Maria Aguillera, Lacey Miller, Shagun Sharma, V. V. Lemus, Meredith Pittman, Thomas Wallach
We report a case of a 13‐year‐old male who presented to the Pediatric Gastroenterology clinic with complaints of abdominal pain and frequent stooling, worsened by hematochezia. Despite undergoing endoscopic evaluation twice within a 1‐year period, the diagnosis of an Inflammatory Cloacogenic Polyp (ICP) was only revealed during the second evaluation, in which rectal retroflexion was performed. This case highlights the importance of maintaining the ICP at the anorectal transitional zone as part of the differential diagnosis when evaluating patients with symptoms of distal colitis.
{"title":"Don't forget to turn around: A case of hematochezia and tenesmus driven by anorectal inflammatory cloacogenic polyp","authors":"Maria Aguillera, Lacey Miller, Shagun Sharma, V. V. Lemus, Meredith Pittman, Thomas Wallach","doi":"10.1002/jpr3.12074","DOIUrl":"https://doi.org/10.1002/jpr3.12074","url":null,"abstract":"We report a case of a 13‐year‐old male who presented to the Pediatric Gastroenterology clinic with complaints of abdominal pain and frequent stooling, worsened by hematochezia. Despite undergoing endoscopic evaluation twice within a 1‐year period, the diagnosis of an Inflammatory Cloacogenic Polyp (ICP) was only revealed during the second evaluation, in which rectal retroflexion was performed. This case highlights the importance of maintaining the ICP at the anorectal transitional zone as part of the differential diagnosis when evaluating patients with symptoms of distal colitis.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":" 8","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140689010","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}
Omid Madadi-Sanjani, A. Calinescu, Nathalie Rock, V. McLin, Marie Uecker, J. Kuebler, Claus Petersen, Barbara E. Wildhaber
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.
{"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":"13 6","pages":""},"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}
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
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.
{"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":"13 10","pages":""},"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}
{"title":"Pancreatic stones causing secondary biliary obstruction: An uncommon presentation of chronic pancreatitis","authors":"Wesley C. Judy, Tom K. Lin","doi":"10.1002/jpr3.12070","DOIUrl":"https://doi.org/10.1002/jpr3.12070","url":null,"abstract":"","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"41 7‐8","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140726750","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}
Akshat Goel, Bethany Tucker, Lorena Soler Casale, T. Grammatikopoulos
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.
{"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":"2 1","pages":""},"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}
Ana Sofia Figueiredo, Carolina Soares-Aquino, Rita Amorim, L. Melão, C. Espinheira, Isabel Pinto Pais, Miguel Campos, Eunice Trindade
Pyloric stenosis commonly affects infants and rarely causes gastric outlet obstruction in adolescents and older children. We present the case of an 11‐year‐old girl with a 2‐month history of recurrent postprandial vomiting and weight loss. On physical examination, the patient presented with abdominal distension. Upper gastrointestinal endoscopy revealed a very small pyloric orifice through which the endoscope could not be advanced. Abdominal ultrasonography and a computed tomography confirmed pylorus thickening. She underwent Heineke‐Mikulicz pyloroplasty with symptom resolution.
{"title":"Gastric outlet obstruction in an 11‐year‐old girl: A case report","authors":"Ana Sofia Figueiredo, Carolina Soares-Aquino, Rita Amorim, L. Melão, C. Espinheira, Isabel Pinto Pais, Miguel Campos, Eunice Trindade","doi":"10.1002/jpr3.12062","DOIUrl":"https://doi.org/10.1002/jpr3.12062","url":null,"abstract":"Pyloric stenosis commonly affects infants and rarely causes gastric outlet obstruction in adolescents and older children. We present the case of an 11‐year‐old girl with a 2‐month history of recurrent postprandial vomiting and weight loss. On physical examination, the patient presented with abdominal distension. Upper gastrointestinal endoscopy revealed a very small pyloric orifice through which the endoscope could not be advanced. Abdominal ultrasonography and a computed tomography confirmed pylorus thickening. She underwent Heineke‐Mikulicz pyloroplasty with symptom resolution.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"565 ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140784482","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}
Approved options for advanced therapy in pediatric inflammatory bowel disease (IBD) are limited. Although Janus kinase (JAK) inhibitors are approved in adult IBD, their benefit in pediatric populations is not yet delineated. We present a 13‐year‐old female patient with ulcerative colitis (UC) refractory to numerous therapies and courses of prednisone that ultimately responded to a JAK inhibitor. Initial treatment consisted of 5‐aminosalicylate and azathioprine. This was changed to adalimumab due to persistent symptoms. Repeat colonoscopy revealed pancolitis, thus she was transitioned to vedolizumab. She was hospitalized twice for uncontrolled symptoms on vedolizumab and subsequent scope showed continued pancolitis. As a result, she transitioned to ustekinumab without symptomatic relief after adjusting to monthly dosing. The family declined colectomy, opting to exhaust all medical therapies. Upadacitinib was started and her symptoms resolved within 1 week, and she remains in steroid‐free remission. This case illustrates the possible role of JAK inhibitors in extensively refractory pediatric UC patients before colectomy.
小儿炎症性肠病（IBD）晚期治疗的获批方案有限。尽管 Janus 激酶 (JAK) 抑制剂已获准用于成人 IBD，但其对儿科人群的益处尚未明确。我们介绍了一位 13 岁的女性溃疡性结肠炎（UC）患者，她对多种疗法和泼尼松疗程均无效，最终对 JAK 抑制剂产生了反应。最初的治疗包括5-氨基水杨酸盐和硫唑嘌呤。由于症状持续存在，治疗方案改为阿达木单抗。重复结肠镜检查发现了胰腺炎，因此她转而接受了维多珠单抗治疗。使用维多珠单抗后，她两次因症状无法控制而住院，随后的结肠镜检查显示她仍有胰腺炎。因此，在调整为每月给药一次后，她转用了乌司替尼，但症状没有得到缓解。家属拒绝接受结肠切除术，选择用尽所有药物疗法。开始服用乌达替尼后，她的症状在一周内得到缓解，目前仍处于无类固醇缓解期。该病例说明了JAK抑制剂在结肠切除术前对广泛难治性小儿UC患者可能发挥的作用。
{"title":"Rescue therapy with upadacitinib in medically refractory pediatric ulcerative colitis","authors":"Maria Miller, Ashish S. Patel, Brad Pasternak","doi":"10.1002/jpr3.12067","DOIUrl":"https://doi.org/10.1002/jpr3.12067","url":null,"abstract":"Approved options for advanced therapy in pediatric inflammatory bowel disease (IBD) are limited. Although Janus kinase (JAK) inhibitors are approved in adult IBD, their benefit in pediatric populations is not yet delineated. We present a 13‐year‐old female patient with ulcerative colitis (UC) refractory to numerous therapies and courses of prednisone that ultimately responded to a JAK inhibitor. Initial treatment consisted of 5‐aminosalicylate and azathioprine. This was changed to adalimumab due to persistent symptoms. Repeat colonoscopy revealed pancolitis, thus she was transitioned to vedolizumab. She was hospitalized twice for uncontrolled symptoms on vedolizumab and subsequent scope showed continued pancolitis. As a result, she transitioned to ustekinumab without symptomatic relief after adjusting to monthly dosing. The family declined colectomy, opting to exhaust all medical therapies. Upadacitinib was started and her symptoms resolved within 1 week, and she remains in steroid‐free remission. This case illustrates the possible role of JAK inhibitors in extensively refractory pediatric UC patients before colectomy.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"61 9","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140794448","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}
Steven Lin, Terry C. Dixon, Hamza Hassan Khan, Martha M. Munden, J. N. Anderson
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.
{"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":"38 4","pages":""},"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}
Arun Ajmera, Peter Joseph Pernicone, Nishant Patel, Stefany Hernandez Benabe
Pediatric acute liver failure is a rare but serious complication of Coronavirus infections. Our patient is a previously healthy 8‐year‐old male who presented with acute liver failure in the setting of human coronavirus HKU1 (HCoV‐HKU1) infection while asymptomatic from a respiratory perspective. During the hospital course, he developed acute hepatic encephalopathy and was listed for liver transplantation, but fortunately recovered remaining status 7 (inactive) on the transplant list. With a negative diagnostic evaluation other than his viral infection and hyperdense CD8 T‐cells on liver immunohistochemical staining, pediatric acute liver failure (PALF) immune dysregulation phenotype was diagnosed.
小儿急性肝功能衰竭是冠状病毒感染的一种罕见但严重的并发症。我们的患者是一名原本健康的 8 岁男性，因感染人类冠状病毒 HKU1（HCoV-HKU1）而出现急性肝功能衰竭，但从呼吸系统角度看并无症状。住院期间，他出现了急性肝性脑病，并被列入肝移植名单，但幸运的是，他已经康复，在移植名单上的状态仍为 7（非活动）。除了病毒感染和肝脏免疫组化染色上高密度的 CD8 T 细胞外，诊断评估结果为阴性，因此诊断为小儿急性肝衰竭（PALF）免疫调节失调表型。
{"title":"Coronavirus HKU1 infection and development of pediatric acute liver failure with immune dysregulation phenotype","authors":"Arun Ajmera, Peter Joseph Pernicone, Nishant Patel, Stefany Hernandez Benabe","doi":"10.1002/jpr3.12065","DOIUrl":"https://doi.org/10.1002/jpr3.12065","url":null,"abstract":"Pediatric acute liver failure is a rare but serious complication of Coronavirus infections. Our patient is a previously healthy 8‐year‐old male who presented with acute liver failure in the setting of human coronavirus HKU1 (HCoV‐HKU1) infection while asymptomatic from a respiratory perspective. During the hospital course, he developed acute hepatic encephalopathy and was listed for liver transplantation, but fortunately recovered remaining status 7 (inactive) on the transplant list. With a negative diagnostic evaluation other than his viral infection and hyperdense CD8 T‐cells on liver immunohistochemical staining, pediatric acute liver failure (PALF) immune dysregulation phenotype was diagnosed.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"216 6","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140233993","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}
The dangers of magnet ingestion are well known. When multiple magnets are ingested, interventional removal is often necessary to prevent and/or treat complications. Despite reports of both endoscopic and surgical techniques in the literature, there is a lack of clear guidance on the best method for removal of high‐power magnets when they are embedded within the intestinal wall (increasing concern for fistulation, perforation, and bowel wall necrosis). This case demonstrates the successful endoscopic removal of magnetic balls incidentally identified on X‐ray and found to be embedded in the duodenal wall in a critically ill 2‐year‐old patient. Endoscopic removal can be considered in similar situations, if all resources (interventional endoscopy and pediatric surgery) are available to proceed safely.
误食磁铁的危险众所周知。当摄入多块磁铁时，通常需要进行介入性清除以预防和/或治疗并发症。尽管文献中报道了内窥镜和外科技术，但对于嵌入肠壁内的高功率磁铁的最佳取出方法（增加了对瘘管、穿孔和肠壁坏死的担忧）仍缺乏明确的指导。本病例展示了如何在内窥镜下成功取出在 X 光片上偶然发现并嵌入十二指肠壁的磁球，患者是一名两岁的重症患者。在类似的情况下，如果所有资源（介入内镜和儿科手术）都能保证手术安全进行，也可以考虑使用内镜取出。
{"title":"Successful endoscopic removal of high‐power magnetic balls embedded in the duodenal wall","authors":"Juliana M. Kennedy, Nikhil A. Kumta, Joanne Lai","doi":"10.1002/jpr3.12060","DOIUrl":"https://doi.org/10.1002/jpr3.12060","url":null,"abstract":"The dangers of magnet ingestion are well known. When multiple magnets are ingested, interventional removal is often necessary to prevent and/or treat complications. Despite reports of both endoscopic and surgical techniques in the literature, there is a lack of clear guidance on the best method for removal of high‐power magnets when they are embedded within the intestinal wall (increasing concern for fistulation, perforation, and bowel wall necrosis). This case demonstrates the successful endoscopic removal of magnetic balls incidentally identified on X‐ray and found to be embedded in the duodenal wall in a critically ill 2‐year‐old patient. Endoscopic removal can be considered in similar situations, if all resources (interventional endoscopy and pediatric surgery) are available to proceed safely.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"4 5","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140232313","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}
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