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Salvage of the infected Nuss bar: a protocol and institutional experience. 抢救受感染的纳斯酒吧:协议和机构经验。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-12-15 DOI: 10.1007/s00383-025-06275-5
Christopher Clinker, Jack Scaife, Anastasia Kahan, Nicolas Cordoba, Katie W Russell, Trahern W Jones
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引用次数: 0
Testicular cytology and semen analysis in 46, XY differences/disorders of sex development (DSD): implications for fertility. 性发育障碍(DSD)患者的睾丸细胞学和精液分析:对生育的影响。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-12-15 DOI: 10.1007/s00383-025-06276-4
Suhasini Gazula, Shilpa Sharma, Venkat K Iyer, Devendra K Gupta

Purpose: The fertility potential of with 46,XY DSD individuals has remained poorly understood. This study aimed to assess testicular cytology and semen analysis findings in post-pubertal patients with 46,XY DSD.

Method: Following ethical approval, a cohort of post-pubertal (> 13 years) patients with 46,XY DSD assigned a male gender was evaluated through a personal interview, clinical examination, and hormonal assay. Prostatic and testicular volumes and echotexture were assessed. Semen analysis and testicular cytology were also performed.

Results: Of the 75 eligible patients, 17 responded. The mean age at the time of study was 17(± 3.33) years, with a follow-up of 160 (± 68.28) months. All patients reported comfort with male gender assignment. The median stretched penile length was 6 cm, and clinically assessed testicular volumes were within normal limits. However, prostate volumes were small, and prostate-specific antigen (PSA) levels were undetectable in one-third. Semen analysis revealed that 6 patients were unable to ejaculate, 4 had aspermia, 4 had oligospermia, and 3 had azoospermia. Testicular FNAC demonstrated normal spermatogenesis in only 6 patients. The median spermatogenic-to-Sertoli cell ratio was 0.84, markedly lower than the normal expected value. Notably, several patients with normal spermatogenesis on FNAC had abnormal semen analysis findings.

Conclusions: Despite overall comfort with male gender assignment and normal hormonal profiles, both semen analysis and testicular cytology revealed compromised fertility potential in post-pubertal patients with 46,XY DSD.

目的:46,xy DSD个体的生育潜力仍然知之甚少。本研究旨在评估青春期后46,xy DSD患者的睾丸细胞学和精液分析结果。方法:遵循伦理批准,通过个人访谈、临床检查和激素测定对青春期后(bb0 - 13岁)46xy男性DSD患者进行队列评估。评估前列腺和睾丸体积及超声图。同时进行精液分析和睾丸细胞学检查。结果:75例符合条件的患者中,17例有反应。研究时平均年龄为17(±3.33)岁,随访160(±68.28)个月。所有患者均表示对男性性别分配感到满意。中位伸展阴茎长度为6cm,临床评估睾丸体积在正常范围内。然而,前列腺体积小,前列腺特异性抗原(PSA)水平检测不到三分之一。精液分析显示:不能射精6例,无精症4例,少精症4例,无精症3例。只有6例患者睾丸FNAC显示精子发生正常。生精细胞与支持细胞比值中位数为0.84,明显低于正常期望值。值得注意的是,一些精子发生正常的患者在FNAC上有异常的精液分析结果。结论:尽管总体上对男性性别分配和正常的激素谱感到满意,但精液分析和睾丸细胞学显示青春期后46,xy DSD患者的生育潜力受到损害。
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引用次数: 0
School toilet facilities perception of children with hirschsprung disease: a parents perspective. 学校厕所设施对先天性巨结肠患儿的认知:家长视角。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-12-08 DOI: 10.1007/s00383-025-06263-9
A C Ter Schure, S P van Streun, C M C de Beaufort, J van Leeuwen, M van Dorp, S van Velsen-Knobbout, J Bols, M A Benninga, J P M Derikx, R R Gorter

Purpose: Access to appropriate and clean toilet facilities is essential for children with Hirschsprung Disease (HD). This study aims to assess how parents of children with HD perceive school toilet facilities.

Method: A cross-sectional survey was spread among parents of children aged 4-13 years with HD. The questionnaire addressed the frequency of toilet use and reasons for positive or negative experiences, with open- and closed-ended questions, for quantitatively and qualitatively analyses, respectively.

Results: Forty-four parents completed the questionnaire. The median age of the children was 7 years (IQR 5.3-10.8). Five (11.4%) children never used the school toilet, 16 (36.6%) used it for urination only, and 23 (52.3%) used it for urination and defecation. Positive parental experiences (29, 65.9%) were associated with the possibility for the child to clean themselves at school, while negative experiences (15, 38.6%) were linked to the absence of child-specific toilet rules. Qualitative analysis revealed six influencing themes; assistance, possibilities, school rules, medical nature, toilet facilities, and information.

Conclusion: Toilet avoidance for defecation remains common among children with HD. Improvements in toilet facilities, support, and individualized toilet policies may enhance well-being in this population.

目的:获得适当和清洁的厕所设施对患有先天性巨结肠病(HD)的儿童至关重要。本研究旨在评估自闭症儿童的家长如何看待学校厕所设施。方法:对4 ~ 13岁HD患儿家长进行横断面调查。问卷调查了使用厕所的频率和积极或消极体验的原因,有开放式和封闭式的问题,分别用于定量和定性分析。结果:44名家长完成问卷调查。患儿年龄中位数为7岁(IQR 5.3-10.8)。5名(11.4%)儿童从不使用学校厕所,16名(36.6%)儿童只使用厕所小便,23名(52.3%)儿童既使用厕所又使用厕所。积极的父母经历(29.65.9%)与孩子在学校自己清洁的可能性有关,而消极的经历(15.38.6%)与缺乏儿童专用的厕所规则有关。定性分析揭示了六个影响主题;援助,可能性,学校规则,医疗性质,厕所设施和信息。结论:逃避厕所排便在HD患儿中仍很常见。改善厕所设施、支持和个性化厕所政策可能会提高这一人群的幸福感。
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引用次数: 0
Clinical challenges in the management of neonatal testicular torsion: a single-center perspective. 新生儿睾丸扭转治疗的临床挑战:单中心视角。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-12-08 DOI: 10.1007/s00383-025-06264-8
Chang-Kun Mao, Cheng-Pin Tao, Chao Yang
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引用次数: 0
Elevated day-2 hemoglobin P₅₀ and risk of necrotizing enterocolitis in preterm infants: a pilot study. 第2天血红蛋白P₅0升高和早产儿坏死性小肠结肠炎的风险:一项试点研究。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-12-08 DOI: 10.1007/s00383-025-06269-3
Yakup Karakurt, Çağrı Cumhur Gök, Emrah Can

Purpose: To evaluate whether hemoglobin P₅₀, automatically calculated from routine blood gas analyses, can serve as a practical early biomarker for necrotizing enterocolitis (NEC) in preterm infants.

Methods: We retrospectively analyzed 117 preterm infants born at < 32 weeks' gestation who were admitted to a tertiary NICU between 2022 and 2024. Hemoglobin P₅₀ and serum lactate values were obtained from arterial blood gases on postnatal days 1-3. NEC was defined according to modified Bell's criteria (stage ≥ II). Discriminative performance was assessed using receiver operating characteristic (ROC) analysis, with a prespecified P₅₀ threshold of 21.0 mmHg.

Results: NEC developed in 27 infants (23%). Mean day-2 P₅₀ was significantly higher in the NEC group compared with controls (22.5 ± 6.8 vs. 18.9 ± 3.6 mmHg; p = 0.03). Day-2 P₅₀ predicted NEC with moderate accuracy (AUC 0.70, 95% CI 0.56-0.83), outperforming lactate (AUC 0.52, 95% CI 0.38-0.67). At the 21.0 mmHg cutoff, sensitivity was 67%, specificity 72%, positive predictive value 41.8%, and negative predictive value 87.9%.

Conclusion: Day-2 hemoglobin P₅₀ was elevated in infants who subsequently developed NEC. These preliminary findings indicate that P₅₀ may be a feasible, cost-effective biomarker for early NEC risk stratification; however, larger prospective studies are required to validate its predictive utility.

目的:评估从常规血气分析自动计算的血红蛋白P₅0是否可以作为早产儿坏死性小肠结肠炎(NEC)的实用早期生物标志物。方法:我们回顾性分析了117名在美国出生的早产儿。结果:27名婴儿(23%)发生NEC。NEC组的平均day-2 P₅0明显高于对照组(22.5±6.8 vs. 18.9±3.6 mmHg; P = 0.03)。Day-2 P₅0预测NEC具有中等精度(AUC 0.70, 95% CI 0.56-0.83),优于乳酸(AUC 0.52, 95% CI 0.38-0.67)。在21.0 mmHg临界值时,敏感性为67%,特异性为72%,阳性预测值为41.8%,阴性预测值为87.9%。结论:在随后发展为NEC的婴儿中,第2天血红蛋白P₅0升高。这些初步研究结果表明,P₅0可能是一种可行的、具有成本效益的早期NEC风险分层生物标志物;然而,需要更大规模的前瞻性研究来验证其预测效用。
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引用次数: 0
Investigation of anogenital distance/anal position index and intestinal functional outcomes in children with hypospadias. 尿道下裂患儿肛门生殖器距离/肛门位置指数与肠道功能结局的研究。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-12-06 DOI: 10.1007/s00383-025-06261-x
Caner İsbir, Özlem Elvan, Şener Çolak, Hakan Taşkınlar

Purpose: Perineal morphometric alterations and functional outcomes in patients with hypospadias remain a subject of ongoing debate. This study aimed to evaluate the changes in anogenital distance (AGD)/anal position index (API) values and their intestinal functional impacts in relation to the severity of hypospadias in children.

Method: Fifty-one children who underwent hypospadias surgery (2014-2024) and 19 controls presenting for circumcision (June-September 2024) were assessed. Patients were classified as distal hypospadias (n = 31) or proximal hypospadias (n = 20). Posterior scrotal raphe-anus center (AGD), posterior scrotal raphe-coccyx distance, and API were measured. Constipation was evaluated using Rome IV criteria. Percentile values ​​were calculated.

Results: Patient ages (years): 7.42 ± 1.67/11.51 ± 2.79/8.8 ± 2.37 for control/distal/proximal hypospadias groups. Proximal hypospadias showed significantly lower AGD (33.39 ± 8.11 mm) and API (0.35 ± 0.07) compared to distal hypospadias (54.79 ± 15.65 mm; 0.53 ± 0.07) and controls (55.54 ± 15.57 mm; 0.59 ± 0.08) (p = 0.042), (p < 0.001). Constipation was strongly associated with proximal hypospadias (p < 0.001). AGD distribution varied significantly across percentiles (p < 0.001), whereas API distribution did not significantly (p = 0.587).

Conclusion: Changes in perineal morphometric parameters and their functional outcomes exhibit clinically meaningful distinctions in pediatric patients with proximal hypospadias. Furthermore, it is suggested that these perineal morphometric measurements should be taken into account in predicting and monitoring constipation status during postoperative follow-up in children with hypospadias.

目的:尿道下裂患者的会阴形态改变和功能结局仍然是一个持续争论的主题。本研究旨在评估肛门生殖器距离(AGD)/肛门位置指数(API)值的变化及其对儿童尿道下裂严重程度的肠道功能影响。方法:对51例尿道下裂手术患儿(2014-2024年)和19例对照组患儿(2024年6月- 9月)行包皮环切术进行评估。患者分为远端尿道下裂(n = 31)和近端尿道下裂(n = 20)。测量后阴囊rape -anus中心(AGD)、后阴囊rape -尾骨距离、API。使用Rome IV标准评估便秘。计算百分位数。结果:对照组/远端/近端尿道下裂组患者年龄(岁):7.42±1.67/11.51±2.79/8.8±2.37。尿道下裂近端AGD(33.39±8.11 mm)和API(0.35±0.07)明显低于尿道下裂远端(54.79±15.65 mm; 0.53±0.07)和对照组(55.54±15.57 mm; 0.59±0.08)(p = 0.042)。(p)结论:小儿尿道下裂近端患者会阴形态测量参数及其功能结局的变化具有临床意义。此外,我们建议在尿道下裂患儿术后随访中,应考虑这些会阴形态测量来预测和监测便秘状况。
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引用次数: 0
Preoperative mechanical bowel preparation versus no preparation for pediatric intestinal surgery: a systematic review and meta-analysis. 儿童肠道手术术前机械肠道准备与不准备:一项系统回顾和荟萃分析。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-12-06 DOI: 10.1007/s00383-025-06265-7
Rajpal Singh Sisodiya, Prince Raj, Akanksha Tomar, Himanshu Acharya, Balkrishna Tripathi, Vikesh Agrawal, Abhishek Tiwari

Background: Mechanical bowel preparation (MBP) has traditionally been routine before pediatric intestinal surgery, based on the belief that it reduces the risk of infection. In adults, benefits are only observed when MBP is combined with oral antibiotics; however, evidence in pediatrics remains limited and inconsistent.

Methods: We conducted a systematic review and meta-analysis (PROSPERO CRD420251133552) following PRISMA 2020 and Cochrane guidelines, searching databases up to August 2025. Eligible studies included Randomized controlled trials and comparative observational cohorts comparing MBP with no MBP in elective Pediatric intestinal surgery. The primary outcome was surgical site infection (SSI); secondary outcomes included anastomotic leak (AL), intra-abdominal abscess (IAA), ileus, bowel obstruction, and length of stay (LOS). Random-effects models produced pooled estimates, and certainty was assessed using the GRADE approach.

Results: Seventeen studies involving 3,036 children were included. MBP showed no reduction in SSI, AL, or IAA, with moderate certainty for SSI and low certainty for AL/IAA due to rare events. MBP was associated with longer LOS and possible non-significant increases in ileus and obstruction.

Conclusion: Current evidence suggests that MBP offers no significant benefits in pediatric elective intestinal surgery and may even delay recovery. Within the framework of ERAS protocols, routine use of MBP appears unnecessary. However, MBP might still be required for specific functional or reconstructive indications. Further high-quality multicentre trials are essential before any definitive conclusions can be drawn regarding its discontinuation.

背景:机械肠道准备(MBP)传统上是儿童肠道手术前的常规,基于它可以降低感染风险的信念。在成人中,只有当MBP与口服抗生素联合使用时才能观察到益处;然而,儿科的证据仍然有限且不一致。方法:我们按照PRISMA 2020和Cochrane指南进行了系统评价和荟萃分析(PROSPERO CRD420251133552),检索截至2025年8月的数据库。符合条件的研究包括随机对照试验和比较选择性小儿肠道手术中MBP与不MBP的比较观察队列。主要结局为手术部位感染(SSI);次要结局包括吻合口漏(AL)、腹内脓肿(IAA)、肠梗阻、肠梗阻和住院时间(LOS)。随机效应模型产生汇总估计,使用GRADE方法评估确定性。结果:纳入17项研究,涉及3036名儿童。MBP未显示SSI、AL或IAA的减少,由于罕见事件,SSI有中等确定性,AL/IAA有低确定性。MBP与较长的LOS和可能的肠梗阻和梗阻无显著性增加有关。结论:目前的证据表明,MBP在儿童选择性肠道手术中没有明显的益处,甚至可能延迟恢复。在ERAS协议框架内,常规使用MBP似乎是不必要的。然而,MBP可能仍然需要用于特定的功能或重建适应症。进一步的高质量多中心试验是必不可少的,然后才能得出关于停药的任何明确结论。
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引用次数: 0
Identification of CD7 as a novel biomarker of embryonal hepatoblastoma. CD7作为胚胎性肝母细胞瘤新标志物的鉴定。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-12-04 DOI: 10.1007/s00383-025-06255-9
Ahmad Adawy, Daiki Yoshii, Hiroki Hirao, Yoshihiro Komohara, Yukio Fujiwara, Masahiro Tomita, Masaki Honda, Jia Han, Yao Liu, Sohsuke Yamada, Masahiro Yamamoto, Eiso Hiyama, Taizo Hibi

Purpose: This study aimed to investigate the expression of cluster of differentiation 7 (CD7) in hepatoblastoma (HB) and its potential use as a novel biomarker of HB.

Methods: CD7 expression was investigated in human HB samples at the gene level by bulk, single-cell RNA sequencing, and spatial transcriptomic analyses, in addition to the protein level by immunohistochemical (IHC) staining. CD7 gene expression-based survival analysis was also conducted, along with gene set enrichment analysis (GSEA) of the CD7-SECTM1 receptor-ligand gene pair.

Results: CD7 was differentially expressed in human HB at both the gene level by various bioinformatics analyses, and the protein level by IHC, with remarkably higher expression levels in embryonal HB. Conversely, CD7 was not expressed in other primary adult liver tumors. CD7high HB cases showed poorer 5-year event-free survival (P = 0.016), and GSEA demonstrated that CD7 is linked to the embryonal MYCN transcription factor, as were protumor kinases such as JAK3, and marginally MAPK14 and MAPK3.

Conclusion: CD7 is expressed in human HB, especially the embryonal histological subtype, and appears to be linked to tumor progression and poor clinical outcomes. Nevertheless, CD7-targeted chimeric antigen receptor T cells could be proposed as a promising immunotherapy for embryonal HB.

目的:本研究旨在探讨肝母细胞瘤(HB)中CD7的表达及其作为HB新的生物标志物的潜力。方法:通过单细胞RNA测序和空间转录组学分析,在基因水平上研究CD7在人HB样本中的表达,并通过免疫组化(IHC)染色在蛋白质水平上研究CD7的表达。我们还进行了基于CD7基因表达的生存分析,以及CD7- sectm1受体-配体基因对的基因集富集分析(GSEA)。结果:CD7在人HB中存在基因水平和蛋白水平的差异,且在胚胎HB中的表达水平显著升高。相反,CD7在其他原发性成人肝脏肿瘤中不表达。CD7高的HB患者的5年无事件生存率较差(P = 0.016), GSEA表明CD7与胚胎MYCN转录因子相关,与JAK3等蛋白激酶相关,MAPK14和MAPK3也与之相关。结论:CD7在人HB中表达,尤其是胚胎组织学亚型,并且似乎与肿瘤进展和不良临床结果有关。然而,靶向cd7的嵌合抗原受体T细胞可能被认为是一种有前途的胚胎HB免疫治疗方法。
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引用次数: 0
Investigation of vasoactive intestinal peptide expression and significance in a congenital diaphragmatic hernia animal model. 先天性膈疝动物模型血管活性肠肽表达及意义的研究。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-12-04 DOI: 10.1007/s00383-025-06257-7
Jiajun Chen, Huijiao Xu, Li Yang, Feifan Chen, Kunpeng Li, Bing Xu, Wenying Liu, Fang Hou

Purpose: To investigate vasoactive intestinal peptide (VIP) expression and distribution in fetal Sprague-Dawley (SD) rat lung with congenital diaphragmatic hernia (CDH). Assess the impact of VIP analog Aviptadil on CDH-associated lung hypoplasia.

Methods: CDH was induced in pregnant SD rats by nitrofen gavage on E9.5. The CDH + VIP group received Aviptadil via tail vein from E10.5. Lung development was assessed by hematoxylin and eosin (HE) staining. VIP, α-SMA, and CD31 were evaluated by immunofluorescence (IF). VIP mRNA and protein levels were quantified by RT-qPCR and Western blotting.

Results: The CDH group exhibited a significantly lower lung index compared to the control group (P < 0.001), with no significant difference observed between the CDH and CDH + VIP groups. Compared to controls, VIP expression in CDH lungs was significantly downregulated at both the mRNA (P = 0.049) and protein levels (P = 0.049). HE staining revealed mature alveolar structures in the control group, whereas the CDH group showed disrupted pulmonary architecture. Partial improvement was observed in the CDH + VIP group. IF analysis indicated that VIP was predominantly localized in the bronchi. VIP fluorescence intensity was significantly decreased in the CDH group compared to both the control group (P = 0.002) and the CDH + VIP group (P = 0.005), while no significant difference was found between the CDH + VIP and control groups. α-SMA fluorescence was primarily localized to pulmonary arterioles and bronchial smooth muscle. Compared to the control group, α-SMA expression was significantly upregulated in the CDH group (P < 0.001). The CDH + VIP group showed a significant reduction in α-SMA expression compared to the CDH group (P = 0.026), with no significant difference from the control group. CD31 was mainly localized to the vascular endothelium. CD31 fluorescence intensity was markedly increased in the CDH group compared to both the control (P < 0.001) and CDH + VIP groups (P < 0.001). The CDH + VIP group also had significantly higher CD31 levels than the control group (P = 0.005).

Conclusion: This study revealed that VIP is downregulated in CDH lungs. In this nitrofen-induced SD rat model, Aviptadil partially restored VIP levels and was associated with attenuation of vascular remodeling and alveolar dysplasia, suggesting a possible therapeutic role in CDH-related pulmonary hypoplasia that warrants further investigation.

目的:探讨血管活性肠肽(VIP)在Sprague-Dawley (SD)胎鼠先天性膈疝(CDH)肺中的表达和分布。评估VIP类似物阿维他地尔对冠心病相关性肺发育不全的影响。方法:以妊娠SD大鼠E9.5灌胃硝芬诱导CDH。CDH + VIP组从E10.5开始经尾静脉给予阿维他地尔。苏木精和伊红(HE)染色评估肺发育。免疫荧光(IF)法检测VIP、α-SMA、CD31。RT-qPCR和Western blotting检测VIP mRNA和蛋白水平。结果:CDH组肺指数明显低于对照组(P)。结论:本研究提示CDH组肺VIP下调。在这个硝芬诱导的SD大鼠模型中,Aviptadil部分恢复VIP水平,并与血管重塑和肺泡发育不良的衰减有关,这表明它可能在cdh相关的肺发育不全中具有治疗作用,值得进一步研究。
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引用次数: 0
Outcomes of major liver resection for children with hepatoblastoma weighing less than 10 kg: avoiding liver transplantation with extended or extreme resection. 体重小于10kg的儿童肝母细胞瘤大切除的结果:避免肝移植和扩大或极端切除。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-11-27 DOI: 10.1007/s00383-025-06249-7
Pattamon Sutthatarn, Wipusit Taesombat, Matina Kruephate, Pongserath Sirichindakul, Bunthoon Nonthasoot, Pornchai Achatsachat, Piti Techavichit, Teerasak Phewplung, Paisarn Vejchapipat
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引用次数: 0
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Pediatric Surgery International
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