首页 > 最新文献

Pediatric Surgery International最新文献

英文 中文
Classic divided sigmoidostomy vs loop sigmoidostomy in anorectal malformations: time for change? 肛门直肠畸形中的经典乙状结肠分段造口术与环状乙状结肠造口术:改变的时候到了?
IF 1.8 3区 医学 Q2 PEDIATRICS Pub Date : 2024-09-10 DOI: 10.1007/s00383-024-05834-6
Carla Ramirez-Amoros, Catarina Carvalho, María San Basilio, Leopoldo Martinez, Jose Luis Encinas, Alejandra Vilanova-Sanchez

Purpose

Divided sigmoidostomy (DS) is the classic stoma for patients with anorectal malformations (ARM). Loop sigmoidostomies (LS) in ARM are associated with a higher risk of stoma prolapse and urinary tract infections (UTI). This is not clearly supported by literature. We compared our experience with both techniques.

Methods

Retrospective study of ARM patients who underwent DS or LS between 2013 and 2023. We analysed demographics, associated malformations, intraoperative variables, oral intake and stoma functioning times, hospital stay, complications, prolapses, and UTI.

Results

Of 40 patients, 29 underwent open DS and 11 laparoscopic LS. Demographics, malformation type, associated anomalies, surgical time, intraoperative and anaesthetic complications were comparable. Postoperative complications were higher in DS than LS [14(48.3%) vs 1(9.1%), (p = 0.02)], mostly due to wound complications [12(41.3%) vs 0(0%), (p = 0.01)]; with 3 dehiscenses and 3 strictures reintervened. The hours to oral intake and stoma functioning were higher for DS [48(39–90) and 48(24–48) vs 24(24–48) and 24(24–24), (p < 0.05)], with more days of hospital stay [36(19–60) vs 8(5–10), (p = 0.001)]. Prolapses [1(3.4%) vs 1(9.1%)] and UTIs [3(10.3%) vs 1(9.1%) (p > 0.05)] were comparable.

Conclusion

LS in ARM patients have no higher risk of prolapse or UTI than DS. DS had more complications, mostly wound infections, strictures and dehiscenses.

目的分段乙状结肠造口术(DS)是肛门直肠畸形(ARM)患者的传统造口。对肛门直肠畸形患者实施环状乙状结肠造口术(LS)与造口脱垂和尿路感染(UTI)的高风险相关。这一点并没有得到文献的明确支持。我们对这两种技术的经验进行了比较。方法对 2013 年至 2023 年间接受 DS 或 LS 的 ARM 患者进行了回顾性研究。我们对人口统计学、相关畸形、术中变量、口服和造口功能时间、住院时间、并发症、脱垂和 UTI 进行了分析。结果 40 名患者中,29 人接受了开放式 DS,11 人接受了腹腔镜 LS。人口统计学、畸形类型、相关畸形、手术时间、术中并发症和麻醉并发症具有可比性。DS 术后并发症高于 LS[14(48.3%) vs 1(9.1%), (p = 0.02)],主要是由于伤口并发症[12(41.3%) vs 0(0%), (p = 0.01)];其中 3 例开裂,3 例狭窄需要再次手术。DS患者的口服时间和造口功能时间更长[48(39-90)小时和48(24-48)小时 vs 24(24-48)小时和24(24-24)小时,(p <0.05)],住院天数更多[36(19-60)天 vs 8(5-10)天,(p = 0.001)]。脱垂[1(3.4%) vs 1(9.1%)]和尿毒症[3(10.3%) vs 1(9.1%) (p > 0.05)]的发生率相当。DS的并发症较多,主要是伤口感染、狭窄和开裂。
{"title":"Classic divided sigmoidostomy vs loop sigmoidostomy in anorectal malformations: time for change?","authors":"Carla Ramirez-Amoros, Catarina Carvalho, María San Basilio, Leopoldo Martinez, Jose Luis Encinas, Alejandra Vilanova-Sanchez","doi":"10.1007/s00383-024-05834-6","DOIUrl":"https://doi.org/10.1007/s00383-024-05834-6","url":null,"abstract":"<h3 data-test=\"abstract-sub-heading\">Purpose</h3><p>Divided sigmoidostomy (DS) is the classic stoma for patients with anorectal malformations (ARM). Loop sigmoidostomies (LS) in ARM are associated with a higher risk of stoma prolapse and urinary tract infections (UTI). This is not clearly supported by literature. We compared our experience with both techniques.</p><h3 data-test=\"abstract-sub-heading\">Methods</h3><p>Retrospective study of ARM patients who underwent DS or LS between 2013 and 2023. We analysed demographics, associated malformations, intraoperative variables, oral intake and stoma functioning times, hospital stay, complications, prolapses, and UTI.</p><h3 data-test=\"abstract-sub-heading\">Results</h3><p>Of 40 patients, 29 underwent open DS and 11 laparoscopic LS. Demographics, malformation type, associated anomalies, surgical time, intraoperative and anaesthetic complications were comparable. Postoperative complications were higher in DS than LS [14(48.3%) vs 1(9.1%), (<i>p</i> = 0.02)], mostly due to wound complications [12(41.3%) vs 0(0%), (<i>p</i> = 0.01)]; with 3 dehiscenses and 3 strictures reintervened. The hours to oral intake and stoma functioning were higher for DS [48(39–90) and 48(24–48) vs 24(24–48) and 24(24–24), (<i>p</i> &lt; 0.05)], with more days of hospital stay [36(19–60) vs 8(5–10), (<i>p</i> = 0.001)]. Prolapses [1(3.4%) vs 1(9.1%)] and UTIs [3(10.3%) vs 1(9.1%) (<i>p</i> &gt; 0.05)] were comparable.</p><h3 data-test=\"abstract-sub-heading\">Conclusion</h3><p>LS in ARM patients have no higher risk of prolapse or UTI than DS. DS had more complications, mostly wound infections, strictures and dehiscenses.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":null,"pages":null},"PeriodicalIF":1.8,"publicationDate":"2024-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142211021","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Development and validation of a realistic type III esophageal atresia simulator for the training of pediatric surgeons 开发和验证用于培训小儿外科医生的逼真 III 型食道闭锁模拟器
IF 1.8 3区 医学 Q2 PEDIATRICS Pub Date : 2024-09-09 DOI: 10.1007/s00383-024-05827-5
Javier Arredondo Montero, Blanca Paola Pérez Riveros, Oscar Emilio Bueso Asfura, Nerea Martín-Calvo, Francisco Javier Pueyo, Nicolás López de Aguileta Castaño

Background

The technical complexity and limited casuistry of neonatal surgical pathology limit the possibilities of developing the necessary technical competencies by specialists in training. Esophageal atresia constitutes the paradigm of this problem. The use of synthetic 3D models for training is a promising line of research, although the literature is limited.

Methods

We conceptualized, designed, and produced an anatomically realistic model for the open correction of type III oesophageal atresia. We validated it with two groups of participants (experts and non-experts) through face, construct, and content-validity questionnaires.

Results

The model was validated by nine experts and nine non-experts. The mean procedure time for the experts and non-experts groups was 34.0 and 38.4 min, respectively. Two non-experts did not complete the procedure at the designed time (45 min). Regarding the face validity questionnaire, the mean rating of the model was 3.2 out of 4. Regarding the construct validity, we found statistically significant differences between groups for the equidistance between sutures, 100% correct in the expert group vs. 42.9% correct in the non-expert group (p = 0.02), and for the item “Confirms that tracheoesophageal fistula closure is watertight before continuing the procedure”, correctly assessed by 66.7% of the experts vs. by 11.1% of non-experts (p = 0.05). Concerning content validity, the mean score was 3.3 out of 4 for the experts and 3.4 out of 4 for the non-experts.

Conclusions

The present model is a cost-effective, simple-to-produce, and validated option for training open correction of type III esophageal atresia. However, future studies with larger sample sizes and blinded validators are needed before drawing definitive conclusions.

背景新生儿外科病理学的技术复杂性和有限的病例限制了正在接受培训的专科医生发展必要技术能力的可能性。食道闭锁是这一问题的典型代表。方法我们构思、设计并制作了一个解剖逼真的模型,用于开放式矫正 III 型食道闭锁。我们通过对两组参与者(专家和非专家)进行表面、结构和内容有效性问卷调查,对该模型进行了验证。专家组和非专家组的平均程序时间分别为 34.0 分钟和 38.4 分钟。两名非专家没有在设计时间(45 分钟)内完成程序。在面效度问卷中,模型的平均评分为 3.2 分(满分 4 分)。在构造效度方面,我们发现各组之间在缝线间距的等距方面存在显著的统计学差异,专家组的正确率为 100%,而非专家组的正确率为 42.9%。在 "继续手术前确认气管食管瘘闭合不漏水 "这一项上,66.7% 的专家和 11.1%的非专家都能正确评估(P = 0.05)。在内容效度方面,专家的平均得分是 3.3 分(满分 4 分),非专家的平均得分是 3.4 分(满分 4 分)。然而,在得出明确结论之前,未来的研究还需要更大的样本量和盲法验证。
{"title":"Development and validation of a realistic type III esophageal atresia simulator for the training of pediatric surgeons","authors":"Javier Arredondo Montero, Blanca Paola Pérez Riveros, Oscar Emilio Bueso Asfura, Nerea Martín-Calvo, Francisco Javier Pueyo, Nicolás López de Aguileta Castaño","doi":"10.1007/s00383-024-05827-5","DOIUrl":"https://doi.org/10.1007/s00383-024-05827-5","url":null,"abstract":"<h3 data-test=\"abstract-sub-heading\">Background</h3><p>The technical complexity and limited casuistry of neonatal surgical pathology limit the possibilities of developing the necessary technical competencies by specialists in training. Esophageal atresia constitutes the paradigm of this problem. The use of synthetic 3D models for training is a promising line of research, although the literature is limited.</p><h3 data-test=\"abstract-sub-heading\">Methods</h3><p>We conceptualized, designed, and produced an anatomically realistic model for the open correction of type III oesophageal atresia. We validated it with two groups of participants (experts and non-experts) through face, construct, and content-validity questionnaires.</p><h3 data-test=\"abstract-sub-heading\">Results</h3><p>The model was validated by nine experts and nine non-experts. The mean procedure time for the experts and non-experts groups was 34.0 and 38.4 min, respectively. Two non-experts did not complete the procedure at the designed time (45 min). Regarding the face validity questionnaire, the mean rating of the model was 3.2 out of 4. Regarding the construct validity, we found statistically significant differences between groups for the equidistance between sutures, 100% correct in the expert group vs. 42.9% correct in the non-expert group (<i>p</i> = 0.02), and for the item “Confirms that tracheoesophageal fistula closure is watertight before continuing the procedure”, correctly assessed by 66.7% of the experts vs. by 11.1% of non-experts (<i>p</i> = 0.05). Concerning content validity, the mean score was 3.3 out of 4 for the experts and 3.4 out of 4 for the non-experts.</p><h3 data-test=\"abstract-sub-heading\">Conclusions</h3><p>The present model is a cost-effective, simple-to-produce, and validated option for training open correction of type III esophageal atresia. However, future studies with larger sample sizes and blinded validators are needed before drawing definitive conclusions.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":null,"pages":null},"PeriodicalIF":1.8,"publicationDate":"2024-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142211020","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Improving care and survival of newborns with surgical conditions in Tanzania (TINY Tanzania): a focus on gastroschisis. 改善坦桑尼亚手术新生儿的护理和存活率(坦桑尼亚 TINY):重点关注胃裂。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2024-09-06 DOI: 10.1007/s00383-024-05828-4
Godfrey Sama Philipo, Zaitun Mohamed Bokhary, Melanie Kapapa, Neema Lala Bayyo, Massawa Klint Nyamuryekung'e, Mohamed Salim, Lazaro Mboma, Alicia Massenga, Langa Michael, Meshack Mashara, Baraka Edward Mgaya, Raphael Mwita, Aron Desta, Jay Lodhia, Neema L Gwahela, Suba Martin Sindani, Frank Martin Sudai, Judith Lindert

Purpose: Gastroschisis is associated with over 90% mortality in many sub-Saharan African countries. The introduction of the Gastroschisis Care Bundle at Muhimbili National Hospital (MNH) increased survival up to 60%. We aim to explain the impact of using implementation science methods to decentralize the care of babies with gastroschisis to other parts of Tanzania.

Methods: We used a Step-Wedge Implementation Science design to scale up gastroschisis care through training of providers, dissemination and current revision of evidence-based care protocols, advocacy, and engagement with stakeholders. We used mixed methods for data collection. Anonymous patient and provider evaluation data were collected using a nationwide Gastroschisis Database via REDCap. We evaluated the implementation and effectiveness of the care bundle in different hospitals in Tanzania.

Results: Decentralizing care nationally was feasible, acceptable, and adaptable. A total of nine trainings have been conducted training 420 providers (14 Master Trainers) reaching seven regions of Tanzania. The three advocacy national campaigns have ensured community reach and patient engagement. A countrywide gastroschisis database was developed to collect data on patients with gastroschisis, hosted locally at MNH with 332 patients' data entered in 1 year. The majority (90.2%) were treated using preformed silo bags with an overall survival of 28.5% in all centers. Late presentation and infection remain to be the main challenge.

Conclusion: To achieve quality and sustainable surgical care, there is a need to design, implement, evaluate, and continuously improve context-relevant strategies to achieve and sustain the survival of neonates with congenital anomalies. Decentralization enables clear connectedness of hospitals, bringing care closer to patients.

目的:在撒哈拉以南的许多非洲国家,90%以上的胃十二指肠畸形都与死亡率有关。穆欣比利国立医院(Muhimbili National Hospital,MNH)引进的胃裂护理包将存活率提高了 60%。我们旨在解释使用实施科学方法将胃裂婴儿护理下放到坦桑尼亚其他地区所产生的影响:方法:我们采用了 "阶梯式楔形实施科学 "设计,通过对医疗服务提供者的培训、循证护理方案的传播和现行修订、宣传以及与利益相关者的接触,扩大了胃畸形护理的规模。我们采用混合方法收集数据。通过 REDCap 使用全国范围的胃裂孔数据库收集匿名患者和医疗服务提供者的评估数据。我们评估了护理包在坦桑尼亚不同医院的实施情况和效果:结果:在全国范围内分散护理是可行的、可接受的和可适应的。在坦桑尼亚的 7 个地区共开展了 9 次培训,培训了 420 名医疗服务提供者(14 名主培训师)。三项全国性宣传活动确保了社区覆盖率和患者参与度。开发了一个全国性的胃畸形数据库,以收集胃畸形患者的数据。大多数患者(90.2%)使用预制胃袋进行治疗,所有中心的总存活率为28.5%。晚期发病和感染仍是主要挑战:为实现高质量和可持续的外科治疗,有必要设计、实施、评估并不断改进与具体情况相关的策略,以实现并维持先天性畸形新生儿的存活率。权力下放使医院之间的联系更加紧密,使医疗服务更加贴近患者。
{"title":"Improving care and survival of newborns with surgical conditions in Tanzania (TINY Tanzania): a focus on gastroschisis.","authors":"Godfrey Sama Philipo, Zaitun Mohamed Bokhary, Melanie Kapapa, Neema Lala Bayyo, Massawa Klint Nyamuryekung'e, Mohamed Salim, Lazaro Mboma, Alicia Massenga, Langa Michael, Meshack Mashara, Baraka Edward Mgaya, Raphael Mwita, Aron Desta, Jay Lodhia, Neema L Gwahela, Suba Martin Sindani, Frank Martin Sudai, Judith Lindert","doi":"10.1007/s00383-024-05828-4","DOIUrl":"https://doi.org/10.1007/s00383-024-05828-4","url":null,"abstract":"<p><strong>Purpose: </strong>Gastroschisis is associated with over 90% mortality in many sub-Saharan African countries. The introduction of the Gastroschisis Care Bundle at Muhimbili National Hospital (MNH) increased survival up to 60%. We aim to explain the impact of using implementation science methods to decentralize the care of babies with gastroschisis to other parts of Tanzania.</p><p><strong>Methods: </strong>We used a Step-Wedge Implementation Science design to scale up gastroschisis care through training of providers, dissemination and current revision of evidence-based care protocols, advocacy, and engagement with stakeholders. We used mixed methods for data collection. Anonymous patient and provider evaluation data were collected using a nationwide Gastroschisis Database via REDCap. We evaluated the implementation and effectiveness of the care bundle in different hospitals in Tanzania.</p><p><strong>Results: </strong>Decentralizing care nationally was feasible, acceptable, and adaptable. A total of nine trainings have been conducted training 420 providers (14 Master Trainers) reaching seven regions of Tanzania. The three advocacy national campaigns have ensured community reach and patient engagement. A countrywide gastroschisis database was developed to collect data on patients with gastroschisis, hosted locally at MNH with 332 patients' data entered in 1 year. The majority (90.2%) were treated using preformed silo bags with an overall survival of 28.5% in all centers. Late presentation and infection remain to be the main challenge.</p><p><strong>Conclusion: </strong>To achieve quality and sustainable surgical care, there is a need to design, implement, evaluate, and continuously improve context-relevant strategies to achieve and sustain the survival of neonates with congenital anomalies. Decentralization enables clear connectedness of hospitals, bringing care closer to patients.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142140760","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Global survey on point-of-care ultrasound (pocus) use in child surgery. 关于儿童手术中使用护理点超声波 (pocus) 的全球调查。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2024-09-05 DOI: 10.1007/s00383-024-05797-8
Gerlin Naidoo, Mohammed Salim, Andrew Jackson, Ashok Handa, Kokila Lakhoo, Judith Lindert

Purpose: To undertake a global assessment of existing ultrasound practices, barriers to access, point-of-care ultrasound (POCUS) training pathways, and the perceived clinical utility of POCUS in Child Surgery.

Methods: An electronic survey was disseminated via the GICS (Global Initiative of Children's Surgery) network. 247 anonymized responses from 48 countries were collated. 71.3% (176/247) worked in child surgery.

Results: Ultrasound was critical to practice with 84% (147/176) of requesting one daily or multiple times per week. Only 10% (17/176) could access emergency ultrasound < 1 h from request. The main barrier was a lack of trained personnel. HIC surgeons were more likely to have ultrasound training (24/29; 82.8%) compared with LMICs (74/147; 50.3%) (p = .001319; CI 95%). Self-perceived POCUS competence was associated with regularity of POCUS use (p < 0.001; CI 95%). Those who already practice POCUS most commonly use it for trauma, intussusception, and ultrasound-guided procedures. Majority (90%; 159/176) of child surgeons would attend formal POCUS training if available.

Conclusions: Ultrasound is critically important in children's surgery globally, however, many surgeons experience barriers to timely access. There is a strong interest in learning POCUS for relevant pediatric surgical applications. Further research is needed to evaluate the best methods of training, accreditation, and governance.

目的:对儿童外科中现有的超声检查实践、使用障碍、床旁超声检查(POCUS)培训途径以及床旁超声检查的临床实用性进行全球评估:方法:通过 GICS(全球儿童外科倡议)网络进行电子调查。对来自 48 个国家的 247 份匿名回复进行了整理。71.3%(176/247)的受访者从事儿童外科工作:结果:84%(147/176)的受访者要求每天或每周多次使用超声波。只有 10%(17/176)能获得紧急超声检查结论:在全球范围内,超声波对儿童外科手术至关重要,但许多外科医生在及时获取超声波方面遇到了障碍。人们对学习相关儿科手术应用的 POCUS 有着浓厚的兴趣。需要进一步开展研究,以评估培训、认证和管理的最佳方法。
{"title":"Global survey on point-of-care ultrasound (pocus) use in child surgery.","authors":"Gerlin Naidoo, Mohammed Salim, Andrew Jackson, Ashok Handa, Kokila Lakhoo, Judith Lindert","doi":"10.1007/s00383-024-05797-8","DOIUrl":"10.1007/s00383-024-05797-8","url":null,"abstract":"<p><strong>Purpose: </strong>To undertake a global assessment of existing ultrasound practices, barriers to access, point-of-care ultrasound (POCUS) training pathways, and the perceived clinical utility of POCUS in Child Surgery.</p><p><strong>Methods: </strong>An electronic survey was disseminated via the GICS (Global Initiative of Children's Surgery) network. 247 anonymized responses from 48 countries were collated. 71.3% (176/247) worked in child surgery.</p><p><strong>Results: </strong>Ultrasound was critical to practice with 84% (147/176) of requesting one daily or multiple times per week. Only 10% (17/176) could access emergency ultrasound < 1 h from request. The main barrier was a lack of trained personnel. HIC surgeons were more likely to have ultrasound training (24/29; 82.8%) compared with LMICs (74/147; 50.3%) (p = .001319; CI 95%). Self-perceived POCUS competence was associated with regularity of POCUS use (p < 0.001; CI 95%). Those who already practice POCUS most commonly use it for trauma, intussusception, and ultrasound-guided procedures. Majority (90%; 159/176) of child surgeons would attend formal POCUS training if available.</p><p><strong>Conclusions: </strong>Ultrasound is critically important in children's surgery globally, however, many surgeons experience barriers to timely access. There is a strong interest in learning POCUS for relevant pediatric surgical applications. Further research is needed to evaluate the best methods of training, accreditation, and governance.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11377359/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142140759","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
Genomic instability in congenital lung malformations in children. 儿童先天性肺畸形的基因组不稳定性。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2024-09-05 DOI: 10.1007/s00383-024-05835-5
Ronald Rodrigues de Moura, Sara Patrizi, Emmanouil Athanasakis, Jurgen Schleef, Federica Pederiva, Adamo Pio d'Adamo

Purpose: To study the biological relationship between congenital lung malformations (CLMs) and malignancy.

Methods: Biopsies of 12 CPAMs, 6 intralobar sequestrations and 2 extralobar sequestrations were analyzed through whole-genome sequencing. Blood samples from 10 patients were used to confirm or exclude somatic mosaicism. Putative somatic Single Nucleotide Variants (SNVs) were called for each malformed sample with a Panel of Normals built with control DNA samples extracted from blood. The variants were subsequently confirmed by Sanger sequencing and searched, whenever possible, in the blood samples of patients.

Results: All CLMs but one presented a signature of genomic instability by means of multiple clusters of cells with gene mutations. Seven tumor transformation-related SNVs were detected in 6/20 congenital lung malformations. Four very rare in the general population SNVs were found in a region previously linked to lung cancer in 5p15.33, upstream of TERT oncogene. Furthermore, we identified missense genetic variants, whose tumorigenic role is well known, in the RET, FANCA and MET genes.

Conclusions: Genomic instability in 95% of CLMs and genetic variants linked to tumor development in 30% of them, regardless of histopathology, are predisposing factors to malignancy, that combined with exposure to carcinogens, might trigger the development of malignancy and explain the association between CLMs and lung cancer.

目的:研究先天性肺畸形(CLMs)与恶性肿瘤之间的生物学关系:通过全基因组测序分析了 12 例 CPAM、6 例肺叶内嵌塞和 2 例肺叶外嵌塞的活组织样本。10 例患者的血样用于确认或排除体细胞嵌合。利用从血液中提取的对照 DNA 样本建立的正常组,对每个畸形样本的推测体细胞单核苷酸变异(SNV)进行了调用。这些变异随后通过桑格测序进行了确认,并尽可能在患者的血液样本中进行了搜索:结果:除一种情况外,所有 CLM 都呈现出基因组不稳定的特征,即多个细胞群存在基因突变。在 6/20 例先天性肺畸形中发现了 7 个与肿瘤转化相关的 SNV。在TERT癌基因上游的5p15.33区域发现了4个在普通人群中非常罕见的SNV。此外,我们还在 RET、FANCA 和 MET 基因中发现了错义遗传变异,其致癌作用已众所周知:结论:95%的CLMs存在基因组不稳定性,30%的CLMs存在与肿瘤发生相关的基因变异,无论组织病理学如何,这些都是导致恶性肿瘤的易感因素,再加上接触致癌物质,可能会诱发恶性肿瘤的发生,并解释了CLMs与肺癌之间的关联。
{"title":"Genomic instability in congenital lung malformations in children.","authors":"Ronald Rodrigues de Moura, Sara Patrizi, Emmanouil Athanasakis, Jurgen Schleef, Federica Pederiva, Adamo Pio d'Adamo","doi":"10.1007/s00383-024-05835-5","DOIUrl":"https://doi.org/10.1007/s00383-024-05835-5","url":null,"abstract":"<p><strong>Purpose: </strong>To study the biological relationship between congenital lung malformations (CLMs) and malignancy.</p><p><strong>Methods: </strong>Biopsies of 12 CPAMs, 6 intralobar sequestrations and 2 extralobar sequestrations were analyzed through whole-genome sequencing. Blood samples from 10 patients were used to confirm or exclude somatic mosaicism. Putative somatic Single Nucleotide Variants (SNVs) were called for each malformed sample with a Panel of Normals built with control DNA samples extracted from blood. The variants were subsequently confirmed by Sanger sequencing and searched, whenever possible, in the blood samples of patients.</p><p><strong>Results: </strong>All CLMs but one presented a signature of genomic instability by means of multiple clusters of cells with gene mutations. Seven tumor transformation-related SNVs were detected in 6/20 congenital lung malformations. Four very rare in the general population SNVs were found in a region previously linked to lung cancer in 5p15.33, upstream of TERT oncogene. Furthermore, we identified missense genetic variants, whose tumorigenic role is well known, in the RET, FANCA and MET genes.</p><p><strong>Conclusions: </strong>Genomic instability in 95% of CLMs and genetic variants linked to tumor development in 30% of them, regardless of histopathology, are predisposing factors to malignancy, that combined with exposure to carcinogens, might trigger the development of malignancy and explain the association between CLMs and lung cancer.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142140758","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Correction to: Comprehensive meta‑analysis of surgical procedure for congenital diaphragmatic hernia: thoracoscopic versus open repair. 更正:先天性膈疝外科手术的综合荟萃分析:胸腔镜与开放式修补术。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2024-08-31 DOI: 10.1007/s00383-024-05832-8
Soichi Shibuya, Irene Paraboschi, Stefano Giuliani, Takafumi Tsukui, Andreea Matei, Maricarmen Olivos, Mikihiro Inoue, Simon A Clarke, Atsuyuki Yamataka, Augusto Zani, Simon Eaton, Paolo De Coppi
{"title":"Correction to: Comprehensive meta‑analysis of surgical procedure for congenital diaphragmatic hernia: thoracoscopic versus open repair.","authors":"Soichi Shibuya, Irene Paraboschi, Stefano Giuliani, Takafumi Tsukui, Andreea Matei, Maricarmen Olivos, Mikihiro Inoue, Simon A Clarke, Atsuyuki Yamataka, Augusto Zani, Simon Eaton, Paolo De Coppi","doi":"10.1007/s00383-024-05832-8","DOIUrl":"10.1007/s00383-024-05832-8","url":null,"abstract":"","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11365829/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142110722","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
Improving outcomes for uncomplicated gastroschisis: clinical practice guidelines from the American Pediatric Surgical Association Outcomes and Evidence-based Practice Committee. 改善无并发症胃裂的治疗效果:美国儿科外科学会治疗效果和循证实践委员会的临床实践指南。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2024-08-30 DOI: 10.1007/s00383-024-05819-5
Joanne Baerg, Jarod McAteer, Doug Miniati, Stig Somme, Mark Slidell

Background: The authors sought better outcomes for uncomplicated gastroschisis through development of clinical practice guidelines.

Methods: The authors and the American Pediatric Surgical Association Outcomes and Evidenced-based Practice Committee used an iterative process and chose two questions to develop clinical practice guidelines regarding (1) standardized nutrition protocols and (2) postnatal management strategies. An English language search of PubMed, MEDLINE, OVID, SCOPUS, and the Cochrane Library Database identified literature published between January 1, 1970, and December 31, 2019, with snowballing to 2022. The Appraisal of Guideline, Research and Evaluation reporting checklist was followed.

Results: Thirty-three studies were included with a Level of Evidence that ranged from 2 to 5 and recommendation Grades B-D. Nine evaluated standardized nutrition protocols and 24 examined postnatal management strategies. The adherence to gastroschisis-specific nutrition protocols promotes intestinal feeding and reduces TPN administration. The implementation of a standardized postnatal clinical management protocol is often significantly associated with shorter hospital stays, less mechanical ventilation use, and fewer infections.

Conclusions: There is a lack of comparative studies to guide practice changes that improve uncomplicated gastroschisis outcomes. The implementation of gastroschisis-specific feeding and clinical care protocols is recommended. Feeding protocols often significantly reduce TPN administration, although the length of hospital stay may not consistently decrease.

背景:作者希望通过制定临床实践指南来提高无并发症胃裂的治疗效果:作者希望通过制定临床实践指南来改善无并发症胃裂的治疗效果:作者和美国小儿外科协会结果与循证实践委员会采用迭代过程,选择了两个问题来制定临床实践指南,分别涉及(1)标准化营养方案和(2)产后管理策略。通过对 PubMed、MEDLINE、OVID、SCOPUS 和 Cochrane 图书馆数据库进行英文检索,确定了 1970 年 1 月 1 日至 2019 年 12 月 31 日期间发表的文献,并将其滚雪球式扩展至 2022 年。结果:结果:共纳入 33 项研究,证据等级为 2 至 5 级,建议等级为 B 至 D 级。其中 9 项研究评估了标准化营养方案,24 项研究探讨了产后管理策略。坚持胃十二指肠畸形专用营养方案可促进肠道喂养并减少TPN的使用。实施标准化的产后临床管理方案往往与缩短住院时间、减少机械通气的使用和减少感染有显著关系:结论:目前还缺乏比较研究来指导实践变革,以改善无并发症胃裂的预后。建议实施针对胃裂的喂养和临床护理方案。尽管住院时间可能不会持续缩短,但喂养方案通常会大大减少TPN的使用。
{"title":"Improving outcomes for uncomplicated gastroschisis: clinical practice guidelines from the American Pediatric Surgical Association Outcomes and Evidence-based Practice Committee.","authors":"Joanne Baerg, Jarod McAteer, Doug Miniati, Stig Somme, Mark Slidell","doi":"10.1007/s00383-024-05819-5","DOIUrl":"10.1007/s00383-024-05819-5","url":null,"abstract":"<p><strong>Background: </strong>The authors sought better outcomes for uncomplicated gastroschisis through development of clinical practice guidelines.</p><p><strong>Methods: </strong>The authors and the American Pediatric Surgical Association Outcomes and Evidenced-based Practice Committee used an iterative process and chose two questions to develop clinical practice guidelines regarding (1) standardized nutrition protocols and (2) postnatal management strategies. An English language search of PubMed, MEDLINE, OVID, SCOPUS, and the Cochrane Library Database identified literature published between January 1, 1970, and December 31, 2019, with snowballing to 2022. The Appraisal of Guideline, Research and Evaluation reporting checklist was followed.</p><p><strong>Results: </strong>Thirty-three studies were included with a Level of Evidence that ranged from 2 to 5 and recommendation Grades B-D. Nine evaluated standardized nutrition protocols and 24 examined postnatal management strategies. The adherence to gastroschisis-specific nutrition protocols promotes intestinal feeding and reduces TPN administration. The implementation of a standardized postnatal clinical management protocol is often significantly associated with shorter hospital stays, less mechanical ventilation use, and fewer infections.</p><p><strong>Conclusions: </strong>There is a lack of comparative studies to guide practice changes that improve uncomplicated gastroschisis outcomes. The implementation of gastroschisis-specific feeding and clinical care protocols is recommended. Feeding protocols often significantly reduce TPN administration, although the length of hospital stay may not consistently decrease.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-08-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142110723","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Navigating the transition: a multidisciplinary approach to inflammatory bowel disease in children. 过渡时期的导航:儿童炎症性肠病的多学科方法。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2024-08-27 DOI: 10.1007/s00383-024-05789-8
A Raffaele, C M Ferlini, G Fusi, M V Lenti, E Cereda, S M E Caimmi, M Bertozzi, G Riccipetitoni

Purpose: A multidisciplinary approach to Inflammatory Bowel Disease (IBD) has recently demonstrated a positive impact in pediatric patients, reducing dropout rates and facilitating the transition to adult care. Our study aims to evaluate how this approach influences disease activity, dropout rates, and transition.

Methods: We conducted a longitudinal observational study including all patients diagnosed with IBD during pediatric-adolescent age, with a minimum follow-up period of 12 months. For each patient, endpoints included therapeutic approach, need for surgery and transition features.

Results: We included 19 patients: 13 with Ulcerative Colitis (UC) and 6 with Crohn's disease (CD). Most patients required multiple lines of therapy, with over 50% in both groups receiving biological drugs. Compliance was good, with a single dropout in each group (10, 5%). The need for surgery was significantly higher in the CD group compared to the UC group (16% vs. 7.7%, p < 0.01). Mean age at transition was significantly higher in the UC group compared to the CD group (19.2 ± 0.7 years SD vs. 18.3 ± 0.6 years SD, p < 0.05).

Conclusions: In our experience, the multidisciplinary approach to IBD in transition-age patients appears effective in achieving clinical remission, offering the potential to reduce therapeutic dropouts.

目的:多学科方法治疗炎症性肠病(IBD)最近在儿科患者中产生了积极影响,降低了辍学率并促进了向成人护理的过渡。我们的研究旨在评估这种方法如何影响疾病活动、辍学率和过渡:我们进行了一项纵向观察研究,研究对象包括所有在儿童至青少年时期被诊断为 IBD 的患者,随访期至少为 12 个月。每位患者的终点包括治疗方法、手术需求和转归特征:我们共纳入了 19 名患者:结果:我们纳入了 19 名患者:13 名溃疡性结肠炎(UC)患者和 6 名克罗恩病(CD)患者。大多数患者需要接受多线治疗,两组患者中均有50%以上接受生物药物治疗。患者的依从性良好,两组均有一名患者退出治疗(10 人,5%)。与 UC 组相比,CD 组需要手术治疗的比例明显更高(16% 对 7.7%,P 结论:我们的经验表明,多学科综合疗法能帮助患者在最短的时间内治愈疾病:根据我们的经验,多学科方法治疗过渡年龄段患者的 IBD 似乎能有效实现临床缓解,并有可能减少治疗中途退出的情况。
{"title":"Navigating the transition: a multidisciplinary approach to inflammatory bowel disease in children.","authors":"A Raffaele, C M Ferlini, G Fusi, M V Lenti, E Cereda, S M E Caimmi, M Bertozzi, G Riccipetitoni","doi":"10.1007/s00383-024-05789-8","DOIUrl":"10.1007/s00383-024-05789-8","url":null,"abstract":"<p><strong>Purpose: </strong>A multidisciplinary approach to Inflammatory Bowel Disease (IBD) has recently demonstrated a positive impact in pediatric patients, reducing dropout rates and facilitating the transition to adult care. Our study aims to evaluate how this approach influences disease activity, dropout rates, and transition.</p><p><strong>Methods: </strong>We conducted a longitudinal observational study including all patients diagnosed with IBD during pediatric-adolescent age, with a minimum follow-up period of 12 months. For each patient, endpoints included therapeutic approach, need for surgery and transition features.</p><p><strong>Results: </strong>We included 19 patients: 13 with Ulcerative Colitis (UC) and 6 with Crohn's disease (CD). Most patients required multiple lines of therapy, with over 50% in both groups receiving biological drugs. Compliance was good, with a single dropout in each group (10, 5%). The need for surgery was significantly higher in the CD group compared to the UC group (16% vs. 7.7%, p < 0.01). Mean age at transition was significantly higher in the UC group compared to the CD group (19.2 ± 0.7 years SD vs. 18.3 ± 0.6 years SD, p < 0.05).</p><p><strong>Conclusions: </strong>In our experience, the multidisciplinary approach to IBD in transition-age patients appears effective in achieving clinical remission, offering the potential to reduce therapeutic dropouts.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11349840/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142081189","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
Neonatal surgical mortality at a low resource setting, HEAL Africa tertiary hospital, Eastern Democratic Republic of the Congo. 刚果民主共和国东部一家资源匮乏的 HEAL 非洲三级医院的新生儿外科死亡率。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2024-08-27 DOI: 10.1007/s00383-024-05829-3
Jacques Fadhili Bake, Mechak Barianga Musubao, Sarah Cairo

Purpose: This study describes the experience with common neonatal surgical conditions and their outcomes at a single center in the Eastern Democratic Republic of the Congo (DRC) over a period of 7 years (2016-2022).

Methods: A retrospective review of neonatal surgical admissions and their outcomes was performed for patient admitted between January 2016 and December 2022 at HEAL Africa teaching hospital. Data were collected from the neonatal admission and discharge registry for all patients with a potential surgical condition.

Results: 107 neonates potentially requiring surgery were identified. 81.3% were referred from facilities within 10 km of HEAL Africa. The most common diagnosis was myelomeningocele/meningocele (27.1%). 68.2% of patients had an operation. The overall mortality was 29% for all patients and mean length of stay 9.9 days. Operated patients had a lower mortality at 16.4% (p-value < 0.001, OR 0.155, CI 0.062-0.389) while patients with a birth weight of less than 2500 g were more likely to die (p-value < 0.001, OR 5.333, CI 2.062-13.79).

Conclusion: The neonatal mortality rate for patients presenting with a potential surgical condition is extremely high. This is multifactorial and largely related to patient selection inherent to resource limitations.

目的:本研究描述了刚果民主共和国东部(刚果(金))一家中心在 7 年内(2016-2022 年)对常见新生儿外科疾病及其治疗效果的经验:方法:对HEAL非洲教学医院2016年1月至2022年12月期间收治的新生儿手术入院情况及其结果进行了回顾性审查。从新生儿入院和出院登记处收集了所有可能需要手术的患者的数据:结果:确定了107名可能需要手术的新生儿。81.3%的患者转诊自距离HEAL Africa 10公里以内的医疗机构。最常见的诊断是脊髓膜膨出/脑膜膨出(27.1%)。68.2%的患者接受了手术。所有患者的总死亡率为 29%,平均住院时间为 9.9 天。手术患者的死亡率较低,为 16.4%(P 值为结论):有潜在手术风险的新生儿死亡率极高。这是由多种因素造成的,在很大程度上与资源限制所固有的患者选择有关。
{"title":"Neonatal surgical mortality at a low resource setting, HEAL Africa tertiary hospital, Eastern Democratic Republic of the Congo.","authors":"Jacques Fadhili Bake, Mechak Barianga Musubao, Sarah Cairo","doi":"10.1007/s00383-024-05829-3","DOIUrl":"10.1007/s00383-024-05829-3","url":null,"abstract":"<p><strong>Purpose: </strong>This study describes the experience with common neonatal surgical conditions and their outcomes at a single center in the Eastern Democratic Republic of the Congo (DRC) over a period of 7 years (2016-2022).</p><p><strong>Methods: </strong>A retrospective review of neonatal surgical admissions and their outcomes was performed for patient admitted between January 2016 and December 2022 at HEAL Africa teaching hospital. Data were collected from the neonatal admission and discharge registry for all patients with a potential surgical condition.</p><p><strong>Results: </strong>107 neonates potentially requiring surgery were identified. 81.3% were referred from facilities within 10 km of HEAL Africa. The most common diagnosis was myelomeningocele/meningocele (27.1%). 68.2% of patients had an operation. The overall mortality was 29% for all patients and mean length of stay 9.9 days. Operated patients had a lower mortality at 16.4% (p-value < 0.001, OR 0.155, CI 0.062-0.389) while patients with a birth weight of less than 2500 g were more likely to die (p-value < 0.001, OR 5.333, CI 2.062-13.79).</p><p><strong>Conclusion: </strong>The neonatal mortality rate for patients presenting with a potential surgical condition is extremely high. This is multifactorial and largely related to patient selection inherent to resource limitations.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142073516","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A prospective multicentre study evaluating the performance of the modified simple biliary atresia scoring system in predicting biliary atresia. 一项前瞻性多中心研究,评估改良的简单胆道闭锁评分系统在预测胆道闭锁方面的性能。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2024-08-27 DOI: 10.1007/s00383-024-05830-w
Nooraini Mahat, Li Wei Chiang, Yong Chen, Nazrul Hadi Abdul Razak, Mohd Yusof Abdullah, Anand Sanmugam, Srihari Singaravel, Htoo Htoo Kyaw Soe, Shireen Anne Nah

Purpose: Early diagnosis of biliary atresia (BA) is critical for best outcomes, but is challenged by overlapping clinical manifestations with other causes of obstructive jaundice in neonates. We evaluate the performance of the modified Simple BA Scoring System (SBASS) in diagnosing BA.

Methods: We performed a prospective, cross-sectional study on infants with cholestatic jaundice (June 2021-December 2022). Modified SBASS scoring was applied and compared to the eventual diagnosis (as per intraoperative cholangiogram (IOC) and liver histopathology). The score (0-6), consists of gall bladder length < 1.6 cm (+ 1), presence of triangular cord sign (+ 1), conjugated bilirubin:total bilirubin ratio > 0.7(+ 2), gamma-glutamyl transferase (GGT) ≥ 200 U/L (+ 2).

Results: 73 were included: Fifty-two (71%) had BA. In the non-BA group, 6 (28%) had percutaneous cholangiography (PTC) while 15 (72%) had intraoperative cholangiogram (IOC). At a cut-off of 3, the modified SBASS showed sensitivity of 96.2%, specificity of 61.9% and overall accuracy of 86.3% in diagnosing BA. Area under receiver operating characteristic curve was 0.901. GGT had the highest sensitivity (94.2%), while triangular cord sign showed the highest specificity at 95.2%.

Conclusion: The SBASS provides a bedside, non-invasive scoring system for exclusion of BA in infantile cholestatic jaundice and reduces the likelihood of negative surgical explorations.

目的:早期诊断胆道闭锁(BA)对获得最佳预后至关重要,但由于临床表现与其他原因导致的新生儿阻塞性黄疸重叠,因此面临挑战。我们评估了改良的简易胆道闭锁评分系统(SBASS)在诊断胆道闭锁方面的性能:我们对患有胆汁淤积性黄疸的婴儿进行了一项前瞻性横断面研究(2021 年 6 月至 2022 年 12 月)。采用改良的 SBASS 评分,并与最终诊断(根据术中胆管造影 (IOC) 和肝组织病理学)进行比较。评分(0-6)包括胆囊长度 0.7(+ 2)、γ-谷氨酰转移酶(GGT)≥ 200 U/L(+ 2):共纳入 73 名患者:其中 52 人(71%)患有 BA。非 BA 组中,6 人(28%)进行了经皮胆管造影(PTC),15 人(72%)进行了术中胆管造影(IOC)。以 3 为临界值,改良 SBASS 诊断 BA 的灵敏度为 96.2%,特异度为 61.9%,总体准确率为 86.3%。接收者操作特征曲线下面积为 0.901。GGT 的敏感性最高(94.2%),而三角索征的特异性最高(95.2%):SBASS为排除婴儿胆汁淤积性黄疸中的BA提供了一个床旁无创评分系统,并降低了手术探查阴性的可能性。
{"title":"A prospective multicentre study evaluating the performance of the modified simple biliary atresia scoring system in predicting biliary atresia.","authors":"Nooraini Mahat, Li Wei Chiang, Yong Chen, Nazrul Hadi Abdul Razak, Mohd Yusof Abdullah, Anand Sanmugam, Srihari Singaravel, Htoo Htoo Kyaw Soe, Shireen Anne Nah","doi":"10.1007/s00383-024-05830-w","DOIUrl":"10.1007/s00383-024-05830-w","url":null,"abstract":"<p><strong>Purpose: </strong>Early diagnosis of biliary atresia (BA) is critical for best outcomes, but is challenged by overlapping clinical manifestations with other causes of obstructive jaundice in neonates. We evaluate the performance of the modified Simple BA Scoring System (SBASS) in diagnosing BA.</p><p><strong>Methods: </strong>We performed a prospective, cross-sectional study on infants with cholestatic jaundice (June 2021-December 2022). Modified SBASS scoring was applied and compared to the eventual diagnosis (as per intraoperative cholangiogram (IOC) and liver histopathology). The score (0-6), consists of gall bladder length < 1.6 cm (+ 1), presence of triangular cord sign (+ 1), conjugated bilirubin:total bilirubin ratio > 0.7(+ 2), gamma-glutamyl transferase (GGT) ≥ 200 U/L (+ 2).</p><p><strong>Results: </strong>73 were included: Fifty-two (71%) had BA. In the non-BA group, 6 (28%) had percutaneous cholangiography (PTC) while 15 (72%) had intraoperative cholangiogram (IOC). At a cut-off of 3, the modified SBASS showed sensitivity of 96.2%, specificity of 61.9% and overall accuracy of 86.3% in diagnosing BA. Area under receiver operating characteristic curve was 0.901. GGT had the highest sensitivity (94.2%), while triangular cord sign showed the highest specificity at 95.2%.</p><p><strong>Conclusion: </strong>The SBASS provides a bedside, non-invasive scoring system for exclusion of BA in infantile cholestatic jaundice and reduces the likelihood of negative surgical explorations.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142081188","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
期刊
Pediatric Surgery International
全部 Acc. Chem. Res. ACS Applied Bio Materials ACS Appl. Electron. Mater. ACS Appl. Energy Mater. ACS Appl. Mater. Interfaces ACS Appl. Nano Mater. ACS Appl. Polym. Mater. ACS BIOMATER-SCI ENG ACS Catal. ACS Cent. Sci. ACS Chem. Biol. ACS Chemical Health & Safety ACS Chem. Neurosci. ACS Comb. Sci. ACS Earth Space Chem. ACS Energy Lett. ACS Infect. Dis. ACS Macro Lett. ACS Mater. Lett. ACS Med. Chem. Lett. ACS Nano ACS Omega ACS Photonics ACS Sens. ACS Sustainable Chem. Eng. ACS Synth. Biol. Anal. Chem. BIOCHEMISTRY-US Bioconjugate Chem. BIOMACROMOLECULES Chem. Res. Toxicol. Chem. Rev. Chem. Mater. CRYST GROWTH DES ENERG FUEL Environ. Sci. Technol. Environ. Sci. Technol. Lett. Eur. J. Inorg. Chem. IND ENG CHEM RES Inorg. Chem. J. Agric. Food. Chem. J. Chem. Eng. Data J. Chem. Educ. J. Chem. Inf. Model. J. Chem. Theory Comput. J. Med. Chem. J. Nat. Prod. J PROTEOME RES J. Am. Chem. Soc. LANGMUIR MACROMOLECULES Mol. Pharmaceutics Nano Lett. Org. Lett. ORG PROCESS RES DEV ORGANOMETALLICS J. Org. Chem. J. Phys. Chem. J. Phys. Chem. A J. Phys. Chem. B J. Phys. Chem. C J. Phys. Chem. Lett. Analyst Anal. Methods Biomater. Sci. Catal. Sci. Technol. Chem. Commun. Chem. Soc. Rev. CHEM EDUC RES PRACT CRYSTENGCOMM Dalton Trans. Energy Environ. Sci. ENVIRON SCI-NANO ENVIRON SCI-PROC IMP ENVIRON SCI-WAT RES Faraday Discuss. Food Funct. Green Chem. Inorg. Chem. Front. Integr. Biol. J. Anal. At. Spectrom. J. Mater. Chem. A J. Mater. Chem. B J. Mater. Chem. C Lab Chip Mater. Chem. Front. Mater. Horiz. MEDCHEMCOMM Metallomics Mol. Biosyst. Mol. Syst. Des. Eng. Nanoscale Nanoscale Horiz. Nat. Prod. Rep. New J. Chem. Org. Biomol. Chem. Org. Chem. Front. PHOTOCH PHOTOBIO SCI PCCP Polym. Chem.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
现在去查看 取消
×
提示
确定
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术
文献互助 智能选刊 最新文献 互助须知 联系我们:info@booksci.cn
Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。
Copyright © 2023 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1