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Introduction: Alagille syndrome (ALGS) is a rare, genetic, multisystem disorder commonly associated with cholestatic liver disease; patients with ALGS may experience elevated serum bile acids and severe pruritus with associated impaired sleep. The ileal bile acid transporter (IBAT) is located on the luminal surface of enterocytes in the terminal ileum; this transport protein mediates resorption of conjugated bile acids for recirculation back to the liver. Inhibition of IBAT disrupts the enterohepatic circulation and leads to fecal elimination of bile acids., Areas Covered: Here, the role of odevixibat as a novel, nonsurgical approach to interrupting the enterohepatic circulation from the intestine by inhibition of IBAT is reviewed, specifically in reference to currently available data on pharmacologic IBAT inhibition. IBAT inhibition has been shown to reduce serum bile acids and pruritus in trials of cholestatic liver diseases in children including ALGS., Expert Opinion: Odevixibat or IBAT inhibitor should be considered as a first-line treatment for ALGS to improve pruritis, quality of life and liver-related outcomes including absence of liver transplant, surgical biliary diversion, hepatic decompensation, and death.

Background: Fontan-associated liver disease (FALD) refers to structural and functional changes of the liver caused by the physiology of the Fontan palliation. Currently, liver biopsy is the gold standard to assess liver fibrosis of FALD., Aim: Investigate biomarkers correlating with severity of liver biopsy fibrosis in FALD., Methods: A retrospective study of post-Fontan patients ≥ 10 years of age who underwent liver biopsy was conducted. Advanced liver disease (ALD) was defined as bridging fibrosis and/or cirrhosis on liver biopsy. AST-to-platelet ratio index (APRI), Fibrosis-4 (FIB-4) and Liver Stiffness Measurement (LSM) from FibroScan were used as non-invasive fibrosis scores., Results: Sixty-six patients (26/47; 55.3% adults and 13/19 children; 68.4%) had ALD on biopsy. ALD was associated with lower platelet count (151 vs. 198 K/uL, p = 0.003), higher APRI (0.64 vs. 0.32, p = 0.01), higher FIB-4 (0.64 vs. 0.32, p = 0.02). Liver fibrosis score correlated with APRI (0.34, p = 0.02) and FIB-4 (0.47, p = 0.001) in adults. LSM had a high sensitivity at 81.3% with 45.5% specificity at a cut-off 18.5 kPa., Conclusions: APRI and FIB-4 had modest discrimination to identify adults with advanced liver disease, but not children, indicating that these values may be followed as a marker of FALD progression in older patients., Competing Interests: Conflict of interest All authors have no conflicts of interest to disclose., (Copyright © 2023 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.)

Background: Granulomas in pediatric liver biopsies (GPLB) are rare with the largest pediatric cohort reported over 25 years ago., Methods: Single-center retrospective study of GPLB., Results: Seventeen liver biopsies from 16 patients with granulomas were identified (9 boys, 56%) with a median age of 13 years (range: 1-18) for which the most common indication was the presence of a nodule/mass (47%). Significant comorbidities were seen in 13 patients (81%) and included: liver transplant (25%), history of a neoplasm (25%), autoimmune hepatitis (6%), Crohn disease (6%), bipolar disorder (6%), severe combined immunodeficiency (6%), and sickle cell disease (6%). Eleven patients were taking multiple medications at the time of biopsy. Granulomas were more commonly pan-acinar (11 cases) followed by subcapsular (4 cases), portal (1 case), and periportal (1 case). Necrosis was seen in 10 cases (59%). GMS stain was positive in 2 cases for Histoplasma -like yeast; microbiological cultures were negative in all cases (no: 4). A 18S and 16S rRNA gene sequencing performed in 15 cases revealed only 1 with a pathogenic microorganism, Mycobacterium angelicum ., Conclusion: In our experience, GPLB are heterogenous with only 3 cases having an identifiable infectious etiology and many of the remaining cases being associated to multiple medications, suggesting drug-induced liver injury as possible etiology., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background & Aims: PNPLA3 G-allele is an important determinant of disease severity in nonalcoholic fatty liver disease (NAFLD). Here, we investigated the effect of age, body mass index (BMI), and type 2 diabetes mellitus (T2DM) on the relationship between PNPLA3 G-allele and advanced fibrosis in adults and children with histologically characterized NAFLD., Methods: A total of 1047 children and 2057 adults were included. DNA was genotyped for rs738409 in duplicate. Primary outcome of interest was advanced fibrosis (fibrosis stage ≥3). Regression analyses were performed after controlling for relevant covariates. An additive model was used to assess the effect of PNPLA3 G-allele (CC vs CG vs GG)., Results: PNPLA3 G-allele was significantly associated with advanced fibrosis in children (odds ratio [OR], 1.55; 95% confidence interval [CI], 1.16-2.09) and adults (OR, 1.55; 95% CI, 1.16-1.54). Across the cohort, older age significantly increased the risk for advanced fibrosis for PNPLA3 CC (OR, 1.019; 95% CI, 1.013-1.026), CG (OR, 1.024; 95% CI, 1.018-1.030), and GG (OR, 1.03; 95% CI, 1.023-1.037) genotypes. BMI significantly increased the relationship between PNPLA3 genotypes and advanced fibrosis in children and adults. A BMI of 30 kg/m 2 was the cutoff beyond which PNPLA3 G-allele had exponential effect on the risk for advanced fibrosis in children and adults. T2DM significantly worsened the relationship between PNPLA3 G-allele and advanced fibrosis in children and adults (interaction P < .01 for both)., Conclusions: Age, BMI, and T2DM modify the risk of advanced fibrosis associated with PNPLA3 G-allele. Preventing or reversing T2DM and obesity in persons carrying PNPLA3 G-allele may lower the risk for advanced fibrosis in NAFLD., (Copyright © 2024 AGA Institute. Published by Elsevier Inc. All rights reserved.)

Children represent only a small proportion of those infected with the hepatitis C virus (HCV) compared to adults. Nevertheless, a substantial number of children have chronic HCV infection and are at risk of complications including cirrhosis, portal hypertension, hepatic decompensation with hepatic encephalopathy, and hepatocellular carcinoma in adulthood. The overall prevalence of the HCV in children was estimated to be 0.87% worldwide. The HCV spreads through the blood. Children born to women with chronic hepatitis C should be evaluated and tested for HCV due to the known risk of infection. The course of treatment for hepatitis C depends on the type of HCV. Currently, there are two pan-genotype HCV treatments (Glecaprevir/pibrentasvir and Sofosbuvir/velpatasvir) for children. We aim to review the updated clinical guidelines on the management of HCV infection in children, including screening, diagnosis, and long-term monitoring, as well as currently published clinical trials and ongoing research on direct acting antiviral hepatitis C treatment in children.

Introduction: Glycogenic hepatopathy (GH) is a rare complication of type I diabetes mellitus (DM1), resulting in abnormal deposition of glycogen in the liver due to poor glycemic control. Clinical characteristics and natural history of GH are not completely understood in children. In this study, we investigated clinical, biochemical, histologic parameters and outcomes in children with GH., Method: This was a retrospective review of patients less than 18 years old diagnosed with GH and DM. GH was confirmed on liver biopsy. Medical records were reviewed for clinical presentation, laboratory tests, and clinical outcomes. Liver biopsy findings were reviewed by a pediatric pathologist (I. A. G.)., Results: Nine children were diagnosed with GH and type 1 DM. The median age at diagnosis of GH was 16 (IQR 14.5-17) years. Duration of diagnosis of DM until GH diagnosis was 7 (IQR 5-11) years. The median frequency of diabetic ketoacidosis before GH diagnosis was three times (IQR 2-5.25). Peak Aspartate transaminase (AST) and Alanine transaminase (ALT) ranged from 115 to 797, and 83-389 units/L, respectively. Only two children had mild fibrosis. Seven of nine had steatosis without steatohepatitis. There was no correlation between glycosylated hemoglobin (HbA1c), or other laboratory tests and liver fibrosis on biopsy. HbA1c was 11.2 (IQR 10.2-12.8) at GH diagnosis and 9.8 (IQR 9.5-10.8) with normalization of liver enzymes., Conclusion: GH appears to be related to poor glycemic control in teenagers with long-term diabetes. GH presents with high to very high aminotransferase especially AST > ALT and resolves with modestly improved glycemic control. Diffuse hepatocyte swelling, steatosis, minimal fibrosis without hepatocyte ballooning or lobular inflammation are most common histological features., Competing Interests: The authors declare no conflict of interest., (© 2024 The Authors. JPGN Reports published by Wiley Periodicals LLC on behalf of The European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

The liver coordinates the systemic response to nutrient deprivation and availability by producing glucose from gluconeogenesis during fasting and synthesizing lipids via de novo lipogenesis (DNL) when carbohydrates are abundant. Mitochondrial pyruvate metabolism is thought to play important roles in both gluconeogenesis and DNL. We examined the effects of hepatocyte-specific mitochondrial pyruvate carrier (MPC) deletion on the fasting-refeeding response. Rates of DNL during refeeding were impaired by hepatocyte MPC deletion, but this did not reduce intrahepatic lipid content. During fasting, glycerol is converted to glucose by two pathways; a direct cytosolic pathway and an indirect mitochondrial pathway requiring the MPC. Hepatocyte MPC deletion reduced the incorporation of 13 C-glycerol into TCA cycle metabolites, but not into new glucose. Furthermore, suppression of glycerol and alanine metabolism did not affect glucose concentrations in fasted hepatocyte-specific MPC-deficient mice, suggesting multiple layers of redundancy in glycemic control in mice., Competing Interests: B.N.F is a shareholder and a member of the Scientific Advisory Board for Cirius Therapeutics, which is developing an MPC modulator for treating nonalcoholic steatohepatitis. G.J.P has a research collaboration agreement with Thermo Fisher Scientific and is a scientific advisor for Cambridge Isotope Laboratories., (© 2023 The Author(s).)

Background: Liver transplantation (LT) in infants can be challenging due to their small size and small vasculature. Although both whole LT (WLT) and split LT (SLT) have been described in infants, the head-to-head comparison of these techniques in this population is sparse., Methods: We retrospectively analyzed the records of all patients with age ≤1 year at Indiana University between 2016 and 2022. All SLT were left lateral segment grafts split in situ., Results: A total of 24 infants were transplanted, with 11 SLT and 13 WLT. The median follow-up time was 52.1 months. Donor and recipient characteristics were comparable except for donor age (19 years vs. 2 years; p < .01) and weight (64 kg vs. 14.2 kg; p < .01). Early allograft dysfunction, primary nonfunction, and hepatic artery thrombosis developed more frequently in the WLT group. There were no biliary complications. There were two early deaths (2 and 4 days) in the WLT group. One-year graft survival (100% vs. 77%; p = .10) and patient survival (100% vs. 85%; p = .18) were numerically higher in the SLT group., Conclusions: SLT with LLS offers a safe and viable option for liver transplantation in infants and is associated with a trend toward superior outcomes. SLT should be considered as a strategy to reduce waitlist times for infants in the absence of small, deceased donors for WLT., (© 2023 Wiley Periodicals LLC.)

Objectives: We aimed to evaluate differences in laboratory tests, bleeding, transfusions, and thrombosis between (1) children without and with cirrhosis and (2) children and adults with cirrhosis, and to correlate thromboelastography (TEG) parameters with biomarkers of hemostasis, bleeding, and transfusions in children and adults with cirrhosis., Methods: This single-center, retrospective study included 20 children without cirrhosis, 40 children with cirrhosis, and 40 adults with cirrhosis who underwent a liver transplant (LT). We collected demographic data, preoperative laboratory values, and intraoperative TEG parameters. Biomarkers of hemostasis just prior to the start of LT surgery were analyzed including international normalized ratio (INR), platelet, fibrinogen level, R time, K time, alpha angle (α), and maximum amplitude (MA). We also collected outcome data including blood loss, transfusion requirements, and thrombosis., Results: A significantly higher proportion of children with cirrhosis had abnormal PT ( P = 0.001), platelet ( P = 0.001), K time ( P = 0.02), and MA ( P = 0.05) compared to children without cirrhosis. The incidences of thrombosis, bleeding events, blood loss or PRBC transfusion were not significantly different between these 2 groups. A significantly higher proportion of adults with cirrhosis had abnormal R time ( P = 0.01) and alpha angle ( P = 0.01) than children with cirrhosis., Conclusions: Children with cirrhosis had defects in fibrinogen and platelets compared to children without cirrhosis at time of LT; however, these abnormalities did not translate into higher rates of bleeding in the former. Adults with cirrhosis had more defects in clotting factors compared to children with cirrhosis., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Background: While prior adult studies have shown that approximately 20%-38% of subjects undergoing solid-organ transplant develop neutropenia, similar analyses in pediatric subjects are scarce., Methods: We conducted a retrospective chart review of liver transplant (LT) and kidney transplant (KT) recipients at our center during the period 2008-2018. All of the KT and none of the LT subjects during this time period had induction with either anti-thymocyte globulin (ATG) or basiliximab at time of transplant. Neutropenia was defined as absolute neutrophil count (ANC) value ≤1000/mm 3 ., Results: One hundred subjects with LT and 82 subjects with KT were included. The incidence of neutropenia within the first year of transplant in KT was higher compared to LT (54.8% vs 39%, p = .01). The median number of hospitalizations (p = .001) and infectious complications (p = .04) was significantly higher only in the KT subjects who developed neutropenia (compared to those who did not). Multivariate analysis identified factors associated with severity of liver disease at transplant, namely h/o upper gastrointestinal bleeding (p = .02), weight deficit (p = .01), and pre-LT ANC (p = .01), along with high or moderate risk cytomegalovirus status (p = .05) as predictors of neutropenia in LT subjects. Female gender (p = .03) predicted neutropenia, while BK virus infection was protective for neutropenia (p = .04) in KT subjects., Conclusions: The incidence of and morbidity associated with neutropenia within 1 year post-transplant is higher in KT subjects compared to LT subjects. The likely reason for this is the use of induction therapy (ATG, basiliximab) at the time of transplant in KT subjects., (© 2021 Wiley Periodicals LLC.)

The mitochondrial pyruvate carrier (MPC) is an inner mitochondrial membrane complex that plays a critical role in intermediary metabolism. Inhibition of the MPC, especially in liver, may have efficacy for treating type 2 diabetes mellitus. Herein, we examined the antidiabetic effects of zaprinast and 7ACC2, small molecules which have been reported to act as MPC inhibitors. Both compounds activated a bioluminescence resonance energy transfer-based MPC reporter assay (reporter sensitive to pyruvate) and potently inhibited pyruvate-mediated respiration in isolated mitochondria. Furthermore, zaprinast and 7ACC2 acutely improved glucose tolerance in diet-induced obese mice in vivo. Although some findings were suggestive of improved insulin sensitivity, hyperinsulinemic-euglycemic clamp studies did not detect enhanced insulin action in response to 7ACC2 treatment. Rather, our data suggest acute glucose-lowering effects of MPC inhibition may be due to suppressed hepatic gluconeogenesis. Finally, we used reporter sensitive to pyruvate to screen a chemical library of drugs and identified 35 potentially novel MPC modulators. Using available evidence, we generated a pharmacophore model to prioritize which hits to pursue. Our analysis revealed carsalam and six quinolone antibiotics, as well as 7ACC1, share a common pharmacophore with 7ACC2. We validated that these compounds are novel inhibitors of the MPC and suppress hepatocyte glucose production and demonstrated that one quinolone (nalidixic acid) improved glucose tolerance in obese mice. In conclusion, these data demonstrate the feasibility of therapeutic targeting of the MPC for treating diabetes and provide scaffolds that can be used to develop potent and novel classes of MPC inhibitors., Competing Interests: Conflict of interest Brian Finck is a shareholder and member of the scientific advisory board of Cirius Therapeutics, which is developing the MPC inhibitor MSDC-0602K for clinical use. The other authors declare that they have no conflicts of interest with the contents of this article., (Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.)

Competing Interests: The authors report no conflicts of interest.

Objective: The aim of this prospective cross sectional study was to assess the prevalence of sleep disturbance in children with inflammatory bowel disease (IBD), including the relationships between sleep, inflammatory markers, and disease activity of pediatric patients with IBD., Methods: Pediatric patients with IBD and parents were enrolled in the study. Patients completed the Pittsburgh Sleep Quality Index (PSQI), the Pediatric Daytime Sleepiness Scale, and the Adolescent Sleep Wake Scale (ASWS) surveys. Parents completed the Child Sleep Habits Questionnaire (CSHQ). Disease activity for Crohn disease (CD) was determined by the Pediatric Crohn Disease Activity Index and the Pediatric Ulcerative Colitis Activity Index was used to define disease activity in ulcerative colitis (UC)/indeterminate colitis patients., Results: Fifty-three pediatric patients with IBD (38 CD, 12 UC, and 3 indeterminate colitis) participated in the study. The significant correlations between the CSHQ and Pediatric Crohn Disease Activity Index (P = 0.002) and the PSQI and Pediatric Ulcerative Colitis Activity Index (P = 0.04) were found. Youth with UC and indeterminate colitis significantly reported more sleep disturbance than patients with CD, (P = 0.03, 0.05, and 0.04; PSQI, Pediatric Daytime Sleepiness Scale, ASWS, respectively). Patients self-reported significantly more sleep disturbance than was observed by parents (P < 0.0001). This study showed the significant correlations between CSHQ score compared to erythrocyte sedimentation rate and albumin (P = 0.001 and 0.03, respectively)., Conclusions: Results suggest that increased disease activity is associated with adverse effects on sleep quality. Based on the results of this study, pediatric patients with IBD should be screened for sleep disturbance.

Introduction: A brief resolved unexplained event (BRUE) describes an event associated with a change in muscle tone, color, respiration, and responsiveness that is unexplained after an appropriate examination. Some infants with higher risk BRUE may undergo endoscopy as part of their evaluation., Objective: This retrospective study aimed to identify the endoscopic findings in infants who have experienced a higher risk BRUE. We also compared the characteristics, prenatal, natal, and postnatal risk factors between 23 infants who underwent endoscopic evaluation and 23 race-matched/sex-matched/term-matched/preterm-matched infants who did not undergo endoscopic evaluation., Methods: This was a retrospective descriptive study. Infants were identified from a query of medical records using the ICD-10 code for BRUE (R68.13)., Results: Of 119 infants with BRUE, 23 infants with higher risk BRUE underwent an esophagogastroduodenoscopy and flexible sigmoidoscopy. Apnea (87%) was the most common presentation of BRUE. Most were female (57%) with a mean age at BRUE presentation of 2.73 months. We found 10 (43.5%) term infants and 13 (56.5%) preterm infants in our study. There were no significant differences in characteristics, prenatal, natal, and postnatal risk factors between the infants who underwent endoscopy and those who did not undergo endoscopy. The most common abnormal endoscopic finding was lymphonodular hyperplasia (LNH) associated with eosinophilia in the rectosigmoid colon. The proportion of females in the LNH group was significantly higher than the non-LNH group., Conclusion: Rectosigmoid LNH and eosinophilia, which are associated with milk soy protein intolerance (MSPI), were the most common findings on endoscopic evaluation. Although there is no proof of causation between MSPI and BRUE, MSPI should be considered in the differential diagnosis for higher risk BRUE.

Background: Brief Resolved Unexplained Events (BRUE) is defined as a sudden, brief and now resolved episode characterized by color change, altered respirations, change in tone, and altered level of responsiveness. This study aims to identify the characteristics of esophageal Multichannel Intraluminal Impedance-pH (MII-pH) monitoring in infants who have experienced a BRUE., Methods: This study was a retrospective review of records of infants younger than 12 months who presented to the University of South Alabama Children's and Women's Hospital with an admission diagnosis of BRUE. Patients who underwent esophageal MII-pH monitoring between October 2015 and February 2017 and diagnosed with BRUE were initially included in this study., Results: Fifty-three infants (preterm 25, term 28) who experienced a higher risk BRUE were included in our study. The mean age at diagnosis was 2.25 ± 2.07 months. Apnea (41/53; 77.4%) was the most common manifestation of BRUE. Non-acid reflux events were the most common findings in the MII-pH studies (66%). MII-pH results showed 6/53 (11%) acid reflux, 17/53 (32%) non-acid reflux and 12/53 (23%) both acid/nonacid reflux and 18/53 (34%) were normal. There were significant differences in the longest acid reflux episode and the Reflux Symptom Sensitivity Index (RSSI) of coughing/choking/gagging between preterm and term infants. The Reflux Symptom Index (RSI), RSSI and Reflux Symptom Association Probability (RSAP) were significantly correlated with each other in all symptoms (pain/fussiness, coughing/choking/gagging and vomiting)., Conclusions: Among infants experiencing a higher risk BRUE, esophageal MII-pH monitoring revealed acid or nonacid reflux in 2/3 of patients., Competing Interests: Conflict of Interest: The authors have no conflicts of interest to disclose.

Background: Pediatric patients with inflammatory bowel disease (IBD) are at risk for psychiatric symptoms that impact quality of life (QoL) and psychosocial functioning. Sleep disturbance has been reported to impose adverse effects on host defense mechanisms by affecting the magnitude and characteristics of the inflammatory response. The current study sought to assess the relationships among sleep disturbance, QoL, and psychosocial functioning in children with IBD., Methods: Pediatric IBD patients completed multiple measures of sleep and daytime functioning as well as measures of QoL and psychosocial functioning. The parents completed complementary measures of sleep, QoL, and psychosocial functioning. The HRQOL results for subjects with IBD were compared to a healthy control group., Results: Fifty-three children with pediatric IBD and their parents were enrolled in the study. QoL was positively associated with sleep quality, based on significant negative correlations between QoL and both sleep quality and daytime sleepiness scales (r = -0.62, -0.57; p value <0.001, respectively). Patients with CD reported significantly better QoL and psychosocial functioning than patients with UC. The QoL was similar between IBD patients and healthy controls., Conclusions: The present study suggests that a positive association exists between sleep functioning and QoL in pediatric patients with IBD. Patients with pediatric IBD should be screened for sleep disturbance, QoL and psychosocial functioning. Prevention and intervention strategies of sleep disturbance aimed at improving QoL and psychosocial functioning in children with IBD should be developed and evaluated.

Background: Esophageal multichannel intraluminal impedance-pH monitoring has become one of the preferred tests to correlate observed reflux-like behaviors with esophageal reflux events. The Gastroesophageal reflux disease Assessment Symptom Questionnaire is a validated tool used to distinguish infants with gastroesophageal reflux disease from healthy children. The aim of this study was to determine whether the Gastroesophageal reflux disease Assessment Symptom Questionnaire composite symptom scores and individual symptom scores correlate with outcomes in esophageal multichannel intraluminal impedance-pH monitoring., Methods: A total of 26 patients with gastroesophageal reflux disease-associated symptoms, aged 0-2 years, for whom both esophageal multichannel intraluminal impedance-pH monitoring and Gastroesophageal reflux disease Assessment Symptom Questionnaire survey results were available were included in the study. Gastroesophageal reflux disease Assessment Symptom Questionnaire score data were collected from a 7-day recall of parent's responses about the frequency and severity of gastroesophageal reflux disease symptoms, which determined the individual symptom scores. The composite symptom scores is the sum of all individual symptom scores. Multichannel intraluminal impedance-pH study results were compared to Gastroesophageal reflux disease Assessment Symptom Questionnaire data using Pearson correlation., Results: Among 26 patients, a total number of 2817 (1700 acid and 1117 non-acid) reflux episodes and 845 clinical reflux behaviors were recorded. There were significant correlations between the reflux index and the individual symptom scores for coughing/gagging/choking (r 2 = 0.2842, p = 0.005), the impedance score and individual symptom scores for coughing/gagging/choking (r 2 = 0.2482, p = 0.009), the reflux symptom index for acid reflux-related coughing/gagging/choking and the individual symptom scores for coughing/gagging/choking (r 2 = 0.1900, p = 0.026), the impedance score and individual symptom scores for vomiting (r 2 = 0.1569, p = 0.045), and the impedance score and the composite symptom scores (r 2 = 0.2916, p = 0.004). However, there were no significant correlations between fussiness, irritability, or abdominal pain-related multichannel intraluminal impedance-pH results and the individual symptom scores for abdominal pain., Conclusion: The impedance scores from multichannel intraluminal impedance-pH studies correlate with coughing/gagging/choking and vomiting in infants with gastroesophageal reflux disease. There are no significant correlations among the reflux index and impedance score versus the Gastroesophageal reflux disease Assessment Symptom Questionnaire scores for abdominal pain. We conclude that in infants with gastroesophageal reflux disease, multichannel intraluminal impedance-pH studies are more likely to demonstrate an association between gastroesophageal reflux disease and symptoms of coughing, gagging, or choking compared to an association between gastroesophageal reflux disease and pain in infants., Competing Interests: Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Gastrointestinal bleeding (GIB) is one of the potential causes of increased morbidity and mortality in immunocompromised patients, but data on characteristics of GIB in immunocompromised children are sparse., Objectives: This study aimed to identify the etiology, endoscopic, and histologic findings of GIB in immunocompromised children., Design: This was a retrospective descriptive study., Patients: We identified 33 patients (aged<20 years) and 45 GIB episodes related to GIB between January 2007 and April 2015 from a tertiary care and teaching hospital., Results: The mean age at endoscopy was 10.7±4.6 years. Most common indications for endoscopy were melena in upper GIB and hematochezia in lower GIB. The median delay of duration between GIB presentation to endoscopy was 3 days. All except one child had at least one endoscopic abnormality. The most common cause of upper GIB was cytomegalovirus (CMV)-related gastrointestinal disease (35%), followed by esophageal varices (26%), and the most common cause of lower GIB was CMV-related gastrointestinal disease (55%). Fourteen percent of patients died during upper GIB episodes and 15% died during lower GIB episodes., Conclusion: Among immunocompromised individuals aged younger than 20 years presenting with GIB, CMV-related gastrointestinal disease is the most prevalent in our study population. However, the etiology of immunocompromised state needs to be taken into consideration when evaluating these children presenting with GIB.

Henoch-Schönlein purpura (HSP) is a multi-organ vasculitis involving skin, joints, gastrointestinal tract, and kidneys. The present study reported a 5-year-old boy presenting with intense abdominal pain, bloody diarrhea, and protein-losing enteropathy. Investigations for infectious enteritis were negative. Esophagogastroduodenoscopy showed swelling and erythematous mucosa with hemorrhagic spots at duodenal bulb to the third part of duodenum. Histopathology of endoscopic biopsies revealed non-specific duodenitis. HSP was suspected, based on duodenitis and the presence of inflammatory markers without identifiable causes. Corticosteroid was started resulting in marked improvement of his clinical symptoms. Two weeks later, he developed nephrotic-range proteinuria, thus kidney biopsy was performed. Renal histology was consistent with IgA nephropathy, supporting the diagnosis of HSP This report emphasizes that patients with HSP may not always show visible purpura, and the diagnosis requires a high index of suspicion. GI endoscopy and renal biopsy may be helpful for the diagnosis in selected patients presenting with atypical presentations.

Objective: To determine the efficacy of prophylactic pharmacotherapy on the short- and long-term outcomes of children with cyclic vomiting syndrome (CVS)., Material and Method: Medical records were reviewed in 32 children who were diagnosed with CVS between 2000 and 2013. Efficacy ofprophylactic medications was classified as good vs. no response after treatment for three to six months. Long-term outcome was evaluated inpatients who had been diagnosedfor > or =2 years and classiied as 1) excellent: no episode, 2) good: one to two episodes, and 3) poor: three episodes or more during the past year., Results: At three to six months after treatment, good response to amitriptyline was significantly higher than propranolol (73% vs. 36%, p = 0.04). Of the 24 CVS patients whohad been diagnosed > or =2 years, data was available in 19 patients (mean age, 11.3 +/- 4.9; and mean duration from diagnosis to follow-up, 6.3+3.3 years). Excellent outcome was achieved in seven, good in seven, and poor in five children. Overall, the favorable long-term outcome (good and excellent) was 74%. Most children (86%) who hadfavorable long-term outcome had good response to the prophylactic medications in the early period of treatment., Conclusion: Amitriptyline may be more effective than propranololforprophylaxis of CVS. However, a randomized controlled trial is required to confirm this result. Children with CVS have a relatively favorable long-term outcome, particularly those who initially responded well to the prophylactic medications.

We report here the case of the youngest patient with adenomyomatosis of the gallbladder in a female infant diagnosed at 4 months of age. This diagnosis was made based on characteristic ultrasonography findings in a patient that was undergoing routine surveillance for a suspected clinical diagnosis of Beckwith-Wiedemann syndrome. The patient remains asymptomatic and currently no surgical interventions have been needed. We review the pathophysiology and ultrasonographic findings of this rare condition and present a comparison with the only other four pediatric cases of adenomyomatosis of the gallbladder.