Journal of Pediatric Gastroenterology and Nutrition最新文献

{"title":"Influence of pediatric ERCP positioning on procedural outcomes: A single-center study.","authors":"Michael Joseph, Rebecca Schiff, Jacob Mark, Robert Kramer","doi":"10.1002/jpn3.12438","DOIUrl":"10.1002/jpn3.12438","url":null,"abstract":"<p><strong>Objectives: </strong>Endoscopic retrograde cholangiopancreatography (ERCP) is traditionally done in the prone position. In pediatrics, patient anatomy and anesthesia practices are considerations that affect procedural success and anesthesia time. The aim of our study was to evaluate the safety, procedural success, and efficiency of pediatric ERCP in the prone versus supine positions.</p><p><strong>Methods: </strong>ERCPs from September 2016 to August 2023 were reviewed at our center. Demographic and procedure variables were collected. Multivariate linear regression was performed to determine the effect of patient position on total anesthesia time.</p><p><strong>Results: </strong>Two hundred and eighty-three patients (378 ERCPs) were included. There were significant differences in fellow involvement, proportion of native papillae, procedural indication, and total anesthesia time by ERCP position. Multivariate linear regression found that supine position was associated with 9.3-min decrease in anesthesia time and American Society of Anesthesiologists Class 1 or 2 was associated with 10.6-min decrease in anesthesia time. Factors that were associated with increased anesthesia time were additional procedure, increased procedure time, and native papilla. Finally, we found a learning curve for transitioning from prone to supine position was between 10 and 40 cases. After the learning curve, we found 11-min decrease in mean procedure time and 16-min decrease in total anesthesia time in the supine position.</p><p><strong>Conclusions: </strong>This is the first pediatric study to evaluate the role of patient positioning on ERCP outcomes and total anesthesia time. Given similar procedural outcomes, the impact of increased anesthesia time on neurodevelopment in children, and the cost to the patient, the supine position may be preferred to a prone position.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"345-352"},"PeriodicalIF":2.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142837201","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}

{"title":"Health-related quality of life determinants in Spanish children with eosinophilic esophagitis.","authors":"Ruth García-Martínez de Bartolomé, Josefa Barrio-Torres, Tomás Sebastián-Viana, Víctor Vila-Miravet, Enrique La Orden-Izquierdo, Sonia Fernández-Fernández, Myriam Herrero-Álvarez, Marta Soria-López, Gonzalo Botija-Arcos, Alejandro Rodríguez-Martínez, Gonzalo Galicia-Poblet, Alejandro García-Díaz, Marta Herreros-Sáenz, Javier Blasco-Alonso, Gloria Rodrigo-García, Natalia Alonso-Pérez, Ana Fernández de Valderrama-Rodríguez, Noel Oppenau-López, Begoña Pérez-Moneo, Sara Feo-Ortega, Raquel Vecino-López, Paloma Donado-Palencia, José Ramón Alberto-Alonso, Margarita Revenga-Parra, Helena Lorenzo-Garrido, Miguel Ángel Carro-Rodríguez, Luis Grande-Herrero, Saioa Vicente-Santamaría, Elena Balmaseda-Serrano, María Carmen Miranda-Cid, Jessica Martín-González, Ruth García-Romero, Diana García-Tirado, Jana Rizo-Pascual, Pedro Alonso-López, Miriam Blanco-Rodríguez, Alicia Rendo-Vázquez, Antonio Millán-Jiménez, Ana Castro-Millán, Eduard Bastida-Ratera, M Luz Cilleruelo-Pascual","doi":"10.1002/jpn3.12426","DOIUrl":"10.1002/jpn3.12426","url":null,"abstract":"<p><strong>Objectives: </strong>To study the health-related quality of life (HRQoL) and its possible determinant factors in Spanish children with eosinophilic esophagitis (EoE) and their parents.</p><p><strong>Methods: </strong>Multicenter observational cross-sectional study. The Spanish version of the Pediatric Quality of Life Eosinophilic Esophagitis Module was filled out by EoE patients aged 8-18 and their parents. Demographic, psychosocial, and clinical variables were studied. Multiple linear regression was performed to identify related factors of HRQoL.</p><p><strong>Results: </strong>A total of 279 children and their parents participated (72.7% males). 39.1% received swallowed corticosteroids, 35.5% proton pump inhibitors and 16.8% diet. 1.1% received other treatments and 7.5% received no treatment. Poor compliance to treatment was observed in 11.5% and 6.5% of patients were referred to mental health. The Total Scale Scores were 72.71 ± 17.50 and 75.62 ± 16.73 for children and parents, respectively. \"Communication\" was the dimension with the highest scores (82.14 ± 21.65 and 81.59 ± 24.13) while \"Food and Eating\" (48.92 ± 32.94 and 62.85 ± 28.78), and \"Food Feelings\" (53.55 ± 29.96 and 53.95 ± 27.78) had the lower scores. Patients and parents under dietary treatment showed lower scores than those under pharmacological treatment, 65.77 ± 16.96 versus 74.28 ± 16.96, p = 0.001 and 68.33 ± 17.32 versus 77.24 ± 15.97, p = 0.001, respectively. Factors associated with worse HRQoL scores were symptom frequency, diet, food allergies, and the need for mental health assistance.</p><p><strong>Conclusions: </strong>The HRQoL of children and their parents was \"good.\" However, diet, frequency of symptoms, food allergies, and the need for mental health care were the factors that had a negative impact on HRQoL of children with EoE.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"308-317"},"PeriodicalIF":2.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142813627","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}

{"title":"Current practice in the management of paediatric autoimmune liver disease in Europe.","authors":"Mara Cananzi, Marianne Hørby Jørgensen, Gustav Buescher, Ruth De Bruyne, Marianne Samyn","doi":"10.1002/jpn3.12424","DOIUrl":"10.1002/jpn3.12424","url":null,"abstract":"<p><strong>Objective: </strong>Paediatric autoimmune liver disease (pAILD) is a rare condition with serious health implications. Notwithstanding treatment advancements, areas of uncertainty and knowledge gaps still exist. We here investigated the real-life approach to pAILD management in Europe.</p><p><strong>Methods: </strong>A survey was distributed to members of the European Rare Liver Disease Reference Network (ERN RARE-LIVER) and the European Society for Paediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) Hepatology Interest Group. Information was gathered regarding clinical activity, medications used, and access to paediatric drug formulations at each site.</p><p><strong>Results: </strong>Thirty-six centres from 22 European countries responded to the survey. The majority are exclusively paediatric units (86%). Among participants, 80% follow <50 children with pAILD, of which 25%-50% are <10 years old in 44% of centres. All centres use predniso(lo)ne as first-line therapy, alone (15/36) or with azathioprine (21/36). Azathioprine and mycophenolate are the preferred second-line options in centres using first-line steroid monotherapy (11/15) or combined steroid-azathioprine (19/21), respectively. Tacrolimus is used as third-line agent in 15/36 centres. Proactive measurement of drug metabolites and target levels vary widely among centres. Paediatric predniso(lo)ne formulations are commercially available in 7/22 European countries, azathioprine in 3, mycophenolate in 14, tacrolimus in 15 and ursodeoxycholic acid in 14. When paediatric formulations are unavailable, children are treated with magisterial preparations or 'solid' formulations (crushed or intact).</p><p><strong>Conclusions: </strong>Treatment of pAILD in Europe varies widely in terms of medications used and treatment monitoring. Availability of paediatric drug formulations across Europe is limited. Collaborative initiatives are needed to define evidence-based strategies for management of pAILD and to promote an equal, age-appropriate treatment for affected children.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"260-270"},"PeriodicalIF":2.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142770030","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}

{"title":"Primary sclerosing cholangitis in children with inflammatory bowel disease: An ESPGHAN position paper from the Hepatology Committee and the IBD Porto group.","authors":"Patrick F van Rheenen, Kaija-Leena Kolho, Richard K Russell, Marina Aloi, Annamaria Deganello, Séamus Hussey, Norman Junge, Jan De Laffolie, Mark R Deneau, Emer Fitzpatrick, Anne M Griffiths, Iva Hojsak, Emanuele Nicastro, Andreia Nita, Mikko Pakarinen, Amanda Ricciuto, Lissy de Ridder, Aurelio Sonzogni, Andrea Tenca, Marianne Samyn, Giuseppe Indolfi","doi":"10.1002/jpn3.12378","DOIUrl":"10.1002/jpn3.12378","url":null,"abstract":"<p><strong>Objective: </strong>We aimed to provide an evidence-supported approach to diagnose, monitor, and treat children with inflammatory bowel disease (IBD) and primary sclerosing cholangitis (PSC).</p><p><strong>Methods: </strong>The core group formulated seven PICO-structured clinical questions. A systematic literature search from inception to December 2022 was conducted by a medical librarian using MEDLINE and EMBASE. Core messages from the literature were phrased as position statements and then circulated to a sounding board composed of international experts in pediatric gastroenterology and hepatology, histopathology, adult gastroenterology and hepatology, radiology, and surgery. Statements reaching at least 80% agreement were considered as final. The other statements were refined and then subjected to a second online vote or rejection.</p><p><strong>Results: </strong>Regular screening for gamma-glutamyltransferase (GGT) is essential for detecting possible biliary disease in children with IBD. MR cholangiopancreatography is the radiological modality of choice for establishing the diagnosis of PSC. Liver biopsy is relevant in the evaluation of small duct PSC or autoimmune hepatitis. Children who do not have known IBD at the time of PSC diagnosis should undergo initial screening with fecal calprotectin for asymptomatic colitis, and then at least once yearly thereafter. Children with a cholestatic liver enzyme profile can be considered for treatment with ursodeoxycholic acid and can continue if there is a meaningful reduction or normalization in GGT. Oral vancomycin may have a beneficial effect on GGT and intestinal inflammation, but judicious use is recommended due to the lack of long-term studies. Children with PSC-IBD combined with convincing features of autoimmune hepatitis may benefit from corticosteroids and antimetabolites.</p><p><strong>Conclusions: </strong>We present state-of-the-art guidance on the diagnostic criteria, follow-up strategies, and therapeutic strategies and point out research gaps in children and adolescents with PSC-IBD.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"374-393"},"PeriodicalIF":2.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11788976/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142915248","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A decade of real-world clinical experience with 8-week azithromycin-metronidazole combined therapy in paediatric Crohn's disease.","authors":"Maria Teresa Fioretti, Laura Gianolio, Katherine Armstrong, Rosalind M Rabone, Paul Henderson, David C Wilson, Richard K Russell","doi":"10.1002/jpn3.12430","DOIUrl":"10.1002/jpn3.12430","url":null,"abstract":"<p><strong>Objectives: </strong>The aim of our study was to assess the effectiveness and side-effect profile of a combination of azithromycin and metronidazole (CD AZCRO) as alternative induction therapy for 8 weeks in mild to moderately active paediatric Crohn's disease (CD).</p><p><strong>Methods: </strong>We performed a retrospective cohort study (November 2012 to July 2023) of a regional paediatric inflammatory bowel disease service. Disease activity, faecal calprotectin (FC), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), haematological parameters and albumin were collected at baseline, 8 and 16 weeks. At Week 8, patients were divided based on (paediatric Crohn's disease activity index) score and inflammatory markers (blood and stool) into: Group 1 clinical remission and Group 2 non-remission.</p><p><strong>Results: </strong>A total of 48 patients were initially identified of whom 44 were included in the intention-to-treat analysis. After 8 weeks, the overall remission rate was 64%. Of the 38 patients who completed the CD AZCRO course, 28 patients (74%) entered remission (Group 1) and 10 (26%) did not (Group 2). At baseline a shorter disease duration, low weight z score and higher inflammatory burden (ESR, platelets and FC levels) were observed in Group 2. After 8 weeks, Group 1 showed improved CRP levels and higher albumin and haemoglobin levels than Group 2. Median FC declined significantly from 650 mcg/g at baseline to 190 mcg/g at Week 8 in Group 1 (p < 0.001). At 16 weeks, 23/28 patients (82%) continued in clinical remission. Nausea and vomiting were reported in 4/44 patients.</p><p><strong>Conclusions: </strong>Our real-world data demonstrate that CD AZCRO represents an alternative induction therapy for mild to moderate paediatric CD.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"300-307"},"PeriodicalIF":2.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142794850","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}

{"title":"Unveiling distinctive patterns in pediatric PSC-IBD: Time for a tailored histopathologic index?","authors":"Oscar F Lopez-Nunez, Sarangarajan Ranganathan","doi":"10.1002/jpn3.12435","DOIUrl":"10.1002/jpn3.12435","url":null,"abstract":"","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"257-259"},"PeriodicalIF":2.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142818459","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}

{"title":"Evaluation and management of pediatric refractory constipation: Recommendations from the NASPGHAN neurogastroenterology and motility committee.","authors":"Alexandra L Kilgore, Mary K Rogers Boruta, Lusine Ambartsumyan, Roberto Gomez Suarez, Dhiren Patel, Richard J Wood, Anil Darbari, Leonel Rodriguez","doi":"10.1002/jpn3.12390","DOIUrl":"10.1002/jpn3.12390","url":null,"abstract":"<p><p>Refractory constipation (RC) in pediatric patients should be recognized as a distinct condition with long-term impacts on patient and family quality of life. RC requires a more targeted diagnostic evaluation and complex management strategy that may involve management by pediatric neurogastroenterology and motility specialists and multidisciplinary teams including surgeons. Currently, there is a lack of a clear definition, evaluation, and management strategies for RC. This is the first North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition position paper to address pediatric RC regarding its definition, evaluation, and management.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"353-373"},"PeriodicalIF":2.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142915245","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}

{"title":"Portal hypertension in doublecortin domain-containing protein 2 (DCDC2)-related neonatal sclerosing cholangitis.","authors":"Prabhsaran Kaur, Bikrant Bihari Lal, Deepa Janakiraman, Nirmala Dheivamani, Snehavardhan Pandey, Ashish Bavdekar, Aashay Shah, Sanjeev Kumar Verma, Vaibhav Shah, Arjun Maria, Nishant Wadhwa, Sumit Kumar Singh, Vikrant Sood, Rajeev Khanna, Seema Alam","doi":"10.1002/jpn3.12414","DOIUrl":"10.1002/jpn3.12414","url":null,"abstract":"<p><p>Mutations in doublecortin domain-containing protein 2 (DCDC2) lead to neonatal sclerosing cholangitis (NSC), and portal hypertension (PHTN). The objective of the study was to systematically evaluate PHTN, variceal bleeding, and outcomes of patients with DCDC2-related NSC. The study included children with homozygous or compound heterozygous variants in DCDC2. All 14 children with DCDC2-related NSC had PHTN. Eight (57.1%) developed variceal bleed at a median age of 3 years (range: 1.9-5 years). Eleven (78.6%) children with high-risk varices underwent endotherapy. Varices were completely eradicated in three, downstaged to low-risk in five, and there was no response with endotherapy in three. All three children with failure to eradicate/downstage varices had rebleed, and required listing for liver transplantation (LT). The study shows that children with variants in DCDC2 have a high incidence of variceal bleed at a very young age. Variceal eradication may often be difficult and rebleed rates are high; often necessitating LT.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"189-196"},"PeriodicalIF":2.4,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142647908","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}

{"title":"Considerations in the development of the International Multicenter Pediatric Portal Hypertension Registry.","authors":"Tassos Grammatikopoulos, Catalina Jaramillo, Jean Molleston, Julio Pimenta, Oanez Ackermann, Riccardo Superina, Roberto De Franchis, Serpil Tutan, Simon Ling, Uma Ramamurthy, Benjamin L Shneider","doi":"10.1002/jpn3.12415","DOIUrl":"10.1002/jpn3.12415","url":null,"abstract":"<p><p>Portal hypertension, a common sequela of chronic liver disease, is complicated by variceal hemorrhage, one of its most serious complications. Evidence-based approaches to managing variceal hemorrhage are limited by the scarcity of data related to this rare entity. Multicenter international registries are increasingly utilized to garner critical information about rare diseases. The International Multicenter Pediatric Portal Hypertension Registry (IMPPHR) was developed to acquire pediatric data about the mortality of first variceal hemorrhage and approaches to primary and second prophylaxis of variceal hemorrhage with a goal of improving outcomes in children with portal hypertension. IMPPHR evolved from pediatric portal hypertension symposia at the Baveno V and VI meetings in 2010 and 2015, with a formal executive committee initiating the development of IMPPHR in 2019. The registry opened in 2020, with data closure in 2024, including information from 44 centers and >700 subjects. The complexities and approaches to developing IMPPHR are described.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"197-202"},"PeriodicalIF":2.4,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142647955","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}

{"title":"Personalized management of hepatic glycogen storage disorders: The role of continuous glucose monitoring.","authors":"Ambika Gupta, Anuja Agarwala, Mani Kalaivani, Rohan Malik, Rajni Sharma, Madhulika Kabra, Neerja Gupta","doi":"10.1002/jpn3.12391","DOIUrl":"10.1002/jpn3.12391","url":null,"abstract":"<p><strong>Objective: </strong>Glycogen storage disorders (GSD), inherent disorders of carbohydrate metabolism, feature hypoglycemia as a hallmark. Normoglycemia and glucose monitoring are pivotal in disease management. Conventional glucometer-based monitoring may overlook hypoglycemic trends. This study assesses glycemic control in Asian Indian GSD children using continuous glucose monitoring (CGM) and its role in facilitating dietary adjustments.</p><p><strong>Methods: </strong>A pre-post study enrolled molecularly confirmed GSDI, GSDIII, GSDVI, and GSDIX patients for baseline dietary compliance and CGM-based glycemic status evaluation. Hypoglycemic patients were stratified into diet-compliant and diet-noncompliant groups. Noncompliant patients received dietary reinforcement; compliant individuals underwent dietary adjustments. Repeat CGM (rCGM) was performed 6 weeks to 6 months postadjustments. Clinical and metabolic parameters were re-evaluated at 6 months.</p><p><strong>Results: </strong>Of the 20 patients assessed at baseline, 11 were diet compliant. Six among these exhibited hypoglycemia, prompting diet adjustments. Among nine noncompliant patients, eight experienced hypoglycemia and received diet reinforcement. rCGM in 10 patients (five GSDI, three GSDIII, and two GSDIXc) showed a significant reduction in hypoglycemia duration in all. An improvement in height and body mass index was observed in all GSDI and GSDIII patients. Triglyceride levels, raised at baseline in two GSDI and one GSDIII, showed a substantial decline in one GSDI patient. Hepatic transaminase levels decreased in both GSDIXc patients. Plasma lactate levels decreased in all GSDI patients.</p><p><strong>Conclusion: </strong>CGM is an efficacious adjunct in the personalized management of hepatic GSD patients, in the Asian Indian population. The study also underscores the need for long-term follow-up to determine the role of glycemic management in growth, general well-being, and metabolic control in the GSD subtypes.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"151-162"},"PeriodicalIF":2.4,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142564047","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}