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Aim: Patients who undergo the Fontan procedure for complex congenital heart disease are prone to liver cirrhosis. Liver stiffness (LS) reflects liver fibrosis stage in patients with chronic viral hepatitis; however, its accuracy in predicting liver fibrosis stage in Fontan patients is controversial. We aimed to clarify the correlation between LS and liver fibrosis stage in Fontan patients. Methods: Fifty‐eight Fontan patients were prospectively measured for LS with transient elastography. We undertook liver biopsy, cardiac catheterization, and laboratory tests in 22 of these patients (median age, 14.7 years; range, 9.9–32.1 years) with LS > 11.0 kPa (median, 19.2 kPa; range, 12.2–39.8 kPa); these elevated LS values suggest liver cirrhosis. Results: Histologically, all patients showed mild‐to‐severe portal and sinusoidal fibrosis but no cirrhosis. Statistically, LS did not predict histological liver fibrosis scores (p = 0.175). Liver stiffness was not correlated with central venous pressure (p = 0.456) or with the hepatic venous pressure gradient (HVPG; p = 0.062), although the p value for HVPG was only slightly above the threshold for significance. Conclusions: Fontan patients are prone to developing both portal and sinusoidal fibrosis. Liver stiffness could be influenced by HVPG, and using the conventional cut‐off values for LS overestimates and overtreats liver fibrosis in these patients. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 16-year-old girl who had congenital hypoplastic left heart syndrome and underwent a Fontan procedure at the age of 1.7 years. It mentions esophageal retention of a patency capsule due to extraesophageal compression from pulmonary artery stent grafts; and also mentions how physical development influences the sizes or distances associated with mediastinal organs.

Background and Aims: Pediatric use of liver transient elastography (TE) is attractive for its non-invasiveness, but reference values have not been established. We aimed to determine reference values for TE in children. Methods: In pediatric patients (1 to 18 years), TE (FibroScan®) with an M probe was used for both liver stiffness measurement (LSM) and measurement of hepatic fat deposition by using a controlled attenuation parameter (CAP). The patients were divided into three relevant age groups: preschoolers (1 to 5 years), elementary school children (6 to 11 years), and adolescents (12 to 18 years). Overweight or obese patients or those with known liver disease, elevated serum liver enzymes, or hepatic echogenic abnormality were excluded from the study. Results: Among 139 children, 123 (88.5%; 62 male; median age, 11.7 years; age range, 1.3 to 17.2 years) were successfully subjected to M-probe TE without anesthesia. Median LSM increased with age: it was 3.4 kPa (2.3 to 4.6 kPa, 5th to 95th percentiles) at ages 1 to 5 years; 3.8 (2.5 to 6.1) kPa at ages 6 to 11; and 4.1 (3.3 to 7.9) kPa at ages 12 to 18 (P = 0.001). Median CAP was not age dependent: it was 183 (112 to 242) for ages 1 to 18 years. Conclusions: M-probe TE is suitable in a wide age range of children from age 1 year up. In children without evidence of liver disease, LSM has an age-dependent increase, whereas CAP does not differ between ages 1 and 18. [ABSTRACT FROM AUTHOR]

Background & Aims: The utility of transient elastography (FibroScan) is well studied in adults but not in children. We sought to assess the feasibility of performing FibroScans and the characteristics of FibroScan-based liver profiles in Japanese obese and non-obese children. Methods: FibroScan examinations were performed in pediatric patients (age, 1–18 yr) who visited Osaka City University Hospital. Liver steatosis measured by controlled attenuation parameter (CAP), and hepatic fibrosis evaluated as the liver stiffness measurement (LSM), were compared among obese subjects (BMI percentile ≥90%), non-obese healthy controls, and non-obese patients with liver disease. Results: Among 214 children examined, FibroScans were performed successfully in 201 children (93.9%; median, 11.5 yr; range, 1.3–17.6 yr; 115 male). CAP values (mean±SD) were higher in the obese group (n = 52, 285±60 dB/m) compared with the liver disease (n = 40, 202±62, P<0.001) and the control (n = 107, 179±41, P<0.001) group. LSM values were significantly higher in the obese group (5.5±2.3 kPa) than in the control (3.9±0.9, P<0.001), but there were no significant differences in LSM between the liver disease group (5.4±4.2) and either the obese or control group. LSM was highly correlated with CAP in the obese group (ρ = 0.511) but not in the control (ρ = 0.129) or liver disease (ρ = 0.170) groups. Conclusions: Childhood obesity carries a high risk of hepatic steatosis associated with increased liver stiffness. FibroScan methodology provides simultaneous determination of CAP and LSM, is feasible in children of any age, and is a non-invasive and effective screening method for hepatic steatosis and liver fibrosis in Japanese obese children. [ABSTRACT FROM AUTHOR]

Hepatic focal nodular hyperplasia (FNH) is a rare benign tumor in children. Vascular anomalies have been identified as pathological features of FNH, but the etiology remains unclear. We describe a rare case including the time course of formation of hepatic FNH in response to congenital portosystemic shunt (PSS). A 4-month-old girl was identified on newborn mass screening to have hypergalactosemia, but no inherited deficiencies in galactose-metabolizing enzymes were found. Ultrasonography and per-rectal portal scintigraphy showed intrahepatic PSS of the right lobe as a cause of the hypergalactosemia. At age 12 months, the patient had elevated hepatic enzymes and small hypoechoic hepatic lesions around the shunt. On abdominal contrast-enhanced ultrasonography spoke-wheel sign and central stellate scar were seen, which are typical features of hepatic FNH without biopsy. Congenital intrahepatic PSS should be evaluated on abdominal contrast-enhanced ultrasonography and observed over time because of its potential to develop into hepatic FNH. [ABSTRACT FROM AUTHOR]

• Neonates have limited innate immune responses to infection. • We compared cord blood (CD14+CD16high) and adult (CD14+CD16intermediate) monocytes. • Cord blood monocytes had low basal expression of MHC class II, CD80, and CD11b. • TLR4 or TLR2/6 stimulation increased the antigens more in cord than adult monocytes. • TLR agonists may have utility as vaccine adjuvants in infants. Toll-like receptors (TLRs) are important components of the innate immune system, but how neonatal TLR-mediated immune responses differ from those of adults is unknown. We aimed to clarify the TLR-mediated expression profiles of cell surface antigens related to antigen presentation in neonates. CD14-positive monocytes were isolated from human cord blood and adult peripheral blood and then stimulated with lipopolysaccharide (LPS; TLR4 agonist) or zymosan (TLR2/6 agonist) or left unstimulated. Expression levels of the surface antigens major histocompatibility (MHC)-class II, CD80, CD86, CD11b, CD11c, CD14, and CD16 were then evaluated by flow cytometry. Cord blood CD14+CD16high monocytes (CBM) showed significantly lower basal levels of MHC-class II, CD80, and CD11b than adult blood CD14+CD16intermediate monocytes (ABM) (P < 0.01, P < 0.001, P < 0.001, respectively). LPS stimulation enhanced expression of MHC class II, CD80, and CD11b significantly more in CBM than in ABM (P < 0.001, P < 0.01, P < 0.01, respectively), resulting in levels that did not differ between CBM and ABM. Zymosan stimulation also enhanced expression of MHC class II, CD86, CD11b, and CD11c significantly more in CBM than in ABM (P < 0.001, P < 0.01, P < 0.001, P < 0.01, respectively), resulting in levels of CD86 and CD11c that did not differ in CBM and ABM. However, MHC class II, CD80, and CD11b remained significantly higher in ABM than in CBM (P < 0.05, P < 0.01, P < 0.05, respectively). These data indicate that CBM and ABM have distinct phenotypes and responses to stimulation. [ABSTRACT FROM AUTHOR]

Objective: Patients who undergo Fontan surgery for complex cardiac anomalies are prone to developing liver and gastrointestinal complications. In particular, gastroesophageal varices (GEVs) can occur, but their prevalence is unknown. We aimed to elucidate the occurrence of GEVs and the predicting parameters of GEVs in these patients. Materials and methods: Twenty-seven patients (median age, 14.8 years; median time since surgery, 12.9 years) who had undergone the Fontan surgery and were examined by abdominal dynamic computed tomography (CT) for the routine follow-up were included in the study. Radiological findings including GEVs and extraintestinal complications were retrospectively evaluated by experienced radiologists in a blinded manner. Relationships between blood-biochemical and demographic parameters and the presence of GEVs were statistically analyzed. Results: Dynamic CT revealed gastric varices (n = 3, 11.1%), esophageal varices (n = 1, 3.7%), and gastrorenal shunts (n = 5, 18.5%). All patients with gastric varices had gastrorenal shunts. All gastric varices were endoscopically confirmed as being isolated and enlarged, with indications for preventive interventional therapy. A platelet count lower than 119 × 109 /L was identified as a predictor of GEV (area under the receiver operating curve, 0.946; sensitivity, 100%; and specificity, 87%). Conclusions: GEVs are important complications that should not be ignored in patients who have undergone a Fontan procedure. Platelet counts lower than 119 × 109 /L may help to prompt patient screening by using abdominal dynamic CT to identify GEVs and their draining collateral veins in these patients. [ABSTRACT FROM AUTHOR]

Background: A patency capsule (PC) can help predict capsule endoscope (CE) retention; however, PC tolerability is unknown in children. We retrospectively evaluated PC tolerability in school-aged children. Methods: Sixty-one patients (median age, 12.9 years; range 7.4-17.3 years) who underwent PC examination were analyzed for occurrence and determinants of ingestion difficulty and relationships between ingestion of the 2 capsules. We defined ingestion difficulty as taking 30 min or more, or failure, to ingest the PC. Results: Thirty-nine patients (64%) successfully ingested the PC without ingestion difficulty. The other 22 had ingestion difficulty and were significantly younger (11.7 ± 2.2 vs. 13.0 ± 1.8 years; p = 0.04) and shorter (143.3 ± 14.0 vs. 154.6 ± 12.5 cm; p = 0.003) than those without ingestion difficulty. Multivariate analysis showed that the most significant factor for predicting PC ingestion difficulty was height (cutoff value, 152 cm). Time to ingest the CE was significantly shorter than that for PC ingestion (8 ± 32 vs. 20 ± 58 min; p = 0.01). All patients indicated that ingestion of the CE was easier because of its smooth surface compared with the PC. Conclusions: PC ingestion is not guaranteed in school-aged children. PC ingestion ability should be evaluated by considering the child's height and lack of experience ingesting capsules prior to PC examination. [ABSTRACT FROM AUTHOR]

Immunosuppressive therapies can affect the immune response to or safety of vaccination in patients with inflammatory bowel disease (IBD). The appropriateness of vaccination should be assessed prior to the initiation of IBD treatment because patients with IBD frequently undergo continuous treatment with immunosuppressive drugs. This consensus was developed to support the decision-making process regarding appropriate vaccination for pediatric and adult patients with IBD and physicians by providing critical information according to the published literature and expert consensus about vaccine-preventable diseases (VPDs) [excluding cervical cancer and coronavirus disease 2019 (COVID-19)] in Japan. This consensus includes 19 important clinical questions (CQs) on the following 4 topics: VPDs (6 CQs), live attenuated vaccines (2 CQs), inactivated vaccines (6 CQs), and vaccination for pregnancy, childbirth, and breastfeeding (5 CQs). These topics and CQs were selected under unified consensus by the members of a committee on intractable diseases with support by a Health and Labour Sciences Research Grant. Physicians should provide necessary information on VPDs to their patients with IBD and carefully manage these patients' IBD if various risk factors for the development or worsening of VPDs are present. This consensus will facilitate informed and shared decision-making in daily IBD clinical practice. [ABSTRACT FROM AUTHOR]

The article presents a case study of an11-year-old boy presented with a 2 week history of fever, vomiting, abdominal pain, anorexia, and massive melena. Topics include capsule endoscopy showed multiple hematoma-like protrusions over the duodenum and proximal jejunum; and palpable purpura has the common manifestation of immunoglobulin A vasculitis, but abdominal symptoms precede the purpuric rash typical of IgA vasculitis in 14–36% of patients.

Background & Aims: Although transient elastography (TE) is used to determine liver stiffness as a surrogate to hepatic fibrosis, the normal range in children is not well defined. We performed a systematic review and individual participant data (IPD) meta‐analysis to determine the range of liver stiffness in healthy children and evaluate the influence of important biological parameters. Methods: We pooled data from 10 studies that examined healthy children using TE. We divided 1702 children into two groups: ≥3 years (older group) and < 3 years of age (younger group). Univariate and multivariate linear regression models predicting liver stiffness were conducted. Results: After excluding children with obesity, diabetes, or abnormal liver tests, 652 children were analysed. Among older children, mean liver stiffness was 4.45 kPa (95% confidence interval 4.34‐4.56), and increased liver stiffness was associated with age, sedation status, and S probe use. In the younger group, the mean liver stiffness was 4.79 kPa (95% confidence interval 4.46‐5.12), and increased liver stiffness was associated with sedation status and Caucasian race. In a subgroup analysis, hepatic steatosis on ultrasound was significantly associated with increased liver stiffness. We define a reference range for normal liver stiffness in healthy children as 2.45‐5.56 kPa. Conclusions: We have established TE‐derived liver stiffness ranges for healthy children and propose an upper limit of liver stiffness in healthy children to be 5.56 kPa. We have identified increasing age, use of sedation, probe size, and presence of steatosis on ultrasound as factors that can significantly increase liver stiffness. [ABSTRACT FROM AUTHOR]

Insulinoma is generally identified as a single tumor and seldom occurs in children or adolescents. A 14-year-old girl with difficulty in waking was found to have hyperinsulinemic hypoglycemia. On abdominal ultrasonography two hypoechoic masses (8 and 12 mm in diameter) were seen in the pancreatic body: the larger mass was hypervascular, whereas the smaller one was hypovascular. Contrast-enhanced computed tomography showed enhancement of the larger mass, but did not delineate the smaller mass. On fat-suppressed T1-weighted magnetic resonance imaging, the larger mass was hypointense, but the smaller mass was hyperintense. Pathologically, the larger tumor was normal density, insulin positive, and rich in vascularity, whereas the smaller tumor was high density, insulin negative, and poor in vascularity. The present case suggests that difficulty waking should be considered as a potential etiology in insulinoma, and multiple suspected pancreatic insulinomas should be evaluated using a combination of imaging modalities to characterize each tumor. [ABSTRACT FROM AUTHOR]

Motivation is an important psychological concept in academic learning. Subjects performed jigsaw puzzle and square puzzle sessions (as difficulty variant task) and 80%, 50%, and 20% completion sessions (as completion variant task). After square puzzle or 20% completion sessions, subjective motivation decreased. Although baseline scores on an academic motivation scale were negatively correlated with changes in subjective motivation for the square puzzle session, a positive correlation was observed for the 20% completion session. These suggest that while continual completion of facile task trials may support the motivation of college students with lower academic motivation, attempting difficult task trials may sustain that of those with higher academic motivation. [ABSTRACT FROM AUTHOR]