JPGN reports最新文献_第10页

Immunotherapy withdrawal by step‐down to mesalamine in pediatric patients with ulcerative colitis 儿科溃疡性结肠炎患者逐步停用美沙拉嗪后的免疫疗法

JPGN reports Pub Date : 2024-02-05 DOI: 10.1002/jpr3.12048

R. Szigeti, R. Kellermayer

{"title":"Immunotherapy withdrawal by step‐down to mesalamine in pediatric patients with ulcerative colitis","authors":"R. Szigeti, R. Kellermayer","doi":"10.1002/jpr3.12048","DOIUrl":"https://doi.org/10.1002/jpr3.12048","url":null,"abstract":"Parents and pediatric patients with ulcerative colitis (UC) who progressed to systemic immunotherapy are concerned about lifelong risks from such treatments. There is limited knowledge about withdrawal of such agents and step‐down (SD) to enteral 5‐aminosalicylic acid (mesalamine) before transitioning to adult care.We studied nine pediatric cases with moderate to severe UC who after a median of 2.18 years of clinical remission on systemic immunotherapy stepped down to oral mesalamine treatment.Average follow‐up time from SD was 3.49 years. Five patients (55.5%) had sustained remission (without any flare noted) after SD during follow‐up. Sustained clinical remission was 88.9% (8/9) at 1 year, 87.5% (7/8) at 2 years, and 66.7% (4/6) at 3 years after SD. Out of those tested (one patient was not tested), 62.5% (5/8) had fecal calprotectin <50 μg/g. Four out of six patients examined (66.6%) had mucosal healing on post‐SD colonoscopy.We propose that SD to mesalamine can be a reasonable therapeutic consideration for pediatric patients with UC before transitioning to adult gastroenterology care. Shared decision‐making is important before such treatment changes.","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"33 4","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139866035","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: 1

Invited commentary to immunotherapy withdrawal by step‐down to mesalamine in pediatrics patients with ulcerative colitis 应邀评论儿科溃疡性结肠炎患者逐步停用美沙拉嗪后的免疫疗法问题

JPGN reports Pub Date : 2024-02-05 DOI: 10.1002/jpr3.12047

Daniel M. O'Connell, Jonathan Moses

引用次数: 0

Characteristics, clinical laboratory, histopathology, and outcomes of glycogenic hepatopathy in children 儿童糖源性肝病的特征、临床实验室、组织病理学和预后

JPGN reports Pub Date : 2024-02-05 DOI: 10.1002/jpr3.12046

Chaowapong Jarasvaraparn, Iván A. González, Kyla Tolliver, Nadine G. Haddad, Jean P. Molleston

{"title":"Characteristics, clinical laboratory, histopathology, and outcomes of glycogenic hepatopathy in children","authors":"Chaowapong Jarasvaraparn, Iván A. González, Kyla Tolliver, Nadine G. Haddad, Jean P. Molleston","doi":"10.1002/jpr3.12046","DOIUrl":"https://doi.org/10.1002/jpr3.12046","url":null,"abstract":"Glycogenic hepatopathy (GH) is a rare complication of type I diabetes mellitus (DM1), resulting in abnormal deposition of glycogen in the liver due to poor glycemic control. Clinical characteristics and natural history of GH are not completely understood in children. In this study, we investigated clinical, biochemical, histologic parameters and outcomes in children with GH.This was a retrospective review of patients less than 18 years old diagnosed with GH and DM. GH was confirmed on liver biopsy. Medical records were reviewed for clinical presentation, laboratory tests, and clinical outcomes. Liver biopsy findings were reviewed by a pediatric pathologist (I. A. G.).Nine children were diagnosed with GH and type 1 DM. The median age at diagnosis of GH was 16 (IQR 14.5−17) years. Duration of diagnosis of DM until GH diagnosis was 7 (IQR 5−11) years. The median frequency of diabetic ketoacidosis before GH diagnosis was three times (IQR 2−5.25). Peak Aspartate transaminase (AST) and Alanine transaminase (ALT) ranged from 115 to 797, and 83−389 units/L, respectively. Only two children had mild fibrosis. Seven of nine had steatosis without steatohepatitis. There was no correlation between glycosylated hemoglobin (HbA1c), or other laboratory tests and liver fibrosis on biopsy. HbA1c was 11.2 (IQR 10.2−12.8) at GH diagnosis and 9.8 (IQR 9.5−10.8) with normalization of liver enzymes.GH appears to be related to poor glycemic control in teenagers with long‐term diabetes. GH presents with high to very high aminotransferase especially AST > ALT and resolves with modestly improved glycemic control. Diffuse hepatocyte swelling, steatosis, minimal fibrosis without hepatocyte ballooning or lobular inflammation are most common histological features.","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"16 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139804588","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

Characteristics, clinical laboratory, histopathology, and outcomes of glycogenic hepatopathy in children 儿童糖源性肝病的特征、临床实验室、组织病理学和预后

JPGN reports Pub Date : 2024-02-05 DOI: 10.1002/jpr3.12046

Chaowapong Jarasvaraparn, Iván A. González, Kyla Tolliver, Nadine G. Haddad, Jean P. Molleston

{"title":"Characteristics, clinical laboratory, histopathology, and outcomes of glycogenic hepatopathy in children","authors":"Chaowapong Jarasvaraparn, Iván A. González, Kyla Tolliver, Nadine G. Haddad, Jean P. Molleston","doi":"10.1002/jpr3.12046","DOIUrl":"https://doi.org/10.1002/jpr3.12046","url":null,"abstract":"Glycogenic hepatopathy (GH) is a rare complication of type I diabetes mellitus (DM1), resulting in abnormal deposition of glycogen in the liver due to poor glycemic control. Clinical characteristics and natural history of GH are not completely understood in children. In this study, we investigated clinical, biochemical, histologic parameters and outcomes in children with GH.This was a retrospective review of patients less than 18 years old diagnosed with GH and DM. GH was confirmed on liver biopsy. Medical records were reviewed for clinical presentation, laboratory tests, and clinical outcomes. Liver biopsy findings were reviewed by a pediatric pathologist (I. A. G.).Nine children were diagnosed with GH and type 1 DM. The median age at diagnosis of GH was 16 (IQR 14.5−17) years. Duration of diagnosis of DM until GH diagnosis was 7 (IQR 5−11) years. The median frequency of diabetic ketoacidosis before GH diagnosis was three times (IQR 2−5.25). Peak Aspartate transaminase (AST) and Alanine transaminase (ALT) ranged from 115 to 797, and 83−389 units/L, respectively. Only two children had mild fibrosis. Seven of nine had steatosis without steatohepatitis. There was no correlation between glycosylated hemoglobin (HbA1c), or other laboratory tests and liver fibrosis on biopsy. HbA1c was 11.2 (IQR 10.2−12.8) at GH diagnosis and 9.8 (IQR 9.5−10.8) with normalization of liver enzymes.GH appears to be related to poor glycemic control in teenagers with long‐term diabetes. GH presents with high to very high aminotransferase especially AST > ALT and resolves with modestly improved glycemic control. Diffuse hepatocyte swelling, steatosis, minimal fibrosis without hepatocyte ballooning or lobular inflammation are most common histological features.","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"1 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139864188","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

Immunotherapy withdrawal by step‐down to mesalamine in pediatric patients with ulcerative colitis 儿科溃疡性结肠炎患者逐步停用美沙拉嗪后的免疫疗法

JPGN reports Pub Date : 2024-02-05 DOI: 10.1002/jpr3.12048

R. Szigeti, R. Kellermayer

{"title":"Immunotherapy withdrawal by step‐down to mesalamine in pediatric patients with ulcerative colitis","authors":"R. Szigeti, R. Kellermayer","doi":"10.1002/jpr3.12048","DOIUrl":"https://doi.org/10.1002/jpr3.12048","url":null,"abstract":"Parents and pediatric patients with ulcerative colitis (UC) who progressed to systemic immunotherapy are concerned about lifelong risks from such treatments. There is limited knowledge about withdrawal of such agents and step‐down (SD) to enteral 5‐aminosalicylic acid (mesalamine) before transitioning to adult care.We studied nine pediatric cases with moderate to severe UC who after a median of 2.18 years of clinical remission on systemic immunotherapy stepped down to oral mesalamine treatment.Average follow‐up time from SD was 3.49 years. Five patients (55.5%) had sustained remission (without any flare noted) after SD during follow‐up. Sustained clinical remission was 88.9% (8/9) at 1 year, 87.5% (7/8) at 2 years, and 66.7% (4/6) at 3 years after SD. Out of those tested (one patient was not tested), 62.5% (5/8) had fecal calprotectin <50 μg/g. Four out of six patients examined (66.6%) had mucosal healing on post‐SD colonoscopy.We propose that SD to mesalamine can be a reasonable therapeutic consideration for pediatric patients with UC before transitioning to adult gastroenterology care. Shared decision‐making is important before such treatment changes.","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"24 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139806172","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: 1

Letter to the editor: A response to Hildreth and Schwimmer. 致编辑的信回应 Hildreth 和 Schwimmer。

JPGN reports Pub Date : 2024-02-05 eCollection Date: 2024-05-01 DOI: 10.1002/jpr3.12027

Rachel B Schenker, Brian Kim, George Yanni

引用次数: 0

Invited commentary to immunotherapy withdrawal by step‐down to mesalamine in pediatrics patients with ulcerative colitis 应邀评论儿科溃疡性结肠炎患者逐步停用美沙拉嗪后的免疫疗法问题

JPGN reports Pub Date : 2024-02-05 DOI: 10.1002/jpr3.12047

Daniel M. O'Connell, Jonathan Moses

引用次数: 0

Idiopathic fibrosing pancreatitis: A rare cause of obstructive jaundice 特发性纤维性胰腺炎：阻塞性黄疸的罕见病因

JPGN reports Pub Date : 2024-01-30 DOI: 10.1002/jpr3.12018

Mindy Huynh, Rodolfo Silva, Nikhil Thiruvengadam, K. Parashette

引用次数: 0

Mucosal prolapse syndrome mimicking Peutz–Jeghers syndrome in a pediatric patient 一名儿科患者模仿 Peutz-Jeghers 综合征的粘膜脱垂综合征

JPGN reports Pub Date : 2024-01-29 DOI: 10.1002/jpr3.12022

Phinga Do, Claudia Phen, Michele Alkalay, Vivekanand Singh, Isabel Rojas

引用次数: 0

Gallbladder remnant: A potential source for biliary stones postcholecystectomy; a case report in a child with sickle cell disease 胆囊残余：胆囊切除术后胆道结石的潜在来源；镰状细胞病患儿的病例报告

JPGN reports Pub Date : 2024-01-25 DOI: 10.1002/jpr3.12039

Ahmad Miri, Shahab Abdessalam, Andria M. Powers, Ruben E. Quiros‐Tejeira, Chinenye R Dike

{"title":"Gallbladder remnant: A potential source for biliary stones postcholecystectomy; a case report in a child with sickle cell disease","authors":"Ahmad Miri, Shahab Abdessalam, Andria M. Powers, Ruben E. Quiros‐Tejeira, Chinenye R Dike","doi":"10.1002/jpr3.12039","DOIUrl":"https://doi.org/10.1002/jpr3.12039","url":null,"abstract":"Stone formation in a gallbladder remnant is a rare postcholecystectomy complication. This report describes the case of gallstones in a gallbladder remnant of an adolescent with sickle cell disease (SCD) years after laparoscopic cholecystectomy. A 15‐year‐old female with SCD presented to our gastroenterology clinic with concerns of recurrent choledocholithiasis despite cholecystectomy 2 years before presentation. About 4 months before presentation to our clinic, she was evaluated at the referring physician's emergency department for recurrent severe abdominal pain of 1 month duration. After admission to the hospital, common bile duct stones were seen on magnetic resonance cholangiopancreatography (MCRP) imaging and subsequently removed via endoscopic retrograde cholangiopancreatography (ERCP). On review of her MRCP and ERCP at our hospital, a remnant of gallbladder containing multiple stones was identified. She subsequently underwent a laparoscopic resection of the gallbladder remnant. Clinicians should consider biliary duct imaging in children with biliary colic following cholecystectomy, especially those with history of chronic hemolysis.","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"3 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-01-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139596886","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