Journal of Pediatric Gastroenterology and Nutrition最新文献

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Lymphocytic esophagitis causing esophageal stenosis in children: Part of a disease spectrum or a new disease? 淋巴细胞性食管炎导致儿童食管狭窄:疾病谱的一部分还是一种新疾病?
IF 2.4 3区 医学
Journal of Pediatric Gastroenterology and Nutrition Pub Date : 2024-10-01 Epub Date: 2024-08-20 DOI: 10.1002/jpn3.12353
Joana Filipe Ribeiro, Margarida Vicente-Ferreira, Joana Lage, Isabel Pinto Pais, Céu Espinheira, Joanne Lopes, Fátima Carneiro, Eunice Trindade
{"title":"Lymphocytic esophagitis causing esophageal stenosis in children: Part of a disease spectrum or a new disease?","authors":"Joana Filipe Ribeiro, Margarida Vicente-Ferreira, Joana Lage, Isabel Pinto Pais, Céu Espinheira, Joanne Lopes, Fátima Carneiro, Eunice Trindade","doi":"10.1002/jpn3.12353","DOIUrl":"10.1002/jpn3.12353","url":null,"abstract":"","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142004407","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
Clinical presentation and factors associated with gluten exposure in children with celiac disease. 乳糜泻患儿的临床表现以及与麸质暴露相关的因素。
IF 2.4 3区 医学
Journal of Pediatric Gastroenterology and Nutrition Pub Date : 2024-10-01 Epub Date: 2024-08-16 DOI: 10.1002/jpn3.12321
Andrew Krueger, Lisa Fahey, Qin Sun, Stephanie Regis, Nasim Khavari, Hilary Jericho, Vahe Badalyan, Imad Absah, Ritu Verma, Maureen M Leonard, Vanessa Weisbrod, Temara Hajjat, Dale Lee, Mary Shull, Jocelyn A Silvester, Daniel Mallon
{"title":"Clinical presentation and factors associated with gluten exposure in children with celiac disease.","authors":"Andrew Krueger, Lisa Fahey, Qin Sun, Stephanie Regis, Nasim Khavari, Hilary Jericho, Vahe Badalyan, Imad Absah, Ritu Verma, Maureen M Leonard, Vanessa Weisbrod, Temara Hajjat, Dale Lee, Mary Shull, Jocelyn A Silvester, Daniel Mallon","doi":"10.1002/jpn3.12321","DOIUrl":"10.1002/jpn3.12321","url":null,"abstract":"<p><strong>Objectives: </strong>The prevalence of celiac disease (CeD) is increasing, yet it is still underdiagnosed, in part because of its heterogeneous presentation. Diagnostic criteria are evolving and management with strict adherence to a gluten-free diet is challenging for many. We aimed to characterize the clinical presentation of CeD among a large multicenter cohort of pediatric patients and to identify factors associated with gluten-free diet adherence.</p><p><strong>Methods: </strong>Patients with CeD aged 0-18 years were recruited from 11 United States health centers. Parents completed surveys about gluten-free diet adherence and patient electronic health records were reviewed. Logistic regression analyses were performed to identify risk factors associated with gluten exposure.</p><p><strong>Results: </strong>Charts were reviewed for 460 children with a median age of 6.4 years. Abdominal pain was reported in 57% of the cohort, but diverse symptoms were identified. Parent surveys were completed for 455 participants. Sixty-five (14%) participants were at high risk for gluten exposure based on parental reports of weekly or daily gluten exposure or eating gluten by choice in the past year. Participants under the age of 5 years had a lower risk of gluten exposure, while participants without repeat serology testing 18 months after initial diagnosis were at higher risk of gluten exposure.</p><p><strong>Conclusions: </strong>In a large, multicenter cohort of pediatric CeD patients, clinical presentation is highly variable, necessitating a high index of suspicion to make a diagnosis. Parent surveys indicate that 14% of patients are at high risk of gluten exposure, with patient age and lack of close follow-up associated with gluten-free diet adherence.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141988193","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
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
Journal of Pediatric Gastroenterology and Nutrition 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
{"title":"Role of ileal diversion in pediatric inflammatory bowel disease.","authors":"Amanda A Simard, Swetha Kotamraju, Jennifer R DeFazio, Joseph A Picoraro","doi":"10.1002/jpn3.12331","DOIUrl":"10.1002/jpn3.12331","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141792744","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
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
Journal of Pediatric Gastroenterology and Nutrition 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
{"title":"Posttransplant inflammatory bowel disease after successful solid organ transplantation: Not out of the woods yet.","authors":"Amanda A Wenzel, Samantha Saul, Teresa Kodiak, Bridget Whitehead, Jennifer Strople, Jeffrey B Brown, Valeria Cohran","doi":"10.1002/jpn3.12347","DOIUrl":"10.1002/jpn3.12347","url":null,"abstract":"<p><strong>Objectives: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141906905","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
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
Journal of Pediatric Gastroenterology and Nutrition 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
{"title":"Clinical impacts of immunomodulator withdrawal from anti-tumor necrosis factor combination therapy in pediatric inflammatory bowel disease.","authors":"Nicholas A Iovino, Madeline G McClinchie, Mahmoud Abdel-Rasoul, Brendan Boyle, Jennifer L Dotson, Hilary K Michel, Ross M Maltz","doi":"10.1002/jpn3.12299","DOIUrl":"10.1002/jpn3.12299","url":null,"abstract":"<p><strong>Objectives: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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).</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141468852","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
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
Journal of Pediatric Gastroenterology and Nutrition 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
{"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":null,"pages":null},"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
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
Journal of Pediatric Gastroenterology and Nutrition Pub Date : 2024-10-01 Epub Date: 2024-07-16 DOI: 10.1002/jpn3.12322
Marie L Neumann, Swapna Kakani, Amy Ladner
{"title":"Quality of life from childhood to adulthood: Perspectives from adult patients with pediatric-onset intestinal failure.","authors":"Marie L Neumann, Swapna Kakani, Amy Ladner","doi":"10.1002/jpn3.12322","DOIUrl":"10.1002/jpn3.12322","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141626979","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
Journal of Pediatric Gastroenterology and Nutrition Pub Date : 2024-10-01 Epub Date: 2024-07-24 DOI: 10.1002/jpn3.12329
Faria Hasan, Vivian Kim, Ellen J Silver, Gitit Tomer
{"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":null,"pages":null},"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
Harmonization is key for transition of care. 统一是护理过渡的关键。
IF 2.4 3区 医学
Journal of Pediatric Gastroenterology and Nutrition 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":null,"pages":null},"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
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
Journal of Pediatric Gastroenterology and Nutrition 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
{"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":null,"pages":null},"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
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