Pediatric inflammatory bowel disease (IBD) is a chronic illness with various treatments, including ostomy surgery. Ostomy‐related medical decision‐making (MDM) is complex for multidisciplinary healthcare professionals (HCPs). This study sought to understand national multidisciplinary HCPs' perceptions about pediatric IBD ostomy surgery in the United States.This cross‐sectional REDCap survey recruited HCPs in pediatric IBD HCP roles from the ImproveCareNow (ICN) Learning Health System. The survey gathered data on, (1) demographics, (2) participants' perspectives on patients/families' ostomy perceptions, (3) participants' own perspectives on ostomies, and (4) participants' perspectives on multidisciplinary collaboration in ostomy surgery.Participants (n = 69, across n = 30 American ICN sites) were 84% White, 62% female, and 54% gastroenterologists. In reflecting on patients/families' perceptions, participants endorsed most (86%) view ostomies as a “failure,” various psychosocial concerns (e.g., 97% endorsed body image concerns), and most (81%) have moderate to significant stigma about ostomies. In reflecting on their own perceptions, nearly half (44%) endorsed some to moderate stigma about ostomies and felt ostomy discussions were difficult when families had negative perceptions (88%). Over half (54%) endorsed currently having multidisciplinary care for pediatric IBD surgery. Most (70%) endorsed variability in the amount and/or quality of multidisciplinary collaboration.MDM related to pediatric IBD ostomy surgery presents nuanced clinical complexities for HCPs. This work underscores interest in multidisciplinary care, notable ostomy‐related psychosocial needs, and ostomy‐related stigma. Future work should develop systematic approaches to multidisciplinary pediatric IBD ostomy care, bolster psychosocial support, and evaluate how systematic care may impact psychosocial, and healthcare utilization outcomes.
{"title":"Is it “all in the bag?” Multidisciplinary perspectives on ostomy surgery in pediatric IBD across the ImproveCareNow network","authors":"J. David, Jennifer L. Dotson, Laura Mackner","doi":"10.1002/jpr3.12044","DOIUrl":"https://doi.org/10.1002/jpr3.12044","url":null,"abstract":"Pediatric inflammatory bowel disease (IBD) is a chronic illness with various treatments, including ostomy surgery. Ostomy‐related medical decision‐making (MDM) is complex for multidisciplinary healthcare professionals (HCPs). This study sought to understand national multidisciplinary HCPs' perceptions about pediatric IBD ostomy surgery in the United States.This cross‐sectional REDCap survey recruited HCPs in pediatric IBD HCP roles from the ImproveCareNow (ICN) Learning Health System. The survey gathered data on, (1) demographics, (2) participants' perspectives on patients/families' ostomy perceptions, (3) participants' own perspectives on ostomies, and (4) participants' perspectives on multidisciplinary collaboration in ostomy surgery.Participants (n = 69, across n = 30 American ICN sites) were 84% White, 62% female, and 54% gastroenterologists. In reflecting on patients/families' perceptions, participants endorsed most (86%) view ostomies as a “failure,” various psychosocial concerns (e.g., 97% endorsed body image concerns), and most (81%) have moderate to significant stigma about ostomies. In reflecting on their own perceptions, nearly half (44%) endorsed some to moderate stigma about ostomies and felt ostomy discussions were difficult when families had negative perceptions (88%). Over half (54%) endorsed currently having multidisciplinary care for pediatric IBD surgery. Most (70%) endorsed variability in the amount and/or quality of multidisciplinary collaboration.MDM related to pediatric IBD ostomy surgery presents nuanced clinical complexities for HCPs. This work underscores interest in multidisciplinary care, notable ostomy‐related psychosocial needs, and ostomy‐related stigma. Future work should develop systematic approaches to multidisciplinary pediatric IBD ostomy care, bolster psychosocial support, and evaluate how systematic care may impact psychosocial, and healthcare utilization outcomes.","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"23 7","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139784161","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":"Reply to letter to the editor in response to: Distinguishing autoimmune hepatitis from steatohepatitis in adolescents with obesity and positive screening alanine aminotransferase","authors":"Amber Hildreth, J. Schwimmer","doi":"10.1002/jpr3.12049","DOIUrl":"https://doi.org/10.1002/jpr3.12049","url":null,"abstract":"","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"20 5","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139784861","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}
Julia Esswein, Maggie Vickers, Michael Kleinman, John Whitworth, Mark Corkins, S. Riley Pace
Crohn's disease has been described as the “great mimicker” with a wide array of presentations. We describe a case of a teenager who presented with tetany and undetectable vitamin D as initial presentation of Crohn's disease. There are reports of adults in tetany due to electrolyte derangements in chronic gastrointestinal diseases secondary to malabsorption. However, the role of deficient vitamin D as it contributes to immune system dysfunction has only begun to be explored. Vitamin D is essential for calcium absorption, immune regulation, and gut epithelial barrier. This case report discusses vitamin D physiology and its potential mediation in the pathogenesis of inflammatory bowel disease.
克罗恩病被称为 "伟大的模仿者"，表现形式多种多样。我们描述了一例以四肢抽搐和检测不到维生素 D 作为克罗恩病初始表现的青少年病例。有报告称，成人四肢抽搐是由于慢性胃肠道疾病继发吸收不良导致电解质失衡所致。然而，人们对缺乏维生素 D 导致免疫系统功能紊乱的作用的研究才刚刚开始。维生素 D 对钙吸收、免疫调节和肠道上皮屏障至关重要。本病例报告讨论了维生素 D 的生理学及其在炎症性肠病发病机制中的潜在中介作用。
{"title":"Cause or effect? Undetectable vitamin D in a patient with Crohn's disease","authors":"Julia Esswein, Maggie Vickers, Michael Kleinman, John Whitworth, Mark Corkins, S. Riley Pace","doi":"10.1002/jpr3.12045","DOIUrl":"https://doi.org/10.1002/jpr3.12045","url":null,"abstract":"Crohn's disease has been described as the “great mimicker” with a wide array of presentations. We describe a case of a teenager who presented with tetany and undetectable vitamin D as initial presentation of Crohn's disease. There are reports of adults in tetany due to electrolyte derangements in chronic gastrointestinal diseases secondary to malabsorption. However, the role of deficient vitamin D as it contributes to immune system dysfunction has only begun to be explored. Vitamin D is essential for calcium absorption, immune regulation, and gut epithelial barrier. This case report discusses vitamin D physiology and its potential mediation in the pathogenesis of inflammatory bowel disease.","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"125 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139852816","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}
Julia Esswein, Maggie Vickers, Michael Kleinman, John Whitworth, Mark Corkins, S. Riley Pace
Crohn's disease has been described as the “great mimicker” with a wide array of presentations. We describe a case of a teenager who presented with tetany and undetectable vitamin D as initial presentation of Crohn's disease. There are reports of adults in tetany due to electrolyte derangements in chronic gastrointestinal diseases secondary to malabsorption. However, the role of deficient vitamin D as it contributes to immune system dysfunction has only begun to be explored. Vitamin D is essential for calcium absorption, immune regulation, and gut epithelial barrier. This case report discusses vitamin D physiology and its potential mediation in the pathogenesis of inflammatory bowel disease.
克罗恩病被称为 "伟大的模仿者"，表现形式多种多样。我们描述了一例青少年四肢抽搐和检测不到维生素 D 的病例，这是克罗恩病的最初表现。有报告称，成人四肢抽搐是由于慢性胃肠道疾病继发吸收不良导致的电解质紊乱。然而，人们对缺乏维生素 D 导致免疫系统功能紊乱的作用的研究才刚刚开始。维生素 D 对钙吸收、免疫调节和肠道上皮屏障至关重要。本病例报告讨论了维生素 D 的生理学及其在炎症性肠病发病机制中的潜在中介作用。
{"title":"Cause or effect? Undetectable vitamin D in a patient with Crohn's disease","authors":"Julia Esswein, Maggie Vickers, Michael Kleinman, John Whitworth, Mark Corkins, S. Riley Pace","doi":"10.1002/jpr3.12045","DOIUrl":"https://doi.org/10.1002/jpr3.12045","url":null,"abstract":"Crohn's disease has been described as the “great mimicker” with a wide array of presentations. We describe a case of a teenager who presented with tetany and undetectable vitamin D as initial presentation of Crohn's disease. There are reports of adults in tetany due to electrolyte derangements in chronic gastrointestinal diseases secondary to malabsorption. However, the role of deficient vitamin D as it contributes to immune system dysfunction has only begun to be explored. Vitamin D is essential for calcium absorption, immune regulation, and gut epithelial barrier. This case report discusses vitamin D physiology and its potential mediation in the pathogenesis of inflammatory bowel disease.","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" 29","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139792903","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}
Protein‐losing enteropathy associated with collagenous colitis (CC) is a rare but described entity in the adult population. However, literature regarding this in the pediatric population is scarce. Here we describe a 2‐year‐old female who presented with fevers, accompanied by nonbloody, watery diarrhea, and decreased oral intake. Work‐up was significant for severe hypoalbuminemia at 1.5 grams per deciliter (g/dL), pancytopenia, and elevated fecal alpha‐1‐antitrypsin at 1.13 milligrams per grams (mg/g). Gastrointestinal mucosal evaluation was normal endoscopically; however, histology was consistent with CC. She responded to 12‐week treatment with budesonide with resolution of symptoms and laboratory values. At this point, she has not had a recurrence 1 year later.
{"title":"Protein‐losing enteropathy secondary to collagenous colitis in a 2‐year‐old","authors":"Hamza Hassan Khan, David N. Lewin, Carmine Suppa","doi":"10.1002/jpr3.12051","DOIUrl":"https://doi.org/10.1002/jpr3.12051","url":null,"abstract":"Protein‐losing enteropathy associated with collagenous colitis (CC) is a rare but described entity in the adult population. However, literature regarding this in the pediatric population is scarce. Here we describe a 2‐year‐old female who presented with fevers, accompanied by nonbloody, watery diarrhea, and decreased oral intake. Work‐up was significant for severe hypoalbuminemia at 1.5 grams per deciliter (g/dL), pancytopenia, and elevated fecal alpha‐1‐antitrypsin at 1.13 milligrams per grams (mg/g). Gastrointestinal mucosal evaluation was normal endoscopically; however, histology was consistent with CC. She responded to 12‐week treatment with budesonide with resolution of symptoms and laboratory values. At this point, she has not had a recurrence 1 year later.","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"97 8","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139802157","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}
Protein‐losing enteropathy associated with collagenous colitis (CC) is a rare but described entity in the adult population. However, literature regarding this in the pediatric population is scarce. Here we describe a 2‐year‐old female who presented with fevers, accompanied by nonbloody, watery diarrhea, and decreased oral intake. Work‐up was significant for severe hypoalbuminemia at 1.5 grams per deciliter (g/dL), pancytopenia, and elevated fecal alpha‐1‐antitrypsin at 1.13 milligrams per grams (mg/g). Gastrointestinal mucosal evaluation was normal endoscopically; however, histology was consistent with CC. She responded to 12‐week treatment with budesonide with resolution of symptoms and laboratory values. At this point, she has not had a recurrence 1 year later.
{"title":"Protein‐losing enteropathy secondary to collagenous colitis in a 2‐year‐old","authors":"Hamza Hassan Khan, David N. Lewin, Carmine Suppa","doi":"10.1002/jpr3.12051","DOIUrl":"https://doi.org/10.1002/jpr3.12051","url":null,"abstract":"Protein‐losing enteropathy associated with collagenous colitis (CC) is a rare but described entity in the adult population. However, literature regarding this in the pediatric population is scarce. Here we describe a 2‐year‐old female who presented with fevers, accompanied by nonbloody, watery diarrhea, and decreased oral intake. Work‐up was significant for severe hypoalbuminemia at 1.5 grams per deciliter (g/dL), pancytopenia, and elevated fecal alpha‐1‐antitrypsin at 1.13 milligrams per grams (mg/g). Gastrointestinal mucosal evaluation was normal endoscopically; however, histology was consistent with CC. She responded to 12‐week treatment with budesonide with resolution of symptoms and laboratory values. At this point, she has not had a recurrence 1 year later.","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"2 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139862201","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}
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.
{"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}
{"title":"Invited commentary to immunotherapy withdrawal by step‐down to mesalamine in pediatrics patients with ulcerative colitis","authors":"Daniel M. O'Connell, Jonathan Moses","doi":"10.1002/jpr3.12047","DOIUrl":"https://doi.org/10.1002/jpr3.12047","url":null,"abstract":"","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"27 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139803226","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}
Chaowapong Jarasvaraparn, Iván A. González, Kyla Tolliver, Nadine G. Haddad, Jean P. Molleston
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.
{"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}
Chaowapong Jarasvaraparn, Iván A. González, Kyla Tolliver, Nadine G. Haddad, Jean P. Molleston
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.
{"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}
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