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Surgical treatment of invasive pulmonary fungal infections in immunocompromised pediatric patients: Aspergillus spp. and other emerging fungi. 免疫力低下的儿科患者侵袭性肺部真菌感染的外科治疗:曲霉菌属和其他新出现的真菌。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2024-10-05 DOI: 10.1007/s00383-024-05851-5
Sergio López-Fernández, José Andrés Molino, Pere Soler-Palacín, Natalia Mendoza-Palomar, Maria Luz Uria Oficialdegui, Marta Martos Rodríguez, Manuel López, Gabriela Guillén

Purpose: Invasive Pulmonary Fungal Infections (IPFIs) represent a diagnostic and therapeutic challenge. The exact role of surgery is not well defined. This study analyzes our experience with surgical treatment of IPFI in immunocompromised pediatric patients and, secondarily, compares IPFI caused by Aspergillus spp. with other fungal infections.

Methods: This is a retrospective review (2000-2019) of patients with IPFI surgically treated at our pediatric institution. Statistical analysis was used to compare data between Aspergillus spp. and non-Aspergillus IPFI.

Results: Twenty-five patients (64% female) underwent 29 lung resections. Median age at surgery was 7.19 years (1.63-19.14). The most frequent underlying condition (64%) was acute leukemia. Surgical indications included persistence or worsening of symptoms and pathological image findings (52%) or asymptomatic suspicious lesions in patients scheduled for intensive cytotoxic treatments or hematopoietic stem cell transplantation (48%). All patients underwent atypical lung resections, except one lobectomy. Aspergillus spp. was the most frequently isolated pathogen (68%). Follow-up was 4.07 years (0.07-18.07). Surgery-related mortality was 0%, but 4 patients died in the 100 days following surgery (2 due to disseminated fungal infection); the remaining 21 did not show signs of IPFI recurrence. Non-specific consolidations on CT scan were more frequent in non-Aspergillus IPFI (p < 0.05).

Conclusion: Surgical treatment of IPFI should be considered as a part of the treatment in selected pediatric immunocompromised patients, and it may have both diagnostic and therapeutic advantages over non-surgical management. When there is clinical suspicion of IPFI but CT scan shows unspecific alterations, the possibility of a non-Aspergillus IPFI should be considered.

目的:侵袭性肺真菌感染(IPFIs)是诊断和治疗方面的难题。手术的确切作用尚未明确。本研究分析了我们对免疫力低下的儿科患者进行 IPFI 手术治疗的经验,并将曲霉菌属引起的 IPFI 与其他真菌感染进行了比较:这是一项回顾性研究(2000-2019 年),研究对象是在本院儿科接受手术治疗的 IPFI 患者。统计分析用于比较曲霉菌属和非曲霉菌 IPFI 之间的数据:25名患者(64%为女性)接受了29例肺切除手术。手术时的中位年龄为 7.19 岁(1.63-19.14)。最常见的基础疾病(64%)是急性白血病。手术适应症包括症状和病理图像结果持续存在或恶化(52%),或计划接受强化细胞毒治疗或造血干细胞移植的患者出现无症状可疑病灶(48%)。所有患者均接受了非典型肺切除术,只有一名患者接受了肺叶切除术。曲霉菌属是最常见的分离病原体(68%)。随访时间为 4.07 年(0.07-18.07)。手术相关死亡率为0%,但有4名患者在术后100天内死亡(其中2人死于播散性真菌感染);其余21名患者未显示IPFI复发迹象。在非曲霉菌 IPFI 中,CT 扫描显示的非特异性合并症更为常见(P 结论:应考虑将 IPFI 的手术治疗作为选定的儿科免疫功能低下患者治疗的一部分,与非手术治疗相比,手术治疗可能在诊断和治疗方面都有优势。当临床怀疑有 IPFI,但 CT 扫描显示无特异性改变时,应考虑非曲霉菌 IPFI 的可能性。
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引用次数: 0
National centralization of Hirschsprung's disease in Sweden: a comparison of postoperative outcome. 瑞典赫氏胃肠病的全国集中治疗:术后效果比较。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2024-10-05 DOI: 10.1007/s00383-024-05842-6
Linnea Söderström, Christina Graneli, Daniel Rossi, Kristine Hagelsteen, Anna Gunnarsdottir, Jenny Oddsberg, Pär-Johan Svensson, Helena Borg, Matilda Bräutigam, Elisabet Gustafson, Anna Löf Granström, Pernilla Stenström, Tomas Wester

Background: In Sweden, surgical treatment of Hirschsprung's disease (HSCR) was centralized from four to two pediatric surgery centers 1st of July 2018. In adults, centralization of surgical care for complex or rare diseases seems to improve quality of care. There is little evidence supporting centralization of pediatric surgical care. The aim of this study was to assess surgical management and postoperative outcome in HSCR patients following centralization of care.

Methods: This study retrospectively analyzed data of patients with HSCR that had undergone pull-through at a pediatric surgery center in Sweden from 1st of July 2013 to 30th of June 2023. Patients managed from 1st of July 2013 to 30th of June 2018 (before centralization) were compared with patients managed from 1st of July 2018 to 30th of June 2023 (after centralization) regarding surgical treatment, unplanned procedures under general anesthesia or readmissions up to 90 days after pull-through as well as complications classified according to Clavien-Madadi up to 30 days after pull-through.

Results: In the 5-year period prior to centralization, 114 individuals from 4 treating centers were included and compared to 83 patients from 2 treating centers in the second period. There was no difference regarding age at pull-through or proportion of patients with a stoma prior to pull-through. An increase of laparoscopically assisted endorectal pull-through (8.8% to 39.8%) was observed (p < 0.001). No significant differences were seen in postoperative hospital stay, unplanned procedures under general anesthesia, or readmissions up to 90 days after pull-through. There was no difference in severe complications (Clavien-Madadi ≥ 3); however, HAEC treated with antibiotics increased following centralization (10.5-24.1%; p = 0.018).

Conclusion: Centralization of care for HSCR does not seem to delay time to pull-through nor reduce severe complications, unplanned procedures under general anesthesia or readmissions up to 90 days after pull-through. The increased HAEC rate may be due to increased awareness of mild HAEC.

Level of evidence:  Level III.

背景:在瑞典,赫氏胃肠病(HSCR)的手术治疗从 2018 年 7 月 1 日开始从四个儿科手术中心集中到两个。在成人中,集中手术治疗复杂或罕见疾病似乎可以提高医疗质量。支持儿科手术护理集中化的证据很少。本研究旨在评估集中护理后 HSCR 患者的手术管理和术后效果:本研究回顾性分析了2013年7月1日至2023年6月30日期间在瑞典一家儿科外科中心接受拉通手术的HSCR患者的数据。将2013年7月1日至2018年6月30日(集中管理前)接受治疗的患者与2018年7月1日至2023年6月30日(集中管理后)接受治疗的患者在手术治疗、全身麻醉下的计划外手术或拔管后90天内的再入院以及拔管后30天内根据Clavien-Madadi分类的并发症等方面进行比较:在集中管理前的 5 年中,共有来自 4 个治疗中心的 114 名患者接受了集中管理,而在集中管理后的 5 年中,共有来自 2 个治疗中心的 83 名患者接受了集中管理。拔管时的年龄和拔管前有造口的患者比例没有差异。观察到腹腔镜辅助肛门直肠内引流术的比例有所上升(8.8% 升至 39.8%)(p 结论:腹腔镜辅助肛门直肠内引流术的比例有所上升(8.8% 升至 39.8%):对 HSCR 进行集中护理似乎并不能延迟穿刺时间,也不能减少严重并发症、全身麻醉下的意外手术或穿刺后 90 天内的再入院率。HAEC 发生率增加的原因可能是对轻度 HAEC 的认识有所提高: 证据等级:三级。
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引用次数: 0
Utility of the oxygenation index in management of congenital diaphragmatic hernia: a report from a Thai University Surgical Centre. 氧合指数在治疗先天性膈疝中的作用:泰国大学外科中心的报告。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2024-10-05 DOI: 10.1007/s00383-024-05848-0
Issariyaporn Kulngamnetr, Pharuhad Pongmee, Paul D Losty, Suraida Aeesoa, Ampaipan Boonthai

Background: Oxygenation index (OI) is associated with severity of newborn pulmonary hypertension (PH) in congenital diaphragmatic hernia (CDH). Higher OI may indicate worst degree(s) of PH.

Objectives: This study reports OI dynamic(s) over the first 72 h of life and its correlation with (1) perioperative morbidity and (2) CDH mortality.

Methods: Medical records of inborn CDH babies during 2002-2022 were examined. OI on Days (s) 1-3 and perioperative OI trends were recorded. Operation (primary vs patch repair) and survival rates (%) were studied.

Results: Fifty-five CDH newborns (54.5% male: 45.5% female)-mean birth GA 37.5 ± 2.7 wks. had a mean birth weight 2813 ± 684 g with prenatal diagnosis in 32.7% cases. 52/55 (94.5%) were intubated at birth and HFOV deployed in 29 (55.8%). Those requiring HFOV had higher OI on DOL1 (24.8 ± 17 vs 10.3 ± 11.5; p < 0.05), DOL 2 (26.3 ± 22.9 vs 6.7 ± 12.1; p < 0.05) and DOL 3 (21.9 ± 33.8 vs 5.5 ± 9.3; p = 0.04). Operation was undertaken in 36/55 (65.5%). Preoperative mortality group had significant higher OI on DOL 2 (42.1 ± 21.0 vs 14.9 ± 9.3; p = 0.04). CDH defects were-Type A N = 27 (75%), Type B N = 7 (19.4%) and Type C N = 2 (5.6%). Overall mortality was 40% (22/55). Statistically significant OI trends were recorded in non-survival vs. survival groups on DOL 1 (31.6 ± 16.8. vs 10.5 ± 9.0; p < 0.05, DOL 2 (38.1 ± 21.9 vs 6.3 ± 7.1; p < 0.05), and DOL 3 (38.8 ± 39.4; p = 0.012).

Conclusions: OI dynamics are highly predictive for accurate monitoring of CDH cardiorespiratory physiology and crucially may guide ventilatory management as well as timing of surgery.

背景:氧合指数(OI)与先天性膈疝(CDH)新生儿肺动脉高压(PH)的严重程度有关。较高的氧合指数可能预示着 PH 的最严重程度:本研究报告了出生后 72 小时内 OI 的动态变化及其与(1)围手术期发病率和(2)CDH 死亡率的相关性:方法:研究人员查阅了 2002-2022 年间 CDH 新生儿的医疗记录。记录了第 1-3 天的 OI 和围手术期的 OI 趋势。研究了手术(初次手术与修补手术)和存活率(%):55 名 CDH 新生儿(54.5% 为男性:45.5% 为女性),平均出生体重为 2813±684 克(37.5±2.7 周),32.7% 的病例经产前诊断。52/55(94.5%)例婴儿出生时已插管,29(55.8%)例使用了高频氧合监护。需要 HFOV 的患儿在 DOL1 时的 OI 较高(24.8 ± 17 vs 10.3 ± 11.5;P 结论:OI 动态可高度预测胎儿的预后:OI 动态对准确监测 CDH 心肺生理学具有很高的预测性,关键是可以指导通气管理和手术时机的选择。
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引用次数: 0
Appendicitis in children: correlation between the surgical and histological diagnosis. 儿童阑尾炎:手术诊断与组织学诊断之间的相关性。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2024-10-04 DOI: 10.1007/s00383-024-05846-2
Balazs Fadgyas, Georgina Monostori, Dorottya Ori, Peter Vajda

Purpose: Study was designed in order to evaluate the discrepancies between surgical and histological diagnosis in pediatric acute appendicitis (AA) and to compare the outcomes of laparoscopic (LA) and open appendectomies (OA).

Methods: In a retrospective observational cohort, AA patients were included under 18 years of age, operated between 2011 and 2020. Surgical diagnosis was defined by the operating surgeon. The histological findings were classified as uncomplicated and complicated AA. The LOS and complications were also statistically analyzed.

Results: Altogether, 1444 patients were included. Significant strong correlation and a moderate to substantial agreement were found between the surgeon's and the histopathological findings in all appendectomy cases (weighted kappa value in OA: 0.633, LA: 0.639, total sample: 0.637). If the surgeon's diagnosis was less severe than the pathologist's, the LOS was 4 (3;7) days, whereas if the surgical diagnosis was correct, the LOS was 3 (3;5) days (p < 0.0001).

Conclusions: In contrary to the literature, our study revealed a strong correlation and moderate agreement between the intraoperative and histopathological findings regarding the severity of AA. Complicated cases are distinctly recognizable during the surgery. In case the surgeon underestimates the severity of AA, the chance of complications is higher.

Level of evidence: II.

目的:本研究旨在评估小儿急性阑尾炎(AA)手术诊断与组织学诊断之间的差异,并比较腹腔镜阑尾切除术(LA)与开腹阑尾切除术(OA)的疗效:在一项回顾性观察队列中,纳入了2011年至2020年间接受手术的18岁以下AA患者。手术诊断由手术医生确定。组织学检查结果分为非复杂性和复杂性 AA。此外,还对住院时间和并发症进行了统计分析:结果:共纳入 1444 例患者。在所有阑尾切除术病例中,外科医生的诊断与组织病理学结果之间均存在显著的强相关性和中度至高度的一致性(加权卡帕值:OA:0.633;LA:0.639;总样本:0.637)。如果外科医生的诊断结果比病理学家的诊断结果轻,则患者的生命周期为 4 (3;7) 天,而如果手术诊断结果正确,则患者的生命周期为 3 (3;5) 天(P 结论:如果外科医生的诊断结果比病理学家的诊断结果轻,则患者的生命周期为 4 (3;7) 天,而如果手术诊断结果正确,则患者的生命周期为 3 (3;5) 天:与文献报道相反,我们的研究显示,术中和组织病理学检查结果与 AA 的严重程度之间存在很强的相关性和适度的一致性。并发症病例在手术过程中可明显识别。如果外科医生低估了 AA 的严重程度,出现并发症的几率就会更高:证据等级:II.
{"title":"Appendicitis in children: correlation between the surgical and histological diagnosis.","authors":"Balazs Fadgyas, Georgina Monostori, Dorottya Ori, Peter Vajda","doi":"10.1007/s00383-024-05846-2","DOIUrl":"10.1007/s00383-024-05846-2","url":null,"abstract":"<p><strong>Purpose: </strong>Study was designed in order to evaluate the discrepancies between surgical and histological diagnosis in pediatric acute appendicitis (AA) and to compare the outcomes of laparoscopic (LA) and open appendectomies (OA).</p><p><strong>Methods: </strong>In a retrospective observational cohort, AA patients were included under 18 years of age, operated between 2011 and 2020. Surgical diagnosis was defined by the operating surgeon. The histological findings were classified as uncomplicated and complicated AA. The LOS and complications were also statistically analyzed.</p><p><strong>Results: </strong>Altogether, 1444 patients were included. Significant strong correlation and a moderate to substantial agreement were found between the surgeon's and the histopathological findings in all appendectomy cases (weighted kappa value in OA: 0.633, LA: 0.639, total sample: 0.637). If the surgeon's diagnosis was less severe than the pathologist's, the LOS was 4 (3;7) days, whereas if the surgical diagnosis was correct, the LOS was 3 (3;5) days (p < 0.0001).</p><p><strong>Conclusions: </strong>In contrary to the literature, our study revealed a strong correlation and moderate agreement between the intraoperative and histopathological findings regarding the severity of AA. Complicated cases are distinctly recognizable during the surgery. In case the surgeon underestimates the severity of AA, the chance of complications is higher.</p><p><strong>Level of evidence: </strong>II.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"262"},"PeriodicalIF":1.5,"publicationDate":"2024-10-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11452465/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142375736","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
Enhanced lymphangiogenesis in the left lateral segment of a biopsied liver during portoenterostomy for biliary atresia. 在对胆道闭锁进行造口术时,活检肝脏左外侧部分的淋巴管生成增强。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2024-10-04 DOI: 10.1007/s00383-024-05845-3
Yudai Tsuruno, Koshiro Sugita, Toshihiro Muraji, Ryuta Masuya, Toshio Harumatsu, Keisuke Yano, Shun Onishi, Takafumi Kawano, Chihiro Ichikawa, Haruo Ohtani, Yuko Bitoh, Satoshi Ieiri

Purpose: We investigate the histopathology of the portal vein branches and lymphatic vessels to elucidate the mechanism of atrophy of the left lateral segment (LLS) of the liver in biliary atresia (BA).

Methods: LLS and right anterior segment (RAS) liver biopsy samples obtained during Kasai portoenterostomy (KPE) from ten consecutive patients with BA underwent histopathological investigation of the portal vein and lymphatic vessels using double chromogenic immunostaining for CD31/D2-40 and the hepatitis-like findings (HLF) score. Each parameter and clinical data were compared between prognostic groups.

Results: HLF scores in the LLS were always higher than those in the RAS. There was no difference in portal vein and lymphatic vascular morphology, whereas the number of lymphatic vessels was correlated with the fibrotic area of all specimen areas. Left-to-right ratio of the number of lymphatic vessels was correlated with the age at KPE (r = 0.784, p = 0.007) and the pre-KPE CRP value (r = 0.723, p = 0.018).

Conclusions: Lymphangiogenesis on the LLS compared to the RAS was significantly correlated with the degree of fibrosis and the age at KPE. Further investigation is warranted to clarify the causes of LLS atrophy and lymphangiogenesis relevant to immune dysregulation.

目的:我们研究了门静脉分支和淋巴管的组织病理学,以阐明胆道闭锁(BA)患者肝脏左外侧段(LLS)萎缩的机制:方法:对连续十例胆道闭锁患者在葛西肠管造口术(KPE)中获得的肝左外侧段(LLS)和肝右前段(RAS)活检样本进行门静脉和淋巴管组织病理学检查,使用CD31/D2-40双色原免疫染色法和肝炎样病变(HLF)评分。对预后组的各项参数和临床数据进行比较:结果:LLS 组的 HLF 评分始终高于 RAS 组。门静脉和淋巴管形态无差异,而淋巴管数量与所有标本区域的纤维化面积相关。淋巴管数量的左右比值与KPE时的年龄(r = 0.784,p = 0.007)和KPE前的CRP值(r = 0.723,p = 0.018)相关:结论:LLS淋巴管生成与RAS淋巴管生成相比,与纤维化程度和KPE年龄显著相关。有必要进行进一步研究,以明确与免疫调节失调相关的 LLS 萎缩和淋巴管生成的原因。
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引用次数: 0
Effect of cryoablation in Nuss bar placement on opioid utilization and length of stay. 冷冻消融术在努斯杆置入术中对阿片类药物使用和住院时间的影响。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2024-10-03 DOI: 10.1007/s00383-024-05838-2
Christopher Clinker, Jack Scaife, Davian Martinez, Anastasia M Kahan, R Scott Eldredge, Katie W Russell

Background: Our institution recently transitioned from paravertebral nerve blocks (PVBs) to intercostal nerve cryoablation (INC) for pain control following minimally invasive repair of pectus excavatum (MIRPE). This study aimed to determine how INC affected the operative time, length of stay, complication rates, inpatient opioid use, and outpatient prescription of opioids at a single center.

Methods: A retrospective review was performed at a single pediatric referral center of all patients who underwent MIRPE between 2018 and 2023. Patient demographics, operative details, and perioperative course were collected. The use of INC versus PVB was recorded. Univariate analyses were performed using Wilcoxon rank sum tests for continuous variables and chi-squared tests for categorical variables.

Results: 255 patients were included with a median age of 15 years, median BMI of 18.50 kg/m2, and median Haller index of 4.40. INC was utilized in 41% (105/255), and 59% (150/255) received PVB. The two groups did not differ significantly in BMI, Haller index, or complications, though the INC patients were older by 1 year (15.0 vs. 16.0, p = 0.034). INC was associated with an increased operative time (INC: 92 min vs. PVB: 67 min, p < 0.001), decreased length of stay (3 vs. 4 days, p =  < 0.001), more than twofold decrease in inpatient opioids per day (INC: 16 MME vs. PVB: 41 MME, p < 0.001), and a fourfold decrease in the amount of opioids prescribed at discharge (INC: 90 MME vs. PVB: 390 MME, p < 0.001).

Conclusion: INC after MIRPE significantly decreased both the inpatient opioid utilization and our outpatient prescribing practices while also decreasing our overall length of stay without increasing complications.

Level of evidence: Level III.

背景:我院最近从椎旁神经阻滞术(PVB)过渡到了肋间神经低温消融术(INC),用于微创修复开胸手术(MIRPE)后的疼痛控制。本研究旨在确定 INC 如何影响一个中心的手术时间、住院时间、并发症发生率、住院患者阿片类药物的使用以及门诊患者阿片类药物的处方:一家儿科转诊中心对 2018 年至 2023 年期间接受 MIRPE 的所有患者进行了回顾性审查。收集了患者的人口统计学资料、手术细节和围手术期过程。记录了 INC 与 PVB 的使用情况。使用Wilcoxon秩和检验对连续变量进行单变量分析,使用秩方检验对分类变量进行单变量分析。结果:共纳入255名患者,中位年龄为15岁,中位BMI为18.50 kg/m2,中位Haller指数为4.40。41%的患者(105/255)使用了 INC,59%的患者(150/255)接受了 PVB。两组患者在体重指数、霍勒指数或并发症方面无明显差异,但 INC 患者的年龄比 PVB 患者大 1 岁(15.0 对 16.0,P = 0.034)。INC 与手术时间延长有关(INC:92 分钟对 PVB:67 分钟,P=0.034):MIRPE 后的 INC 大幅减少了住院患者的阿片类药物使用量和门诊处方,同时也缩短了总住院时间,而不会增加并发症:证据等级:III 级。
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引用次数: 0
Congenital diaphragmatic hernia and cleft lip and palate: looking for a common genetic etiology. 先天性膈疝和唇腭裂:寻找共同的遗传病因。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2024-10-01 DOI: 10.1007/s00383-024-05843-5
Petra Nord, Ashley H Ebanks, Petra Peterson, Erik Iwarsson, Matthew T Harting, Carmen Mesas Burgos

Purpose: Congenital diaphragmatic hernia (CDH) and cleft lip and/or palate (CL/P) are inborn closure defects. Genetic factors in and outcomes for patients with both anomalies (CDH+CL/P) remain unclear. We aimed to investigate associated genetic aberrations, prevalence of, and outcomes for, CDH+CL/P.

Methods: Data from Congenital Diaphragmatic Hernia Study Group (CDHSG) registry were collected. CL/P prevalence in CDH patients was determined. Genetic abnormalities and additional malformations in CDH+CL/P were explored. Patient characteristics and outcomes were compared between CDH+CL/P and isolated CDH (CDH-) using Fisher's Exact Test for categorical, and t-test or Mann-Whitney U-test for continuous, data. p < 0.05 was considered statistically significant.

Results: Genetic anomalies in CDH+CL/P included trisomy 13, 8p23.1 deletion, and Wolf-Hirschhorn syndrome (4p16.3 deletion). CL/P prevalence in CDH was 0.7%. CDH+CL/P had lower survival rates than CDH-, a nearly fourfold risk of death within 7 days, were less supported with extracorporeal life support (ECLS), had higher non-repair rates, and survivors had longer length of hospital stay.

Conclusion: Genetic anomalies, e.g. trisomy 13, 8p23.1 deletion, and Wolf-Hirschhorn syndrome, are seen in patients with the combination of CDH and orofacial clefts. CL/P in CDH patients is rare and associated with poorer outcomes compared to CDH-, influenced by goals of care decision-making.

目的:先天性膈疝(CDH)和唇腭裂(CL/P)是先天性闭合缺陷。这两种畸形(CDH+CL/P)患者的遗传因素和预后仍不清楚。我们旨在调查 CDH+CL/P 的相关遗传畸变、患病率和预后:从先天性膈疝研究组(CDHSG)登记处收集数据。确定CDH患者中CL/P的患病率。探讨了 CDH+CL/P 中的遗传异常和其他畸形。使用费舍尔精确检验(Fisher's Exact Test)比较 CDH+CL/P 和孤立型 CDH(CDH-)患者的特征和预后,使用 t 检验或 Mann-Whitney U 检验比较连续性数据:CDH+CL/P 的遗传异常包括 13 三体综合征、8p23.1 缺失和 Wolf-Hirschhorn 综合征(4p16.3 缺失)。CL/P在CDH中的发病率为0.7%。CDH+CL/P的存活率低于CDH-,7天内死亡的风险几乎是CDH-的4倍,体外生命支持(ECLS)的支持较少,非修复率较高,幸存者的住院时间较长:结论:遗传异常,如13三体综合征、8p23.1缺失和Wolf-Hirschhorn综合征,可见于合并CDH和口面裂隙的患者。CDH 患者中的 CL/P 很少见,与 CDH- 相比,CL/P 与较差的预后有关,并受护理决策目标的影响。
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引用次数: 0
Alpha-1-antitrypsin improves anastomotic healing in intestinal epithelial cells model. α-1-抗胰蛋白酶可改善肠上皮细胞模型中吻合口的愈合。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2024-09-30 DOI: 10.1007/s00383-024-05841-7
Nagoud Schukfeh, Kokilavani Sivaraman, Aileen Schmidt, Gertrud Vieten, Jens Dingemann, Johannes Weidner, Ruth Olmer, Sabina Janciauskiene

Purpose: Intestinal anastomosis is a routine procedure in pediatric surgery, with leakage being a significant complication. Human alpha1-antitrypsin (AAT), whose physiological serum concentrations range from 0.9-2.0 mg/ml, is known to accelerate wound healing and stimulate the expression of cell proliferation-related genes. We hypothesized that AAT might enhance anastomotic healing.

Methods: In a monolayer of non-tumorigenic HIEC-6 epithelial cells derived from fetal intestine a scratch was created. Standard medium without (control) or with AAT (0.5 and 1 mg/ml) was added. Cells were observed using a Life-Cell Imaging System. Cell proliferation was assessed, and the expression of proliferation-related genes was measured by qRT-PCR.

Results: In the presence of AAT, the scratch closed significantly faster. Cells treated with 1 mg/ml AAT showed 53% repopulation after 8 h and 97% after 18 h, while control cells showed 24% and 60% repopulation, respectively (p < 0.02). The treatment with AAT induced HIEC-6-cell proliferation and significantly increased the mRNA-expression of CDKN1A, CDKN2A, ANGPTL4, WNT3 and COL3A1 genes. AAT did not change the mRNA-expression of CXCL8 but decreased levels of IL-8 as compared to controls.

Conclusion: At physiological concentrations AAT accelerates the confluence of intestinal cells and increases cell proliferation. The local administration of AAT may bear therapeutic potential to improve anastomotic healing.

目的:肠吻合术是小儿外科的常规手术,渗漏是一个重要的并发症。人α1-抗胰蛋白酶(AAT)的生理血清浓度为 0.9-2.0 毫克/毫升,已知它能加速伤口愈合并刺激细胞增殖相关基因的表达。我们假设 AAT 可促进吻合口愈合:方法:在取自胎儿肠道的非肿瘤性 HIEC-6 上皮细胞单层中制造划痕。加入不含(对照组)或含 AAT(0.5 和 1 毫克/毫升)的标准培养基。使用 Life-Cell 成像系统观察细胞。评估细胞增殖,并通过 qRT-PCR 测定增殖相关基因的表达:结果:在有 AAT 存在的情况下,划痕闭合的速度明显加快。用 1 mg/ml AAT 处理的细胞在 8 小时后有 53% 的细胞再增殖,18 小时后有 97% 的细胞再增殖,而对照组细胞分别有 24% 和 60% 的细胞再增殖(p < 0.02)。AAT 可诱导 HIEC-6 细胞增殖,并显著增加 CDKN1A、CDKN2A、ANGPTL4、WNT3 和 COL3A1 基因的 mRNA 表达。与对照组相比,AAT 没有改变 CXCL8 的 mRNA 表达,但降低了 IL-8 的水平:结论:在生理浓度下,AAT 可加速肠细胞的汇合并增加细胞增殖。结论:在生理浓度下,AAT 可加速肠道细胞的汇合并增加细胞增殖,局部给药 AAT 可能具有改善吻合口愈合的治疗潜力。
{"title":"Alpha-1-antitrypsin improves anastomotic healing in intestinal epithelial cells model.","authors":"Nagoud Schukfeh, Kokilavani Sivaraman, Aileen Schmidt, Gertrud Vieten, Jens Dingemann, Johannes Weidner, Ruth Olmer, Sabina Janciauskiene","doi":"10.1007/s00383-024-05841-7","DOIUrl":"10.1007/s00383-024-05841-7","url":null,"abstract":"<p><strong>Purpose: </strong>Intestinal anastomosis is a routine procedure in pediatric surgery, with leakage being a significant complication. Human alpha1-antitrypsin (AAT), whose physiological serum concentrations range from 0.9-2.0 mg/ml, is known to accelerate wound healing and stimulate the expression of cell proliferation-related genes. We hypothesized that AAT might enhance anastomotic healing.</p><p><strong>Methods: </strong>In a monolayer of non-tumorigenic HIEC-6 epithelial cells derived from fetal intestine a scratch was created. Standard medium without (control) or with AAT (0.5 and 1 mg/ml) was added. Cells were observed using a Life-Cell Imaging System. Cell proliferation was assessed, and the expression of proliferation-related genes was measured by qRT-PCR.</p><p><strong>Results: </strong>In the presence of AAT, the scratch closed significantly faster. Cells treated with 1 mg/ml AAT showed 53% repopulation after 8 h and 97% after 18 h, while control cells showed 24% and 60% repopulation, respectively (p < 0.02). The treatment with AAT induced HIEC-6-cell proliferation and significantly increased the mRNA-expression of CDKN1A, CDKN2A, ANGPTL4, WNT3 and COL3A1 genes. AAT did not change the mRNA-expression of CXCL8 but decreased levels of IL-8 as compared to controls.</p><p><strong>Conclusion: </strong>At physiological concentrations AAT accelerates the confluence of intestinal cells and increases cell proliferation. The local administration of AAT may bear therapeutic potential to improve anastomotic healing.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"258"},"PeriodicalIF":1.5,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142351654","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
Parents caring and sham-feeding their child born with Esophageal atresia at home while waiting for reconstructive surgery. 食道闭锁患儿在等待重建手术期间,家长在家中对其进行护理和假性喂养。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2024-09-29 DOI: 10.1007/s00383-024-05839-1
AnnaMaria Tollne, Elin Öst, Tuva Nilsson, Markus Almström, Jan F Svensson

Purpose: For children with Esophageal atresia who have to wait for reconstructive surgery, long hospital stay, delayed introduction of oral feeds and hampered oro-motor function has traditionally been draw-backs for this treatment as the patients have minimal training of oro-motor function while waiting for surgery. In this paper, we present the concept of sham-feed at home awaiting reconstructive surgery with the aim to obliviate these problems. The aim was to describe the characteristics of patients with Esophageal atresia waiting for reconstructive surgery sham-feeding at home by their parents and further describe adverse events that arose.

Methods: The study is a retrospective descriptive single center study on all children with a delayed reconstruction of Esophageal atresia who was sham-fed by their parents at home before reconstructive surgery between January 2010 and January 2023 at the Karolinska University Hospital, Stockholm.

Results: Nine patients where home waiting for reconstructive surgery for a median of 72 days. No adverse events were reported related to the sham-feed procedure at home. The children had reconstructive surgery at a median 120 days of age. Five of the children ate full meals orally day 8-27 after surgery. Two children ate fully before 1 year after surgery. Two children had surgery less than 1 year ago and were not eating fully orally at the time of data collection.

Conclusion: Sham-feeding at home by the parents was safe and feasible with the benefits of a prolonged time out of hospital awaiting reconstructive surgery.

目的:对于需要等待重建手术的食道闭锁患儿来说,漫长的住院时间、延迟口腔喂养和口腔运动功能受阻一直是这种治疗方法的弊端,因为患者在等待手术期间只能接受最低限度的口腔运动功能训练。在本文中,我们提出了在家等待重建手术的假性喂养概念,目的是消除这些问题。目的是描述食道闭锁患者在等待重建手术期间由父母在家进行假性喂养的特点,并进一步描述出现的不良事件:该研究是一项回顾性描述性单中心研究,研究对象为2010年1月至2023年1月期间在斯德哥尔摩卡罗林斯卡大学医院接受重建手术前由父母在家假喂养的所有延迟重建食道闭锁的儿童:九名患者在家等待重建手术的时间中位数为 72 天。没有关于在家进行假性喂养的不良事件报告。这些儿童在中位 120 天时接受了重建手术。其中五名患儿在术后第 8-27 天口服了全餐。两名患儿在术后 1 年前吃饱了饭。两名儿童的手术时间不到一年,在收集数据时还没有完全口服进食:结论:父母在家进行假性喂养是安全可行的,其好处是延长了等待整形手术的住院时间。
{"title":"Parents caring and sham-feeding their child born with Esophageal atresia at home while waiting for reconstructive surgery.","authors":"AnnaMaria Tollne, Elin Öst, Tuva Nilsson, Markus Almström, Jan F Svensson","doi":"10.1007/s00383-024-05839-1","DOIUrl":"10.1007/s00383-024-05839-1","url":null,"abstract":"<p><strong>Purpose: </strong>For children with Esophageal atresia who have to wait for reconstructive surgery, long hospital stay, delayed introduction of oral feeds and hampered oro-motor function has traditionally been draw-backs for this treatment as the patients have minimal training of oro-motor function while waiting for surgery. In this paper, we present the concept of sham-feed at home awaiting reconstructive surgery with the aim to obliviate these problems. The aim was to describe the characteristics of patients with Esophageal atresia waiting for reconstructive surgery sham-feeding at home by their parents and further describe adverse events that arose.</p><p><strong>Methods: </strong>The study is a retrospective descriptive single center study on all children with a delayed reconstruction of Esophageal atresia who was sham-fed by their parents at home before reconstructive surgery between January 2010 and January 2023 at the Karolinska University Hospital, Stockholm.</p><p><strong>Results: </strong>Nine patients where home waiting for reconstructive surgery for a median of 72 days. No adverse events were reported related to the sham-feed procedure at home. The children had reconstructive surgery at a median 120 days of age. Five of the children ate full meals orally day 8-27 after surgery. Two children ate fully before 1 year after surgery. Two children had surgery less than 1 year ago and were not eating fully orally at the time of data collection.</p><p><strong>Conclusion: </strong>Sham-feeding at home by the parents was safe and feasible with the benefits of a prolonged time out of hospital awaiting reconstructive surgery.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"257"},"PeriodicalIF":1.5,"publicationDate":"2024-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11439852/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142351658","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
Management of pediatric lower extremity vascular trauma: adult vs pediatric level I trauma centers. 小儿下肢血管创伤的处理:成人与小儿一级创伤中心的对比。
IF 1.5 3区 医学 Q2 PEDIATRICS Pub Date : 2024-09-28 DOI: 10.1007/s00383-024-05837-3
Goeto Dantes, Zachary J Grady, Ahna Weeks, Nathaniel Forrester, Jose B Trinidad, Alexis Stokes, Valerie L Dutreuil, Annie Cheng, Phillip Kim, Randi N Smith, Christopher R Ramos, Samual R Todd, Alexis Smith, Jason D Sciarretta

Background: Pediatric lower extremity vascular injury (PLEVI) is uncommon and the availability of granular data is sparse. This study evaluated the surgical management of PLEVIs between a Level I adult (ATC) vs pediatric (PTC) trauma center.

Methods: We performed a retrospective review of PLEVIs (< 18 years) managed surgically between 01/2009-12/2022. Demographics and outcome data were obtained. Primary outcomes included amputation and fasciotomy rates. Secondary outcomes included type of vessel repair, mortality, and hospital length of stay.

Results: Seventy-nine patients were identified, 41 at the ATC and 38 at the PTC, totaling 112 vessels injured. ATC patients were older (median years 16.0 vs 12.5) and almost exclusively (97.6% vs 29.0%) gunshot wounds. Vascular surgeons managed 50% of injuries at the ATC vs 73.7% at the PTC (p = 0.10). Amputations were uncommon and not significantly different between centers. Seventeen patients (44.7%) required fasciotomies at the PTC vs 21 (51.2%) at the ATC (p = 0.56). Rates of vessel repair, ligation, grafting, mortality, and hospital length of stay were not significantly different.

Conclusions: PLEVI can be managed safely at ATCs and PTCs with acceptable outcomes. However, important nuances in patient triage and management need to be considered. Multi-institutional comprehensive datasets are needed.

Level of evidence:  Level III.

背景:小儿下肢血管损伤(PLEVI)并不常见,可获得的详细数据也很少。本研究评估了一级成人创伤中心(ATC)与儿科创伤中心(PTC)对下肢血管损伤的手术治疗情况:方法:我们对 PLEVIs 进行了回顾性分析:结果:共发现79名患者,其中41名在ATC,38名在PTC,受伤血管总数为112条。ATC患者年龄较大(中位年龄为16.0岁对12.5岁),几乎全部(97.6%对29.0%)为枪伤。在 ATC,血管外科医生处理了 50%的伤员,而在 PTC,这一比例为 73.7%(P = 0.10)。截肢的情况并不常见,各中心之间的差异也不大。PTC有17名患者(44.7%)需要进行筋膜切开术,而ATC有21名患者(51.2%)(P = 0.56)。血管修复率、结扎率、移植率、死亡率和住院时间无明显差异:结论:PLEVI 可在 ATC 和 PTC 安全治疗,且治疗效果可接受。结论:PLEVI 可在 ATC 和 PTC 安全治疗,且疗效可接受。然而,在患者分流和管理方面需要考虑重要的细微差别。需要多机构综合数据集: 证据等级:三级。
{"title":"Management of pediatric lower extremity vascular trauma: adult vs pediatric level I trauma centers.","authors":"Goeto Dantes, Zachary J Grady, Ahna Weeks, Nathaniel Forrester, Jose B Trinidad, Alexis Stokes, Valerie L Dutreuil, Annie Cheng, Phillip Kim, Randi N Smith, Christopher R Ramos, Samual R Todd, Alexis Smith, Jason D Sciarretta","doi":"10.1007/s00383-024-05837-3","DOIUrl":"10.1007/s00383-024-05837-3","url":null,"abstract":"<p><strong>Background: </strong>Pediatric lower extremity vascular injury (PLEVI) is uncommon and the availability of granular data is sparse. This study evaluated the surgical management of PLEVIs between a Level I adult (ATC) vs pediatric (PTC) trauma center.</p><p><strong>Methods: </strong>We performed a retrospective review of PLEVIs (< 18 years) managed surgically between 01/2009-12/2022. Demographics and outcome data were obtained. Primary outcomes included amputation and fasciotomy rates. Secondary outcomes included type of vessel repair, mortality, and hospital length of stay.</p><p><strong>Results: </strong>Seventy-nine patients were identified, 41 at the ATC and 38 at the PTC, totaling 112 vessels injured. ATC patients were older (median years 16.0 vs 12.5) and almost exclusively (97.6% vs 29.0%) gunshot wounds. Vascular surgeons managed 50% of injuries at the ATC vs 73.7% at the PTC (p = 0.10). Amputations were uncommon and not significantly different between centers. Seventeen patients (44.7%) required fasciotomies at the PTC vs 21 (51.2%) at the ATC (p = 0.56). Rates of vessel repair, ligation, grafting, mortality, and hospital length of stay were not significantly different.</p><p><strong>Conclusions: </strong>PLEVI can be managed safely at ATCs and PTCs with acceptable outcomes. However, important nuances in patient triage and management need to be considered. Multi-institutional comprehensive datasets are needed.</p><p><strong>Level of evidence: </strong> Level III.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"256"},"PeriodicalIF":1.5,"publicationDate":"2024-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142351657","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
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