• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.21 no.2
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• pp.111-117
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• 2018

Purpose: The prevalence of obesity has significantly increased among children and adolescents worldwide and is becoming an important health care problem in parallel with the increased prevalence of obesity pediatric non-alcoholic fatty liver disease. Betatrophin is a newly define hormone that is commonly secreted by liver and plays role in glucose tolerance. This study aimed to investigate the relationship between serum betatrophin levels and non-alcoholic fatty liver disease in obese children. Methods: The study included 40 obese children with a body mass index (BMI) above 95th centile, and 35 non-obese subjects with a BMI 3-85th centile, whose age and gender were similar to those of the patient group. For the evaluation of metabolic parameters fasting serum glucose, insulin, alanine aminotransferase, aspartate aminotransferase, lipid profile and serum betatrophin levels were measured. Total cholesterol: high-density lipoprotein cholesterol and low-density lipoprotein cholesterol: high-density lipoprotein cholesterol ratios were calculated as "atherogenic indices." Results: Serum betatrophin levels of the obese subjects were similar to that of non-obese subjects (p=0.90). Betatrophin levels were not correlated with the metabolic parameters. Conclusion: In the present study, levels of betatrophin are not different between obese and insulin resistant children and non-obese subjects, and they are not correlated with atherogenic indices. To elucidate the exact role of betatrophin in obesity, further studies are required to identify the betatrophin receptor and/or other possible cofactors.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.21 no.2
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• pp.147-153
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• 2018

Epstein-Barr virus (EBV) infection can be presented with various clinical manifestations and different levels of severity when infected. Infectious mononucleosis, which is most commonly caused by EBV infection in children and adolescents, is a clinical syndrome characterized by fatigue, malaise, fever, sore throat, and generalized lymphadenopathy. But rarely, patients with infectious mononucleosis may present with gastrointestinal symptoms and complicated by gastritis, splenic infarction, and splenic rupture. We encountered a 16-year-old girl who presented with fever, fatigue, and epigastric pain. Splenic infarction and EBV-associated gastritis were diagnosed by using esophagogastroduodenoscopy and abdominal computed tomography. Endoscopy revealed a generalized hyperemic nodular lesion in the stomach, and the biopsy findings were chronic gastritis with erosion and positive in situ hybridization for EBV. As splenic infarction and acute gastritis are rare in infectious mononucleosis and are prone to be overlooked, we must consider these complications when an infectious mononucleosis patient presents with gastrointestinal symptom.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.21 no.1
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• pp.28-33
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• 2018

Purpose: To evaluate the efficacy of synbiotic formula with partial whey hydrolysate and high magnesium content in infants presenting with functional constipation. Methods: Sixty-five infants with functional constipation were included. Forty infants were treated during one month with parental reassurance and the intervention formula and were compared to a control group of 25 infants treated with parental reassurance only. Parents completed a quality of life (QoL) questionnaire at baseline and during the last week of the study. Results: At inclusion, stool characteristics and QoL were similar in both groups. The control group was slightly older than the intervention group ($7.5{\pm}3.9$ vs. $6.2{\pm}3.6$ weeks). At onset, stool composition was "hard and tight" (Bristol stool scale 1 and 2) in all infants. After one month, stool composition remained unchanged in the control group except in two infants that developed "creamy" stools (Bristol stool scale type 3 and 4). In the intervention group, stools remained "hard and tight" in 27.5%, and became "creamy" in 47.5%, "loose" (Bristol stool scale type 5) in 22.5% and "watery" (Bristol stool type 6 and 7) in 2.5%. The benefit of the intervention formula was estimated to be "very important" in 70%. The median scores for QoL improved significantly in the intervention group for all parameters and for one in the control group. Conclusion: The intervention formula significantly improved functional constipation resulting in a better QoL of the parents and infants.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.21 no.1
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• pp.59-67
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• 2018

Purpose: to compare cut off points corrected for age and gender (COOP) with fixed cut off points (FCOP) for fasting plasma insulin and Homeostatic model assessment-insulin resistance (HOMA-IR) for the diagnosis of IR in obese children and adolescents and their correlation with dyslipidemia. Methods: A multicenter, cross-sectional study including 383 subjects aged 7 to 18 years, evaluating fasting blood glucose, plasma insulin, and lipid profile. Subjects with high insulin levels and/or HOMA-IR were considered as having IR, based on two defining criteria: FCOP or CCOP. The frequency of metabolic abnormalities, the presence of IR, and the presence of dyslipidemia in relation to FCOP or CCOP were analyzed using Fisher and Mann-Whitney exact tests. Results: Using HOMA-IR, IR was diagnosed in 155 (40.5%) and 215 (56.1%) patients and, using fasting insulin, 150 (39.2%) and 221 (57.7%), respectively applying FCOP and CCOP. The use of CCOP resulted in lower insulin and HOMA-IR values than FCOP. Dyslipidemia was not related to FCOP or CCOP. Blood glucose remained within normal limits in all patients with IR. There was no difference in the frequency of IR identified by plasma insulin or HOMA-IR, both for FCOP and CCOP. Conclusion: The CCOP of plasma insulin or of HOMA-IR detected more cases of IR as compared to the FCOP, but were not associated with the frequency of dyslipidemia. As blood glucose has almost no fluctuation in this age group, even in the presence of IR, fasting plasma insulin detected the same cases of IR that would be detected by HOMA-IR.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.21 no.1
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• pp.20-27
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• 2018

Purpose: Minimal change esophagitis (MCE) is a reflux disease without mucosal breaks, known to be partially associated with abnormal gastric motor function. Electrogastrography (EGG) is commonly applied to assess gastric motor function in a noninvasive fashion. We aimed to determine the relationship between MCE and gastric myoelectrical activity (GME) recorded on EGG in children. Methods: We retrospectively assessed the records of 157 children without underlying disease who underwent both EGG and upper gastrointestinal endoscopy at Gachon University Gil Medical Center between January 2010 and June 2015. The children were stratified according to the appearance of the esophagus (normal vs. MCE). Between-group differences in EGG parameters and their correlation with each MCE finding were statistically analyzed. Results: Only the power ratio, one of the EGG parameters analyzed, differed significantly between the two groups (MCE, $1.68{\pm}3.37$ vs. normal, $0.76{\pm}1.06$; p<0.05), whereas the other parameters, such as dominant frequency, dominant power, and the ratio of abnormal rhythm, showed no differences. Among children with MCE, significant correlations were noted between erythema and power ratio (p<0.05), friability and postprandial dominant frequency (p<0.05), and edema and/or accentuation of mucosal folds and pre-prandial frequency (p<0.05). Helicobacter pylori infection correlated with postprandial arrhythmia (MCE, $33.59{\pm}15.52$ vs. normal, $28.10{\pm}17.23$; p<0.05). EGG parameters did not differ between children with normal esophagus and those with biopsy-proven chronic esophagitis. Conclusion: In children with MCE, gastric dysmotility may affect the development of MCE, manifesting as EGG abnormalities. H. pylori infection may also affect GME. However, larger prospective investigations are needed to confirm these findings.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.21 no.1
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• pp.34-42
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• 2018

Purpose: Monogenic inflammatory bowel disease (IBD) patients do not respond to conventional therapy and are associated with a higher morbidity. We summarized the clinical characteristics of monogenic IBD patients and compared their clinical outcomes to that of non-monogenic IBD patients. Methods: We performed a retrospective cohort study of all children <18 years old who were diagnosed with IBD between 2005 and 2016. A total of 230 children were enrolled. Monogenic IBD was defined as a presentation age less than 6 years old with confirmation of a genetic disorder. We subdivided the groups into monogenic IBD (n=18), non-monogenic very early-onset IBD (defined as patients with a presentation age <6 years old without a confirmed genetic disorder, n=12), non-monogenic IBD (defined as all patients under 18 years old excluding monogenic IBD, n=212), and severe IBD (defined as patients treated with an anti-tumor necrosis factor excluding monogenic IBD, n=92). We compared demographic data, initial pediatric Crohn disease activity index/pediatric ulcerative colitis activity index (PCDAI/PUCAI) score, frequency of hospitalizations, surgical experiences, and height and weight under 3rd percentile among the patients enrolled. Results: The initial PCDAI/PUCAI score (p<0.05), incidence of surgery per year (p<0.05), and hospitalization per year (p<0.05) were higher in the monogenic IBD group than in the other IBD groups. Additionally, the proportion of children whose weight and height were less than the 3rd percentile (p<0.05 and p<0.05, respectively) was also higher in the monogenic IBD group. Conclusion: Monogenic IBD showed more severe clinical manifestations than the other groups.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.21 no.1
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• pp.43-50
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• 2018

Purpose: Clostridium difficile colonization and infection are commonly associated with poor outcomes in patients with pediatric inflammatory bowel disease (PIBD). We aimed to investigate the prevalence of C. difficile colonization and infection at the time of diagnosis and to evaluate risk factors associated with the development of C. difficile infection during the course of PIBD treatment. Methods: We retrospectively enrolled a total of 59 children who were newly diagnosed with PIBD at the tertiary medical center. All patients underwent C. difficile toxin assays and cultures initially and at every follow-up during the disease course. Kaplan-Meier survival analysis and Cox regression test were used for statistical analysis. Results: Initial cultures for C. difficile were positive in 13 (22.0%) of 59 PIBD patients, whereas initial toxin assays were positive in 3 patients (5.1%). During treatment, C. difficile cultures converted to positive in 28 (47.5%) in addition to 13 patients who were initially culture-positive, and C. difficile toxins converted to positive in 13 (22.0%) in addition to 3 originally toxin-positive patients. Antibiotic usage alone was significantly associated with the development of C. difficile colonization (p=0.011), and the length of hospitalization was associated with the development of C. difficile infection (p=0.032). Conclusion: C. difficile colonization and infection occur frequently during the disease course of PIBD. Antibiotic usage and longer hospital stay were significant risks factors for the conversion of C. difficile status in PIBD patients undergoing treatment.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.21 no.1
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• pp.51-58
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• 2018

Purpose: To clarify the clinical features of appendicitis in preschool children and to explore clinical appendicitis scoring systems in this age group. Methods: We retrospectively collected data on 142 children, aged 10 years or younger, with confirmed diagnosis of appendicitis based on surgical and pathologic findings. Enrolled subjects were divided into two groups: Group 1 (preschool children aged ${\leq}5$ years, n=41) and Group 2 (school children aged >5 to ${\leq}10$ years, n=101). Data analyzed included clinical presentation, laboratory findings, the pediatric appendicitis score (PAS), and the modified Alvarado score (MAS). Results: The most common presenting symptom was abdominal pain in both groups (92.7% vs. 97.0%). Other presenting symptoms were as follows: fever (65.9%), vomiting (68.3%), right lower quadrant (RLQ) localization (24.4%), anorexia (14.6%), and diarrhea (7.3%) in Group 1, and RLQ localization (74.3%), vomiting (71.3%), anorexia (52.5%), fever (47.5%), and diarrhea (11.9%) in Group 2. Perforation and abscess occurred more frequently in Group 1 than in Group 2 (43.9% vs. 12.9%, p<0.001; 34.1% vs. 5.0%, p<0.001; respectively). PAS and MAS were lower in Group 1 than in Group 2 ($4.09{\pm}1.97$ vs. $6.91{\pm}1.61$, p=0.048; $4.65{\pm}1.79$ vs. $6.51{\pm}1.39$, p=0.012; respectively). Conclusion: In preschool children, appendicitis often presents with atypical features, more rapid progression, and higher incidence of complications. This age group is more likely to have lower PAS and MAS than those of school children.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.2
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• pp.174-179
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• 2020

We present an 8 years old girl who was diagnosed at 6 months of age of Progressive Familial Intrahepatic Cholestasis type 2. Although liver transplantation (LT) was classically considered curative for these patients, cholestasis recurrence with normal gamma-glutamyl transpeptidase (GGT), mediated by anti-bile salt export pump (BSEP) antibodies after LT (auto-antibody Induced BSEP Deficiency, AIBD) has been recently reported. Our patient underwent LT at 14 months. During her evolution, patient presented three episodes of acute rejection. Seven years after the LT, the patient presented pruritus with cholestasis and elevation of liver enzymes with persistent normal GGT. Liver biopsy showed intrahepatic cholestasis and giant-cell transformation with very low BSEP activity. Auto-antibodies against BSEP were detected therefore an AIBD was diagnosed. She was treated with Rituximab and immunoadsorption with resolution of the AIBD. As a complication of the treatment she developed a pneumocystis infection successfully treated with corticoids, cotrimoxazol and anidulafungin.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.2
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• pp.154-162
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• 2020

Purpose: The Cow's Milk-related Symptom Score (CoMiSS^TM), which considers crying, regurgitation, stools, skin and respiratory symptoms, was developed as an awareness tool for evaluating cow's milk-related symptoms. The scoring ranges from 0 to 33. A score ≥12 was proposed as being likely cow's milk-related and suggestive of allergy to cow's milk. This study aimed to determine the age-related CoMiSS^TM values in presumed healthy infants in Poland. Methods: This was a cross-sectional study conducted in well-child clinics in two locations. Parents of the presumed healthy infants aged ≤6 months were approached during a routine checkup/vaccination visit. The exclusion criteria were as follows: presence of acute or chronic diseases, preterm delivery, treatment with therapeutic formula, and use of any food supplements (except vitamins) or medications. Results: Data from 226 infants were obtained (median age [Q1-Q3], 4 months [3-4]). The overall median (Q1-Q3) and mean (standard deviation) CoMiSS^TM values were 4 (2-7) and 4.7 (3.5), respectively. The 95th percentile was 11. Scores on some, albeit not all, components of the CoMiSS^TM significantly differed between age groups (crying, stools) or feeding type groups (stools and skin symptoms). Eleven children (4.9%) scored ≥12. Conclusion: This study adds to earlier age-related CoMiSS^TM data by providing CoMiSS^TM values in presumed healthy infants in Poland.