Pub Date : 2026-02-01Epub Date: 2025-12-09DOI: 10.1002/jpn3.70298
Sharef Al-Mulaabed, Joanne Thio, Vijay Mehta, Justin de Boer, Shaista Safder, Mary Schreck, Yamen Smadi
Objectives: We aim to categorize the mechanical properties of the esophagus in children with eosinophilic esophagitis (EoE) in different disease stages and based on different management modalities, using endoscopic functional luminal imaging probe (EndoFLIP) which has been established to objectively evaluate mechanical properties of the esophagus in patients with EoE.
Methods: We performed a retrospective analysis of children who completed EndoFLIP during sedated endoscopy over 2 years. Patients with EoE were categorized into EoE remission or active EoE based on published guidelines. Control subjects were those with normal endoscopy, esophageal biopsies, and EndoFLIP parameters. EndoFLIP studies were analyzed for distensibility index (DI) and maximum diameter (MD) at the esophagogastric junction (EGJ), as well as contractile response (CR).
Results: We included 130 subjects, 60 controls, and 70 with EoE (34 [49%] had active EoE and 36 [51%] were in remission). DI and MD were significantly lower in active EoE compared to controls (p < 0.001). DI and MD were significantly lower in initial (baseline) EoE compared to active EoE on therapy (p < 0.05) and EoE remission (p < 0.001). There was no statistically significant difference in DI and MD between EoE subgroups on different therapies.
Conclusion: Assessment of esophageal mechanical properties using EndoFLIP provides valuable information on disease severity in pediatric EoE. In our cohort, histological remission achieved with therapies such as proton pump inhibitors, topical steroids, and dupilumab was associated with improved distensibility and diameter at the EGJ. However, a subset of patients continued to demonstrate abnormal EndoFLIP findings despite histologic improvement.
{"title":"Esophageal mechanical properties in different stages of pediatric eosinophilic esophagitis and based on different therapy interventions.","authors":"Sharef Al-Mulaabed, Joanne Thio, Vijay Mehta, Justin de Boer, Shaista Safder, Mary Schreck, Yamen Smadi","doi":"10.1002/jpn3.70298","DOIUrl":"10.1002/jpn3.70298","url":null,"abstract":"<p><strong>Objectives: </strong>We aim to categorize the mechanical properties of the esophagus in children with eosinophilic esophagitis (EoE) in different disease stages and based on different management modalities, using endoscopic functional luminal imaging probe (EndoFLIP) which has been established to objectively evaluate mechanical properties of the esophagus in patients with EoE.</p><p><strong>Methods: </strong>We performed a retrospective analysis of children who completed EndoFLIP during sedated endoscopy over 2 years. Patients with EoE were categorized into EoE remission or active EoE based on published guidelines. Control subjects were those with normal endoscopy, esophageal biopsies, and EndoFLIP parameters. EndoFLIP studies were analyzed for distensibility index (DI) and maximum diameter (MD) at the esophagogastric junction (EGJ), as well as contractile response (CR).</p><p><strong>Results: </strong>We included 130 subjects, 60 controls, and 70 with EoE (34 [49%] had active EoE and 36 [51%] were in remission). DI and MD were significantly lower in active EoE compared to controls (p < 0.001). DI and MD were significantly lower in initial (baseline) EoE compared to active EoE on therapy (p < 0.05) and EoE remission (p < 0.001). There was no statistically significant difference in DI and MD between EoE subgroups on different therapies.</p><p><strong>Conclusion: </strong>Assessment of esophageal mechanical properties using EndoFLIP provides valuable information on disease severity in pediatric EoE. In our cohort, histological remission achieved with therapies such as proton pump inhibitors, topical steroids, and dupilumab was associated with improved distensibility and diameter at the EGJ. However, a subset of patients continued to demonstrate abnormal EndoFLIP findings despite histologic improvement.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"541-548"},"PeriodicalIF":2.6,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145708043","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 : 2026-02-01Epub Date: 2025-11-23DOI: 10.1002/jpn3.70272
Sakil S Kulkarni, Tess Coker, Neeta A Vachharajani, Amen Z Kiani, Angela L Hill, Janis M Stoll, Sarah Henkel, William C Chapman, Darren Cullinan, Maria M Doyle, Adeel S Khan
Objectives: Timely availability of a suitable allograft helps offset pediatric liver transplant (pLT) waitlist mortality. Candidate-donor blood type matching (CDBM) is an important attribute for allograft allocation. Current policy allows for blood type (ABO) compatible non-identical transplants for pLT candidates with high acuity. Additionally, centers may use ABO incompatible allografts for pLT candidates when required.
Methods: We conducted a retrospective analysis of the United Network for Organ Sharing (UNOS) database. We analyzed pediatric (<18 years) candidates listed for deceased donor liver-only transplant (DDLT) during the time-period 1/2008 to 12/2022. Specifically, we analyzed the impact of CDBM, including ABO non-identical LT on outcomes.
Results: 72.4% of the 8976 pLT candidates included in our analyses underwent DDLT. 19.9% of pLTs were ABO nonidentical, and 4.9% were ABO incompatible. Nonidentical pLT recipients had significantly higher listing urgency and lower waitlist times. Nonidentical pLTs led to a net loss of allografts for blood type O and net gain of allografts for all other blood types. Candidates with blood type O had significantly higher waitlist time (62.5 vs. 48 days) and lower proportion of candidates undergoing DDLT (70.2% vs. 74.7%), when compared to blood type A.
Conclusion: ABO nonidentical allografts result in timely transplant for pLT candidates with high acuity. Blood type O candidates are disadvantaged, while blood type A candidates are advantaged by ABO nonidentical allografts. The observations of our study, which differ from those of prior adult studies, could inform future policy changes pertaining to organ allocation based on CDBM.
{"title":"ABO nonidentical liver transplantation disadvantages pediatric waitlist candidates with blood group O.","authors":"Sakil S Kulkarni, Tess Coker, Neeta A Vachharajani, Amen Z Kiani, Angela L Hill, Janis M Stoll, Sarah Henkel, William C Chapman, Darren Cullinan, Maria M Doyle, Adeel S Khan","doi":"10.1002/jpn3.70272","DOIUrl":"10.1002/jpn3.70272","url":null,"abstract":"<p><strong>Objectives: </strong>Timely availability of a suitable allograft helps offset pediatric liver transplant (pLT) waitlist mortality. Candidate-donor blood type matching (CDBM) is an important attribute for allograft allocation. Current policy allows for blood type (ABO) compatible non-identical transplants for pLT candidates with high acuity. Additionally, centers may use ABO incompatible allografts for pLT candidates when required.</p><p><strong>Methods: </strong>We conducted a retrospective analysis of the United Network for Organ Sharing (UNOS) database. We analyzed pediatric (<18 years) candidates listed for deceased donor liver-only transplant (DDLT) during the time-period 1/2008 to 12/2022. Specifically, we analyzed the impact of CDBM, including ABO non-identical LT on outcomes.</p><p><strong>Results: </strong>72.4% of the 8976 pLT candidates included in our analyses underwent DDLT. 19.9% of pLTs were ABO nonidentical, and 4.9% were ABO incompatible. Nonidentical pLT recipients had significantly higher listing urgency and lower waitlist times. Nonidentical pLTs led to a net loss of allografts for blood type O and net gain of allografts for all other blood types. Candidates with blood type O had significantly higher waitlist time (62.5 vs. 48 days) and lower proportion of candidates undergoing DDLT (70.2% vs. 74.7%), when compared to blood type A.</p><p><strong>Conclusion: </strong>ABO nonidentical allografts result in timely transplant for pLT candidates with high acuity. Blood type O candidates are disadvantaged, while blood type A candidates are advantaged by ABO nonidentical allografts. The observations of our study, which differ from those of prior adult studies, could inform future policy changes pertaining to organ allocation based on CDBM.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"331-340"},"PeriodicalIF":2.6,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145588027","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 : 2026-02-01Epub Date: 2025-11-18DOI: 10.1002/jpn3.70263
Maria Graciela Parra Villasmil, Melena Bellin, Catherina Pinnaro, Fati Craighead, Gretchen Cress, Aliye Uc, Mark Lowe, James S Hodges, Katie Larson Ode
Objectives: Approximately 9% of children with acute recurrent pancreatitis (ARP) or chronic pancreatitis (CP) have pancreatogenic diabetes during childhood; lifetime risk approaches 50%. To date, data are limited on pathophysiology and biomarkers identifying those at highest risk. This pilot study investigated glycemic physiology in children with ARP or CP.
Methods: Children (5-21 years) with an established diagnosis of CP or ARP, and participants of INSPPIRE-2 (INternational Study Group of Pediatric Pancreatitis: In search for a cuRE) were enrolled. Mixed meal tolerance testing (MMTT) measured glucose, insulin, C-peptide, glucagon, glucagon-like peptide-1 (GLP-1) and pancreatic polypeptide (PP) at -5, -1, 0, 30, 60, 90, 120 min before/after a Boost HP beverage. Other measures included hemoglobin A1c, continuous glucose monitoring (CGM, Dexcom Pro), HLA haplotype, and diabetes autoantibodies. Glycemic variability metrics were calculated using "cgmanalysis." Dysglycemia was defined by fasting glucose ≥100 mg/dL or HbA1c ≥ 5.7%.
Results: Twenty participants were enrolled (mean age 16.3 years; 65% female, 60% non-Hispanic white, 2 with pre-existing diabetes). Mean HbA1c was 5.7% (range 5.0-8.9); 7/20 had dysglycemia, 1 with previously unrecognized diabetes. Those with dysglycemia differed from normoglycemic participants by having greater insulin resistance, lower GLP-1, and trend toward lower insulin and C-peptide but higher PP on MMTT.
Conclusions: In this small study, 35% of children with pancreatitis had dysglycemia, which may be mechanistically related to insulin resistance. Other trends associated with dysglycemia included impaired insulin secretion, reduced GLP-1, and unexpectedly elevated PP.
{"title":"Early mechanisms of diabetes development in pediatric pancreatitis: A pilot study.","authors":"Maria Graciela Parra Villasmil, Melena Bellin, Catherina Pinnaro, Fati Craighead, Gretchen Cress, Aliye Uc, Mark Lowe, James S Hodges, Katie Larson Ode","doi":"10.1002/jpn3.70263","DOIUrl":"10.1002/jpn3.70263","url":null,"abstract":"<p><strong>Objectives: </strong>Approximately 9% of children with acute recurrent pancreatitis (ARP) or chronic pancreatitis (CP) have pancreatogenic diabetes during childhood; lifetime risk approaches 50%. To date, data are limited on pathophysiology and biomarkers identifying those at highest risk. This pilot study investigated glycemic physiology in children with ARP or CP.</p><p><strong>Methods: </strong>Children (5-21 years) with an established diagnosis of CP or ARP, and participants of INSPPIRE-2 (INternational Study Group of Pediatric Pancreatitis: In search for a cuRE) were enrolled. Mixed meal tolerance testing (MMTT) measured glucose, insulin, C-peptide, glucagon, glucagon-like peptide-1 (GLP-1) and pancreatic polypeptide (PP) at -5, -1, 0, 30, 60, 90, 120 min before/after a Boost HP beverage. Other measures included hemoglobin A1c, continuous glucose monitoring (CGM, Dexcom Pro), HLA haplotype, and diabetes autoantibodies. Glycemic variability metrics were calculated using \"cgmanalysis.\" Dysglycemia was defined by fasting glucose ≥100 mg/dL or HbA1c ≥ 5.7%.</p><p><strong>Results: </strong>Twenty participants were enrolled (mean age 16.3 years; 65% female, 60% non-Hispanic white, 2 with pre-existing diabetes). Mean HbA1c was 5.7% (range 5.0-8.9); 7/20 had dysglycemia, 1 with previously unrecognized diabetes. Those with dysglycemia differed from normoglycemic participants by having greater insulin resistance, lower GLP-1, and trend toward lower insulin and C-peptide but higher PP on MMTT.</p><p><strong>Conclusions: </strong>In this small study, 35% of children with pancreatitis had dysglycemia, which may be mechanistically related to insulin resistance. Other trends associated with dysglycemia included impaired insulin secretion, reduced GLP-1, and unexpectedly elevated PP.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"549-556"},"PeriodicalIF":2.6,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145541008","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 : 2026-02-01Epub Date: 2025-11-29DOI: 10.1002/jpn3.70304
Melanie P Parziale, Julia R Kleinhenz, Taylor Abbey, Ritu Verma
Objectives: Celiac disease (CeD) is an autoimmune disease induced by ingestion of gluten. Patients may present with stereotypical gastrointestinal symptoms, including bloating and diarrhea, but can also present with nongastrointestinal symptoms such as migraines or rashes. The clinical presentation is well described in older pediatric and adult populations; however, the clinical picture is less clear for younger, preschool-aged pediatric patients. This retrospective chart review aims to describe the clinical presentation and serological values seen in patients diagnosed with CeD whose symptoms began before 5 years of age.
Methods: A retrospective review of a single pediatric CeD institution was conducted, and 82 children diagnosed with CeD with symptom onset before 5 years of age were identified.
Results: Children with symptom onset on or before 24 months of age were significantly more likely to have higher symptom burden (5 vs. 4, p = 0.0120). Most notable are vomiting (52.5% vs. 19%, p = 0.0024), mood changes (40% vs. 7.1%, p = 0.0005), poor weight gain (67.5% vs. 41.5%, p = 0.0164), and poor linear growth (35% vs. 11.9%, p = 0.0182). This subgroup was more likely to be hospitalized within 3 months of diagnosis (12.5% vs. 0, p = 0.0241). Children who had symptom onset after 24 months were more likely to have abdominal pain (30% vs. 59.5%, p = 0.0086) and sparser extraintestinal symptoms (p = 0.0224). No matter the age of symptom onset, there was no difference in the time interval to diagnosis.
Conclusions: This study adds to the literature evidence of the extraintestinal presentation of CeD that is seen in toddler-aged patients, as well as increased incidence of hospitalization at the time of diagnosis in this age group.
目的:乳糜泻(CeD)是一种由摄入谷蛋白引起的自身免疫性疾病。患者可能出现典型的胃肠道症状,包括腹胀和腹泻,但也可能出现非胃肠道症状,如偏头痛或皮疹。临床表现很好地描述了老年儿科和成人人群;然而,对于年龄较小的学龄前儿童患者,临床情况就不太清楚了。本回顾性图表综述旨在描述在5岁之前出现症状的诊断为CeD的患者的临床表现和血清学价值。方法:回顾性分析某儿科CeD机构82例5岁前出现症状的CeD患儿。结果:24月龄或之前出现症状的儿童更有可能出现更高的症状负担(5比4,p = 0.0120)。最显著的是呕吐(52.5%对19%,p = 0.0024),情绪变化(40%对7.1%,p = 0.0005),体重增加不佳(67.5%对41.5%,p = 0.0164),线性增长不佳(35%对11.9%,p = 0.0182)。该亚组更有可能在诊断后3个月内住院(12.5% vs. 0, p = 0.0241)。24个月后出现症状的儿童更容易出现腹痛(30% vs. 59.5%, p = 0.0086)和较少的肠外症状(p = 0.0224)。不论出现症状的年龄,到诊断的时间间隔没有差异。结论:本研究增加了文献证据,证明幼儿期患者肠外表现为CeD,并且该年龄组在诊断时住院率增加。
{"title":"Celiac disease symptomatology in patients with disease onset prior to age five.","authors":"Melanie P Parziale, Julia R Kleinhenz, Taylor Abbey, Ritu Verma","doi":"10.1002/jpn3.70304","DOIUrl":"10.1002/jpn3.70304","url":null,"abstract":"<p><strong>Objectives: </strong>Celiac disease (CeD) is an autoimmune disease induced by ingestion of gluten. Patients may present with stereotypical gastrointestinal symptoms, including bloating and diarrhea, but can also present with nongastrointestinal symptoms such as migraines or rashes. The clinical presentation is well described in older pediatric and adult populations; however, the clinical picture is less clear for younger, preschool-aged pediatric patients. This retrospective chart review aims to describe the clinical presentation and serological values seen in patients diagnosed with CeD whose symptoms began before 5 years of age.</p><p><strong>Methods: </strong>A retrospective review of a single pediatric CeD institution was conducted, and 82 children diagnosed with CeD with symptom onset before 5 years of age were identified.</p><p><strong>Results: </strong>Children with symptom onset on or before 24 months of age were significantly more likely to have higher symptom burden (5 vs. 4, p = 0.0120). Most notable are vomiting (52.5% vs. 19%, p = 0.0024), mood changes (40% vs. 7.1%, p = 0.0005), poor weight gain (67.5% vs. 41.5%, p = 0.0164), and poor linear growth (35% vs. 11.9%, p = 0.0182). This subgroup was more likely to be hospitalized within 3 months of diagnosis (12.5% vs. 0, p = 0.0241). Children who had symptom onset after 24 months were more likely to have abdominal pain (30% vs. 59.5%, p = 0.0086) and sparser extraintestinal symptoms (p = 0.0224). No matter the age of symptom onset, there was no difference in the time interval to diagnosis.</p><p><strong>Conclusions: </strong>This study adds to the literature evidence of the extraintestinal presentation of CeD that is seen in toddler-aged patients, as well as increased incidence of hospitalization at the time of diagnosis in this age group.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"524-530"},"PeriodicalIF":2.6,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145634602","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 : 2026-02-01Epub Date: 2025-11-29DOI: 10.1002/jpn3.70286
Aysenur Demirok, Hedy A van Oers, Marc A Benninga, Lotte Haverman, Merit M Tabbers
Objectives: Chronic intestinal failure (CIF) is a rare and complex disease, requiring home parenteral nutrition (HPN) to sustain growth and development. The impact on parents taking care for children with HPN remains underexplored. This qualitative study aimed to elucidate the experiences and challenges faced by parents in caring for HPN-dependent children.
Methods: Parents of children aged 0-18 years, diagnosed with CIF receiving HPN in the Emma Children's Hospital-Amsterdam UMC, were eligible. One-to-one semistructured interviews were conducted with parents at their home with open-ended questions regarding experiences around diagnosis, experiences with healthcare (both home care and at the hospital), mental health, holidays, social functioning and leisure time, work, relationship with the partner and family, fear for the future, and overall impact.
Results: Parents of 24 children were invited. Thirteen parents (four fathers and nine mothers) of 10 children (four females, median age 9 years) with HPN agreed to participate and were interviewed. Most important recurring themes among all parents, emerging from open-ended questions, were as follows: control in caregiving and reluctance to trust others to provide care over their child, social isolation due to limited time and flexibility, and importance of maintaining their own identity by self-fulfillment activities such as work, and physical or social activities.
Conclusions: This qualitative study underlines the profound impact of managing a child with HPN on daily life, relationships and well-being. There is need for tailored support and interventions to help families face the burden of having a child with HPN.
{"title":"What is the impact of having a child dependent on home parenteral nutrition? A qualitative study in mothers and fathers.","authors":"Aysenur Demirok, Hedy A van Oers, Marc A Benninga, Lotte Haverman, Merit M Tabbers","doi":"10.1002/jpn3.70286","DOIUrl":"10.1002/jpn3.70286","url":null,"abstract":"<p><strong>Objectives: </strong>Chronic intestinal failure (CIF) is a rare and complex disease, requiring home parenteral nutrition (HPN) to sustain growth and development. The impact on parents taking care for children with HPN remains underexplored. This qualitative study aimed to elucidate the experiences and challenges faced by parents in caring for HPN-dependent children.</p><p><strong>Methods: </strong>Parents of children aged 0-18 years, diagnosed with CIF receiving HPN in the Emma Children's Hospital-Amsterdam UMC, were eligible. One-to-one semistructured interviews were conducted with parents at their home with open-ended questions regarding experiences around diagnosis, experiences with healthcare (both home care and at the hospital), mental health, holidays, social functioning and leisure time, work, relationship with the partner and family, fear for the future, and overall impact.</p><p><strong>Results: </strong>Parents of 24 children were invited. Thirteen parents (four fathers and nine mothers) of 10 children (four females, median age 9 years) with HPN agreed to participate and were interviewed. Most important recurring themes among all parents, emerging from open-ended questions, were as follows: control in caregiving and reluctance to trust others to provide care over their child, social isolation due to limited time and flexibility, and importance of maintaining their own identity by self-fulfillment activities such as work, and physical or social activities.</p><p><strong>Conclusions: </strong>This qualitative study underlines the profound impact of managing a child with HPN on daily life, relationships and well-being. There is need for tailored support and interventions to help families face the burden of having a child with HPN.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"584-591"},"PeriodicalIF":2.6,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12864186/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145634710","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}
Objectives: Primary intestinal lymphangiectasia (PIL) is a very rare disease responsible for protein-losing enteropathy. There is little published data about treatments efficacy and outcomes. Our main objective was to describe the clinical profile, response to therapy, and outcomes of children with PIL.
Methods: We conducted a national retrospective study including children with PIL followed in French university hospitals between 2010 and 2022. Response to treatment was defined as clinical remission and no need for albumin infusion. Response was considered complete if albuminemia was normal (>35 g/l) during follow-up and partial if less than 35 g/l.
Results: Thirty-four children (22 males) were included; median age at diagnosis was 7 (3-29,5) months. The median follow-up in our cohort was 4.5 years. Edema (79%), chronic diarrhea (50%), and ascites (35%) were the main symptoms. Thirty-one patients received a low long-chain triglycerides dietary therapy and 25 (81%) responded: 15 had a partial response and 10 a complete response. Four patients (12%) required a second-line drug treatment. The presence of lymphedema or an identified genetic variant were associated with a partial response to diet (p < 0.05). A normal diet could be reintroduced in 14 patients (45%) without relapse during the follow-up. Among them, nine children (26%) were considered cured with a complete and prolonged remission under a normal diet.
Conclusions: Most children with PIL responded to diet therapy and about a quarter of the cohort had a good prognosis with complete remission even after discontinuation of the diet. Presence of lymphedema or a genetic variant was associated with a chronic condition.
{"title":"Clinical presentation, treatment, and outcome of children with primary intestinal lymphangiectasia: A national retrospective study.","authors":"Noémie Goret, Cécile Lambe, Emmanuelle Ecochard-Dugelay, Alexandre Fabre, Alain Dabadie, Aurélie Comte, Julie Rebeuh, Pierre Poinsot, Emilie Chaillou, Haude Clouzeau, Aurélie Sabard, Béatrice Dubern, Stéphanie Willot","doi":"10.1002/jpn3.70266","DOIUrl":"10.1002/jpn3.70266","url":null,"abstract":"<p><strong>Objectives: </strong>Primary intestinal lymphangiectasia (PIL) is a very rare disease responsible for protein-losing enteropathy. There is little published data about treatments efficacy and outcomes. Our main objective was to describe the clinical profile, response to therapy, and outcomes of children with PIL.</p><p><strong>Methods: </strong>We conducted a national retrospective study including children with PIL followed in French university hospitals between 2010 and 2022. Response to treatment was defined as clinical remission and no need for albumin infusion. Response was considered complete if albuminemia was normal (>35 g/l) during follow-up and partial if less than 35 g/l.</p><p><strong>Results: </strong>Thirty-four children (22 males) were included; median age at diagnosis was 7 (3-29,5) months. The median follow-up in our cohort was 4.5 years. Edema (79%), chronic diarrhea (50%), and ascites (35%) were the main symptoms. Thirty-one patients received a low long-chain triglycerides dietary therapy and 25 (81%) responded: 15 had a partial response and 10 a complete response. Four patients (12%) required a second-line drug treatment. The presence of lymphedema or an identified genetic variant were associated with a partial response to diet (p < 0.05). A normal diet could be reintroduced in 14 patients (45%) without relapse during the follow-up. Among them, nine children (26%) were considered cured with a complete and prolonged remission under a normal diet.</p><p><strong>Conclusions: </strong>Most children with PIL responded to diet therapy and about a quarter of the cohort had a good prognosis with complete remission even after discontinuation of the diet. Presence of lymphedema or a genetic variant was associated with a chronic condition.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"398-406"},"PeriodicalIF":2.6,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12864169/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145588057","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}
Pub Date : 2026-02-01Epub Date: 2025-11-17DOI: 10.1002/jpn3.70268
Jinjin Rong, Yanxue Lian, Pincheng Luo
{"title":"Letter to the editor: Impaired physical fitness in pediatric inflammatory bowel disease.","authors":"Jinjin Rong, Yanxue Lian, Pincheng Luo","doi":"10.1002/jpn3.70268","DOIUrl":"10.1002/jpn3.70268","url":null,"abstract":"","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"632"},"PeriodicalIF":2.6,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145540968","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 : 2026-02-01Epub Date: 2025-11-17DOI: 10.1002/jpn3.70269
Saija Kantanen, Pekka Arikoski
{"title":"Impaired physical fitness in pediatric inflammatory bowel disease.","authors":"Saija Kantanen, Pekka Arikoski","doi":"10.1002/jpn3.70269","DOIUrl":"10.1002/jpn3.70269","url":null,"abstract":"","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"633"},"PeriodicalIF":2.6,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145540992","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 : 2026-02-01Epub Date: 2025-11-18DOI: 10.1002/jpn3.70243
Manar Matar, Dita Cebecauerova, Kaija-Leena Kolho, Luca Scarallo, Seamus Hussey, Anat Yerushalmy-Feler, Gemma Pujol-Muncunill, Helena Rolandsdotter, Manuela Distante, Maya Granot, Christine Olbjørn, Cecilia K Zetterström, Marta Velasco Rodríguez-Belvís, Jan de Laffolie, Oren Ledder, Stephanie Van Biervliet, Victor Manuel Navas-López, Johan Van Limbergen, Anastasia Konidari, Ivana Čopová, Dror S Shouval
Objectives: Chronic nonbacterial osteomyelitis (CNO) is a rare, sterile autoinflammatory bone disorder that can develop in patients with inflammatory bowel disease (IBD). We aimed to identify the clinical features and natural history of patients with a dual diagnosis of CNO and IBD.
Methods: Medical records of patients with a dual diagnosis of IBD and CNO were reviewed in centers from the Paediatric IBD Porto Group and IBD Interest Group of European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN). Collected data included demographic characteristics, disease features, laboratory studies, bone imaging findings, and clinical outcomes.
Results: Forty-five patients (24 [53%] males), were included. Median age at the time of dual diagnosis was 10 (interquartile range [IQR]: 12-13) years. Thiry-two (71%) patients were diagnosed with Crohn's disease, and 14 (44%) of them exhibited perianal disease. CNO presented in 15 patients (33%) within 3 months of IBD diagnosis, and in additional 20 (44%) patients after IBD diagnosis. In most patients, CNO manifested while IBD was clinically active, but not necessarily. However, in 10 (22%) patients CNO preceded the diagnosis of IBD with a median time 46 (25-248) weeks. Upon diagnosis of CNO, most patients were treated with anti-tumor necrosis factor. CNO remission was achieved in all patients at some point during follow-up; However, complications occurred in six patients and included vertebral collapse, bone fracture, and bone deformity.
Conclusions: CNO can be associated with active or quiescent intestinal inflammation, manifesting before, during, or after the diagnosis of IBD. While CNO remission was achieved in all patients, some developed significant bone complications.
{"title":"Chronic nonbacterial osteomyelitis associated with pediatric inflammatory bowel disease: A multicenter retrospective study from the Paediatric inflammatory bowel disease Porto Group of ESPGHAN.","authors":"Manar Matar, Dita Cebecauerova, Kaija-Leena Kolho, Luca Scarallo, Seamus Hussey, Anat Yerushalmy-Feler, Gemma Pujol-Muncunill, Helena Rolandsdotter, Manuela Distante, Maya Granot, Christine Olbjørn, Cecilia K Zetterström, Marta Velasco Rodríguez-Belvís, Jan de Laffolie, Oren Ledder, Stephanie Van Biervliet, Victor Manuel Navas-López, Johan Van Limbergen, Anastasia Konidari, Ivana Čopová, Dror S Shouval","doi":"10.1002/jpn3.70243","DOIUrl":"10.1002/jpn3.70243","url":null,"abstract":"<p><strong>Objectives: </strong>Chronic nonbacterial osteomyelitis (CNO) is a rare, sterile autoinflammatory bone disorder that can develop in patients with inflammatory bowel disease (IBD). We aimed to identify the clinical features and natural history of patients with a dual diagnosis of CNO and IBD.</p><p><strong>Methods: </strong>Medical records of patients with a dual diagnosis of IBD and CNO were reviewed in centers from the Paediatric IBD Porto Group and IBD Interest Group of European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN). Collected data included demographic characteristics, disease features, laboratory studies, bone imaging findings, and clinical outcomes.</p><p><strong>Results: </strong>Forty-five patients (24 [53%] males), were included. Median age at the time of dual diagnosis was 10 (interquartile range [IQR]: 12-13) years. Thiry-two (71%) patients were diagnosed with Crohn's disease, and 14 (44%) of them exhibited perianal disease. CNO presented in 15 patients (33%) within 3 months of IBD diagnosis, and in additional 20 (44%) patients after IBD diagnosis. In most patients, CNO manifested while IBD was clinically active, but not necessarily. However, in 10 (22%) patients CNO preceded the diagnosis of IBD with a median time 46 (25-248) weeks. Upon diagnosis of CNO, most patients were treated with anti-tumor necrosis factor. CNO remission was achieved in all patients at some point during follow-up; However, complications occurred in six patients and included vertebral collapse, bone fracture, and bone deformity.</p><p><strong>Conclusions: </strong>CNO can be associated with active or quiescent intestinal inflammation, manifesting before, during, or after the diagnosis of IBD. While CNO remission was achieved in all patients, some developed significant bone complications.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"487-494"},"PeriodicalIF":2.6,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145540854","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 : 2026-02-01Epub Date: 2025-11-27DOI: 10.1002/jpn3.70265
Patil Kavarian, Parnia Abrishamchian, Peacha Sokzini, Nasim Sabery Khavari, Javier A Lopez-Rivera, Hilary Jericho
Objective: Celiac disease (CeD) is a chronic autoimmune condition triggered by gluten consumption. CeD is screened for using total serum immunoglobulin A (IgA) and tissue transglutaminase immunoglobulin A antibodies (tTG IgA Ab). This study aimed to evaluate IgA patterns over time in patients diagnosed with CeD during childhood.
Methods: A retrospective chart review was performed at Lucile Packard Children's Hospital (January 2012-December 2023). Patients diagnosed before 18 years of age with biopsy-confirmed CeD or those who met European Society of Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) nonbiopsy diagnostic criteria were included. Total serum IgA and tTG IgA Ab levels were required at diagnosis and two subsequent time points. Patients with missing data were excluded.
Results: Of 1861 identified patients, 365 met study criteria. The average age at diagnosis was 9 years old, with an average follow-up time of 5 years. Sixteen patients (4.4%) were IgA insufficient at diagnosis; 11 (69%) remained insufficient while five (31%) became sufficient over the observation period. Nineteen patients (5.2%) initially identified as IgA sufficient developed IgA insufficiency.
Conclusions: IgA levels can fluctuate over time and should be monitored routinely to assess IgA sufficiency and ensure reliable tTG IgA Ab levels. Patients who regain IgA sufficiency can transition to tTG IgA Ab testing, allowing for more reliable assessments of mucosal healing and reducing the need for invasive procedures.
目的:乳糜泻(CeD)是一种由麸质摄入引发的慢性自身免疫性疾病。使用血清总免疫球蛋白A (IgA)和组织转谷氨酰胺酶免疫球蛋白A抗体(tTG IgA Ab)筛选CeD。本研究旨在评估儿童期诊断为CeD的患者的IgA模式随时间的变化。方法:回顾性分析Lucile Packard儿童医院(2012年1月- 2023年12月)的病历。患者在18岁之前被诊断为活检证实的CeD或符合ESPGHAN非活检诊断标准。在诊断和随后的两个时间点需要血清总IgA和tTG IgA Ab水平。排除资料缺失的患者。结果:1861例确诊患者中,365例符合研究标准。确诊时平均年龄为9岁,平均随访时间为5年。16例(4.4%)诊断时IgA不足;11例(69%)仍然不足,5例(31%)在观察期间变得足够。最初确定为IgA充足的19例患者(5.2%)发展为IgA不足。结论:IgA水平可随时间波动,应常规监测,以评估IgA充分性,并确保可靠的tTG IgA Ab水平。恢复IgA充足的患者可以过渡到tTG IgA Ab检测,允许更可靠的评估粘膜愈合并减少侵入性手术的需要。
{"title":"The role of continued immunoglobulin A monitoring in celiac disease management.","authors":"Patil Kavarian, Parnia Abrishamchian, Peacha Sokzini, Nasim Sabery Khavari, Javier A Lopez-Rivera, Hilary Jericho","doi":"10.1002/jpn3.70265","DOIUrl":"10.1002/jpn3.70265","url":null,"abstract":"<p><strong>Objective: </strong>Celiac disease (CeD) is a chronic autoimmune condition triggered by gluten consumption. CeD is screened for using total serum immunoglobulin A (IgA) and tissue transglutaminase immunoglobulin A antibodies (tTG IgA Ab). This study aimed to evaluate IgA patterns over time in patients diagnosed with CeD during childhood.</p><p><strong>Methods: </strong>A retrospective chart review was performed at Lucile Packard Children's Hospital (January 2012-December 2023). Patients diagnosed before 18 years of age with biopsy-confirmed CeD or those who met European Society of Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) nonbiopsy diagnostic criteria were included. Total serum IgA and tTG IgA Ab levels were required at diagnosis and two subsequent time points. Patients with missing data were excluded.</p><p><strong>Results: </strong>Of 1861 identified patients, 365 met study criteria. The average age at diagnosis was 9 years old, with an average follow-up time of 5 years. Sixteen patients (4.4%) were IgA insufficient at diagnosis; 11 (69%) remained insufficient while five (31%) became sufficient over the observation period. Nineteen patients (5.2%) initially identified as IgA sufficient developed IgA insufficiency.</p><p><strong>Conclusions: </strong>IgA levels can fluctuate over time and should be monitored routinely to assess IgA sufficiency and ensure reliable tTG IgA Ab levels. Patients who regain IgA sufficiency can transition to tTG IgA Ab testing, allowing for more reliable assessments of mucosal healing and reducing the need for invasive procedures.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"516-523"},"PeriodicalIF":2.6,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145634755","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}