• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제20권2호
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• pp.114-123
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• 2017

Purpose: The goal of this study was the early diagnosis of ABCB11 spectrum liver disorders, especially those focused on benign recurrent intrahepatic cholestasis and progressive familial intrahepatic cholestasis. Methods: Fifty patients presenting neonatal cholestasis were evaluated to identify underlying etiologies. Genetic analysis was performed on patients suspected to have syndromic diseases or ABCB11 spectrum liver disorders. Two families with proven ABCB11 spectrum liver disorders were subjected to genetic analyses to confirm the diagnosis and were provided genetic counseling. Whole exome sequencing and Sanger sequencing were performed on the patients and the family members. Results: Idiopathic or viral hepatitis was diagnosed in 34%, metabolic disease in 20%, total parenteral nutrition induced cholestasis in 16%, extrahepatic biliary atresia in 14%, genetic disease in 10%, neonatal lupus in 2%, congenital syphilis in 2%, and choledochal cyst in 2% of the patients. The patient with progressive familial intrahepatic cholestasis had novel heterozygous mutations of ABCB11 c.11C>G (p.Ser4*) and c.1543A>G (p.Asn515Asp). The patient with benign recurrent intrahepatic cholestasis had homozygous mutations of ABCB11 c.1331T>C (p.Val444Ala) and heterozygous, c.3084A>G (p.Ala1028Ala). Genetic confirmation of ABCB11 spectrum liver disorder led to early liver transplantation in the progressive familial intrahepatic cholestasis patient. In addition, the atypically severe benign recurrent intrahepatic cholestasis patient was able to avoid unnecessary liver transplantation after genetic analysis. Conclusion: ABCB11 spectrum liver disorders can be clinically indistinguishable as they share similar characteristics related to acute episodes. A comprehensive genetic analysis will facilitate optimal diagnosis and treatment.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제20권2호
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• pp.94-99
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• 2017

Purpose: Abdominal cysts of gastrointestinal origin are rare. Their rarity and varied clinical presentations make their pre-operative diagnosis difficult. Methods: Fourteen patients with histological diagnosis of cysts of gastrointestinal origin admitted between 2009 and 2015 were retrospectively analyzed with respect to age, sex, clinical presentation, diagnostic modality, site and type of cyst, management, outcome and follow-up. Results: The mean age at presentation was 4 years and there were six males and eight females. Abdominal pain was the most common presenting symptom. Five patients had an acute presentation-three had distal ileal mesenteric cysts and two had ileal duplication cyst sharing a common wall with ileum. Six patients presented with chronic abdominal pain and lump-three patients had omental cysts and three had mesenteric cysts-two of these in distal ileum and one in sigmoid colon. Two patients presented with antenatally diagnosed palpable abdominal lump. One had a mesenteric cyst of the ileum and the other had a distal ileal duplication cyst which required excision with resection and anastomosis. One patient had an atypical presentation. He was a known case of sickle cell trait and had presented with vague abdominal pain, recurrent cough and multiple episodes of haemoptysis over a period of one year. At laparotomy, gastric duplication cyst was found which was excised completely. Histopathology confirmed the diagnosis. Conclusion: Cysts of gastrointestinal origin are rare and have varied presentation. Surgical excision is the mainstay of treatment. The results and prognosis are good.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제20권2호
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• pp.100-106
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• 2017

Purpose: The purpose of this paper is to evaluate the efficacy of a lactose- reduced synbiotic partial whey hydrolysate in formula fed infants presenting with colic and the impact of this dietary intervention in mean crying time and quality of life. Methods: Forty infants with infantile colic were treated during one month with parental reassurance and the intervention formula (partial whey hydrolysate, reduced lactose, Bifidobacterium lactis BB12 and galacto-oligosaccharides) and were compared to a control group of 20 infants with infantile colic treated with parental reassurance and a standard infant formula. Parents completed a quality of life (QoL) questionnaire assessing the burden of infantile colic. Wilcoxon test, t-test and Mann-Whitney test were used to compare QoL scores before and after intervention as well as between the intervention and control group. Results: At inclusion, duration of crying did not differ between both groups. Crying duration decreased with 2.7 hours (from 3.2 to 0.5 hours) in the intervention group while duration of crying decreased only with 1.2 hours in the control group (p<0.001). Stool composition became looser in the intervention group, but defecation frequency did not change. The median scores of the QoL questionnaire improved significantly in the intervention group for all parameters. In the control group, parameters improved significantly also but not for the parent-child and social interaction. The score changes were significantly greater in the intervention than in the control group. Conclusion: The intervention formula (partial whey hydrolysate, synbiotic, reduced lactose) significantly reduced the duration of crying and improved QoL of the parents and infants.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제20권2호
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• pp.107-113
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• 2017

Purpose: This study clarified the bacterial pathogens currently causing acute infectious enterocolitis (AIE) in children and evaluated the clinical characteristics and ultrasonographic findings according to the different pathogens. Methods: Medical records regarding age, sex, clinical symptoms, laboratory data, identified enteropathogens, ultrasonographic findings, treatment, and outcome of 34 patients who were diagnosed with AIE via stool examination using multiplex polymerase chain reaction (PCR) or culture, were retrospectively reviewed. Results: Twenty-four patients (70.6%) were male. The mean age of the patients was $8.5{\pm}6.2$ (range, 1.1-17.1) years. Six bacterial pathogens were isolated: Salmonella species (spp.) (32.4%), Campylobacter spp. (20.6%), verotoxin-producing Escherichia coli (14.7%), Staphylococcus aureus (11.8%), Clostridium difficile (8.8%), and Shigella spp. (2.9%). Abdominal pain occurred in all patients regardless of pathogen. The patients infected with Salmonella were older than those infected with verotoxin-producing E. coli (p<0.05). C-reactive protein levels were higher in patients with Salmonella and Campylobacter infections than in those with verotoxin-producing E. coli infection (p< 0.05), the other clinical and laboratory data were indistinguishable between pathogens. Ultrasonography demonstrated diverse involvement of bowel segments according to pathogen. Wall thickening of both the ileum and the entire colon was the most common lesion site regardless of pathogen. Conclusion: Various bacterial agents cause AIE and the symptoms are diverse symptoms, however, all most children recovered spontaneously. Use of multiplex PCR on stool samples warrants improvement of its sensitivity for diagnosis of enteropathogenic bacteria. Ultrasonographic examination is useful for diagnosis of AIE; it can also detect the disease extent and severity.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제21권4호
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• pp.315-320
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• 2018

Purpose: This study set out to evaluate the compliance to, and efficacy of oral supplementation, using a 1.5 kcal/mL or 1 kcal/mL sip feed, in children with mild to moderate malnutrition. Methods: This was a parallel, randomized, controlled open-label trial in children aged 3 to 6 years with a weight for height Z (WHZ) score <-1 and ${\geq}-3$, who were randomized to receive a total of 600 kcal/day from either a 1.5 kcal/mL or a 1.0 kcal/mL pediatric sip feed for 28 days. Assessments included daily study product intake, body weight, tolerance and dietary intake from solid food. Results: Of 110 children recruited, 98 ($mean{\pm}standard$ deviation of age $49{\pm}7months$) completed the study. Both sip feeds were well tolerated, with high compliance ($80{\pm}24%$ and $81{\pm}22%$ of prescribed volume in 1.5 kcal/mL and 1.0 kcal/mL groups respectively, p=0.79). Both study groups gained similar weight during the 28 days intervention period ($0.42{\pm}0.40kg$ in 1.5 kcal/mL group vs. $0.49{\pm}0.49kg$ in 1.0 kcal/mL group, p=0.43). There were no significant differences between the groups in weight gain and in the change in WHZ score over the intervention period. Dietary analysis at the end of the study did not show replacement of solid food by the oral nutritional supplements. Conclusion: In children with mild to moderate malnutrition, both 1.5 kcal/mL and 1 kcal/mL pediatric sip feeds had high compliance and were well tolerated, and were equally effective in promoting weight gain in the 28 days study period.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제21권4호
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• pp.321-328
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• 2018

Purpose: Feeding children is a problem in pediatric intensive care units (PICU) and it is difficult to know the correct amount. The purpose of this study is to evaluate if prealbumin or retinol binding proteins (RBP) are effective relative to daily enteral nutrition, without being affected by severity of diseases or infections and can be used to follow up nutritional amount. Methods: This is a prospective observational study that includes 81 patients admitted to PICU in Akdeniz University with estimated duration >72 hours, age between 1 month and 8 years. Daily calorie and protein intake were calculated and prealbumin, RBP and C-reactive protein (CRP) levels were measured on the first, third, fifth and seventh mornings. Results: We find moderate correlation between daily calorie intake and prealbumin levels (r=0.432, p<0.001), RBP levels and daily protein intake (r=0.330, p<0.001). When we investigated the relationship between changes of prealbumin, RBP, CRP, calorie and protein intake during intensive care stay, we found that increase of Prealbumin and RBP levels are explained by decrease of CRP levels (r=-0.546 and -0.645, p<0.001) and not with increase of nourishment. Conclusion: Even adjusted for PRISM3, age and CRP, prealbumin and RBP are correlated with last 24 hours' diet. However, it is not convenient to use as a follow up biomarker because increase of their levels is related with decrease of CRP levels.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제21권4호
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• pp.278-288
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• 2018

Purpose: Dehydration is a paediatric medical emergency but there is no single standard parameter to evaluate it at the emergency department. Our aim was to evaluate the reliability and validity of capillary refilling time as a triage parameter to assess dehydration in children. Methods: This was a prospective pilot cohort study of children who presented to two paediatric emergency departments in Italy, with symptoms of dehydration. Reliability was assessed by comparing the triage nurse's measurements with those obtained by the physician. Validity was demonstrated by using 6 parameters suggestive of dehydration. Comparison between refilling time (RT) and a validated Clinical Dehydration Score (CDS) was also considered. The scale's discriminative ability was evaluated for the outcome of starting intravenous rehydration therapy by using a receiver operating characteristic (ROC) curve. Results: Participants were 242 children. All nurses found easy to elicit the RT after being trained. Interobserver reliability was fair, with a Cohen's kappa of 0.56 (95% confidence interval [CI], 0.41 to 0.70). There was a significant correlation between RT and weight loss percentage (r-squared=-0.27; 95% CI, -0.47 to -0.04). The scale's discriminative ability yielded an area under the ROC curve (AUC) of 0.65 (95% CI, 0.57 to 0.73). We found a similarity between RT AUC and CDS-scale AUC matching the two ROC curves. Conclusion: The study showed that RT represents a fast and handy tool to recognize dehydrated children who need a prompt rehydration and may be introduced in the triage line-up.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제21권4호
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• pp.257-263
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• 2018

Purpose: Studies on the physiology of the transposed stomach as an esophageal substitute in the form of a gastric pull-up or a gastric tube in children are limited. We conducted a study of motility and the pH of gastric esophageal substitutes using manometry and 24-hour pH measurements in 10 such patients. Methods: Manometry and 24 hour pH studies were performed on 10 children aged 24 to 55 months who had undergone gastric esophageal replacement. Results: Six gastric tubes (4, isoperistaltic; 2, reverse gastric tubes) and 4 gastric pull-ups were studied. Two gastric tubes and 4 gastric pull-ups were transhiatal. Four gastric tubes were retrosternal. The mean of the lowest pH at the midpoint of the substitute was 4.0 (range, 2.8-5.0) and in the stomach remaining below the diaphragm was 3.3 (range, 1.9-4.2). In both types of substitute, the difference between the peak and the nadir pH recorded in the intra-thoracic and the sub-diaphragmatic portion of the stomach was statistically significant (p<0.05), with the pH in the portion below the diaphragm being lower. The lowest pH values in the substitute and in the remnant stomach were noted mainly in the evening hours whereas the highest pH was noted mainly in the morning hours. All the cases showed a simultaneous rise in the intra-cavitatory pressure along the substitute while swallowing. Conclusion: The study suggested a normal gastric circadian rhythm in the gastric esophageal substitute. Mass contractions occurred in response to swallowing. The substitute may be able to effectively clear contents.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제23권1호
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• pp.98-104
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• 2020

Purpose: To investigate the impact of omega-3-enriched lipid emulsion (LE) on liver enzyme (aspartate transaminase [AST] and alanine aminotransferase [ALT]) and triglyceride (TG) levels of children undergoing gastrointestinal surgery. Methods: This experimental randomized controlled group pretest-posttest design study included 14 children who underwent gastrointestinal surgery due to duodenal atresia, jejunal atresia, esophageal atresia, and need for parenteral nutrition for a minimum of 3 days at RSUD Dr. Soetomo Surabaya between August 2018 and January 2019. These children were divided into two groups, those who received standard intravenous LE (medium-chain triglyceride [MCT]/long-chain triglyceride [LCT]) and those who received intravenous omega-3-enriched LE. Differences in AST, ALT, and TG levels were measured before surgery and 3 days after the administration of parenteral nutrition. Results: Liver enzyme and TG levels in each group did not differ significantly before versus 3 days after surgery. However, TG levels were significantly lower in the omega-3-enriched intravenous LE group (p=0.041) at 3 days after surgery, and statistically significant difference in changes in TG levels was noted at 3 days after surgery between MCT/LCT intravenous LE group and the omega-3-enriched intravenous LE group (p=0.008). Conclusion: The intravenous omega-3-enriched LE had a better TG-lowering effect than the MCT/LCT intravenous LE in children undergoing gastrointestinal surgery.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제23권1호
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• pp.1-14
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• 2020

The latest definition of a prebiotic is "a substrate that is selectively utilized by host microorganisms conferring a health benefit"; it now includes non-food elements and is applicable to extra-intestinal tissues. Prebiotics are recognized as a promising tool in the promotion of general health and in the prevention and treatment of numerous juvenile diseases. Prebiotics are considered an immunoactive agent, with the potential for long-lasting effects extending past active administration of the prebiotic. Because of its extremely low risk of serious adverse effects, ease of administration, and strong potential for influencing the composition and function of the microbiota in the gut and beyond, the beneficial clinical applications of prebiotics are expanding. Prebiotics are the third largest component of human breast milk. Preparations including galactooligosaccharides (GOS), fructooligosaccharides (FOS), 2'-fucosyllactose, lacto-N-neo-tetraose are examples of commonly used and studied products for supplementation in baby formula. In particular, the GOS/FOS combination is the most studied. Maintaining a healthy microbiome is essential to promote homeostasis of the gut and other organs. With more than 1,000 different microbial species in the gut, it is likely more feasible to modify the gut microbiota through the use of certain prebiotic mixtures rather than supplementing with a particular probiotic strain. In this review, we discuss the latest clinical evidence regarding prebiotics and its role in gut immunity, allergy, infections, inflammation, and functional gastrointestinal disorders.