JPGN Reports最新文献_第2页

Don't forget to turn around: A case of hematochezia and tenesmus driven by anorectal inflammatory cloacogenic polyp 别忘了转身一例由肛门直肠炎性泄殖腔息肉引起的血便和肛门疼痛

JPGN Reports Pub Date : 2024-04-18 DOI: 10.1002/jpr3.12074

Maria Aguillera, Lacey Miller, Shagun Sharma, V. V. Lemus, Meredith Pittman, Thomas Wallach

引用次数: 0

Retrospective analysis of the standardized BARD criteria for acute cholangitis in biliary atresia patients 对胆道闭锁患者急性胆管炎标准化 BARD 标准的回顾性分析

JPGN Reports Pub Date : 2024-04-12 DOI: 10.1002/jpr3.12071

Omid Madadi-Sanjani, A. Calinescu, Nathalie Rock, V. McLin, Marie Uecker, J. Kuebler, Claus Petersen, Barbara E. Wildhaber

{"title":"Retrospective analysis of the standardized BARD criteria for acute cholangitis in biliary atresia patients","authors":"Omid Madadi-Sanjani, A. Calinescu, Nathalie Rock, V. McLin, Marie Uecker, J. Kuebler, Claus Petersen, Barbara E. Wildhaber","doi":"10.1002/jpr3.12071","DOIUrl":"https://doi.org/10.1002/jpr3.12071","url":null,"abstract":"In 2022, the Biliary Atresia and Related Diseases (BARD) community reached a consensus for the definition of suspected and confirmed cholangitis for biliary atresia (BA) patients after hepatoportoenterostomy (HPE). This study assessed the new standardized BARD definition in a retrospective, multicenter cohort study.We included BA cases managed between 2010 and 2020 at the Hannover Medical School and Geneva University Hospitals' Swiss Pediatric Liver Center. The standardized BARD cholangitis definition assesses four clinical items and four imaging/laboratory items to define cholangitis. The definition was retrospectively applied to all BA cases having presented, according to their physician, cholangitis within the first year after the HPE. The diagnosis defined by the standardized BARD definition was compared with the final clinical diagnosis made by physicians. The Spearman's correlation coefficient was used to test for correlation between diagnoses made by standardized and clinical appreciation.Of 185 consecutive BA patients, 59 (32%) had at least one episode of cholangitis within the first year after HPE. The correlation between the clinician's impression and the standardized BARD definition was very strong (r = 0.8). Confirmed cholangitis definition coincided with the clinician's impression (2.5 [±0.7]/4 clinical items, 2.6 [±0.5]/4 imaging/laboratory items). For suspected cholangitis, the threshold for diagnosis was lower within the standardized BARD definition (1.1 [±0.3]/4 clinical items, 2.2 [±0.8]/4 laboratory/imaging items).This first retrospective application of the standardized BARD cholangitis definition reveals a very strong correlation with the physician's assessment before standardization. A prospective study is needed to further refine the standardized definition for cholangitis in BA patients.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-04-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140710370","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Patient perspective on exercise practices, preferences, and barriers in pediatric nonalcoholic fatty liver disease: A multicenter survey 从患者角度看儿科非酒精性脂肪肝患者的运动习惯、偏好和障碍：多中心调查

JPGN Reports Pub Date : 2024-04-12 DOI: 10.1002/jpr3.12072

Elizabeth L. Yu, Hyeri You, Bryan Rudolph, Jennifer A. Panganiban, Taisa J. Kohut, Henry C. Lin, Amanda C Fifi, Rasmita Budhathoki, Megan W. Butler, Sabina R. Anderson, Nidhi P. Goyal, K. Newton, J. Schwimmer

{"title":"Patient perspective on exercise practices, preferences, and barriers in pediatric nonalcoholic fatty liver disease: A multicenter survey","authors":"Elizabeth L. Yu, Hyeri You, Bryan Rudolph, Jennifer A. Panganiban, Taisa J. Kohut, Henry C. Lin, Amanda C Fifi, Rasmita Budhathoki, Megan W. Butler, Sabina R. Anderson, Nidhi P. Goyal, K. Newton, J. Schwimmer","doi":"10.1002/jpr3.12072","DOIUrl":"https://doi.org/10.1002/jpr3.12072","url":null,"abstract":"Nonalcoholic fatty liver disease (NAFLD) is prevalent among children, and lifestyle modification is the primary treatment approach. However, the optimal exercise duration, frequency, and intensity for managing NAFLD remain undefined. This study aimed to gain insights from the patient perspective by examining exercise behaviors, preferences, and barriers in children with NAFLD.A multicenter survey was conducted among children 8–18 years with NAFLD in pediatric gastroenterology clinics. Participants completed a questionnaire on exercise practices, preferences, and barriers, while parents completed a questionnaire on their willingness and ability to support their child's exercise. Data were analyzed using χ2 test with Yates' correction and two‐sample t test.The study included 408 children with NAFLD, with a mean age of 13.8 years. Approximately 52.5% of participants had physical education classes at school, while 59.5% engaged in extracurricular exercise, averaging 3.7 days per week. However, 11.5% reported no physical activity. A significant majority (81.1%) expressed interest in increasing their exercise levels, primarily driven by health‐related factors. Time‐related constraints were the most cited barriers to exercise (53.7%). Approximately 80% of parents demonstrated willingness and ability to support their child's exercise regimen.This study provides insights into exercise behaviors, preferences, and barriers among children with NAFLD. Half of the children lacked exercise opportunities at school but expressed interest in increasing their physical activity. Time limitation was the major obstacle cited. Parents are motivated to support increased physical activity. Exercise intervention programs for NAFLD should consider the perspective of the children and their families.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-04-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140710366","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Pancreatic stones causing secondary biliary obstruction: An uncommon presentation of chronic pancreatitis 胰腺结石导致继发性胆道梗阻：慢性胰腺炎的罕见表现

JPGN Reports Pub Date : 2024-04-09 DOI: 10.1002/jpr3.12070

Wesley C. Judy, Tom K. Lin

引用次数: 0

Odevixibat as an adjunctive treatment for refractory pruritus in rare variants of cholestatic liver disease 奥德韦希巴特辅助治疗罕见变异型胆汁淤积性肝病的难治性瘙痒症

JPGN Reports Pub Date : 2024-04-04 DOI: 10.1002/jpr3.12069

Akshat Goel, Bethany Tucker, Lorena Soler Casale, T. Grammatikopoulos

{"title":"Odevixibat as an adjunctive treatment for refractory pruritus in rare variants of cholestatic liver disease","authors":"Akshat Goel, Bethany Tucker, Lorena Soler Casale, T. Grammatikopoulos","doi":"10.1002/jpr3.12069","DOIUrl":"https://doi.org/10.1002/jpr3.12069","url":null,"abstract":"Odevixibat, a reversible ileal bile acid transport (iBAT) inhibitor, has been shown to reduce serum bile acids (sBA) and pruritus mostly in children with progressive familial intrahepatic cholestasis (PFIC) 1 and 2 in clinical trials and case reports. There are currently no published case reports or series describing its use in rare variants of cholestatic liver disease.We describe three children with progressive cholestatic liver disease who developed refractory pruritus, who had a genotypic diagnosis of AKR1D1, ABCB4 variant, and PKHD1 and PKHD2 variants; all being variants of unknown significance as per the American College of Medical Genetics and Genomics guidelines.On Odevixibat there was a significant improvement in sBA (absolute change from baseline: −196 and −393 μmol/L) and pruritus in two children with heterozygous AKR1D1 and ABCB4 mutations. The child with ABCB4 variants was found to have features of sclerosing cholangitis along with a diagnosis of Crohn's disease, which represents the first reported usage of Odevixibat in such a case with good response. There was some reported improvement in the third child with PKHD1 and PKHD2 variants; however, we hypothesize that no sustained improvement could be due to severe and progressive nature of the disease. There were no side effects reported and it was well tolerated in all.We suggest that Odevixibat may be used as an adjunctive drug in refractory pruritus and could be started early in the course of disease if clinically and phenotypically indicated.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-04-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140744279","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Gastric outlet obstruction in an 11‐year‐old girl: A case report 一名 11 岁女孩的胃出口梗阻：病例报告

JPGN Reports Pub Date : 2024-04-01 DOI: 10.1002/jpr3.12062

Ana Sofia Figueiredo, Carolina Soares-Aquino, Rita Amorim, L. Melão, C. Espinheira, Isabel Pinto Pais, Miguel Campos, Eunice Trindade

引用次数: 0

Rescue therapy with upadacitinib in medically refractory pediatric ulcerative colitis 用达帕替尼对药物难治性小儿溃疡性结肠炎进行抢救性治疗

JPGN Reports Pub Date : 2024-04-01 DOI: 10.1002/jpr3.12067

Maria Miller, Ashish S. Patel, Brad Pasternak

引用次数: 0

Unique sequelae of portal vein thrombosis in a pediatric patient with cystic echinococcosis: A case report 一名患有囊性棘球蚴病的儿科患者因门静脉血栓形成而留下的独特后遗症：病例报告

JPGN Reports Pub Date : 2024-03-19 DOI: 10.1002/jpr3.12066

Steven Lin, Terry C. Dixon, Hamza Hassan Khan, Martha M. Munden, J. N. Anderson

{"title":"Unique sequelae of portal vein thrombosis in a pediatric patient with cystic echinococcosis: A case report","authors":"Steven Lin, Terry C. Dixon, Hamza Hassan Khan, Martha M. Munden, J. N. Anderson","doi":"10.1002/jpr3.12066","DOIUrl":"https://doi.org/10.1002/jpr3.12066","url":null,"abstract":"This case report presents a rare complication of hepatic cystic echinococcosis in a 12‐year‐old Latino male, residing in a nonendemic region, who developed long‐term sequelae of portal vein thrombosis accompanied by the emergence of a hyper‐vascular sigmoid colon mass. Portal vein involvement in hepatic cystic echinococcosis is exceedingly uncommon, with limited documented cases. The presentation of the patient included intermittent hematochezia, abdominal pain, and fatigue. Imaging revealed liver cysts and chronic portal vein thrombosis with cavernous transformation, resulting in portal hypertension. Notably, the patient also exhibited mesenteric venous thrombosis, further complicating the clinical picture. The diagnosis was confirmed through echinococcus serology testing. Treatment involved a six month course of Albendazole, puncture‐aspiration‐injection‐reaspiration procedure, splenectomy, and splenorenal shunt to alleviate portal hypertension. This case underscores the significance of considering portal hypertension secondary to hepatic cystic echinococcosis, even in nonendemic regions, particularly in pediatric patients with unique clinical presentations.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140229461","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Coronavirus HKU1 infection and development of pediatric acute liver failure with immune dysregulation phenotype 冠状病毒 HKU1 感染与小儿急性肝衰竭免疫调节失调表型的形成

JPGN Reports Pub Date : 2024-03-18 DOI: 10.1002/jpr3.12065

Arun Ajmera, Peter Joseph Pernicone, Nishant Patel, Stefany Hernandez Benabe

引用次数: 0

Successful endoscopic removal of high‐power magnetic balls embedded in the duodenal wall 通过内窥镜成功取出嵌入十二指肠壁的高能磁球

JPGN Reports Pub Date : 2024-03-18 DOI: 10.1002/jpr3.12060

Juliana M. Kennedy, Nikhil A. Kumta, Joanne Lai

引用次数: 0