Pub Date : 2024-08-01Epub Date: 2024-06-14DOI: 10.1002/jpn3.12276
Paul W Wales, Susan Hill, Ian Robinson, Bram P Raphael, Cheney Matthews, Valeria Cohran, Beth Carter, Robert Venick, Samuel Kocoshis
Objectives: Patients with short bowel syndrome-associated intestinal failure (SBS-IF) require long-term parenteral nutrition and/or intravenous fluids (PN/IV) to maintain fluid or nutritional balance. We report the long-term safety, efficacy, and predictors of response in pediatric patients with SBS-IF receiving teduglutide over 96 weeks.
Methods: This was a pooled, post hoc analysis of two open-label, long-term extension (LTE) studies (NCT02949362 and NCT02954458) in children with SBS-IF. Endpoints included treatment-emergent adverse events (TEAEs) and clinical response (≥20% reduction in PN/IV volume from baseline). A multivariable linear regression identified predictors of teduglutide response; the dependent variable was mean change in PN/IV volume at each visit over 96 weeks.
Results: Overall, 85 patients were analyzed; 78 patients received teduglutide in the parent and/or LTE studies (any teduglutide [TED] group), while seven patients did not receive teduglutide in either the parent or LTE studies. Most TEAEs were moderate or severe in intensity in both groups. By week 96, 82.1% of patients from the any TED group achieved a clinical response, with a mean fluid decrease of 30.1 mL/kg/day and an energy decrease of 21.6 kcal/kg/day. Colon-in-continuity, non-White race, older age at baseline, longer duration of teduglutide exposure, and increasing length of remaining small intestine were significantly associated with a reduction in mean PN/IV volume requirements.
Conclusions: In pediatric patients with SBS-IF, teduglutide treatment resulted in long-term reductions in PN/IV requirements. The degree of PN/IV volume reduction depended on the duration of teduglutide exposure, underlying bowel anatomy, and demographics.
{"title":"Long-term teduglutide associated with improved response in pediatric short bowel syndrome-associated intestinal failure.","authors":"Paul W Wales, Susan Hill, Ian Robinson, Bram P Raphael, Cheney Matthews, Valeria Cohran, Beth Carter, Robert Venick, Samuel Kocoshis","doi":"10.1002/jpn3.12276","DOIUrl":"10.1002/jpn3.12276","url":null,"abstract":"<p><strong>Objectives: </strong>Patients with short bowel syndrome-associated intestinal failure (SBS-IF) require long-term parenteral nutrition and/or intravenous fluids (PN/IV) to maintain fluid or nutritional balance. We report the long-term safety, efficacy, and predictors of response in pediatric patients with SBS-IF receiving teduglutide over 96 weeks.</p><p><strong>Methods: </strong>This was a pooled, post hoc analysis of two open-label, long-term extension (LTE) studies (NCT02949362 and NCT02954458) in children with SBS-IF. Endpoints included treatment-emergent adverse events (TEAEs) and clinical response (≥20% reduction in PN/IV volume from baseline). A multivariable linear regression identified predictors of teduglutide response; the dependent variable was mean change in PN/IV volume at each visit over 96 weeks.</p><p><strong>Results: </strong>Overall, 85 patients were analyzed; 78 patients received teduglutide in the parent and/or LTE studies (any teduglutide [TED] group), while seven patients did not receive teduglutide in either the parent or LTE studies. Most TEAEs were moderate or severe in intensity in both groups. By week 96, 82.1% of patients from the any TED group achieved a clinical response, with a mean fluid decrease of 30.1 mL/kg/day and an energy decrease of 21.6 kcal/kg/day. Colon-in-continuity, non-White race, older age at baseline, longer duration of teduglutide exposure, and increasing length of remaining small intestine were significantly associated with a reduction in mean PN/IV volume requirements.</p><p><strong>Conclusions: </strong>In pediatric patients with SBS-IF, teduglutide treatment resulted in long-term reductions in PN/IV requirements. The degree of PN/IV volume reduction depended on the duration of teduglutide exposure, underlying bowel anatomy, and demographics.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141317590","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}
Pub Date : 2024-08-01Epub Date: 2024-06-07DOI: 10.1002/jpn3.12280
Hania Szajewska, Raanan Shamir, Renata Auricchio, Anna Chmielewska, Jernej Dolinsek, Laura Kivelä, Sibylle Koletzko, Ilma R Korponay-Szabo, Elin M Hård Af Segerstad, M Luisa Mearin, Caroline Meijer-Boekel, Carmen Ribes Konickx, Alfonso Rodriguez-Herrera, Ketil Stordal, Riccardo Troncone, Margreet Wessels
This position paper by the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) Special Interest Group on Coeliac Disease (SIG-CD) presents an update to the 2016 recommendations concerning early diet and the risk of coeliac disease (CD). This update adheres to the policy that mandates reviewing guidelines every 5 years, particularly when new data emerge. The 2024 statements and recommendations are essentially similar to the 2016 recommendations. Breastfeeding, whether any amount, exclusive, or of any duration, does not reduce the risk of developing CD. Introducing gluten into an infant's diet at any time between completed 4 months (≥17 weeks) and 12 months of age does not affect the cumulative incidence of CD, although earlier introduction may lead to earlier seroconversion and CD. In observational studies involving cohorts with a known risk for CD, consuming a high amount of gluten compared to a low amount during weaning and in the subsequent childhood years-specifically the first 2-3 years, and even up to 5 years in some studies-was associated with an increased risk for CD. However, the specific optimal amounts of gluten consumption remain undetermined due to insufficient evidence on safe thresholds, and the impact of restricting gluten in the diet of healthy children of unknown risk for CD is unknown. Thus, any recommendation on the gluten amount is currently unjustifiable for the general population and infants with known HLA risk types. There is no specific guidance on the type of gluten-containing foods to be introduced at weaning.
本立场文件由欧洲儿科胃肠病学、肝脏病学和营养学学会(ESPGHAN)乳糜泻特别兴趣小组(SIG-CD)撰写,对2016年有关早期饮食和乳糜泻(CD)风险的建议进行了更新。此次更新遵循了每 5 年审查一次指南的政策,尤其是在出现新数据时。2024 年的声明和建议与 2016 年的建议基本相似。母乳喂养,无论是母乳喂养量、纯母乳喂养还是母乳喂养持续时间,都不会降低罹患 CD 的风险。在婴儿满 4 个月(≥17 周)至 12 个月期间的任何时间将麸质引入婴儿饮食不会影响 CD 的累积发病率,尽管较早引入麸质可能会导致较早的血清转换和 CD。在涉及已知有 CD 风险的队列的观察性研究中,在断奶期间和随后的童年时期(特别是最初的 2-3 年,在某些研究中甚至长达 5 年),摄入大量麸质比摄入少量麸质与 CD 风险的增加有关。然而,由于安全阈值方面的证据不足,具体的最佳麸质摄入量仍未确定,而且对 CD 风险未知的健康儿童饮食中限制麸质的影响也不得而知。因此,目前对于普通人群和已知 HLA 风险类型的婴儿而言,任何关于麸质含量的建议都是不合理的。关于断奶时应添加何种含麸质食物,目前还没有具体的指导意见。
{"title":"Early diet and the risk of coeliac disease. An update 2024 position paper by the ESPGHAN special interest group on coeliac disease.","authors":"Hania Szajewska, Raanan Shamir, Renata Auricchio, Anna Chmielewska, Jernej Dolinsek, Laura Kivelä, Sibylle Koletzko, Ilma R Korponay-Szabo, Elin M Hård Af Segerstad, M Luisa Mearin, Caroline Meijer-Boekel, Carmen Ribes Konickx, Alfonso Rodriguez-Herrera, Ketil Stordal, Riccardo Troncone, Margreet Wessels","doi":"10.1002/jpn3.12280","DOIUrl":"10.1002/jpn3.12280","url":null,"abstract":"<p><p>This position paper by the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) Special Interest Group on Coeliac Disease (SIG-CD) presents an update to the 2016 recommendations concerning early diet and the risk of coeliac disease (CD). This update adheres to the policy that mandates reviewing guidelines every 5 years, particularly when new data emerge. The 2024 statements and recommendations are essentially similar to the 2016 recommendations. Breastfeeding, whether any amount, exclusive, or of any duration, does not reduce the risk of developing CD. Introducing gluten into an infant's diet at any time between completed 4 months (≥17 weeks) and 12 months of age does not affect the cumulative incidence of CD, although earlier introduction may lead to earlier seroconversion and CD. In observational studies involving cohorts with a known risk for CD, consuming a high amount of gluten compared to a low amount during weaning and in the subsequent childhood years-specifically the first 2-3 years, and even up to 5 years in some studies-was associated with an increased risk for CD. However, the specific optimal amounts of gluten consumption remain undetermined due to insufficient evidence on safe thresholds, and the impact of restricting gluten in the diet of healthy children of unknown risk for CD is unknown. Thus, any recommendation on the gluten amount is currently unjustifiable for the general population and infants with known HLA risk types. There is no specific guidance on the type of gluten-containing foods to be introduced at weaning.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141283949","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}
Pub Date : 2024-08-01Epub Date: 2024-06-27DOI: 10.1002/jpn3.12241
Peri Millman, Ramit M Rimon, Chani Toff, Martin Engvall, Ron Shaoul, Michael Wilschanski, Hila Elyashar, Harland S Winter
Objectives: Diacylglycerol acyltransferase (DGAT) catalyzes the final step in triglyceride synthesis. DGAT1 is expressed in human enterocytes and is essential for fat absorption. Homozygous DGAT1 deficiency often presents with severe diarrhea and protein-losing enteropathy (PLE) in the 1st weeks of life. Because severe restriction of fat intake controls diarrhea and decreases PLE, total parenteral nutrition (TPN) was the initial standard therapy in infants and children. We present tertiary center experience managing infants and children with DGAT1 deficiency resulting in the development of a nutritional approach that minimizes the use of TPN.
Methods: From 2014 to 2020, 12 infants with DGAT1 deficiency were treated. Stool output, growth, and development, as well as essential fatty acid status, were monitored. This retrospective experience formed the basis for treatment recommendations, which include an ultralow fat formula with intermittent peripheral intravenous lipid infusions during the 1st year of life.
Results: All patients with prolonged intestinal fat exposure had PLE, which resolved when treated with the nutrition protocol. Essential fatty acid status as measured by triene:tetraene ratios normalized in all treated patients. Over time, early genetic diagnosis and prompt initiation of an ultralow fat diet with peripheral intravenous lipid infusions replaced the need for TPN.
Conclusions: Children with DGAT1 deficiency respond to dietary restriction of lipids. Management with a novel nutritional approach provides effective treatment for infants with DGAT1 deficiency, treats diarrhea and PLE, promotes growth and development, avoids TPN dependency, and decreases the potential for essential fatty acid deficiency.
{"title":"A novel nutritional approach to infants and children with congenital diarrhea due to homozygous DGAT1 mutations.","authors":"Peri Millman, Ramit M Rimon, Chani Toff, Martin Engvall, Ron Shaoul, Michael Wilschanski, Hila Elyashar, Harland S Winter","doi":"10.1002/jpn3.12241","DOIUrl":"10.1002/jpn3.12241","url":null,"abstract":"<p><strong>Objectives: </strong>Diacylglycerol acyltransferase (DGAT) catalyzes the final step in triglyceride synthesis. DGAT1 is expressed in human enterocytes and is essential for fat absorption. Homozygous DGAT1 deficiency often presents with severe diarrhea and protein-losing enteropathy (PLE) in the 1st weeks of life. Because severe restriction of fat intake controls diarrhea and decreases PLE, total parenteral nutrition (TPN) was the initial standard therapy in infants and children. We present tertiary center experience managing infants and children with DGAT1 deficiency resulting in the development of a nutritional approach that minimizes the use of TPN.</p><p><strong>Methods: </strong>From 2014 to 2020, 12 infants with DGAT1 deficiency were treated. Stool output, growth, and development, as well as essential fatty acid status, were monitored. This retrospective experience formed the basis for treatment recommendations, which include an ultralow fat formula with intermittent peripheral intravenous lipid infusions during the 1st year of life.</p><p><strong>Results: </strong>All patients with prolonged intestinal fat exposure had PLE, which resolved when treated with the nutrition protocol. Essential fatty acid status as measured by triene:tetraene ratios normalized in all treated patients. Over time, early genetic diagnosis and prompt initiation of an ultralow fat diet with peripheral intravenous lipid infusions replaced the need for TPN.</p><p><strong>Conclusions: </strong>Children with DGAT1 deficiency respond to dietary restriction of lipids. Management with a novel nutritional approach provides effective treatment for infants with DGAT1 deficiency, treats diarrhea and PLE, promotes growth and development, avoids TPN dependency, and decreases the potential for essential fatty acid deficiency.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141457576","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}
Pub Date : 2024-08-01Epub Date: 2024-06-14DOI: 10.1002/jpn3.12284
Emmanuel Schneck, Fabienne Knittel, Melanie Markmann, Felix Balzer, Kerstin Rubarth, Thomas Zajonz, Anna-Lena Schreiner, Andreas Hecker, Lutz Naehrlich, Christian Koch, Jan de Laffolie, Michael Sander
Objectives: Data regarding the occurrence of complications specifically during pediatric anesthesia for endoscopic procedures is limited. By evaluating such data, factors could be identified to assure proper staffing and preparation to minimize adverse events and improve patient safety during flexible endoscopy.
Methods: This retrospective cohort study included children undergoing anesthesia for gastroscopy, colonoscopy, bronchoscopy, or combined endoscopic procedures over 10-year period. The primary study aim was to evaluate the incidence of complications and identify risk factors for adverse events.
Results: Overall, 2064 endoscopic procedures including 1356 gastroscopies (65.7%), 93 colonoscopies (4.5%), 235 bronchoscopies (11.4%), and 380 combined procedures (18.4%) were performed. Of the 1613 patients, 151 (7.3%) patients exhibited an adverse event, with respiratory complications being the most common (65 [3.1%]). Combination of gastrointestinal endoscopies did not lead to an increased adverse event rate (gastroscopy: 5.5%, colonoscopy: 3.2%). Diagnostic endoscopy as compared to interventional had a lower rate. If bronchoscopy was performed, the rate was similar to that of bronchoscopy alone (19.5% vs. 20.4%). Age < 5.8 years or body weight less than 20 kg, bronchoscopy, American Society of Anesthesiologists status ≥ 2 or pre-existing anesthesia-relevant diseases, and urgency of the procedure were independent risk factors for adverse events. For each risk factor, the risk for events increased 2.1-fold [1.8-2.4].
Conclusions: This study identifies multiple factors that increase the rate of adverse events associated anesthesia-based endoscopy. Combined gastrointestinal procedures did not increase the risk for adverse events while combination of bronchoscopy to gastrointestinal endoscopy showed a similar risk as bronchoscopy alone.
{"title":"Assessment of risk factors for adverse events in analgosedation for pediatric endoscopy: A 10-year retrospective analysis.","authors":"Emmanuel Schneck, Fabienne Knittel, Melanie Markmann, Felix Balzer, Kerstin Rubarth, Thomas Zajonz, Anna-Lena Schreiner, Andreas Hecker, Lutz Naehrlich, Christian Koch, Jan de Laffolie, Michael Sander","doi":"10.1002/jpn3.12284","DOIUrl":"10.1002/jpn3.12284","url":null,"abstract":"<p><strong>Objectives: </strong>Data regarding the occurrence of complications specifically during pediatric anesthesia for endoscopic procedures is limited. By evaluating such data, factors could be identified to assure proper staffing and preparation to minimize adverse events and improve patient safety during flexible endoscopy.</p><p><strong>Methods: </strong>This retrospective cohort study included children undergoing anesthesia for gastroscopy, colonoscopy, bronchoscopy, or combined endoscopic procedures over 10-year period. The primary study aim was to evaluate the incidence of complications and identify risk factors for adverse events.</p><p><strong>Results: </strong>Overall, 2064 endoscopic procedures including 1356 gastroscopies (65.7%), 93 colonoscopies (4.5%), 235 bronchoscopies (11.4%), and 380 combined procedures (18.4%) were performed. Of the 1613 patients, 151 (7.3%) patients exhibited an adverse event, with respiratory complications being the most common (65 [3.1%]). Combination of gastrointestinal endoscopies did not lead to an increased adverse event rate (gastroscopy: 5.5%, colonoscopy: 3.2%). Diagnostic endoscopy as compared to interventional had a lower rate. If bronchoscopy was performed, the rate was similar to that of bronchoscopy alone (19.5% vs. 20.4%). Age < 5.8 years or body weight less than 20 kg, bronchoscopy, American Society of Anesthesiologists status ≥ 2 or pre-existing anesthesia-relevant diseases, and urgency of the procedure were independent risk factors for adverse events. For each risk factor, the risk for events increased 2.1-fold [1.8-2.4].</p><p><strong>Conclusions: </strong>This study identifies multiple factors that increase the rate of adverse events associated anesthesia-based endoscopy. Combined gastrointestinal procedures did not increase the risk for adverse events while combination of bronchoscopy to gastrointestinal endoscopy showed a similar risk as bronchoscopy alone.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141317588","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}
Pub Date : 2024-08-01Epub Date: 2024-06-24DOI: 10.1002/jpn3.12294
Valeria De Toro, Gigliola Alberti, Angelica Dominguez, Karina Carrasco-Negüe, Pedro Ferrer, Rodrigo Valenzuela, Maria Luisa Garmendia, Paola Casanello
Background: Previous studies of maternal docosahexaenoic acid (DHA) supplementation during pregnancy have controversial and contrasting results on the short and long-term effects on early child growth. The impact of this nutritional intervention on the postnatal growth patterns in the offspring of women with pregestational overweight/obesity (PGO) also remains controversial.
Objective: To analyze the postnatal growth patterns during the first 4 months of life in the offspring of women with PGO randomly supplemented with 800 mg/day (PGO-800) compared with normative doses of 200 mg/day (PGO-200) of DHA during pregnancy (<15 weeks of gestation until delivery).
Methods: This study evaluated the growth patterns during the first 4 months of life of 169 infants of the women that participated in the MIGHT study (NCT02574767). We included the infants of women from the PGO-200 (n = 81) and PGO-800 group (n = 88). The growth patterns (weight, length, and head circumference) and change in z-score (World health Organization charts) were evaluated.
Results: Throughout the first 4 months of life, the infants of the PGO-800 group had lower weight-for-length z-score (coef. -0.65, 95% confidence interval [CI] -1.07, -0.22, p = 0.003) and lower body mass index-for-age z-score (coef. -0.56, 95% CI -0.99, -0.12, p = 0.012) compared with the PGO-200 group adjusted by maternal body mass index, gestational weight gain, gestational age, insulin in cord blood and infant feeding (exclusive breastfed, not breastfed, and partially breastfed).
Conclusions: Maternal supplementation with DHA during pregnancy could beneficially limit the offspring's postnatal weight gain during the first 4 months of life.
{"title":"Growth patterns in infants born to women with pregestational overweight/obesity supplemented with docosahexaenoic acid during pregnancy.","authors":"Valeria De Toro, Gigliola Alberti, Angelica Dominguez, Karina Carrasco-Negüe, Pedro Ferrer, Rodrigo Valenzuela, Maria Luisa Garmendia, Paola Casanello","doi":"10.1002/jpn3.12294","DOIUrl":"10.1002/jpn3.12294","url":null,"abstract":"<p><strong>Background: </strong>Previous studies of maternal docosahexaenoic acid (DHA) supplementation during pregnancy have controversial and contrasting results on the short and long-term effects on early child growth. The impact of this nutritional intervention on the postnatal growth patterns in the offspring of women with pregestational overweight/obesity (PGO) also remains controversial.</p><p><strong>Objective: </strong>To analyze the postnatal growth patterns during the first 4 months of life in the offspring of women with PGO randomly supplemented with 800 mg/day (PGO-800) compared with normative doses of 200 mg/day (PGO-200) of DHA during pregnancy (<15 weeks of gestation until delivery).</p><p><strong>Methods: </strong>This study evaluated the growth patterns during the first 4 months of life of 169 infants of the women that participated in the MIGHT study (NCT02574767). We included the infants of women from the PGO-200 (n = 81) and PGO-800 group (n = 88). The growth patterns (weight, length, and head circumference) and change in z-score (World health Organization charts) were evaluated.</p><p><strong>Results: </strong>Throughout the first 4 months of life, the infants of the PGO-800 group had lower weight-for-length z-score (coef. -0.65, 95% confidence interval [CI] -1.07, -0.22, p = 0.003) and lower body mass index-for-age z-score (coef. -0.56, 95% CI -0.99, -0.12, p = 0.012) compared with the PGO-200 group adjusted by maternal body mass index, gestational weight gain, gestational age, insulin in cord blood and infant feeding (exclusive breastfed, not breastfed, and partially breastfed).</p><p><strong>Conclusions: </strong>Maternal supplementation with DHA during pregnancy could beneficially limit the offspring's postnatal weight gain during the first 4 months of life.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141457579","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}
Objective: Biliary atresia (BA) is the leading cause of liver cirrhosis and chronic liver insufficiency in children in the world. Gastroesophageal varices bleeding is an ominous complication of cirrhosis in BA patients and is associated with high morbidity and mortality. In this study, we aimed to investigate the utility of noninvasive Baveno VI and Baveno VII criteria for the screening of varices need treatment (VNT) and the need for liver transplantation in BA patients.
Methods: This study enrolled 48 BA patients (23 females and 25 males) who underwent an esophagogastroduodenoscopy (EGD) and transient elastography at a mean age of 11.18 ± 1.48 years; the clinical data were surveyed in a retrospective design.
Results: The sensitivity and negative predictive value of Baveno VI and Baveno VII criteria for the prediction of VNT in BA patients are both 100% and 100%, respectively. The VNT missing rate of Baveno VI and Baveno VII criteria are both 0% in our cohort. The Baveno VI, expanded Baveno VI, and Baveno VII criteria are also predictive of the need for liver transplantation in our cohort (OR = 10.33, 4.24, and 21.33; p = 0.009, 0.03, and 0.007, respectively).
Conclusion: The Baveno VI and Baveno VII criteria are useful for the screening of VNT and minimize non-necessary invasive EGD in BA patients with low VNT missing rates. The Baveno VI, expanded Baveno VI, and Baveno VII criteria are associated with the need for liver transplantation.
目的:胆道闭锁(BA)是世界上导致儿童肝硬化和慢性肝功能不全的主要原因。胃食管静脉曲张出血是 BA 患者肝硬化的一种不祥并发症,与高发病率和高死亡率相关。在这项研究中,我们旨在探讨无创的巴韦诺 VI 和巴韦诺 VII 标准在筛查胃食管静脉曲张是否需要治疗(VNT)以及 BA 患者是否需要肝移植方面的实用性:该研究共纳入了48名接受食管胃十二指肠镜(EGD)和瞬态弹性成像检查的BA患者(23名女性和25名男性),他们的平均年龄为(11.18 ± 1.48)岁;临床数据采用回顾性设计:结果:巴韦诺 VI 和巴韦诺 VII 标准预测 BA 患者 VNT 的灵敏度和阴性预测值分别为 100%和 100%。在我们的队列中,巴韦诺 VI 和巴韦诺 VII 标准的 VNT 缺失率均为 0%。在我们的队列中,巴韦诺 VI、扩展巴韦诺 VI 和巴韦诺 VII 标准也能预测肝移植的需求(OR = 10.33、4.24 和 21.33;P = 0.009、0.03 和 0.007):结论:巴韦诺 VI 和巴韦诺 VII 标准有助于筛查 VNT,并最大限度地减少对 VNT 缺失率低的 BA 患者进行不必要的侵入性 EGD。巴韦诺 VI、扩展巴韦诺 VI 和巴韦诺 VII 标准与肝移植的需求有关。
{"title":"Performance of Baveno VII criteria for the screening of varices needing treatment in patients with biliary atresia.","authors":"Yu-Chieh Ling, Chien-Ting Hsu, Cheng-Yu Chen, Chi-San Tai, Kai-Chi Chang, Jia-Feng Wu","doi":"10.1002/jpn3.12278","DOIUrl":"10.1002/jpn3.12278","url":null,"abstract":"<p><strong>Objective: </strong>Biliary atresia (BA) is the leading cause of liver cirrhosis and chronic liver insufficiency in children in the world. Gastroesophageal varices bleeding is an ominous complication of cirrhosis in BA patients and is associated with high morbidity and mortality. In this study, we aimed to investigate the utility of noninvasive Baveno VI and Baveno VII criteria for the screening of varices need treatment (VNT) and the need for liver transplantation in BA patients.</p><p><strong>Methods: </strong>This study enrolled 48 BA patients (23 females and 25 males) who underwent an esophagogastroduodenoscopy (EGD) and transient elastography at a mean age of 11.18 ± 1.48 years; the clinical data were surveyed in a retrospective design.</p><p><strong>Results: </strong>The sensitivity and negative predictive value of Baveno VI and Baveno VII criteria for the prediction of VNT in BA patients are both 100% and 100%, respectively. The VNT missing rate of Baveno VI and Baveno VII criteria are both 0% in our cohort. The Baveno VI, expanded Baveno VI, and Baveno VII criteria are also predictive of the need for liver transplantation in our cohort (OR = 10.33, 4.24, and 21.33; p = 0.009, 0.03, and 0.007, respectively).</p><p><strong>Conclusion: </strong>The Baveno VI and Baveno VII criteria are useful for the screening of VNT and minimize non-necessary invasive EGD in BA patients with low VNT missing rates. The Baveno VI, expanded Baveno VI, and Baveno VII criteria are associated with the need for liver transplantation.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141200206","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}
Background: Meso-Rex bypass is the surgical intervention of choice for children with extrahepatic portal vein obstruction (EHPVO). Patency of Rex vein, umbilical recessus of the portal vein, is a prerequisite for this surgery. Conventional diagnostic modalities poorly detect patency, while transjugular wedged hepatic vein portography (WHVP) accurately detects patency in 90%.
Objectives: We aimed to assess Rex vein patency and portal vein branching pattern in children with EHPVO using transjugular WHVP and to identify factors associated with Rex vein patency.
Methods: Transjugular WHVP was performed in 31 children with EHPVO by selective cannulation of left and right hepatic veins. Rex vein patency, type of intrahepatic portal venous anatomy (Types A-E), and factors associated with patency of Rex vein were studied.
Results: The patency of Rex recess on transjugular WHVP was 29%. Complete obliteration of intrahepatic portal venous radicles was the commonest pattern (Type E, 38.7%) while Type A, the favorable anatomy for meso-Rex bypass, was seen in only 12.9%. Patency of the Rex vein, but not the anatomical pattern, was associated with younger age at evaluation (patent Rex: 6.6 ± 4.9 years vs. nonpatent Rex: 12.7 ± 3.9 years, p = 0.001). Under-5-year children had a 12 times greater chance of having a patent Rex vein (odds ratio: 12.22, 95% confidence interval: 1.65-90.40, p = 0.004). Patency or pattern was unrelated to local factors like umbilical vein catheterization, systemic thrombophilia, or disease severity.
Conclusion: Less than one-third of our pediatric EHPVO patients have a patent Rex vein. Younger age at evaluation is significantly associated with Rex vein patency.
{"title":"Wedged hepatic vein portovenography for assessment of Rex vein patency in children with extrahepatic portal venous obstruction.","authors":"Prabhsaran Kaur, Rajeev Khanna, Vikrant Sood, Bikrant Bihari Lal, Amar Mukund, Ragini Kilambi, Seema Alam","doi":"10.1002/jpn3.12282","DOIUrl":"10.1002/jpn3.12282","url":null,"abstract":"<p><strong>Background: </strong>Meso-Rex bypass is the surgical intervention of choice for children with extrahepatic portal vein obstruction (EHPVO). Patency of Rex vein, umbilical recessus of the portal vein, is a prerequisite for this surgery. Conventional diagnostic modalities poorly detect patency, while transjugular wedged hepatic vein portography (WHVP) accurately detects patency in 90%.</p><p><strong>Objectives: </strong>We aimed to assess Rex vein patency and portal vein branching pattern in children with EHPVO using transjugular WHVP and to identify factors associated with Rex vein patency.</p><p><strong>Methods: </strong>Transjugular WHVP was performed in 31 children with EHPVO by selective cannulation of left and right hepatic veins. Rex vein patency, type of intrahepatic portal venous anatomy (Types A-E), and factors associated with patency of Rex vein were studied.</p><p><strong>Results: </strong>The patency of Rex recess on transjugular WHVP was 29%. Complete obliteration of intrahepatic portal venous radicles was the commonest pattern (Type E, 38.7%) while Type A, the favorable anatomy for meso-Rex bypass, was seen in only 12.9%. Patency of the Rex vein, but not the anatomical pattern, was associated with younger age at evaluation (patent Rex: 6.6 ± 4.9 years vs. nonpatent Rex: 12.7 ± 3.9 years, p = 0.001). Under-5-year children had a 12 times greater chance of having a patent Rex vein (odds ratio: 12.22, 95% confidence interval: 1.65-90.40, p = 0.004). Patency or pattern was unrelated to local factors like umbilical vein catheterization, systemic thrombophilia, or disease severity.</p><p><strong>Conclusion: </strong>Less than one-third of our pediatric EHPVO patients have a patent Rex vein. Younger age at evaluation is significantly associated with Rex vein patency.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141283950","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}
Pub Date : 2024-08-01Epub Date: 2024-06-24DOI: 10.1002/jpn3.12188
Jorge Amil-Dias, Salvatore Oliva, Alexandra Papadopoulou, Mike Thomson, Carolina Gutiérrez-Junquera, Nicolas Kalach, Rok Orel, Marcus Karl-Heinz Auth, Danielle Nijenhuis-Hendriks, Caterina Strisciuglio, Olivia Bauraind, Sonny Chong, Gloria Dominguez Ortega, Sonia Férnandez Férnandez, Mark Furman, Roger Garcia-Puig, Frederic Gottrand, Matjaz Homan, Koen Huysentruyt, Aco Kostovski, Sebastian Otte, Francesca Rea, Eleftheria Roma, Claudio Romano, Christos Tzivinikos, Vaidotas Urbonas, Saskia Vande Velde, Tsili Zangen, Noam Zevit
Introduction: Eosinophilic esophagitis (EoE) is a chronic inflammatory disease of the esophagus characterized by symptoms of esophageal dysfunction and histologically by predominantly eosinophilic infiltration of the squamous epithelium. European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) published a guideline in 2014; however, the rapid evolution of knowledge about pathophysiology, diagnostic criteria, and therapeutic options have made an update necessary.
Methods: A consensus group of pediatric gastroenterologists from the ESPGHAN Working Group on Eosinophilic Gastrointestinal Diseases (ESPGHAN EGID WG) reviewed the recent literature and proposed statements and recommendations on 28 relevant questions about EoE. A comprehensive electronic literature search was performed in MEDLINE, EMBASE, and Cochrane databases from 2014 to 2022. The Grading of Recommendations Assessment, Development and Evaluation system was used to assess the quality of evidence and formulate recommendations.
Results: A total of 52 statements based on the available evidence and 44 consensus-based recommendations are available. A revision of the diagnostic protocol, options for initial drug treatment, and the new concept of simplified empiric elimination diets are now available. Biologics are becoming a part of the potential armamentarium for refractory EoE, and systemic steroids may be considered as the initial treatment for esophageal strictures before esophageal dilation. The importance and assessment of quality of life and a planned transition to adult medical care are new areas addressed in this guideline.
Conclusion: Research in recent years has led to a better understanding of childhood EoE. This guideline incorporates the new findings and provides a practical guide for clinicians treating children diagnosed with EoE.
{"title":"Diagnosis and management of eosinophilic esophagitis in children: An update from the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN).","authors":"Jorge Amil-Dias, Salvatore Oliva, Alexandra Papadopoulou, Mike Thomson, Carolina Gutiérrez-Junquera, Nicolas Kalach, Rok Orel, Marcus Karl-Heinz Auth, Danielle Nijenhuis-Hendriks, Caterina Strisciuglio, Olivia Bauraind, Sonny Chong, Gloria Dominguez Ortega, Sonia Férnandez Férnandez, Mark Furman, Roger Garcia-Puig, Frederic Gottrand, Matjaz Homan, Koen Huysentruyt, Aco Kostovski, Sebastian Otte, Francesca Rea, Eleftheria Roma, Claudio Romano, Christos Tzivinikos, Vaidotas Urbonas, Saskia Vande Velde, Tsili Zangen, Noam Zevit","doi":"10.1002/jpn3.12188","DOIUrl":"10.1002/jpn3.12188","url":null,"abstract":"<p><strong>Introduction: </strong>Eosinophilic esophagitis (EoE) is a chronic inflammatory disease of the esophagus characterized by symptoms of esophageal dysfunction and histologically by predominantly eosinophilic infiltration of the squamous epithelium. European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) published a guideline in 2014; however, the rapid evolution of knowledge about pathophysiology, diagnostic criteria, and therapeutic options have made an update necessary.</p><p><strong>Methods: </strong>A consensus group of pediatric gastroenterologists from the ESPGHAN Working Group on Eosinophilic Gastrointestinal Diseases (ESPGHAN EGID WG) reviewed the recent literature and proposed statements and recommendations on 28 relevant questions about EoE. A comprehensive electronic literature search was performed in MEDLINE, EMBASE, and Cochrane databases from 2014 to 2022. The Grading of Recommendations Assessment, Development and Evaluation system was used to assess the quality of evidence and formulate recommendations.</p><p><strong>Results: </strong>A total of 52 statements based on the available evidence and 44 consensus-based recommendations are available. A revision of the diagnostic protocol, options for initial drug treatment, and the new concept of simplified empiric elimination diets are now available. Biologics are becoming a part of the potential armamentarium for refractory EoE, and systemic steroids may be considered as the initial treatment for esophageal strictures before esophageal dilation. The importance and assessment of quality of life and a planned transition to adult medical care are new areas addressed in this guideline.</p><p><strong>Conclusion: </strong>Research in recent years has led to a better understanding of childhood EoE. This guideline incorporates the new findings and provides a practical guide for clinicians treating children diagnosed with EoE.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141457578","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}
Pub Date : 2024-08-01Epub Date: 2024-06-14DOI: 10.1002/jpn3.12281
Marta Biernacka, Anna Jakubowska-Winecka, Marcin Biernacki, Kamil Janowski, Wojciech Jańczyk, Piotr Socha
Patients with Wilson's disease (WD) are at increased risk of poor quality of life (QoL) and social-emotional outcomes. The above data has been well established in the adult population. What are the predictors of QoL in children and adolescents with WD are unknown. Our study examined whether subjective feelings about QoL are related to the psychosocial functioning in paediatric patients. A cross-sectional study among 50 children with WD, aged 7-18 years. Participants completed the KINDL QoL questionnaire and the Child Behavior Checklist assessing internalizing and externalizing behaviors. Internalizing and externalizing behaviors and their interaction are significant in predicting the QoL of children with WD. Internalizing behaviors are significant predictor of the QoL β = -0.328 (p < 0.05). The effect of internalizing behavior on the QoL varies with the level of externalizing behavior β = -0.344* (p < 0.05). Simple effects analysis indicates that the highest QoL for children with WD is in the group characterized by both low levels of internalizing and medium levels of externalizing behaviors, t = -3.052 (df = 46) and p < 0.01, or high levels of externalizing behaviors, t = -2.725 (df = 46) p < 0.01. The interaction between internalizing behaviors explained an additional 7.5% of the variance in scores on the QoL scale. Overall, the final regression model explained 14.9% of the scores on the QoL scale. Monitoring internalizing and externalizing behaviors will allow a better understanding of the course of treatment. In chronic disease, the QoL is an aspect that determines the doctor-patient relationship and often determines the course of the therapeutic process.
威尔逊氏病(WD)患者生活质量(QoL)和社会情感状况不佳的风险增加。上述数据已在成人人群中得到证实。对于患有 WD 的儿童和青少年来说,生活质量的预测因素是什么尚不清楚。我们的研究探讨了有关 QoL 的主观感受是否与儿科患者的社会心理功能有关。这是一项横断面研究,研究对象为 50 名患有 WD 的 7-18 岁儿童。参与者填写了 KINDL QoL 问卷和评估内化和外化行为的儿童行为核对表。内化行为和外化行为及其交互作用对预测 WD 儿童的 QoL 有显著影响。内化行为可显著预测 QoL β = -0.328 (p
{"title":"Internalizing and externalizing behaviors in children and adolescents with Wilson's disease in the context of quality of life.","authors":"Marta Biernacka, Anna Jakubowska-Winecka, Marcin Biernacki, Kamil Janowski, Wojciech Jańczyk, Piotr Socha","doi":"10.1002/jpn3.12281","DOIUrl":"10.1002/jpn3.12281","url":null,"abstract":"<p><p>Patients with Wilson's disease (WD) are at increased risk of poor quality of life (QoL) and social-emotional outcomes. The above data has been well established in the adult population. What are the predictors of QoL in children and adolescents with WD are unknown. Our study examined whether subjective feelings about QoL are related to the psychosocial functioning in paediatric patients. A cross-sectional study among 50 children with WD, aged 7-18 years. Participants completed the KINDL QoL questionnaire and the Child Behavior Checklist assessing internalizing and externalizing behaviors. Internalizing and externalizing behaviors and their interaction are significant in predicting the QoL of children with WD. Internalizing behaviors are significant predictor of the QoL β = -0.328 (p < 0.05). The effect of internalizing behavior on the QoL varies with the level of externalizing behavior β = -0.344* (p < 0.05). Simple effects analysis indicates that the highest QoL for children with WD is in the group characterized by both low levels of internalizing and medium levels of externalizing behaviors, t = -3.052 (df = 46) and p < 0.01, or high levels of externalizing behaviors, t = -2.725 (df = 46) p < 0.01. The interaction between internalizing behaviors explained an additional 7.5% of the variance in scores on the QoL scale. Overall, the final regression model explained 14.9% of the scores on the QoL scale. Monitoring internalizing and externalizing behaviors will allow a better understanding of the course of treatment. In chronic disease, the QoL is an aspect that determines the doctor-patient relationship and often determines the course of the therapeutic process.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141317589","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}