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Role of ileal diversion in pediatric inflammatory bowel disease. 回肠转流术在小儿炎症性肠病中的作用。
IF 2.4 3区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-10-01 Epub Date: 2024-07-29 DOI: 10.1002/jpn3.12331
Amanda A Simard, Swetha Kotamraju, Jennifer R DeFazio, Joseph A Picoraro

Surgical intervention is often indicated in pediatric inflammatory bowel disease (IBD) for medically refractory disease or complications of severe disease. Specifically, surgical intervention via ileal diversion allows for fecal flow to be redirected away from diseased distal bowel and through an ileostomy. It is utilized in patients who have medically refractory colitis, severe perianal disease, or irreversible bowel damage. In patients with ulcerative colitis, it is primarily performed during a restorative proctocolectomy with ileal pouch anal anastomosis to protect the high-risk anastomoses. In the setting of Crohn's disease, ileal diversion reduces the exposure of diseased distal intestine to pro-inflammatory stool. During perioperative planning, it is crucial for the gastroenterologist to partner early with a multidisciplinary team including surgeons, nutritionists, wound ostomy care nurses, psychologists, and social workers. Patients should be assessed for malnutrition and should be optimized nutritionally with enteral or parenteral nutrition. As they are associated with increased risk of postoperative complications, corticosteroids should be significantly reduced or completely discontinued preoperatively. Though ileal diversion may reduce the complications associated with anastomosis, serious postoperative complications can include diversion colitis and high-output fistulae. This review aims to provide an overview of the role of ileal diversion in the treatment of pediatric IBD to pediatric gastroenterologists to inform their medical decision-making and discussions with patients and families.

小儿炎症性肠病(IBD)的药物难治性疾病或严重疾病的并发症通常需要手术干预。具体来说,通过回肠转流术进行手术干预,可以将粪便从病变的远端肠道转移到回肠造口。这种方法适用于药物难治性结肠炎、严重肛周疾病或不可逆转的肠道损伤患者。对于溃疡性结肠炎患者,主要是在恢复性直肠结肠切除术中进行回肠袋肛门吻合术,以保护高风险吻合口。在克罗恩病的情况下,回肠转流可减少病变远端肠道暴露于促炎粪便的机会。在制定围手术期计划时,胃肠病学家必须尽早与包括外科医生、营养学家、伤口造口护理护士、心理学家和社会工作者在内的多学科团队合作。应评估患者是否存在营养不良,并通过肠内或肠外营养来优化营养。由于皮质类固醇会增加术后并发症的风险,因此术前应大幅减少或完全停用皮质类固醇。虽然回肠转流可减少吻合术的相关并发症,但严重的术后并发症可能包括转流性结肠炎和高输出性瘘管。本综述旨在向小儿胃肠病学家概述回肠转流术在治疗小儿 IBD 中的作用,为他们的医疗决策以及与患者和家属的讨论提供参考。
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引用次数: 0
Posttransplant inflammatory bowel disease after successful solid organ transplantation: Not out of the woods yet. 实体器官移植成功后的移植后炎症性肠病:尚未走出困境
IF 2.4 3区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-10-01 Epub Date: 2024-08-09 DOI: 10.1002/jpn3.12347
Amanda A Wenzel, Samantha Saul, Teresa Kodiak, Bridget Whitehead, Jennifer Strople, Jeffrey B Brown, Valeria Cohran

Objectives: Gastrointestinal symptoms can occur following pediatric solid organ transplantation (SOT), and a subset of children will develop chronic inflammatory bowel disease (IBD) posttransplant. The goal of this study was to characterize patients who developed IBD following SOT, their treatment modalities, and clinical course.

Methods: A retrospective review was performed of electronic medical records of patients 0-18 years of age who underwent heart, kidney, liver, or intestinal transplantation at our center from January 2009 to April 2019. Patients who developed IBD were included in the final analysis. Demographics, symptoms, and clinical information were recorded. Endoscopic and histologic data and initial and current medications were noted for each patient. Outcomes of interest included phenotype at the time of IBD diagnosis, surgical interventions for IBD, and clinical trajectory at last median follow-up.

Results: Eight patients with IBD after heart (n = 3, 37.5%), kidney (n = 2, 25.0%), liver (n = 1, 12.5%), intestinal (n = 1, 12.5%), or multivisceral (heart and kidney, n = 1, 12.5%) transplants were included. Before IBD diagnosis, most patients developed diarrhea (n = 5, 62.5%) and abdominal pain (n = 5, 62.5%). Abnormal endoscopic findings were most common in the colon. Patients were started on medications including 5-aminosalicylates, steroids, and azathioprine. Two patients required biologic therapy and were receiving vedolizumab at last follow-up. Some patients required adjustment of immune suppression.

Conclusions: Posttransplant IBD can occur following SOT. Patients exhibit inflammatory, nonstricturing disease though one patient experienced fistulizing disease. Complications are uncommon and many patients enter remission with 5-aminosalicylates alone, though some require adjustment in primary immune suppression.

目的:小儿实体器官移植(SOT)后可能会出现胃肠道症状,一部分患儿会在移植后患上慢性炎症性肠病(IBD)。本研究的目的是了解SOT术后出现IBD的患者的特征、治疗方式和临床过程:方法:我们对本中心 2009 年 1 月至 2019 年 4 月期间接受心脏、肾脏、肝脏或肠道移植的 0-18 岁患者的电子病历进行了回顾性审查。最终分析纳入了出现肠道疾病的患者。记录了人口统计学、症状和临床信息。记录了每位患者的内镜和组织学数据以及最初和目前的用药情况。相关结果包括IBD诊断时的表型、IBD手术干预以及最后一次中位随访时的临床轨迹:结果:共纳入了 8 名心脏(3 人,37.5%)、肾脏(2 人,25.0%)、肝脏(1 人,12.5%)、肠道(1 人,12.5%)或多脏器(心脏和肾脏,1 人,12.5%)移植后的 IBD 患者。在确诊 IBD 之前,大多数患者会出现腹泻(5 例,62.5%)和腹痛(5 例,62.5%)。异常的内镜检查结果最常见于结肠。患者开始接受药物治疗,包括 5-氨基水杨酸盐、类固醇和硫唑嘌呤。两名患者需要接受生物治疗,最后一次随访时正在接受维多珠单抗治疗。一些患者需要调整免疫抑制:结论:SOT 可导致移植后 IBD。患者表现为炎症性、非溃疡性疾病,但有一名患者出现了瘘管病。并发症并不常见,许多患者只需服用 5-aminosalicylates 即可缓解病情,但有些患者需要调整原发性免疫抑制。
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引用次数: 0
Clinical impacts of immunomodulator withdrawal from anti-tumor necrosis factor combination therapy in pediatric inflammatory bowel disease. 在小儿炎症性肠病的抗肿瘤坏死因子联合疗法中停用免疫调节剂的临床影响。
IF 2.4 3区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-10-01 Epub Date: 2024-07-01 DOI: 10.1002/jpn3.12299
Nicholas A Iovino, Madeline G McClinchie, Mahmoud Abdel-Rasoul, Brendan Boyle, Jennifer L Dotson, Hilary K Michel, Ross M Maltz

Objectives: Combination therapy consists of both anti-tumor necrosis factor (anti-TNF) and an immunomodulator (IMM) and has been shown to improve outcomes in patients with inflammatory bowel disease (IBD). This study assesses the impacts of IMM withdrawal from combination therapy to anti-TNF monotherapy in children with IBD.

Methods: This single-center retrospective cohort study included children with IBD initiated on combination therapy between 2014 and 2019 who discontinued the IMM. We evaluated whether IMM withdrawal impacts laboratory values and disease activity. Linear mixed effects models with random intercepts were used to compare differences between groups. Chi-square and Kruskal-Wallis tests were used for comparisons between patients who did and did not require subsequent escalation of therapy.

Results: One hundred and fifty-two patients discontinued the IMM which did not significantly affect disease activity. However, 18% of patients escalated therapy after IMM withdrawal, primarily due to low anti-TNF levels. Lower anti-TNF and higher erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels before IMM withdrawal were associated with subsequent escalation of therapy. Overall, there was no statistically significant effect on anti-TNF drug levels. Patients with Crohn's disease (CD) on infliximab (IFX) and methotrexate (MTX) who discontinued the IMM had an increase in mean ESR and CRP (p < 0.05).

Conclusions: IMM withdrawal from anti-TNF combination therapy may be considered safe in the setting of higher anti-TNF levels and normal serum inflammatory markers. Clinicians should consider assessing anti-TNF levels and inflammatory markers after IMM withdrawal, especially in patients with CD receiving IFX who discontinued MTX.

目的:联合疗法由抗肿瘤坏死因子(anti-TNF)和免疫调节剂(IMM)组成,已被证明可改善炎症性肠病(IBD)患者的预后。本研究评估了IBD儿童患者从联合疗法转为抗肿瘤坏死因子单药疗法后IMM停药的影响:这项单中心回顾性队列研究纳入了2014年至2019年期间开始接受联合疗法并停用IMM的IBD患儿。我们评估了IMM停药是否会影响实验室值和疾病活动。采用随机截距的线性混合效应模型来比较组间差异。对需要和不需要后续升级治疗的患者之间的比较采用了Chi-square和Kruskal-Wallis检验:结果:152 名患者停用了 IMM,但这对疾病活动性并无明显影响。然而,18% 的患者在停用 IMM 后升级了治疗,主要原因是抗肿瘤坏死因子水平较低。停用 IMM 前抗 TNF 水平较低、红细胞沉降率 (ESR) 和 C 反应蛋白 (CRP) 水平较高与随后的治疗升级有关。总体而言,抗肿瘤坏死因子药物水平没有统计学意义上的显著影响。服用英夫利昔单抗(IFX)和甲氨蝶呤(MTX)的克罗恩病(CD)患者停用 IMM 后,平均血沉和 CRP 均有所升高(p 结论:停用 IMM 后,患者的血沉和 CRP 均有所升高(p 结论:停用 IMM 后,患者的血沉和 CRP 均有所升高):在抗肿瘤坏死因子水平较高、血清炎症指标正常的情况下,抗肿瘤坏死因子联合疗法中停用 IMM 是安全的。临床医生应考虑在停用 IMM 后评估抗肿瘤坏死因子水平和炎症指标,尤其是接受 IFX 且停用 MTX 的 CD 患者。
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引用次数: 0
Quality of life from childhood to adulthood: Perspectives from adult patients with pediatric-onset intestinal failure. 从童年到成年的生活质量:儿科肠功能衰竭成年患者的观点。
IF 2.4 3区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-10-01 Epub Date: 2024-07-16 DOI: 10.1002/jpn3.12322
Marie L Neumann, Swapna Kakani, Amy Ladner

Adult patients with pediatric onset short bowel syndrome (SBS) or intestinal failure (IF) have been described as a distinct population warranting further research. The aim of this exploratory study aimed was to offer initial insights into this population's navigation of childhood, adolescence, and transition into adulthood. Both quantitative and qualitative data were collected from a convenience sample of adults with pediatric-onset SBS/IF using a disease-specific pilot survey; 14 questionnaires were completed. Responses indicated childhood and adulthood were complex and marked by joys and trials, while adolescence was experienced by many as a particularly challenging time. As adults, numerous patients experienced barriers to accessing the medical care they desired and described difficulties finding experienced and knowledgeable providers who listened and offered individualized care. This study highlights the importance of further studying this unique patient population, suggesting it can offer critical insights to inform the development of interventions and transition programs.

患有儿科发病的短肠综合征(SBS)或肠功能衰竭(IF)的成年患者被描述为一个需要进一步研究的独特人群。这项探索性研究的目的是初步了解这一人群在童年、青春期和成年过渡期的生活情况。研究人员通过一项针对特定疾病的试点调查,从方便抽样的小儿SBS/IF成人患者中收集了定量和定性数据;共完成了14份问卷。调查结果表明,童年和成年期是一个复杂的时期,充满了欢乐和考验,而许多人认为青春期是一个特别具有挑战性的时期。成年后,许多患者在获得他们想要的医疗服务时遇到了障碍,并描述了在寻找经验丰富、知识渊博、愿意倾听并提供个性化医疗服务的医疗服务提供者时遇到的困难。这项研究强调了进一步研究这一独特患者群体的重要性,并指出它可以为制定干预措施和过渡计划提供重要的启示。
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引用次数: 0
Performance of pan-enteroscopy in children with intestinal failure due to short bowel syndrome: A single-center retrospective study. 对因短肠综合征导致肠功能衰竭的儿童进行泛肠镜检查:单中心回顾性研究。
IF 2.4 3区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-10-01 Epub Date: 2024-07-20 DOI: 10.1002/jpn3.12316
Jonathan A Salazar, Christina Chan, Enju Liu, Fatima Hamroud, Amit S Grover, Victor L Fox, Peter D Ngo, Lissette Jimenez, Christopher P Duggan, Alexandra N Carey

Objectives: In pediatric patients with intestinal failure (IF) due to short bowel syndrome (SBS), we hypothesized that young children, those with shorter residual small bowel and those with congenital malrotation of the bowel would be more likely to undergo pan-enteroscopy. We aimed to determine the feasibility and diagnostic yield of pan-enteroscopy in this cohort.

Methods: We performed a single-center, retrospective study of pediatric patients with IF due to SBS who had undergone at least one GI endoscopic evaluation between January 1, 2018 and January 1, 2023.

Results: A pan-enteroscopy might have been possible in 381 of the 431 procedures (206 patients) reviewed. Forty-four (21%) patients underwent 54 pan-enteroscopies. Children with a residual bowel length <35 cm had higher odds of undergoing pan-enteroscopy (odds ratio [OR] 3.72, 95% confidence interval [CI] [1.32, 10.48], p = 0.01), as did patients with periprocedural glucagon-like peptide 2 (GLP-2) analog use (OR 4.30, 95% CI [1.24, 14.95], p = 0.02). Patients with diagnoses other than necrotizing enterocolitis (NEC) tended to be more likely to achieve a pan-enteroscopy (OR 2.73, 95% CI [0.95,7.88], p = 0.06). Evidence of gross and histopathologic abnormalities were found in 77.8% and 78% of the procedures, respectively. No complications were identified.

Conclusion: In a large cohort of children with SBS, pan-enteroscopy was successfully performed in 14.2% of the procedures and microscopic abnormalities were common. Shorter residual bowel length, underlying diagnoses of non-NEC, and GLP-2 analog use were generally associated with successful pan-enteroscopy, independent of age and several other factors. These data suggest that pan-enteroscopy is feasible and of high-yield in a subset of patients with SBS.

目的:对于因短肠综合征导致肠功能衰竭(IF)的儿科患者,我们假设年幼儿童、残余小肠较短的儿童和先天性肠旋转不良的儿童更有可能接受泛肠镜检查。我们的目的是确定在这一人群中进行泛肠镜检查的可行性和诊断率:我们对2018年1月1日至2023年1月1日期间至少接受过一次消化道内镜评估的因短肠综合征(SBS)导致IF的儿科患者进行了单中心回顾性研究:在审查的 431 例手术(206 名患者)中,有 381 例可能接受了泛内镜检查。44名(21%)患者接受了54次泛肠镜检查。有残余肠管长度的儿童 结论:在一大批 SBS 患儿中,14.2% 的手术成功进行了泛肠镜检查,显微镜下异常情况很常见。较短的残肠长度、非 NEC 潜在诊断和 GLP-2 类似物的使用通常与泛肠镜检查的成功率有关,与年龄和其他一些因素无关。这些数据表明,在部分 SBS 患者中,泛肠镜检查是可行的,而且收益很高。
{"title":"Performance of pan-enteroscopy in children with intestinal failure due to short bowel syndrome: A single-center retrospective study.","authors":"Jonathan A Salazar, Christina Chan, Enju Liu, Fatima Hamroud, Amit S Grover, Victor L Fox, Peter D Ngo, Lissette Jimenez, Christopher P Duggan, Alexandra N Carey","doi":"10.1002/jpn3.12316","DOIUrl":"10.1002/jpn3.12316","url":null,"abstract":"<p><strong>Objectives: </strong>In pediatric patients with intestinal failure (IF) due to short bowel syndrome (SBS), we hypothesized that young children, those with shorter residual small bowel and those with congenital malrotation of the bowel would be more likely to undergo pan-enteroscopy. We aimed to determine the feasibility and diagnostic yield of pan-enteroscopy in this cohort.</p><p><strong>Methods: </strong>We performed a single-center, retrospective study of pediatric patients with IF due to SBS who had undergone at least one GI endoscopic evaluation between January 1, 2018 and January 1, 2023.</p><p><strong>Results: </strong>A pan-enteroscopy might have been possible in 381 of the 431 procedures (206 patients) reviewed. Forty-four (21%) patients underwent 54 pan-enteroscopies. Children with a residual bowel length <35 cm had higher odds of undergoing pan-enteroscopy (odds ratio [OR] 3.72, 95% confidence interval [CI] [1.32, 10.48], p = 0.01), as did patients with periprocedural glucagon-like peptide 2 (GLP-2) analog use (OR 4.30, 95% CI [1.24, 14.95], p = 0.02). Patients with diagnoses other than necrotizing enterocolitis (NEC) tended to be more likely to achieve a pan-enteroscopy (OR 2.73, 95% CI [0.95,7.88], p = 0.06). Evidence of gross and histopathologic abnormalities were found in 77.8% and 78% of the procedures, respectively. No complications were identified.</p><p><strong>Conclusion: </strong>In a large cohort of children with SBS, pan-enteroscopy was successfully performed in 14.2% of the procedures and microscopic abnormalities were common. Shorter residual bowel length, underlying diagnoses of non-NEC, and GLP-2 analog use were generally associated with successful pan-enteroscopy, independent of age and several other factors. These data suggest that pan-enteroscopy is feasible and of high-yield in a subset of patients with SBS.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"915-923"},"PeriodicalIF":2.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141731294","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
Relationships of race and area deprivation indices to outcomes in pediatric patients with inflammatory bowel disease. 种族和地区贫困指数与儿科炎症性肠病患者预后的关系。
IF 2.4 3区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-10-01 Epub Date: 2024-07-24 DOI: 10.1002/jpn3.12329
Faria Hasan, Vivian Kim, Ellen J Silver, Gitit Tomer

Objectives: Social determinants of health (SDOH) are nonmedical factors that influence health outcomes. Children with chronic illnesses living with socioeconomic risk factors have higher rates of unfavorable health outcomes. Our study aimed to assess the association between area deprivation indices (ADI), as a proxy for SDOH, and outcomes in pediatric patients with inflammatory bowel disease (IBD).

Methods: A retrospective chart review was conducted on 134 pediatric patients with IBD, ages 0-21 years. Explanatory variables were the patient's ADI and demographics. Outcomes were assessed from time of diagnosis over a 1-year follow-up period. The primary outcome was the ratio of missed to completed appointments; secondary outcomes were the numbers of IBD-related emergency department (ED) visits and IBD-related hospitalizations.

Results: Race/ethnicity was significantly associated with ADI (p < 0.001). In a multivariable regression model, no variables were associated with ratio of missed to completed appointments. Notably, ADI was not significantly associated with the ratio of missed to completed appointments. In a Poisson regression, Black (non-Hispanic) and Hispanic race/ethnicity, Medicaid insurance, female gender, and lower age were significantly associated with more IBD-related ED visits; ADI was not. In a similar model, Black (non-Hispanic) race, Medicaid insurance status, and lower age were significantly associated with more IBD-related hospitalizations; ADI was not.

Conclusions: In our cohort, ADI was not significantly associated with the ratio of missed to completed appointments, IBD-related ED visits, and IBD-related hospitalizations; however, race/ethnicity, age at diagnosis, insurance, and gender were associated with these outcomes.

目的:健康的社会决定因素(SDOH)是影响健康结果的非医疗因素。具有社会经济风险因素的慢性病患儿的不良健康后果发生率较高。我们的研究旨在评估代表 SDOH 的地区贫困指数(ADI)与儿科炎症性肠病(IBD)患者的预后之间的关系:我们对 134 名 0-21 岁的儿科 IBD 患者进行了回顾性病历审查。解释变量为患者的 ADI 和人口统计学特征。结果评估从诊断开始,随访 1 年。主要结果是错过预约与完成预约的比率;次要结果是与 IBD 相关的急诊就诊人数和与 IBD 相关的住院人数:结果:种族/人种与 ADI 显著相关(P 结论:ADI 与种族/人种无关:在我们的队列中,ADI 与失约与完成预约的比率、IBD 相关的急诊就诊人数以及 IBD 相关的住院人数无明显相关性;但是,种族/民族、诊断时的年龄、保险和性别与这些结果相关。
{"title":"Relationships of race and area deprivation indices to outcomes in pediatric patients with inflammatory bowel disease.","authors":"Faria Hasan, Vivian Kim, Ellen J Silver, Gitit Tomer","doi":"10.1002/jpn3.12329","DOIUrl":"10.1002/jpn3.12329","url":null,"abstract":"<p><strong>Objectives: </strong>Social determinants of health (SDOH) are nonmedical factors that influence health outcomes. Children with chronic illnesses living with socioeconomic risk factors have higher rates of unfavorable health outcomes. Our study aimed to assess the association between area deprivation indices (ADI), as a proxy for SDOH, and outcomes in pediatric patients with inflammatory bowel disease (IBD).</p><p><strong>Methods: </strong>A retrospective chart review was conducted on 134 pediatric patients with IBD, ages 0-21 years. Explanatory variables were the patient's ADI and demographics. Outcomes were assessed from time of diagnosis over a 1-year follow-up period. The primary outcome was the ratio of missed to completed appointments; secondary outcomes were the numbers of IBD-related emergency department (ED) visits and IBD-related hospitalizations.</p><p><strong>Results: </strong>Race/ethnicity was significantly associated with ADI (p < 0.001). In a multivariable regression model, no variables were associated with ratio of missed to completed appointments. Notably, ADI was not significantly associated with the ratio of missed to completed appointments. In a Poisson regression, Black (non-Hispanic) and Hispanic race/ethnicity, Medicaid insurance, female gender, and lower age were significantly associated with more IBD-related ED visits; ADI was not. In a similar model, Black (non-Hispanic) race, Medicaid insurance status, and lower age were significantly associated with more IBD-related hospitalizations; ADI was not.</p><p><strong>Conclusions: </strong>In our cohort, ADI was not significantly associated with the ratio of missed to completed appointments, IBD-related ED visits, and IBD-related hospitalizations; however, race/ethnicity, age at diagnosis, insurance, and gender were associated with these outcomes.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"877-884"},"PeriodicalIF":2.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141752023","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
Portal vein hypoplasia is present in patients with biliary atresia at the time of diagnosis. 胆道闭锁患者在诊断时会出现门静脉发育不良。
IF 2.4 3区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-10-01 Epub Date: 2024-08-11 DOI: 10.1002/jpn3.12349
Aitaro Takimoto, Nicholas Wolfe, Liu Jiahui, Daiki Kato, Akihiro Yasui, Hiroo Uchida, Akihiro Asai

Background: In patients with biliary atresia (BA), severe portal hypertension (HTN) develops even with successful bile flow restoration, suggesting an intrinsic factor driving portal HTN independent from bile obstruction. We hypothesize that patients with BA have abnormal portal vein (PV) development, leading to PV hypoplasia.

Methods: In this observational cohort study, we enrolled patients who were referred to a tertiary center from 2017 to 2021 to rule out BA. Newborns who underwent computed tomography angiogram as a clinical routine before intraoperative cholangiogram, and laparoscopic Kasai hepatoportoenterostomy. The diameter of the PV and hepatic artery (HA) were compared to the degree of liver fibrosis in the wedge biopsies. The jaundice clearance, native liver survival, and clinical portal hypertensive events, including ascites development and intestinal bleeding, were assessed.

Results: 47 newborns with cholestasis were included in the cohort; 35 were diagnosed with BA. The patients with BA had a smaller median PV diameter (4.3 vs. 5.1 mm; p < 0.001) and larger median HA diameter (1.4 vs. 1.2 mm; p < 0.05) compared to the patients with other forms of cholestasis. The median PV and HA diameter did not correlate with the degree of liver fibrosis. Among 35 patients with BA, 29 patients (82.9%) achieved jaundice clearance, and 23 patients (65.7%) were alive with their native liver at two years of age. Seven patients (20%) developed intestinal bleeding, and seven patients (20%) developed ascites, with one overlapping patient.

Conclusion: PV hypoplasia is present in patients with BA independent of liver fibrosis at the time of diagnosis.

背景:在胆道闭锁(BA)患者中,即使成功恢复了胆汁流,也会出现严重的门静脉高压(HTN),这表明门静脉高压的内在驱动因素独立于胆汁阻塞。我们假设 BA 患者的门静脉(PV)发育异常,导致门静脉发育不良:在这项观察性队列研究中,我们招募了 2017 年至 2021 年期间转诊至一家三级中心以排除 BA 的患者。新生儿在术中胆管造影前作为临床常规接受计算机断层扫描(CT)血管造影,并在腹腔镜下接受 Kasai 肝门肠管造口术。将 PV 和肝动脉(HA)的直径与楔形活组织切片的肝纤维化程度进行比较。对黄疸清除率、原肝存活率和临床门静脉高压事件(包括腹水形成和肠道出血)进行了评估:结果:47 名新生儿患有胆汁淤积症,其中 35 名被诊断为 BA。胆汁淤积症患者的中位 PV 直径较小(4.3 毫米对 5.1 毫米;P 结论:胆汁淤积症患者的中位 PV 直径较小:胆汁淤积症患者的肺泡发育不良与诊断时的肝纤维化无关。
{"title":"Portal vein hypoplasia is present in patients with biliary atresia at the time of diagnosis.","authors":"Aitaro Takimoto, Nicholas Wolfe, Liu Jiahui, Daiki Kato, Akihiro Yasui, Hiroo Uchida, Akihiro Asai","doi":"10.1002/jpn3.12349","DOIUrl":"10.1002/jpn3.12349","url":null,"abstract":"<p><strong>Background: </strong>In patients with biliary atresia (BA), severe portal hypertension (HTN) develops even with successful bile flow restoration, suggesting an intrinsic factor driving portal HTN independent from bile obstruction. We hypothesize that patients with BA have abnormal portal vein (PV) development, leading to PV hypoplasia.</p><p><strong>Methods: </strong>In this observational cohort study, we enrolled patients who were referred to a tertiary center from 2017 to 2021 to rule out BA. Newborns who underwent computed tomography angiogram as a clinical routine before intraoperative cholangiogram, and laparoscopic Kasai hepatoportoenterostomy. The diameter of the PV and hepatic artery (HA) were compared to the degree of liver fibrosis in the wedge biopsies. The jaundice clearance, native liver survival, and clinical portal hypertensive events, including ascites development and intestinal bleeding, were assessed.</p><p><strong>Results: </strong>47 newborns with cholestasis were included in the cohort; 35 were diagnosed with BA. The patients with BA had a smaller median PV diameter (4.3 vs. 5.1 mm; p < 0.001) and larger median HA diameter (1.4 vs. 1.2 mm; p < 0.05) compared to the patients with other forms of cholestasis. The median PV and HA diameter did not correlate with the degree of liver fibrosis. Among 35 patients with BA, 29 patients (82.9%) achieved jaundice clearance, and 23 patients (65.7%) were alive with their native liver at two years of age. Seven patients (20%) developed intestinal bleeding, and seven patients (20%) developed ascites, with one overlapping patient.</p><p><strong>Conclusion: </strong>PV hypoplasia is present in patients with BA independent of liver fibrosis at the time of diagnosis.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"818-825"},"PeriodicalIF":2.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141916916","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
Harmonization is key for transition of care. 统一是护理过渡的关键。
IF 2.4 3区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-10-01 Epub Date: 2024-08-20 DOI: 10.1002/jpn3.12354
Miguel Saps, Samantha Arrizabalo, Carlos A Velasco-Benitez
{"title":"Harmonization is key for transition of care.","authors":"Miguel Saps, Samantha Arrizabalo, Carlos A Velasco-Benitez","doi":"10.1002/jpn3.12354","DOIUrl":"10.1002/jpn3.12354","url":null,"abstract":"","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"926"},"PeriodicalIF":2.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142004406","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
Infant growth and tolerance with a formula based on novel native demineralized whey: A randomized double-blind pilot study. 基于新型原生去矿物质乳清的婴儿生长和耐受性配方:随机双盲试验研究
IF 2.4 3区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-10-01 Epub Date: 2024-07-11 DOI: 10.1002/jpn3.12305
Anthony Pinon, Claire Trentesaux, Camille Chaffaut, Marion Lemaire, Xavier Parere, Jean-Michel Lecerf, Coralie Schnebelen-Berthier

Objectives: The aim of the study was to evaluate the effects on infant growth and tolerance of a Test infant formula based on a novel whey extraction and demineralization process, compared to a Standard formula and a breastfed reference arm.

Methods: Healthy term infants (n = 61) aged up to 21 days were randomized to Test or Control formula. A breastfed group (n = 39) served as a reference. Growth, tolerance, adverse events, and sleep were evaluated every month until 6 months of age. Plasma amino-acid concentrations at 3 months of age were measured in a subgroup population.

Results: Growth curves of all infants globally agreed with World Health Organization standards across the 6-month period study. Regarding tolerance, no difference between the formula-fed groups was observed on daily number of crying episodes, intensity or time to onset of regurgitations, and stool frequency or consistency, except at 5 months with infants in the Control group having more watery stools. Plasma concentration of some amino acids differed between the groups, especially tryptophan concentration which was higher in infants fed with the Test formula. In parallel, total sleep duration was longer in these infants at 2, 3, and 5 months of age, corresponding to an increase in daytime sleep.

Conclusions: Test formula supported an adequate infant growth from birth to 6 months of age and was well-tolerated by all infants. An increase in total sleep at several months was also observed with the Test formula.

研究目的研究旨在评估基于新型乳清提取和去矿物质工艺的试验婴儿配方奶粉与标准配方奶粉和母乳喂养参照组相比,对婴儿生长和耐受性的影响。母乳喂养组(n = 39)作为参照组。每月对婴儿的生长、耐受性、不良反应和睡眠情况进行评估,直至婴儿满 6 个月。在一个亚组人群中测量了 3 个月大时的血浆氨基酸浓度:结果:在 6 个月的研究期间,所有婴儿的生长曲线均符合世界卫生组织的标准。在耐受性方面,配方奶喂养组之间在每天哭闹次数、反胃强度或开始反胃的时间、大便次数或稠度方面没有发现差异,但在 5 个月时,对照组婴儿的水样便较多。两组婴儿血浆中某些氨基酸的浓度不同,尤其是色氨酸的浓度,试验配方奶粉组婴儿的色氨酸浓度更高。与此同时,这些婴儿在 2、3 和 5 个月大时的总睡眠时间更长,这与白天睡眠时间增加有关:结论:测试配方奶粉有助于婴儿从出生到 6 个月期间的充分成长,并且所有婴儿都能很好地接受。测试配方奶粉还能增加几个月大婴儿的总睡眠时间。
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引用次数: 0
Editing combined multichannel intraluminal impedance and pH monitoring tracings. 编辑合并的多通道腔内阻抗和 pH 值监测记录。
IF 2.4 3区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-10-01 Epub Date: 2024-05-27 DOI: 10.1002/jpn3.12255
Frederick W Woodley, Carlo Di Lorenzo, Raul E Sanchez
{"title":"Editing combined multichannel intraluminal impedance and pH monitoring tracings.","authors":"Frederick W Woodley, Carlo Di Lorenzo, Raul E Sanchez","doi":"10.1002/jpn3.12255","DOIUrl":"10.1002/jpn3.12255","url":null,"abstract":"","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"797-799"},"PeriodicalIF":2.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141155610","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
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Journal of Pediatric Gastroenterology and Nutrition
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