In children with acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP), circulating islet autoantibodies (auto-Ab) may influence β-cell function. This study reports Ab prevalence in youth with ARP or CP and investigates effects on indices of insulin secretion during mixed meal tolerance testing (MMTT) and diabetes status.

This was a retrospective cross-sectional analysis of 234 youth with ARP or CP who had islet Ab testing (Ab+ group, n = 28 [12%]; Ab- group, n = 206 [88%]). Fasting glucose and HbA1c were collected. MMTT was performed in 78% of participants (183 of 234). MMTT-derived indices were calculated and compared between groups.

The Ab+ and Ab- groups did not differ in age, sex, race, ethnicity, BMI percentile, or fasting glucose. Of Ab+ patients, 54% had one Ab+ islet and 46% had multiple Ab+ islets. Comparing the Ab+ to Ab- groups, HbA1c was higher (median 5.7 vs. 5.2%, P < 0.01), and C-peptide was lower (median 2.4 vs. 3.7 ng/mL, P = 0.01) in the Ab+ group. The Ab+ compared with the Ab- group had a higher proportion of prediabetes/diabetes (57% vs. 32%, P < 0.001). In survival analysis, the Ab+ group had significantly shorter time from first acute pancreatitis episode to diabetes development (P = 0.02).

In children with ARP or CP, Ab+ was associated with higher risk of diabetes/diabetes development and shorter time to diabetes development, suggesting that islet Ab+ is associated with β-cell dysfunction in this patient cohort. Islet Ab+ was also associated with higher HbA1c and lower C-peptide levels. Future studies are needed to validate the role of islet Ab positivity in pancreatitis.

Celiac disease (CD) is a chronic and immune-mediated disorder triggered by the ingestion of gluten in some genetically predisposed individuals. CD can be associated with extra-gastrointestinal manifestations and diseases affecting several organs. In this review, the aim is to analyze and discuss the pancreatic alterations and/or comorbidities that could arise in the context of pediatric CD. Exocrine pancreatic insufficiency (EPI) can be observed in a variable fraction (up to 30%) of children diagnosed with CD at the diagnosis; indeed, it usually resolves after the implementation of a gluten-free diet (GFD). The main pathophysiological mechanisms of EPI could be represented by the impaired pattern of gastrointestinal hormones in CD patients. Conversely, pancreatitis seems to be a very rare comorbidity in CD children, since very few cases have been described in children. Therefore, there is no evidence that pancreatitis (including autoimmune forms) represents a relevant comorbidity in pediatric CD. Type 1 diabetes mellitus (T1DM) is a well-known and frequent comorbidity in CD children. The main determinant of this epidemiological association is the common HLA-related predisposing background, even if other (non-HLA-related) genetic and environmental factors (viruses, gut microbiome, and others) are likely to be also implicated in the development of both these autoimmune diseases. T1DM children with concomitant CD may experience specific challenges in the adherence to GFD, which has no negative impact on the glycemic and, in general, metabolic control of diabetes, if it is properly implemented and followed up.

Acute pancreatitis (AP) in children presents unique challenges distinct from adult manifestations, requiring specialized diagnostic and therapeutic approaches. Compared with adults, pediatric AP has lower mortality rates but still carries significant morbidity and potential long-term complications. This review examines current evidence on pediatric AP, highlighting recent advances in diagnosis, risk stratification, and management strategies. Current diagnostic approaches use serum lipase and amylase testing, along with various imaging modalities that have different diagnostic values. Recent research has identified promising biomarkers for predicting severe AP, including blood urea nitrogen, C-reactive protein, and specific cytokine signals. Emerging evidence suggests a role of gut microbiome dysbiosis in disease pathogenesis, opening new therapeutic possibilities targeting the gut-pancreas axis. Genetic factors, specifically pancreatitis risk genes, influence disease progression to recurrent and chronic pancreatitis. In this review, we summarize the consequences of an isolated AP episode in children. Our review highlights for the first time how AP can lead to significant long-term sequelae, including exocrine/nutritional deficiencies, endocrine pancreatic dysfunction, diabetes, recurrent pain, and decreased quality of life compared with healthy population controls. The goal of this review is to summarize advances in understanding of pediatric AP and to emphasize the importance of early recognition, appropriate risk stratification, and comprehensive follow-up after the first pediatric AP episode, while highlighting areas requiring future research to optimize patient outcomes.

Chronic pancreatitis is a fibro-inflammatory syndrome of the pancreas with a prevalence in children of approximately 6 per 100,000. Diagnosis of chronic pancreatitis in a child depends on the presence of imaging findings of chronic pancreatitis in the context of specific clinical features. Currently, a standardized reporting system for imaging findings of chronic pancreatitis in children is lacking, and imaging-based thresholds for abnormality have not been defined. Standardized reporting elements were defined for adults in 2019. Not all of these criteria are directly applicable to children due to recognized changes in the pancreas with normal growth and development. To address the lack of accepted pediatric chronic pancreatitis reporting standards and encourage standardized communication of imaging findings, we convened a group of experienced pediatric radiologists and pediatric gastroenterologists with expertise in pancreatology and interventional endoscopy to define consensus reporting elements and interpretive criteria for findings of pediatric chronic pancreatitis. Based on the existing literature and panel opinion, while leveraging a modified Delphi approach, we propose reporting standards for CT, MRI, and MRCP of pediatric chronic pancreatitis.

Our primary purpose was to understand the correlation between pancreas T1-weighted signal intensity ratio (SIR) and T1 relaxation time in children. We also sought to characterize differences in T1 SIR between children without and with pancreatitis.

Retrospective study of patients < 18-years-old. SIR-pancreas:spleen (SIR-PS) and SIR-pancreas:paraspinal muscle (SIR-PM) were generated from T1-weighted gradient recalled echo images. Subdivided by field strength, T1 SIR was correlated (Spearman's) with T1 relaxation time.

220 participants were included, 144 imaged at 1.5T (mean: 11.4 ± 4.2 years) and 76 imaged at 3T (mean: 10.9 ± 4.5 years). At 1.5T, SIR-PS (rho=-0.62, 95% CI: -0.71 to -0.51, p < 0.0001) and SIR-PM (rho=-0.57, 95% CI: -0.67 to -0.45, p < 0.0001) moderately negatively correlated with T1 relaxation time. At 3T, correlations between T1 SIR and T1 relaxation time were moderate (rho=-0.40 to -0.43, p ≤ 0.0003). SIR-PS was significantly different between patient groups at 1.5T (p < 0.0001) with pairwise differences between: normal vs. acute on chronic pancreatitis (1.52 vs. 1.13; p < 0.0001). SIR-PM was also significantly different between groups at 1.5T (p < 0.0001) with differences between: normal vs. acute pancreatitis (1.65 vs. 1.40; p = 0.0006), normal vs. acute on chronic pancreatitis (1.65 vs. 1.18; p < 0.0001), and normal vs. chronic pancreatitis (1.65 vs. 1.52; p = 0.0066). A SIR-PS cut-off of ≤ 1.31 had 44% sensitivity and 95% specificity and SIR-PM cut-off of ≤ 1.53 had 69% sensitivity and 70% specificity for pancreatitis. At 3T, SIR-PS was significantly different between groups (p = 0.033) but without significant pairwise differences.

At 1.5T pancreas T1 SIR moderately to strongly correlates with estimated T1 relaxation time and is significantly lower in children with pancreatitis.

Chronic pancreatitis (CP) in children has exhibited an annual increase in incidence in recent years. Pediatric CP presents unique clinical features compared to adult cases. Endoscopic retrograde pancreatography (ERP) serves as a valuable and safe tool for diagnosing and treating CP in adults. However, data on endoscopic treatment of CP in children are still limited.

Demographics, etiology, surgical indications, diagnosis, treatment details, associated complications, and follow-up information were retrospectively studied in consecutive patients (<18 years old) who underwent ERP for CP between January 2020 and October 2024.

A total of 17 children (7 male, 10 female) with a mean age of 10.0 ± 2.7 years were included in the study. A total of 34 endoscopic treatments were conducted. Recurrent abdominal pain was the primary clinical symptom. Imaging predominantly revealed pancreatic duct abnormalities such as tortuous dilatation and the presence of pancreatic duct stones. Notably, 41.2% (7 cases) involved genetic and congenital anatomical variations. Pancreatic duct stent placement was successfully performed in all 17 children (100.0% success rate). Stent replacements occurred on average 2.2 times (range 1-5) at intervals of 3-6 months. Postoperative pancreatitis developed in 2 cases (5.9%, 2/34), and hyperamylasemia occurred in 5 cases (14.7%, 5/34). The postprocedure visual analogue scale (VAS) score for abdominal pain significantly decreased from 6 to 1 (P < 0.001). The annual frequency of pancreatitis episodes showed a significant reduction, decreasing from 2.4 times pre-treatment to 0.6 times post-treatment (P < 0.05). Body mass index (BMI) also showed a significant improvement post-treatment compared to pre-treatment (P < 0.05).

ERP performed by trained endoscopists utilizing standard adult endoscopes and accessories proved a safe and effective treatment option for pediatric CP, with complication rates comparable to those reported in adult cases.

Some clinical trials have validated a new perspective on continuous renal replacement therapy (CRRT) in combination with oXiris haemofilter to more effectively enhance endotoxins clearance and circulating cytokines adsorption, stabilise vital signs, correct haemodynamic disorders and accelerate the recovery of organ function, thus providing survival benefits. However, no such combination mode has been reported in paediatric patients with sodium valproate (SV)-induced acute pancreatitis (AP).An early childhood boy was admitted to the department of critical care medicine, due to sudden upper abdominal pain lasting for 2 days. The child had a 2-year history of epilepsy and took 120 mg of SV two times per day for more than 2 months, after which he was diagnosed with the first episode of AP, bronchopneumonia and paroxysmal supraventricular tachycardia. The culprit of AP was identified as SV. After admission to the intensive care unit, the child immediately stopped taking SV and received comprehensive treatment for AP, followed by CRRT in combination with oXiris haemofilter. On the 3rd day after admission, a nasojejunal tube was implanted, followed by enteral nutrition, probiotics and levetiracetam. On the 22nd day after admission, the child was discharged from the hospital. At a 3-month follow-up after discharge, the child has returned to normal life without any sequelae.To the best of our knowledge, this is the first clinical case of SV-induced AP in a paediatric patient who achieved complete recovery after undergoing CRRT in combination with oXiris haemofilter, suggesting that this combination mode could be safely and effectively applied in patients with similar pathogenesis mechanisms as adjuvant therapy.

Thiopurine drugs are effective treatment options in inflammatory bowel disease and other conditions but discontinued in some patients due to toxicity.

We investigated thiopurine-induced toxicity in a pediatric inflammatory bowel disease cohort by utilizing exome sequencing data across a panel of 46 genes, including TPMT and NUDT15.

The cohort included 487 patients with a median age of 13.1 years. Of the 396 patients exposed to thiopurines, myelosuppression was observed in 11%, gastroenterological intolerance in 11%, hepatotoxicity in 4.5%, pancreatitis in 1.8%, and "other" adverse effects in 2.8%. TPMT (thiopurine S-methyltransferase) enzyme activity was normal in 87.4%, intermediate 12.3%, and deficient in 0.2%; 26% of patients with intermediate activity developed toxicity to thiopurines. Routinely genotyped TPMT alleles associated with defective enzyme activity were identified in 28 (7%) patients: TPMT*3A in 4.5%, *3B in 1%, and *3C in 1.5%. Of these, only 6 (21%) patients developed toxic responses. Three rare TPMT alleles (*3D, *39, and *40) not assessed on routine genotyping were identified in 3 patients, who all developed toxic responses. The missense variant p.R139C (NUDT15*3 allele) was identified in 4 patients (azathioprine 1.6 mg/kg/d), but only 1 developed toxicity. One patient with an in-frame deletion variant p.G13del in NUDT15 developed myelosuppression at low doses. Per-gene deleteriousness score GenePy identified a significant association for toxicity in the AOX1 and DHFR genes.

A significant association for toxicity was observed in the AOX1 and DHFR genes in individuals negative for the TPMT and NUDT15 variants. Patients harboring the NUDT15*3 allele, which is associated with myelosuppression, did not show an increased risk of toxicity.

Acute pancreatitis (AP) increases the risk of diabetes mellitus (DM). Our aim was to identify clinical, laboratory and imaging predictors of preDM/DM in youth post index AP.

This was a prospective cohort study of patients ≤21 years-old with an index admission for AP and follow up at 3 and/or 12 months. Clinical laboratory values, imaging findings, admission course, and plasma chemokine and cytokine measures collected at index admission were tested for association with preDM/DM development. A multivariable regression model was used to predict preDM/DM.

Among 187 enrolled participants, 137 (73 %) and 144 (77 %) underwent DM screening at 3 and 12 months respectively, and 137 (73 %) had imaging available. PreDM/DM occurred in 22/137 (16 %; preDM n = 21, DM n = 1) at 3 months and 23/144 (16 %; preDM n = 18, DM n = 5) participants at 12 months. Univariate associations with preDM/DM at 12 months included: severe AP (SAP) (52 % preDM/DM vs. 17 % no DM; p = 0.0008), median [IQR] IL-6 (910 pg/ml [618-3438] vs. 196 pg/ml [71-480], p < 0.05) and CRP (4.16 mg/L [1.67-10.7] vs. 1.55 mg/L [0.4-3.68], p = 0.1) at time of AP attack. The optimal multivariable model to predict preDM/DM included with clinical variables was severe acute pancreatitis (SAP), c reactive protein (CRP), interleukin-6 (IL-6), and age [AUC = 0.80; (0.70, 0.88)]. Including imaging markers, the ideal model included SAP, CRP, IL-6, subcutaneous fat area, age and presence of autoimmune disease with an AUC [0.82 (0.71, 0.90)].

Development of preDM/DM following an index AP episode can be predicted by baseline AP severity, baseline CRP, IL-6 levels, and subcutaneous fat area.

This study aimed to identify the risk profile for postpancreatitis diabetes mellitus (PPDM) in children after a single acute pancreatitis (AP) episode and to investigate the association between episode severity and complications, length of stay (LOS), and intensive care unit (ICU) needs.

This cross-sectional study used the Pediatric Health Information System database for patients ≤19 years old with index AP admissions (2011-2020). A query (2012-2021) was performed for diabetes mellitus (DM). Patients with prior DM or pancreatic surgery before AP or DM diagnoses were excluded.

Out of 12,822 patients with index AP admissions (median age, 13.4 years; 54% female), 686 (5.4%) developed PPDM, with 320 (2.5%) during and 366 (2.9%) at a later admission. The median time to PPDM was 1.9 months (interquartile range: 0-20.8 months). Patients with PPDM experienced extended LOS, heightened ICU needs, and increased complications such as sepsis, pancreatic necrosis, and cyst formation, along with higher rates of organ dysfunction and comorbidities.

Children face DM risks during or after AP episodes. Enhanced DM screening and close outpatient follow-up within 3 to 6 months post-discharge are recommended.

T1-weighted signal intensity ratios (SIR) comparing pancreas to spleen (SIRps) or muscle (SIRpm) can semiquantitatively assess T1 signal change associated with pancreatitis. However, there is no standardized methodology for generating these ratios. We set out to determine the impact of MRI sequence as well as region of interest (ROI) location, shape, and size on T1 SIR.

Retrospective analysis of T1-weighted MR images from 118 patients acquired 2018-2023. A single observer placed ovoid ROIs in the pancreas body/tail and head/uncinate, spleen, and left erector spinae muscle and large irregular ROIs in the pancreas tail and spleen. ROIs were placed on images from two sequences: 3D radial 2 point mDIXON RF spoiled gradient recalled echo sequence (radial) and breath-hold 3D 2-point mDIXON RF spoiled gradient echo (BH). T1 SIR were calculated from mean signal intensity, and agreement was calculated with intraclass correlations coefficients (ICC) and Bland-Altman difference analyses.

118 participants, 57 (48%) female, with mean age 13.7 ± 5.6 years (48%) were included. Agreement was good for SIRps based on irregular versus round ROIs (radial: ICC = 0.90; BH: ICC = 0.91). Agreement was moderate for SIR based on sampling the pancreas body/tail versus head/uncinate (ICC = 0.67-0.76) and poor to moderate based on reference organ (muscle vs. spleen) (ICC = 0.41-0.61). Between sequences, agreement was moderate (ICC = 0.55-0.72, mean difference 0.04-0.09).

The size and shape of the ROI used to sample the pancreas does not meaningfully change T1 SIR but the location sampled, the reference organ used, and the MRI sequence used meaningfully change T1 SIR, potentially impacting disease diagnosis and staging.

Congenital biliary dilatation (CBD) is a congenital malformation of the main biliary tract usually associated with the pancreatobiliary maljunction (PBM), determining stone formation, cholangitis, pancreatitis, and cholangiocarcinoma. The role of endoscopic retrograde cholangiopancreatography (ERCP) in treatment and diagnosis has not been established yet. Therefore, the aim of our study is to define the actual role of ERCP in children with CBD.

A retrospective review of consecutive patients with congenital biliary dilatation undergoing preoperative ERCP and subsequent surgical treatment at our pediatric tertiary referral center (Endoscopy and Digestive Surgery, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy) was performed between 2012 and 2023.

A total of 31 patients were included in the present study. Preoperative ERCP detected a PBM in 28 patients (90%). According to Todani's classification, 2 patients (6.5%) had choledochal cyst (CC) type IV, and 29 patients (93.5%) were diagnosed with CC type I. In 18 (58%) patients, ERCP was performed for treating acute pancreatitis. Sphincterotomy could be performed in 23 of 31 (74%) patients. Patients who did not undergo sphincterotomy had a higher number of acute episodes while awaiting surgery.

The present study is supportive of an essential role of ERCP in the diagnostic and preoperative management of children with CBD with acute presentation or inconclusive magnetic resonance cholangiopancreatography findings.

The development of targeted therapies that correct the effect of mutations in patients with cystic fibrosis (CF) and the relevant heterogeneity of the clinical expression of the disease require biomarkers correlated to the severity of the disease useful for monitoring the therapeutic effects. We applied a targeted metabolomic approach by LC-MS/MS on saliva samples from 70 adult CF patients and 63 age/sex-matched controls to investigate alterations in metabolic pathways related to pancreatic insufficiency (PI), Pseudomonas aeruginosa (PA) colonization, CF liver disease (CFLD), and CF related diabetes (CFRD). Sixty salivary metabolites were differentially expressed, with 11 being less abundant and 49 more abundant in CF patients. Among these, the most relevant alterations involved salivary ADMA, N-acetylornithine, methionine and methionine sulfoxide levels. Furthermore, methionine was significantly lower in CF patients with PI and salivary histamine levels were significantly lower in patients colonized by PA. Moreover, ADMA as well as N-acetylornithine and methionine were significantly lower in CF patients with CFRD than in patients without CFRD. Finally, the levels of DOPA resulted significantly lower in saliva from patients with liver disease. Our study revealed an imbalance in arginine methylation and tryptophan pathway related to CFRD and PI as well as alterations in dopaminergic pathway and Krebs cycle related to CFLD. This study also highlights different salivary metabolites as new potential biomarkers in a non-invasive sample that could represent a useful tool for the stratification and management of CF patients.

Acute pancreatitis (AP) is a common paediatric condition, yet there is little data to support optimal analgesic practice. The aim of this scoping review was to report analgesic practice, investigate trends in analgesic strategy and evaluate the impact of analgesic modality on outcomes.

A systematic search of Medline, Embase, CENTRAL, Pubmed Central and Google Scholar was performed by two independent investigators. This review adhered to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews checklist.

Three retrospective cohort studies, all conducted in North America, reported on analgesic practice in paediatric AP. The studies included 658 patients (median age, 12 years; female sex, 57%; non-biliary aetiology, 85.9%). Overall, analgesia was administered in 67% of patients, including opioids in 59.5% (43.8%-71.4%). Rates of acetaminophen (17.9% and 77.7%) and non-steroidal anti-inflammatory drugs (7.7% and 40.2%) were reported in two studies. Two studies reported reducing rates of opioid administration or reduced duration of opioid administration since 2017 and 2014, respectively. One study did not find any correlation between opioid administration and sociodemographic factors, length of stay or admission to intensive care units. No studies reported on complications or quality of life. No studies investigated non-medical modalities. There were no long-term data on analgesic use post-discharge.

Opioids are the mainstay of pain treatment in paediatric AP in North America. However, factors that influence the analgesic type, the impact of analgesic modality on the post-pancreatitis outcome and long-term analgesic use constitute a knowledge gap. Future studies are needed to inform analgesic use in paediatric AP.

To evaluate outcomes of children from an observational cohort registry of index acute pancreatitis (AP) admissions managed with different types and rates of intravenous fluid therapy.

Patients with index admission of AP between 2013 and 2023 were included. Those who received >1.5x the maintenance intravenous fluid rate were assigned to the liberal fluid group, and patients who received <1.5x maintenance fluids were assigned to the conservative group. Outcomes including intensive care unit admission rate, organ dysfunction, local pancreatic complications, and AP severity were evaluated. Influence of early enteral feeding and fluid composition on outcomes and clinical course were also analyzed.

Patients who received liberal fluids were less likely to be admitted or transferred to the intensive care unit compared with those receiving conservative management (OR, 0.32; 95% CI, 0.12-0.80; P = .015). The liberal fluid group with early feeding had the lowest rate of moderate/severe manifestations of AP compared with other combinations of diet and fluid orders. Patients within the liberal fluid group who received the highest fluid rates (>2x maintenance) did not have higher rates of organ dysfunction or severe disease.

Children with AP may stand to benefit from liberal fluid therapy and continued diet compared with more conservative fluid resuscitation and nothing by mouth status.

The aim of this study was to determine patient-reported burdensome experiences and research interests in children with acute recurrent pancreatitis or chronic pancreatitis and their families.

Children with pancreatitis and their families completed a web-based survey. Subject prioritized rankings of symptoms or quality of life issues and topics for future research were assessed. Data are presented as family and children scores.

Among 80 participants, 18 were children with pancreatitis and 62 were family members. Top 5 ranked symptoms or quality of life issues were as follows: 1) pain, 2) fatigue, 3) missing school, 4) upset stomach, and 5) not knowing when an attack will occur. Top 5 ranked future research topics were as follows: 1) how to prevent a pancreatitis attack, 2) how pancreatitis affects other parts of the body, 3) ways to treat or handle pain, 4) what is the cause of pancreatitis, and 5) teach doctors about pancreatitis.

This study highlights the importance of patient and family input in caring for children with pancreatitis. The most bothersome symptoms were pain, fatigue, and upset stomach. Children with pancreatitis and families would like future research to primarily focus on prevention of pancreatitis attacks, pain therapy, and complications of pancreatitis.

Pancreatic necrosis, a severe complication of acute pancreatitis, is associated with significant morbidity and mortality. The use of lumen-apposing metal stents (LAMS) as a primary treatment offers a less invasive approach that may improve patient outcomes. This study evaluates the efficacy and safety of endoscopic ultrasound (EUS)-guided LAMS for treating walled-off pancreatic necrosis. In this retrospective cohort study, 95 patients treated with EUS-guided LAMS between March 2020 and October 2023 were included. Data were collected on the technical success of stent placement, clinical improvement, and management of symptomatic patients. Patients with other primary interventions, preexisting chronic pancreatitis, or incomplete clinical data were excluded. The technical success rate for LAMS placement was 100%, with a clinical success rate of 92.63%. Seven patients (7.37%) did not respond to LAMS treatment: five underwent video-assisted retroperitoneal drainage, and two had percutaneous drainage. Stent occlusion occurred in seven patients within the first week, managed through saline irrigation or direct endoscopic necrosectomy. No procedure-related complications were reported. The use of LAMS significantly reduced hospital stays and eliminated the need for additional surgeries in most cases. These findings suggest that LAMS is a highly effective and safe primary treatment for pancreatic necrosis, with high success rates and no related complications. The study's strengths include a large sample size and comprehensive follow-up, although its retrospective, single-center design may limit generalizability. These results support the use of LAMS as a primary treatment option for pancreatic necrosis, with future research needed to refine patient selection and explore long-term outcomes.

Acute pancreatitis in children is a rare condition; however, the incidence of this acute condition has been increasing over the past few decades. In most cases, acute pancreatitis does not cause severe complications in children, but in some cases, it can be complicated by fluid accumulation, fistulas and vascular pathologies, but the most common complication remains the formation of pseudocysts. This article describes the clinical case of a 11-year-old girl from Lithuania, which effectively illustrates a rare condition in children-acute pancreatitis and its developed complication-the formation of a pancreatic pseudocyst. The article discusses the frequency of acute pancreatitis and its complication, pancreatic pseudocyst in children, and the clinical expression of the disease and methods of diagnostics and treatment.

To characterize T1 relaxation times of the pancreas, liver, and spleen in children with and without abdominal pathology.

This retrospective study included pediatric patients (< 18-years-old). T1 mapping was performed with a Modified Look-Locker Inversion Recovery sequence. Patients were grouped based on review of imaging reports and electronic medical records. The Kruskal-Wallis test with Dunn's multiple comparison was used to compare groups.

220 participants were included (mean age: 11.4 ± 4.2 years (1.5 T); 10.9 ± 4.5 years (3 T)). Pancreas T1 (msec) was significantly different between subgroups at 1.5 T (p < 0.0001). Significant pairwise differences included: normal (median: 583; IQR: 561-654) vs. acute pancreatitis (731; 632-945; p = 0.0024), normal vs. chronic pancreatitis (700; 643-863; p = 0.0013), and normal vs. acute + chronic pancreatitis (1020; 897-1099; p < 0.0001). Pancreas T1 was also significantly different between subgroups at 3 T (p < 0.0001). Significant pairwise differences included: normal (779; 753-851) vs. acute pancreatitis (1087; 910-1259; p = 0.0012), and normal vs. acute + chronic pancreatitis (1226; 1025-1367; p < 0.0001). Liver T1 was significantly different between subgroups only at 3 T (p = 0.0011) with pairwise differences between normal (818, 788-819) vs. steatotic (959; 848-997; p = 0.0017) and normal vs. other liver disease (882; 831-904; p = 0.0455). Liver T1 was weakly correlated with liver fat fraction at 1.5 T (r = 0.39; 0.24-0.52; p < 0.0001) and moderately correlated at 3 T (r = 0.64; 0.49-0.76; p < 0.0001). There were no significant differences in splenic T1 relaxation times between subgroups.

Pancreas T1 relaxation times are higher at 1.5 T and 3 T in children with pancreatitis and liver T1 relaxation times are higher in children with steatotic and non-steatotic chronic liver disease at 3 T.

We report a case of moderately severe acute pancreatitis, hyperglycemia, acidosis, splenic, superior mesenteric, and portal vein thrombosis in relation to multisystem inflammatory syndrome in children (MIS-C). The patient responded well to intravenous immune globulin, corticosteroids, antibiotics, systemic anticoagulation, and drainage of peripancreatic fluid. The case highlights the polymorphic presentation of MIS-C and advises high level of suspicion for unusual, severe cases unresponsive to routine care.

Celiac plexus blocks (CPBs) using endoscopic ultrasound (EUS) guidance provide significant pain relief in adults with chronic pancreatitis. We present on EUS-guided CPB for pediatric patients with abdominal pain from chronic pancreatitis or severe functional dyspepsia necessitating clinically assisted nutrition and hydration. Patients who underwent EUS-CPB were included and followed prospectively at 2-, 4-, and 8-weeks postprocedure about pain, enteral tolerance, and school/activity attendance. Thirteen patients underwent EUS-guided CPB with a total of 21 procedures. In the pancreatitis cohort, mean pain relief was 11.7 weeks for those who responded. In the functional dyspepsia cohort, mean improvement (in either pain or enteral tolerance) was 4.8 weeks. Symptom improvement varied between the two cohorts. Acute recurrent/chronic pancreatitis patients demonstrated more sustained relief than the functional dyspepsia cohort. This study adds to the limited data investigating the utility of EUS-CPB as part of a multimodal treatment plan in pediatrics.

Necrotizing gallstone pancreatitis is a rare and severe form of pancreatitis, particularly uncommon in the pediatric population. While gallstone pancreatitis is increasingly recognized in children, necrotizing cases remain exceptional. We report a four-and-a-half-year-old Pakistani American male presenting with generalized weakness, abdominal pain, and vomiting. Initial symptoms followed a recent upper respiratory infection. Clinical evaluation revealed an intermittently drowsy-appearing patient with a Glasgow Coma Scale (GCS) of 15 when fully alert, hypotension, diffuse abdominal tenderness, and signs of possible sepsis. Laboratory tests showed elevated lipase levels and conjugated hyperbilirubinemia. Ultrasound identified gallstones and necrotizing changes in the pancreas, which were later confirmed by CT imaging. The patient was admitted to the pediatric intensive care unit for aggressive management of necrotizing pancreatitis, including fluid resuscitation, antibiotic therapy, and nutritional support. He underwent laparoscopic cholecystectomy and developed Clostridium difficile colitis, which was managed with targeted antibiotics. The patient had a 26-day hospital stay and was followed up with negative results from clinical exome sequencing for related disorders. This case underscores the diagnostic and management complexities associated with pediatric necrotizing gallstone pancreatitis. The need for a multidisciplinary approach and adherence to clinical guidelines is emphasized. This report contributes valuable insights into the rare presentation of necrotizing pancreatitis in children and highlights the importance of early and comprehensive intervention.

Acute pancreatitis (AP) carries the risk of subsequent nutritional deficiencies. The prevalence of these deficiencies following a single episode of AP in children is unknown. We aimed to determine prevalence of anthropometric and laboratory-based measures of nutritional status in children following their first (index) admission for AP.

Prospective observational cohort study of patients ≤21 years of age with first episode of confirmed AP. Anthropometric and laboratory values were obtained at time of AP onset and at follow up time points of 3 and 12 months (m) post AP. AP attack was classified as either: mild, moderately severe or severe (which were combined in one group (SAP)).

181 patients met criteria and were followed prospectively with 52% male, a median age of 13.7 years (IQR 9.4-16.0) and median Body Mass Index (BMI) Z-score of 0.6 (IQR -0.5, 1.6). Most patients had mild AP (140, 77%), with 23% meeting criteria for moderate or severe (41/181). 6 (3%) had diabetes mellitus (DM) predating AP and were excluded from further analysis. BMI Z-score remained stable during the follow up period. 13% of patients developed pre-DM or DM at 3m or 12m. Nearly one third of patients had low ferritin at 3m (29%) or 12m (29%). At 12m, 8% of patients had Vitamin A deficiency. 6% of patients had low Vitamin E levels at 3m and 5% at 12m. Over half of patients at both 3m and 12m had 25 OH Vitamin D insufficiency or deficiency (56% and 56%). Prolonged International Normalized Ratio (INR) (>1.3) was seen in 9% of patients at 12m. Very low albumin (<3.5 g/dL) was found in 24% of patients at 3m and 18% at 12m (Table 1). Patients with very low albumin at 3m were younger (median 10.7 vs. 14.2 years, p = 0.04), however sex, BMI Z-score and AP severity were not associated with albumin level. Although BMI Z-score did not differ between the groups, those with SAP had a significant decrease in BMI Z-score from first attack compared to mild AP at 3m (-0.4 vs. 0.0, p = 0.0002, Figure 2). At 3m, Vitamin E deficiency in SAP versus mild AP was found in 20% vs 2% (p = 0.04) and SAP had a lower median hematocrit (35.8 vs. 37.6, p = 0.046). There were no other laboratory significant differences at 3m in mild versus SAP groups. At 12m, those with SAP were more likely to have pre-DM or DM compared to mild AP (31% vs. 7%, p = 0.002). No other significant laboratory differences occurred at 12m.

After the first AP attack patients experience nutritional deficiencies, including ferritin, all fat-soluble vitamins, and low albumin. SAP is associated with a decrease in BMI Z-score, increased prevalence of vitamin E deficiency at 3m, and an increase in pre-diabetes and diabetes at 12m. Serial monitoring of vitamin and mineral values post AP is warranted and further prospective studies are needed.

Acute pancreatitis (AP) is increasingly recognized as a risk factor for diabetes mellitus (DM). We aimed to study the association of pancreatitis genes with pancreatic endocrine insufficiency (pre-DM and DM) development post-AP in children.

This was an observational cohort study that enrolled subjects ≤21 years with their first episode of AP and followed them for 12 months for the development of pancreatic endocrine insufficiency. Pancreatitis risk genes (CASR, CEL, CFTR, CLDN2, CPA1, CTRC, PRSS1, SBDS, SPINK1, and UBR1) were sequenced. A genetic risk score was derived from all genes with univariable P < .15.

A total 120 subjects with AP were genotyped. Sixty-three subjects (52.5%) had at least 1 reportable variant identified. For modeling the development of pancreatic endocrine insufficiency at 1 year, 6 were excluded (2 with DM at baseline, 3 with total pancreatectomy, and 1 death). From this group of 114, 95 remained normoglycemic and 19 (17%) developed endocrine insufficiency (4 DM, 15 pre-DM). Severe AP (58% vs 20%; P = .001) and at least 1 gene affected (79% vs 47%; P = .01) were enriched among the endocrine-insufficient group. Those with versus without endocrine insufficiency were similar in age, sex, race, ethnicity, body mass index, and AP recurrence. A model for pre-DM/DM development included AP severity (odds ratio, 5.17 [1.66-16.15]; P = .005) and genetic risk score (odds ratio, 4.89 [1.83-13.08]; P = .002) and had an area under the curve of 0.74.

In this cohort of children with AP, pancreatitis risk genes and AP disease severity were associated with pre-DM or DM development post-AP.

Among children who suffer from acute recurrent pancreatitis (ARP) or chronic pancreatitis (CP), acute pancreatitis (AP) episodes are painful, often require hospitalization, and contribute to disease complications and progression. Despite this recognition, there are currently no interventions to prevent AP episodes. In this retrospective cohort study, we assessed the impact of pancreatic enzyme therapy (PERT) use on clinical outcomes among children with pancreatic-sufficient ARP or CP.

Children with pancreatic-sufficient ARP or CP in the INSPPIRE-2 cohort were included. Clinical outcomes were compared for those receiving vs not receiving PERT, as well as frequency of AP before and after PERT. Logistic regression was used to study the association between development of AP episodes after starting PERT and response predictors.

Among 356 pancreatic-sufficient participants, 270 (76%) had ARP, and 60 (17%) received PERT. Among those on PERT, 42% did not have a subsequent AP episode, during a mean 2.1 years of follow-up. Children with a SPINK1 mutation ( P = 0.005) and those with ARP (compared with CP, P = 0.008) were less likely to have an AP episode after starting PERT. After initiation of PERT, the mean AP annual incidence rate decreased from 3.14 down to 0.71 ( P < 0.001).

In a retrospective analysis, use of PERT was associated with a reduction in the incidence rate of AP among children with pancreatic-sufficient ARP or CP. These results support the need for a clinical trial to evaluate the efficacy of PERT to improve clinical outcomes among children with ARP or CP.

Exocrine pancreatic insufficiency (EPI) is highly prevalent among individuals with cystic fibrosis (CF). Individuals diagnosed with EPI are often labeled as having "pancreas insufficient cystic fibrosis (PI-CF)" while those with normal exocrine function are labeled as "pancreas sufficient CF (PS-CF)." This diagnosis of EPI relies on clinical and laboratory features and management involves consumption of pancreas enzyme replacement therapy. In this review, we discuss the nuances of diagnosis and management of EPI in CF. We also present emerging evidence on the effects of CFTR modulating agents on the management of EPI, and speculate that these medications may lead to greater heterogeneity in management of EPI in CF moving forward.

Necrotizing pancreatitis is an uncommon diagnosis in pediatric patients. Early diagnosis is difficult as the presentation varies significantly. However, it should be in the differential diagnosis of abdominal pain in the pediatric age group.

An 8-year-old girl arrived with a 1-day history of vomiting, constipation, and abrupt, increasing epigastric discomfort. She didn't have any noteworthy family or medical background. Upon examination, she seemed to be afebrile but also had discomfort in her stomach and symptoms of dehydration. An enlarged pancreas with necrotizing pancreatitis was seen in the first imaging. She received intravenous fluids, antibiotics, and analgesics as a treatment for her acute severe pancreatitis diagnosis. Since the patient continued to have fever, meropenem was prescribed in place of ceftriaxone at first. After 10 days of uncomplicated hospitalization, she was released from the hospital.

Once rare, pediatric pancreatitis now affects 3-13 out of every 100 000 people yearly. Although it is uncommon (<1% in children), necrotizing pancreatitis can happen. Its causes are similar to those of acute pancreatitis, involving genetic abnormalities and certain drugs. Abdominal discomfort, fever, vomiting, and nausea are among the symptoms. Imaging methods like contrast-enhanced CT are used in diagnosis. Surgery has given way to less intrusive techniques like catheter drainage as a form of treatment. Surgery is seldom required in pediatric instances, which are often handled conservatively.

Childhood necrotizing pancreatitis is uncommon but dangerous; prompt diagnosis and prompt treatment are essential.

Background/Objectives: Sickle cell disease (SCD) impacts about 100,000 people in the US. SCD increases the risk of cholelithiasis and microvascular ischemia, which could increase the risk of acute pancreatitis (AP). Abdominal pain is a common presenting symptom of AP and sickle cell vaso-occlusive crisis. The purpose of our systematic review is to estimate the prevalence and determine the severity of AP in individuals with SCD compared to the general population. Methods: Multiple electronic databases were searched. We included studies that included children and adults (population) and addressed the association of SCD (exposure) with AP (outcome) compared to the same population without SCD (control). Two authors screened titles and abstracts independently, and data were abstracted in duplication from included studies. We registered this protocol in PROSPERO-CRD42023422397. Results: Out of 296 studies screened from multiple electronic databases, we identified 33 studies. These studies included 17 case reports, one case series, and 15 retrospective cohort studies, and 18 studies included children. Eight of the AP case reports were in patients with HbSS genotype, two with sickle beta thalassemia, and one with HbSoArab, and in six case reports, a genotype was not specified. Complications were reported in 11 cases-respiratory complication (in at least four cases), splenic complications (three cases), pancreatic pseudocyst (two cases) and death from AP (one case). Of the four AP cases in the case series, three had HbSS genotype, and two cases had complications and severe pancreatitis. AP prevalence in SCD was estimated to be 2% and 7% in two retrospective studies, but they lacked a comparison group. In retrospective studies that evaluated the etiology of AP in children, biliary disease caused mostly by SCD was present in approximately 12% and 34%, respectively. Conclusions: Data on the prevalence of AP in individuals with SCD are limited. Prospectively designed studies aiming to proactively evaluate AP in individuals with SCD who present with abdominal pain are needed to improve timely diagnosis of AP in SCD and outcomes.

To characterize the clinical course of acute pancreatitis (AP) in pediatric inflammatory bowel disease (IBD) patients compared to children with AP without IBD and to identify risk factors associated with AP among IBD patients.

This retrospective, single-center study compared clinical characteristics of children (<19 years) with AP with and without concomitant IBD who were hospitalized 2005-2019. We also conducted a risk factor analysis of AP development in pediatric IBD.

Sixty-eight (54% males) patients with 120 episodes of AP were admitted at a median age of 15.3 years. Thirteen patients (14 episodes) had a co-diagnosis of IBD, representing 4% of our IBD patient population. The AP-IBD patients presented with lower amylase levels compared to the non-IBD patients (160 [interquartile range, IQR: 83-231] vs. 418 [IQR: 176-874] U/L, p > 0.01), all had a mild pancreatitis, and none required invasive intervention. The presumed etiology for AP in all IBD patients was IBD-related: IBD flare-up in five, side effects of medications in two, and undetermined in seven. The only risk factor for AP development among IBD patients was IBD-associated arthritis (23% vs. 3% for IBD-non-AP, p = 0.04), while extracolonic Crohn's disease and induction therapy with nutrition were negative risk factors (15% vs. 51%, p = 0.05, and 8% vs. 44%, p = 0.04, respectively). Other parameters, including disease type and medications, were nonsignificant.

The clinical course of AP in pediatric IBD patients is mild. Only IBD-associated arthritis emerged as a risk factor for the development of AP, while, unexpectedly, IBD medication did not.

This study aimed to identify the risk factors for acute pancreatitis (AP) and its impact on outcomes in Polish children treated for ALL.

The study group included 2303 children receiving intensive chemotherapy for ALL. The group was divided into patients with at least one episode of AP and those who did not develop AP after treatment for ALL.

The cumulative incidence of AP in the study group was 4.08%. Older age was an independent risk factor for the development of AP (OR = 1.05; 95%CI = 1.006-1.098; p = 0.03). The overall mortality associated with AP was 2.13%. The probabilities of disease-free survival (p-DFS) and event-free survival (p-EFS) in both subgroups were 0.84 vs. 0.86, log-rank p = 0.65 and 0.75 vs. 0.80, log-rank p = 0.12, respectively. A total of 22 out of 94 patients (23.4%) with AP were re-exposed to asparaginase (ASP) during the subsequent treatment phases. Only one patient re-exposed to ASP (4.5%) developed a second episode of AP. There were no significant differences in p-DFS and p-EFS between patients re-exposed and not re-exposed to asparaginase (0.78 vs. 0.86, log-rank p = 0.27 and 0.63 vs. 0.79, log-rank p = 0.09, respectively).

The incidence of AP in children with ALL is low and related to patients' age. The development of AP does not seem to influence p-DFS and p-EFS in children with ALL. Recurrence of AP after re-exposure to asparaginase in patients with ALL and a history of AP is low (4.5%). Re-exposure to asparaginase after the first episode of AP does not improve either p-DFS or p-EFS in children with ALL.

Magnetic resonance (MR) imaging with secretin stimulation (MR-PFTs) is a non-invasive test for pancreatic exocrine function based on assessing the volume of secreted bowel fluid in vivo. Adoption of this methodology in clinical care and research is largely limited to qualitative assessment of secretion as current methods for secretory response quantification require manual thresholding and segmentation of MR images, which can be time-consuming and prone to interrater variability. We describe novel software (PFTquant) that preprocesses and thresholds MR images, performs heuristic detection of non-bowel fluid objects, and provides the user with intuitive semi-automated tools to segment and quantify bowel fluid in a fast and robust manner. We evaluate the performance of this software on a retrospective set of clinical MRIs.

Twenty MRIs performed in children (< 18 years) were processed independently by two observers using a manual technique and using PFTquant. Interrater agreement in measured secreted fluid volume was compared using intraclass correlation coefficients, Bland-Altman difference analysis, and Dice similarity coefficients.

Interrater reliability of measured bowel fluid secretion using PFTquant was 0.90 (0.76-0.96 95% C.I.) with - 4.5 mL mean difference (-39.4-30.4 mL 95% limits of agreement) compared to 0.69 (0.36-0.86 95% C.I.) with - 0.9 mL mean difference (-77.3-75.5 mL 95% limits of agreement) for manual processing. Dice similarity coefficients were better using PFTquant (0.88 +/- 0.06) compared to manual processing (0.85 +/- 0.10) but not significantly (p = 0.11). Time to process was significantly (p < 0.001) faster using PFTquant (412 +/- 177 s) compared to manual processing (645 +/- 305 s).

Novel software provides fast, reliable quantification of secreted fluid volume in children undergoing MR-PFTs. Use of the novel software could facilitate wider adoption of quantitative MR-PFTs in clinical care and research.

Asparaginase-associated pancreatitis (AAP) occurs in up to 18% of patients treated for acute lymphoblastic leukemia (ALL); however, long-term sequelae are largely unexplored. We aimed to explore pancreatic sequelae among ALL survivors with and without AAP.

We investigated pancreatic sequelae in a national cohort of ALL survivors, aged 1-45 years at ALL diagnosis treated according to the NOPHO-ALL2008 protocol and included sex- and age-matched community controls.

We included 368 survivors (median follow-up 6.9 years), including 47 survivors with AAP and 369 controls. The p-lipase and p-pancreas-type amylase levels were lower in AAP survivors compared with both non-AAP survivors (Medians: 23 U/L [IQR 14-32] and 18 U/L [IQR 10-25] versus 29 [IQR 24-35] and 22 [17-28], p < .001 and p = .002) and community controls (28 U/L [IQR 22-33] and 21 U/L [IQR 17-26], both p < .006). Fecal-elastase was more frequently reduced in AAP survivors compared with non-AAP survivors (7/31 vs. 4/144, p = .001). Persisting pancreatic sequelae were found in 15/47 of AAP survivors and 20/323 of non-AAP survivors (p < .001), including diabetes mellitus in 2/39 of AAP survivors and 2/273 of non-AAP survivors.

ALL survivors with AAP are at increased risk of persisting pancreatic dysfunction and require special attention during follow-up.

Pancreatic fluid collections (PFCs) are a well-known complication of pancreatitis. PFCs operative management includes percutaneous, endoscopic or surgical drainage. Even if in adult patients, endoscopic drainage is a well-established treatment, few data are available in pediatric setting. We report our single-center experience of EUS-guided cystogastrostomy and lumen-apposing metal stent (LAMS) positioning in children with PFCs; this, at the best of our knowledge, has never been reported before. All consecutive children with PFCs between April 2020 and November 2022 were enrolled in this retrospective study. PFCs were preoperatively evaluated with MRI or CT scan. All the procedures were performed under general anesthesia. A LAMS Hot-AxiosTM 10 × 15 mm was placed in all patients. We evaluated technical feasibility and clinical outcomes, including complications and recurrence rates. Follow-up included clinical observation, blood tests and US. EUS-guided cystogastrostomy was performed in 3 children (2 males; median age 13.2 years). Median maximum cyst diameter was 14.7 cm (range 10-22 cm). Technical and clinical success rates were 100%. No intra or post-operative complications occurred. Our experience suggests that this can be considered a safe and feasible treatment of PCFs even in the pediatric population, as long as the procedure is performed by an expert Endoscopist in a pediatric tertiary-level Center.

Gastrointestinal hospitalisations in the USA cause over US$130 billion in expenditures, and acute pancreatitis is a leading cause of these hospitalisations. Adequate pain control is one of the primary treatment goals for acute pancreatitis. Though opioids are commonly used for analgesia in these patients, there have been concerns about short-term and long-term side effects of using opioids. Recently, non-opioid medications have been studied to treat pain in patients with acute pancreatitis. This systematic review and network meta-analysis aims to assess the comparative efficacy of analgesic medication for non-severe, acute pancreatitis.

We will search multiple electronic databases for randomised controlled trials that study pain management in patients with non-severe, acute pancreatitis. The intervention will be any analgesic for acute pancreatitis in the hospital setting. The comparison group will be patients who received a placebo or other active interventions for pain management. The primary outcomes of interest include pain scores and the need for supplementary analgesia. The secondary outcomes will be serious adverse events, local complications, progression to severe pancreatitis, transfer to the intensive care unit, length of hospitalisation, time to start enteral feeds, 30-day all-cause mortality and Quality of Life Scale scores. If sufficient homogeneity exists among included studies, the findings will be pooled using a traditional pairwise and network meta-analysis. The risk of bias in randomised control trials will be evaluated using the Cochrane Risk of Bias Tool 2.0. The Grading of Recommendations, Assessment, Development, and Evaluation approach will be used to report the certainty of evidence.

This systematic review will not involve direct contact with human subjects. The findings of this review will be published in a peer-reviewed journal. They will give healthcare providers a better awareness of the optimal analgesic medication for pain treatment in non-severe, acute pancreatitis.

Acute pancreatitis (AP) is common among children in Bangladesh. Its management depends mainly on risk stratification. This study aimed to assess the severity of pediatric AP using computed tomography (CT).

This cross-sectional, descriptive study was conducted in pediatric patients with AP at the Department of Pediatric Gastroenterology and Nutrition, BSMMU, Dhaka, Bangladesh.

Altogether, 25 patients with AP were included, of whom 18 (mean age, 10.27±4.0 years) were diagnosed with mild AP, and 7 (mean age, 10.54±4.0 years) with severe AP. Abdominal pain was present in all the patients, and vomiting was present in 88% of the patients. Etiology was not determined. No significant differences in serum lipase, serum amylase, BUN, and CRP levels were observed between the mild and severe AP groups. Total and platelet counts as well as hemoglobin, hematocrit, serum creatinine, random blood sugar, and serum alanine aminotransferase levels (p>0.05) were significantly higher in the mild AP group than in the severe AP group (p=0.001). The sensitivity, specificity, positive predictive value, and negative predictive value of CT severity index (CTSI) were 71.4%, 72.2%, 50%, and 86.7%, respectively. In addition, significant differences in pancreatic appearance and necrosis were observed between the two groups on CT.

CT can be used to assess the severity of AP. In the present study, the CTSI effectively assessed the severity of AP in pediatric patients.