障碍儿童的体重、进食态度和心理社会因素是否也能改善抑郁症状:本研究在 2014 年 4 月至 2016 年 3 月期间共招募了 91 名患者。体重不足的严重程度采用体重指数标准偏差评分(BMI-SDS)进行评估,饮食行为采用儿童饮食态度测试(ChEAT26)进行评估,儿童饮食失调结局采用儿童饮食失调结局量表进行评估,抑郁症状采用儿童抑郁量表(CDI)评分进行评估:经过 12 个月的治疗后,在初始阶段对抑郁症状进行评估的 91 个病例中,有 62 个病例的抑郁症状得到了评估。纳入治疗的患者与退出治疗的 29 名患者在背景特征上没有差异。配对样本 t 检验显示,经过 12 个月的治疗后,CDI 得分显著下降(p 结论:CDI 得分显著下降的主要原因是饮食失调引起的:通过对体重和饮食态度的治疗干预,进食障碍儿童的抑郁症状有所改善:本研究的临床试验编号为 UMIN000055004。
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引用次数: 0
Accurate prenatal diagnosis of coarctation of the aorta by 3-step echocardiographic diagnostic protocol. 通过三步超声心动图诊断方案准确产前诊断主动脉共动脉瘤。
IF 2 3区 医学 Q2 PEDIATRICS Pub Date : 2024-08-27 DOI: 10.1186/s12887-024-04851-7
Hong Meng, Zhi-Ling Luo, Yan Shen, Qian-Qian Liu, Mu-Zi Li, Yi-Ming Gao
<p><strong>Background: </strong>Coarctation of the aorta (CoA) is the most common undiagnosed congenital heart defect during prenatal screening. High false positive and false negative rates seriously affect prenatal consultation and postnatal management. The objective of the study was to assess the utility of various measurements to predict prenatal CoA and to derive a diagnostic algorithm.</p><p><strong>Methods: </strong>One hundred and fifty-four fetuses with suspected CoA who presented at Fuwai Hospital between December 2017 and August 2021 were enrolled and divided into confirmed CoA cases (n = 47) and false positive cases (n = 107), according to their postnatal outcomes. The transverse aortic arch, isthmus, and descending aorta were measured in the long-axis view of the aortic arch. The angle between the transverse aortic arch (TAO) and the descending aortic arch (DAO) was defined as the TAO-DAO angle and measured in the long axis or sagittal view. Based on the database in GE Voluson E10 and the formula (Z = [Formula: see text]), the standard score (Z-score) of the dimensions of the aorta were calculated in relation to the gestational age. The main echocardiographic indices were combined to design a 3-step diagnostic protocol. The TAO-DAO angle was used as the first step in the diagnostic model. The diameter of the transverse arch and the Z-score of the isthmus were the second step. The third-step indices included a Z-score of the transverse arch, diameter of the isthmus, distance from the left subclavian artery (LSA) to left common carotid artery (LCCA), the ratio of isthmus diameter and LSA diameter and ratio of the distances (the distance between the LSA and LCCA to the distance between the right innominate artery and LCCA). The receiver operating characteristic (ROC) curve determined the predictive capability of each diagnostic parameter, and the kappa test determined the diagnostic accuracy of the proposed model.</p><p><strong>Results: </strong>The cases with confirmed CoA had thinner transverse arches (1.92 ± 0.32 mm vs. 3.06 ± 0.67 mm, P = 0.0001), lower Z-scores of the isthmus (-8.97 ± 1.45 vs. -5.65 ± 1.60, P = 0.0001), smaller TAO-DAO angles (105.54 ± 11.51° vs. 125.29 ± 8.97°, P = 0.0001) and larger distance between the LSA and LCCA (4.45 ± 1.75 mm vs. 2.74 ± 1.07 mm, P = 0.0001) than the false positive cases. The area under the curve (AUC) was 0.947 (95% CI 0.91-0.98) for the TAO-DAO angle ≤ 115.75°, 0.942 (95% CI 0.91-0.98) for the transverse arch diameter ≤ 2.31 mm, 0.937 (95% CI 0.90-0.98) for the Z-score of the isthmus ≤ -7.5, and 0.975 (95% CI 0.95-1.00) for the 3-step diagnostic protocol with 97.8% sensitivity and 97.2% specificity. The kappa test showed that the model's diagnostic accuracy was consistent with postnatal outcomes (kappa value 0.936, P = 0.0001).</p><p><strong>Conclusions: </strong>The 3-step diagnostic protocol included the three most useful measurements and the additional indices with appropriate cut-off va
背景:主动脉共动脉症(CoA)是产前筛查中最常见的未确诊先天性心脏缺陷。高假阳性率和假阴性率严重影响产前咨询和产后管理。本研究的目的是评估各种测量方法对预测产前CoA的实用性,并得出诊断算法:纳入2017年12月至2021年8月期间在阜外医院就诊的154例疑似CoA胎儿,根据其产后结果分为确诊CoA病例(n = 47)和假阳性病例(n = 107)。在主动脉弓长轴切面上测量主动脉横弓、峡部和降主动脉。横主动脉弓(TAO)和降主动脉弓(DAO)之间的角度被定义为 TAO-DAO 角,并在长轴或矢状切面上进行测量。根据 GE Voluson E10 数据库和公式(Z = [公式:见正文]),计算出主动脉尺寸的标准分(Z-score)与胎龄的关系。将主要的超声心动图指标结合起来,设计了一个三步诊断方案。TAO-DAO 角被用作诊断模型的第一步。横弓直径和峡部 Z 评分是第二步。第三步指数包括横弓的 Z 值、峡部直径、左锁骨下动脉(LSA)到左颈总动脉(LCCA)的距离、峡部直径和 LSA 直径的比值以及距离的比值(LSA 和 LCCA 之间的距离与右腹股沟动脉和 LCCA 之间的距离)。接受者操作特征曲线(ROC)确定了各诊断参数的预测能力,卡帕检验确定了所提模型的诊断准确性:结果:确诊为 CoA 的病例的横弓更薄(1.92 ± 0.32 mm vs. 3.06 ± 0.67 mm,P = 0.0001),峡部的 Z 值更低(-8.97 ± 1.45 vs. -5.65 ± 1.60,P = 0.0001)、较小的 TAO-DAO 角(105.54 ± 11.51° vs. 125.29 ± 8.97°,P = 0.0001)和较大的 LSA 与 LCCA 之间的距离(4.45 ± 1.75 mm vs. 2.74 ± 1.07 mm,P = 0.0001)。TAO-DAO角度≤115.75°的曲线下面积(AUC)为0.947(95% CI 0.91-0.98),横弓直径≤2.31 mm的曲线下面积(AUC)为0.942(95% CI 0.91-0.98),峡部Z分数≤-7.5的曲线下面积(AUC)为0.937(95% CI 0.90-0.98),三步诊断方案的曲线下面积(AUC)为0.975(95% CI 0.95-1.00),灵敏度为97.8%,特异度为97.2%。卡帕检验显示,该模型的诊断准确性与产后结果一致(卡帕值为 0.936,P = 0.0001):三步诊断方案包括三个最有用的测量指标和具有适当临界值的附加指标。该算法对胎儿主动脉瓣狭窄的检测非常有用,准确率很高:试验登记:回顾性登记。
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引用次数: 0
Comparing body composition measures in children with end stage liver disease using noninvasive bioimpedance analysis. 利用无创生物阻抗分析比较终末期肝病患儿的身体成分。
IF 2 3区 医学 Q2 PEDIATRICS Pub Date : 2024-08-27 DOI: 10.1186/s12887-024-04974-x
Maryam Ekramzadeh, Seyed Ali Moosavi, Amirali Mashhadiagha, Ali Ghorbanpour, Nasrin Motazedian, Seyed Mohsen Dehghani, Homa Ilkhanipoor, Alireza Mirahmadizadeh

Background: Chronic liver disease (CLD) in children, often leads to cirrhosis and end-stage liver disease (ESLD). CLD poses significant challenges in management and prognosis. Assessing body composition, including sarcopenia, is increasingly recognized as important in understanding outcomes in this population.

Methods: We conducted a prospective observational study, involving children aged 2 to 18 years with ESLD awaiting liver transplantation. Socio-demographic, clinical, and laboratory data were collected, and body composition was assessed using Bioelectrical Impedance Analysis (BIA). Sarcopenia was defined using age-specific cut-off points for appendicular skeletal muscle mass (aSMM) and fat-free mass (FFM).

Results: The study included 57 children (42.1% girls, 57.9% boys; median age: 10.9 years) with liver cirrhosis. Of them 11 (19.3%) died during the study. The mean duration of living with end-stage liver disease prior to participation was 5.43 years [IQR: 3.32, 8.39]. The most common etiology was biliary atresia (24.6%), followed by cryptogenic (22.8%). Deceased children exhibited significantly higher sarcopenia prevalence, lower basal metabolic rate and growth scores compared to survivors (P < 0.05), (771.0 vs. 934.0, P = 0.166) (65.0 vs. 80.5, P = 0.005). Total body and limb-specified lean mass were lower in deceased children, although not statistically significant. Similarly, total mineral (90% normal) and bone mineral content were lower in deceased children, with a significant difference observed only in water-to-FFM percentage (72.5 vs. 73.1, P = 0.009).

Conclusion: This study highlights the high prevalence of sarcopenia among children with ESLD and its association with adverse outcomes, including mortality. Bioimpedance analysis emerges as a promising, non-invasive method for assessing body composition in pediatric ESLD, warranting further investigation and integration into clinical practice.

背景:儿童慢性肝病(CLD)通常会导致肝硬化和终末期肝病(ESLD)。慢性肝病给治疗和预后带来了巨大挑战。评估身体成分(包括肌肉疏松症)对于了解这一人群的预后越来越重要:我们开展了一项前瞻性观察研究,研究对象为 2 至 18 岁患有 ESLD 并等待肝移植的儿童。我们收集了社会人口学、临床和实验室数据,并使用生物电阻抗分析法(BIA)评估了身体成分。根据特定年龄段的骨骼肌质量(aSMM)和无脂肪质量(FFM)的临界点来定义 "肌肉疏松症":研究包括 57 名肝硬化儿童(42.1% 为女孩,57.9% 为男孩;年龄中位数:10.9 岁)。其中 11 人(19.3%)在研究期间死亡。参与研究前,患终末期肝病的平均时间为 5.43 年 [IQR: 3.32, 8.39]。最常见的病因是胆道闭锁(24.6%),其次是隐源性(22.8%)。与存活者相比,死亡儿童的肌肉疏松症发病率明显较高,基础代谢率和生长评分也较低(P 结语:本研究突显了肌肉疏松症的高发病率:本研究强调了肌少症在 ESLD 儿童中的高发病率及其与不良后果(包括死亡率)的关联。生物阻抗分析是评估小儿 ESLD 患者身体成分的一种很有前景的非侵入性方法,值得进一步研究并纳入临床实践。
{"title":"Comparing body composition measures in children with end stage liver disease using noninvasive bioimpedance analysis.","authors":"Maryam Ekramzadeh, Seyed Ali Moosavi, Amirali Mashhadiagha, Ali Ghorbanpour, Nasrin Motazedian, Seyed Mohsen Dehghani, Homa Ilkhanipoor, Alireza Mirahmadizadeh","doi":"10.1186/s12887-024-04974-x","DOIUrl":"10.1186/s12887-024-04974-x","url":null,"abstract":"<p><strong>Background: </strong>Chronic liver disease (CLD) in children, often leads to cirrhosis and end-stage liver disease (ESLD). CLD poses significant challenges in management and prognosis. Assessing body composition, including sarcopenia, is increasingly recognized as important in understanding outcomes in this population.</p><p><strong>Methods: </strong>We conducted a prospective observational study, involving children aged 2 to 18 years with ESLD awaiting liver transplantation. Socio-demographic, clinical, and laboratory data were collected, and body composition was assessed using Bioelectrical Impedance Analysis (BIA). Sarcopenia was defined using age-specific cut-off points for appendicular skeletal muscle mass (aSMM) and fat-free mass (FFM).</p><p><strong>Results: </strong>The study included 57 children (42.1% girls, 57.9% boys; median age: 10.9 years) with liver cirrhosis. Of them 11 (19.3%) died during the study. The mean duration of living with end-stage liver disease prior to participation was 5.43 years [IQR: 3.32, 8.39]. The most common etiology was biliary atresia (24.6%), followed by cryptogenic (22.8%). Deceased children exhibited significantly higher sarcopenia prevalence, lower basal metabolic rate and growth scores compared to survivors (P < 0.05), (771.0 vs. 934.0, P = 0.166) (65.0 vs. 80.5, P = 0.005). Total body and limb-specified lean mass were lower in deceased children, although not statistically significant. Similarly, total mineral (90% normal) and bone mineral content were lower in deceased children, with a significant difference observed only in water-to-FFM percentage (72.5 vs. 73.1, P = 0.009).</p><p><strong>Conclusion: </strong>This study highlights the high prevalence of sarcopenia among children with ESLD and its association with adverse outcomes, including mortality. Bioimpedance analysis emerges as a promising, non-invasive method for assessing body composition in pediatric ESLD, warranting further investigation and integration into clinical practice.</p>","PeriodicalId":9144,"journal":{"name":"BMC Pediatrics","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11348767/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142079187","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Factors affecting maximal oxygen uptake in prepubertal children: a systematic review and meta-analysis. 影响青春期前儿童最大摄氧量的因素:系统回顾和荟萃分析。
IF 2 3区 医学 Q2 PEDIATRICS Pub Date : 2024-08-27 DOI: 10.1186/s12887-024-05013-5
Iva Jurov, Jure Demšar

In pre-pubertal children the factors affecting maximal oxygen uptake have yet to be fully understood. Therefore, the purpose of this analysis is to present cardiorespiratory fitness in prepubertal boys and girls and to determine if there are any differences based on sex, exercise testing modality or if maximal or peak oxygen consumption metrics are used. PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines were followed. For statistical analysis, multilevel models grounded in Bayesian principles were used. Selected studies obtained: maximal effort during the test, peak or maximal oxygen consumption (V̇O2) values, boys and girls (sex specific groups only) age under 11, cycle ergometry or treadmill, pre-intervention or no intervention data. In boys using cycle ergometry, 118 studies were included in the analysis, in boys using treadmill 115, in girls using cycle ergometry 83 and in girls using treadmill testing 95 study entries were included. As children get older, their cardiorespiratory fitness increases (P ≈ 100%). Studies with participants having smaller body mass have higher V̇O2 relative to body mass values (P ≈ 100%). Boys have higher V̇O2 values than girls (P ≈ 100%). Studies using treadmill reported higher values than those using cycle ergometer (P ≈ 100%). Regarding the influence of measurement method (max vs. peak) on V̇O2 values we did not find significant differences. In conclusion, we present reference values for cardiorespiratory fitness in prepubertal boys and girls using cycle ergometry or treadmill. Prepubertal boys have higher cardiorespiratory fitness than girls and using treadmill testing might be a preferred method to cycle ergometry, especially in older children. Maximal or peak oxygen consumption metrics might be used interchangeably in prepubertal children.

对于青春期前的儿童来说,影响最大摄氧量的因素尚不完全清楚。因此,本分析的目的是介绍青春期前男孩和女孩的心肺功能情况,并确定是否存在基于性别、运动测试模式或使用最大或峰值耗氧量指标的差异。研究遵循了 PRISMA(系统综述和元分析首选报告项目)指南。统计分析采用了基于贝叶斯原理的多层次模型。所选研究获得了:测试期间的最大努力、峰值或最大耗氧量(V̇O2)值、男孩和女孩(仅限特定性别组)、11 岁以下、自行车测力或跑步机、干预前或无干预数据。在使用自行车测力法的男孩中,有 118 项研究被纳入分析;在使用跑步机的男孩中,有 115 项研究被纳入分析;在使用自行车测力法的女孩中,有 83 项研究被纳入分析;在使用跑步机测试的女孩中,有 95 项研究被纳入分析。随着年龄的增长,儿童的心肺功能会增强(P≈100%)。参与者体重较小的研究结果显示,相对于体重值,V.M.O.较高(P≈100%)。男生的 V̇O2 值高于女生(P≈100%)。使用跑步机的研究报告的数值高于使用自行车测力计的研究报告的数值(P≈100%)。关于测量方法(最大值与峰值)对 V̇O2 值的影响,我们没有发现显著差异。总之,我们提出了使用自行车测力计或跑步机测量青春期前男孩和女孩心肺功能的参考值。青春期前男孩的心肺功能比女孩高,使用跑步机测试可能是比自行车测力法更好的方法,尤其是对于年龄较大的儿童。在青春期前的儿童中,最大耗氧量或峰值耗氧量指标可交替使用。
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引用次数: 0
NUP98::NSD1 and FLT3/ITD co-expression is an independent predictor of poor prognosis in pediatric AML patients. NUP98::NSD1和FLT3/ITD共同表达是预测小儿急性髓细胞性白血病患者预后不良的独立指标。
IF 2 3区 医学 Q2 PEDIATRICS Pub Date : 2024-08-24 DOI: 10.1186/s12887-024-05007-3
Jing-Wen Wang, Yu-Li, Xing-Ge Yang, Lu-Hong Xu

Objective: Patients who carry NUP98::NSD1 or FLT3/ITD mutations are reported to have poor prognosis. Previous studies have confidently reported that the poor outcome in younger AML patients is owning to dual NUP98::NSD1 and FLT3/ITD positivity, with a high overlap for those two genetic lesions. In this study, we assessed the prognostic value of the presence of both NUP98::NSD1 and FLT3/ITD in pediatric AML patients.

Methods: We screened a large cohort of 885 pediatric cases from the COG-National Cancer Institute (NCI) TARGET AML cohort and found 57 AML patients with NUP98 rearrangements.

Results: The frequency of NUP98 gene fusion was 10.8% in 529 patients. NUP98::NSD1 fusion was the most common NUP98 rearrangement, with a frequency of 59.6%(34 of 57). NUP98::NSD1 -positive patients who carried FLT3/ITD mutations had a decreased CR1 or CR2 rate than those patients carried FLT3/ITD mutation alone (P = 0.0001). Moreover, patients harboring both NUP98::NSD1 fusion and FLT3/ITD mutation exhibited inferior event-free survival (EFS, P < 0.001) and overall survival (OS, P = 0.004) than patients who were dual negative for these two genetic lesions. The presence of only NUP98::NSD1 fusion had no significant impact on EFS or OS. We also found that cases with high FLT3/ITD AR levels ( > = 0.5) with or without NUP98::NSD1 had inferior prognosis. Multivariate analysis demonstrated that the presence of both NUP98::NSD1 and FLT3/ITD was an independent prognostic factors for EFS (hazard ratio: 3.2, P = 0.001) in patients with pediatric AML. However, there was no obvious correlation with OS (hazard ratio: 1.3, P = 0.618). Stem cell transplantation did not improve the survival rate of cases with NUP98 fusion or NUP98::NSD1 AML in terms of EFS or OS.

Conclusion: Presence of both NUP98::NSD1 and FLT3/ITD was found to be an independent factor for dismal prognosis in pediatric AML patients. Notably, lack of FLT3/ITD mutations in NUP98::NSD1 -positive patients did not retain its prognostic value.

目的:据报道,携带NUP98::NSD1或FLT3/ITD突变的患者预后较差。以往的研究确信,年轻急性髓细胞白血病患者的不良预后归因于 NUP98::NSD1 和 FLT3/ITD 双阳性,这两种基因病变具有高度重叠性。在这项研究中,我们评估了NUP98::NSD1和FLT3/ITD同时存在对小儿AML患者预后的影响:我们筛选了来自COG-美国国立癌症研究所(NCI)TARGET AML队列的885例儿科病例,发现57例AML患者存在NUP98基因重排:结果:在529例患者中,NUP98基因融合的频率为10.8%。NUP98::NSD1融合是最常见的NUP98重排,频率为59.6%(57例中有34例)。与仅携带FLT3/ITD突变的患者相比,携带FLT3/ITD突变的NUP98::NSD1阳性患者的CR1或CR2率较低(P = 0.0001)。此外,同时携带NUP98::NSD1融合和FLT3/ITD突变的患者无事件生存期(EFS,P = 0.5)较差,而同时携带或不携带NUP98::NSD1的患者预后较差。多变量分析表明,同时存在NUP98::NSD1和FLT3/ITD是小儿急性髓细胞白血病患者无事件生存期的独立预后因素(危险比:3.2,P = 0.001)。但与OS无明显相关性(危险比:1.3,P = 0.618)。就EFS或OS而言,干细胞移植并未改善NUP98融合或NUP98::NSD1 AML病例的生存率:结论:NUP98::NSD1和FLT3/ITD是导致儿童急性髓细胞白血病患者预后不良的独立因素。值得注意的是,NUP98::NSD1阳性患者缺乏FLT3/ITD突变并不具有预后价值。
{"title":"NUP98::NSD1 and FLT3/ITD co-expression is an independent predictor of poor prognosis in pediatric AML patients.","authors":"Jing-Wen Wang, Yu-Li, Xing-Ge Yang, Lu-Hong Xu","doi":"10.1186/s12887-024-05007-3","DOIUrl":"10.1186/s12887-024-05007-3","url":null,"abstract":"<p><strong>Objective: </strong>Patients who carry NUP98::NSD1 or FLT3/ITD mutations are reported to have poor prognosis. Previous studies have confidently reported that the poor outcome in younger AML patients is owning to dual NUP98::NSD1 and FLT3/ITD positivity, with a high overlap for those two genetic lesions. In this study, we assessed the prognostic value of the presence of both NUP98::NSD1 and FLT3/ITD in pediatric AML patients.</p><p><strong>Methods: </strong>We screened a large cohort of 885 pediatric cases from the COG-National Cancer Institute (NCI) TARGET AML cohort and found 57 AML patients with NUP98 rearrangements.</p><p><strong>Results: </strong>The frequency of NUP98 gene fusion was 10.8% in 529 patients. NUP98::NSD1 fusion was the most common NUP98 rearrangement, with a frequency of 59.6%(34 of 57). NUP98::NSD1 -positive patients who carried FLT3/ITD mutations had a decreased CR1 or CR2 rate than those patients carried FLT3/ITD mutation alone (P = 0.0001). Moreover, patients harboring both NUP98::NSD1 fusion and FLT3/ITD mutation exhibited inferior event-free survival (EFS, P < 0.001) and overall survival (OS, P = 0.004) than patients who were dual negative for these two genetic lesions. The presence of only NUP98::NSD1 fusion had no significant impact on EFS or OS. We also found that cases with high FLT3/ITD AR levels ( > = 0.5) with or without NUP98::NSD1 had inferior prognosis. Multivariate analysis demonstrated that the presence of both NUP98::NSD1 and FLT3/ITD was an independent prognostic factors for EFS (hazard ratio: 3.2, P = 0.001) in patients with pediatric AML. However, there was no obvious correlation with OS (hazard ratio: 1.3, P = 0.618). Stem cell transplantation did not improve the survival rate of cases with NUP98 fusion or NUP98::NSD1 AML in terms of EFS or OS.</p><p><strong>Conclusion: </strong>Presence of both NUP98::NSD1 and FLT3/ITD was found to be an independent factor for dismal prognosis in pediatric AML patients. Notably, lack of FLT3/ITD mutations in NUP98::NSD1 -positive patients did not retain its prognostic value.</p>","PeriodicalId":9144,"journal":{"name":"BMC Pediatrics","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-08-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11344362/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142054950","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Two cases of Leukemoid reaction in premature infants caused by fetal inflammatory response syndrome. 两例由胎儿炎症反应综合征引起的早产儿类白血病反应。
IF 2 3区 医学 Q2 PEDIATRICS Pub Date : 2024-08-24 DOI: 10.1186/s12887-024-05006-4
Meng-Ting Feng, Qiong Ji, Dan-Dan Liu, Wei Xu

Background: Fetal inflammatory response syndrome (FIRS) is a systemic inflammatory response caused by the activation of the fetal immune system. The serological diagnostic criterion for fetal inflammatory response syndrome is a cord blood interleukin-6 concentration that exceeds 11 pg/mL, while pathologic evidence indicates the presence of funisitis or chorionic vasculitis. It can affect all systems of the fetus. Alterations in patients' hematopoietic system are primarily reflected by changes in peripheral blood leukocyte and neutrophil counts.

Case presentation: We performed placental pathology to identify FIRS and showed two cases of neonatal leukemoid reaction caused by FIRS. These two babies' alterations in hematopoietic system resolves spontaneously with the inflammation relief, without specific interventions. During the 16‑month and14- month follow‑up period, their motor and intellectual development was normal.

Conclusions: . Neonatal leukemoid reaction is a reactive disease characterized by abnormal blood parameters similar to those of leukemia, but not leukemia. It is an aberrant hematopoietic response that typically resolves spontaneously with cause relief without requiring specific interventions.

背景:胎儿炎症反应综合征(FIRS)是由胎儿免疫系统激活引起的全身性炎症反应。胎儿炎症反应综合征的血清学诊断标准是脐带血白细胞介素-6 浓度超过 11 pg/mL,而病理学证据则表明存在真菌炎或绒毛膜血管炎。它可影响胎儿的所有系统。患者造血系统的改变主要表现为外周血白细胞和中性粒细胞计数的变化:我们对胎盘进行了病理鉴定,发现了两例由 FIRS 引起的新生儿类白血病反应。这两个婴儿的造血系统改变随着炎症的缓解而自然消退,无需特殊干预。在 16 个月和 14 个月的随访期间,他们的运动和智力发育均正常。新生儿类白血病反应是一种反应性疾病,其特点是血液指标异常,与白血病相似,但不是白血病。它是一种异常的造血反应,通常在病因缓解后自发缓解,无需特殊干预。
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引用次数: 0
Prevalence and associated factors of lipodystrophy in type 1 diabetic children and adolescents at Ayder Comprehensive Specialized Hospital, Tigray, Ethiopia. 埃塞俄比亚提格雷Ayder综合专科医院1型糖尿病儿童和青少年脂肪营养不良的患病率和相关因素。
IF 2 3区 医学 Q2 PEDIATRICS Pub Date : 2024-08-24 DOI: 10.1186/s12887-024-05018-0
Tsehaye Alemseged, Abdikarin Ahmed Mohamed, Abraha Gebreegziabher Hailu, Fikaden Berhe Hadgu, Mohammed Mustefa Mohammedamin

Introduction: Lipodystrophy can cause poor glycemic control in addition to cosmetic problems in children and adolescents with type 1 diabetes mellitus. However, data on its prevalence and associated factors is scarce among children and adolescents who live in developing countries like Ethiopia.

Objective: To determine the prevalence and identify associated factors of lipodystrophy in children and adolescents with type 1 diabetes mellitus who visited the endocrinology clinic of Ayder Comprehensive Specialized Hospital between May 1 and July 31, 2020.

Method: This was an institution-based cross-sectional study conducted on 57 children and 65 adolescents with type 1 diabetes mellitus who had been taking insulin injections for a year or more. The dependent variable was lipodystrophy. A pretested, structured questionnaire was used to collect data related to lipodystrophy and other characteristics. The principal investigator oversaw the data collection, which was done by pediatric and child health specialty residents with training. Data was subjected to descriptive statistics, and predictors of lipodystrophy were identified by fitting a multivariable logistic regression model. Statistical significance was declared at p < 0.05.

Results: More than half (53.3%) of patients were in the age range of 13 to 17. The male-to-female ratio was almost 1:1. Educational status for 63.1% of patients was primary school. Four-fifths of patients were residing in urban areas. Of the 122 participants, 60 (49.2%) had lipodystrophy (48.3% lipohypertrophy and 0.8% lipoatrophy), with grade II lipohypertrophy being the most common type at 81.7%. The thigh was the most common site of lipodystrophy. In multivariable regression analysis, the long duration of insulin injection (AOR = 3.6, 95% CI, 1.5 to 9.0, p = 0.005) and inappropriate rotation of the injection site (AOR = 9.0, 95% CI, 2.2 to 37.0, p = 0.002) were significantly associated with lipodystrophy. HbA1c testing was conducted for 70 patients, and poor glycemic control (HbA1c ≥ 7%) was found in 43 (61.4%) of them. Patients with lipodystrophy were more likely to have poor glycemic control (75%) than those without lipodystrophy (47.1%) (p = 0.016).

Conclusion: The prevalence of lipodystrophy was comparable with other studies. Long duration of insulin injection and improper rotation of the injection site are associated with an increased risk of lipodystrophy. Patients with lipodystrophy were more likely to have poor glycemic control, defined by higher HgA1c, than those without lipodystrophy. Proper education of patients and their parents must include correct injection techniques, rotating injection sites, and changing injection sites intermittently to lessen the risk of developing lipodystrophy.

导言:脂肪营养不良会导致患有 1 型糖尿病的儿童和青少年血糖控制不佳,还会影响美观。然而,在埃塞俄比亚等发展中国家的儿童和青少年中,有关其患病率和相关因素的数据却很少:确定2020年5月1日至7月31日期间在艾德综合专科医院内分泌科门诊就诊的1型糖尿病儿童和青少年中脂肪营养不良的患病率并找出相关因素:这是一项以医院为基础的横断面研究,研究对象是57名儿童和65名青少年1型糖尿病患者,他们已注射胰岛素一年或一年以上。因变量为脂肪营养不良。该研究使用了一份经过预先测试的结构化问卷来收集与脂肪变性和其他特征相关的数据。数据收集工作由接受过培训的儿科和儿童健康专科住院医师完成,主要研究人员负责监督。对数据进行了描述性统计,并通过拟合多变量逻辑回归模型确定了脂肪营养不良的预测因素。统计意义以 p 为界:一半以上(53.3%)的患者年龄在 13 至 17 岁之间。男女比例接近 1:1。63.1%的患者接受过小学教育。五分之四的患者居住在城市地区。在122名参与者中,60人(49.2%)患有脂肪营养不良(48.3%为脂肪肥厚,0.8%为脂肪增生),其中二级脂肪肥厚最为常见,占81.7%。大腿是最常见的脂肪营养不良部位。在多变量回归分析中,胰岛素注射时间过长(AOR = 3.6,95% CI,1.5 至 9.0,p = 0.005)和注射部位旋转不当(AOR = 9.0,95% CI,2.2 至 37.0,p = 0.002)与脂肪变性显著相关。对 70 名患者进行了 HbA1c 检测,发现其中 43 人(61.4%)血糖控制不佳(HbA1c ≥ 7%)。脂肪营养不良患者血糖控制不佳的比例(75%)高于无脂肪营养不良患者(47.1%)(P = 0.016):结论:脂肪营养不良的发病率与其他研究结果相当。胰岛素注射时间过长和注射部位旋转不当与脂肪变性风险增加有关。与无脂肪营养不良的患者相比,有脂肪营养不良的患者更有可能血糖控制不佳,即 HgA1c 较高。对患者及其家长的正确教育必须包括正确的注射技术、旋转注射部位和间歇性更换注射部位,以降低发生脂肪变性的风险。
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引用次数: 0
A randomised controlled clinical study of standard triple therapy, bismuth-based quadruple therapy and sequential therapy for Helicobacter pylori infection in children. 针对儿童幽门螺旋杆菌感染的标准三联疗法、铋剂四联疗法和序贯疗法的随机对照临床研究。
IF 2 3区 医学 Q2 PEDIATRICS Pub Date : 2024-08-23 DOI: 10.1186/s12887-024-05020-6
Ruixue Miao, Jing Chen, Shan Gao, Liyuan Wang, Wei Zhou, Chaomin Wan, Zhiling Wang

Background and aim: Bismuth and non-bismuth quadruple therapy are the guideline-recommended first-line therapy in children with Helicobacter pylori infection in areas with high antibiotic resistance. However, their efficacy in children is uncertain and there are few well-designed studies. Here, we evaluated the eradication rates of standard triple therapy, bismuth-based quadruple therapy and sequential therapy in children with H. pylori infection.

Methods: A randomised controlled trial was conducted in children infected with H. pylori in West China Second Hospital. They were randomly assigned to 14-day standard triple therapy (omeprazole + amoxicillin + clarithromycin), 14-day bismuth quadruple therapy (bismuth + omeprazole + amoxicillin + clarithromycin) and 10-day sequential therapy (omeprazole + amoxicillin for 5 days followed by omeprazole + clarithromycin + metronidazole for 5 days). The eradication rate was assessed by a 13C-urea breath test 4 to 6 weeks after therapy completion. Symptom improvement and adverse events were compared among the groups.

Results: In total, 132 patients were enrolled. The eradication rates of 14-day standard triple therapy, 14-day bismuth quadruple therapy and 10-day sequential therapy were 70.0%, 78.9% and 50.0% in per-protocol analysis and 63.6%, 68.2% and 43.2% in intention-to-treat analysis, respectively. Symptom improvement and adverse drug event rates were similar in the three groups.

Conclusion: The three therapeutic regimens evaluated in this study are equally not recommendable for H. pylori infection treatment due to unsatisfactory eradication rates. The high prevalence of clarithromycin resistance makes the use of clarithromycin-based quadruple therapy not advisable, even in combination with amoxicillin and bismuth salts.

背景和目的:在抗生素耐药性较高的地区,铋剂和非铋剂四联疗法是指南推荐的治疗幽门螺旋杆菌感染儿童的一线疗法。然而,这两种疗法对儿童的疗效尚不确定,设计良好的研究也很少。在此,我们评估了标准三联疗法、铋剂四联疗法和序贯疗法对幽门螺杆菌感染儿童的根除率:方法:华西第二医院对感染幽门螺杆菌的儿童进行了随机对照试验。他们被随机分配到14天的标准三联疗法(奥美拉唑+阿莫西林+克拉霉素)、14天的铋剂四联疗法(铋剂+奥美拉唑+阿莫西林+克拉霉素)和10天的序贯疗法(奥美拉唑+阿莫西林5天,然后奥美拉唑+克拉霉素+甲硝唑5天)。治疗结束后 4 至 6 周,通过 13C 尿素呼气试验评估根除率。比较了各组的症状改善情况和不良反应:结果:共有 132 名患者接受了治疗。在按协议分析中,14 天标准三联疗法、14 天四联铋剂疗法和 10 天序贯疗法的根除率分别为 70.0%、78.9% 和 50.0%;在意向治疗分析中,根除率分别为 63.6%、68.2% 和 43.2%。三组患者的症状改善率和药物不良事件发生率相似:结论:由于根除率不理想,本研究评估的三种治疗方案同样不推荐用于幽门螺杆菌感染的治疗。克拉霉素耐药性的高流行率使得以克拉霉素为基础的四联疗法并不可取,即使与阿莫西林和铋盐联合使用也是如此。
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引用次数: 0
Intestinal parasitic co-infections associated with Helicobacter pylori among paediatric patients with gastrointestinal illness attending a general hospital in southern Ethiopia. 在埃塞俄比亚南部一家综合医院就诊的胃肠道疾病儿科患者中,幽门螺杆菌与肠道寄生虫合并感染。
IF 2 3区 医学 Q2 PEDIATRICS Pub Date : 2024-08-23 DOI: 10.1186/s12887-024-05026-0
Addis Aklilu, Melat Woldemariam, Edilu Wanke, Mohammed Seid, Aseer Manilal, Javed Masood Khan, Idhayadhulla Akbar

Background: Concurrent infections or co-infections caused by intestinal parasites and Helicobacter pylori are quite rampant in paediatrics living in endemic areas of sub-Saharan Africa, including Ethiopia, and if left untreated, can result in severe complications and hence must be addressed to ensure their health and well-being.

Objectives: To determine the prevalence of intestinal parasitic and H. pylori co-infections and associated factors among paediatric patients with gastrointestinal symptoms who attended the Arba Minch General Hospital (AMGH), Arba Minch, southern Ethiopia, from September to November 2020.

Methods: A cross-sectional study was conducted among a study population of 299 paediatric patients with gastrointestinal symptoms who visited AMGH. Stool samples were collected and analysed to detect H. pylori and intestinal parasites. A rapid lateral flow chromatographic immunoassay was employed to identify the H. pylori copra antigen, whereas the latter was detected using wet mount saline preparation and formol-ether concentration method. Socio-demographic, clinical, behavioural and other factors were obtained by means of a pre-tested structured questionnaire. Descriptive statistics and logistic regression analysis were done by Statistical Package for Social Service (SPSS) version 25; P values < 0.05 were considered statistically significant.

Results: The prevalence of Helicobacter pylori and intestinal parasites was 14% (n = 42) and 37.1% (n = 111), respectively, whereas that of the co-infections with these pathogens was 6.4% (n = 19). Giardia lamblia was the most prevailing parasite, 21.4% (n = 64). Informal maternal education [AOR = 5.14; 95% CI: 1.98-15.70] and lack of hand washing practice were significantly associated with the extent of co-infections [AOR = 4.18; 95% CI: 1.36-12.80].

Conclusion: Nearly one in twenty pediatric patients with gastrointestinal symptoms had intestinal parasitic infections and H. pylori co-infections, representing a silent health problem that is to be addressed through effective control strategies. Health administrators should consider the importance of co-infections in clinical diagnosis and planning aimed at its prevention.

背景:肠道寄生虫和幽门螺杆菌引起的并发感染或合并感染在撒哈拉以南非洲(包括埃塞俄比亚)流行地区的儿科患者中相当普遍,如果不及时治疗,可能会导致严重的并发症,因此必须加以解决,以确保他们的健康和福祉:确定2020年9月至11月期间在埃塞俄比亚南部阿尔巴明奇的阿尔巴明奇综合医院(AMGH)就诊的有胃肠道症状的儿科患者中肠寄生虫和幽门螺杆菌合并感染的患病率及相关因素:在 299 名到 AMGH 就诊的有胃肠道症状的儿科患者中开展了一项横断面研究。收集并分析粪便样本,以检测幽门螺杆菌和肠道寄生虫。采用快速侧流色谱免疫测定法鉴定幽门螺杆菌共聚抗原,而后者则采用湿装盐水制备法和甲醇-乙醚浓缩法进行检测。社会人口学、临床、行为和其他因素均通过预先测试的结构式问卷调查获得。使用社会服务统计软件包(SPSS)第 25 版进行了描述性统计和逻辑回归分析;P 值 结果:幽门螺杆菌和肠道寄生虫的感染率分别为 14%(42 人)和 37.1%(111 人),而这些病原体的合并感染率为 6.4%(19 人)。蓝氏贾第鞭毛虫是最常见的寄生虫,占 21.4%(64 人)。产妇的非正规教育程度[AOR = 5.14;95% CI:1.98-15.70]和缺乏洗手习惯与合并感染的程度显著相关[AOR = 4.18;95% CI:1.36-12.80]:近二十分之一有胃肠道症状的儿科患者合并有肠道寄生虫感染和幽门螺杆菌感染,这是一个无声的健康问题,需要通过有效的控制策略加以解决。卫生管理人员应在临床诊断和预防计划中考虑到合并感染的重要性。
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