Publications by authors named "Hye-Ran Yang"

102 Publications

Association between ectopic pancreatic and hepatic fat and metabolic risk factors in children with non-alcoholic fatty liver disease.

Pediatr Obes 2021 May 4:e12793. Epub 2021 May 4.

Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, South Korea.

Background: Few studies have reported an association between ectopic pancreatic and hepatic fat and metabolic factors in children with non-alcoholic fatty liver disease (NAFLD).

Objectives: We investigated this association and also the factors associated with pancreatic and hepatic fat deposition in children with NAFLD.

Methods: This cross-sectional study investigated 65 children with NAFLD (49 boys, 13.0 ± 3.2 years, mean body mass index z-score 2.5 ± 1.2), who underwent liver biopsy and magnetic resonance imaging-based proton density fat fraction, as well as anthropometry, laboratory tests, body composition analysis, and hepatic fat fraction (HFF) and pancreatic fat fraction (PFF) measurements.

Results: HFF and PFF were 4.2%-49.9% (median 24.3) and 0.4%-26.9% (median 3.8), respectively. HFF was not significantly correlated with PFF. HFF was correlated with total body fat% (r = 0.329, p = 0.010) and γ-glutamyl transpeptidase (GGT) (r = 0.260, p = 0.040), while PFF was correlated with the diastolic blood pressure (r = 0.253, p = 0.045), GGT (r = 0.335, p = 0.007) and fasting plasma glucose (r = 0.417, p = 0.001). Multiple linear regression analysis showed that HFF was significantly associated with sex, age, body fat% and GGT, whereas PFF was associated with hypertension and fasting plasma glucose levels but not insulin resistance.

Conclusions: HFF was associated with sex, age and body fat in children with NAFLD, while PFF was associated with hypertension and increased fasting plasma glucose, which suggests that the pathophysiology of ectopic fat accumulation varies across organs in children with NAFLD.
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http://dx.doi.org/10.1111/ijpo.12793DOI Listing
May 2021

Nationwide "Pediatric Nutrition Day" survey on the nutritional status of hospitalized children in South Korea.

Nutr Res Pract 2021 Apr 22;15(2):213-224. Epub 2020 Sep 22.

Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam 13620, Korea.

Background/objectives: To evaluate the nutritional status and prevalence of malnutrition in hospitalized children at admission and during hospitalization in South Korea.

Subjects/methods: This first cross-sectional nationwide "Pediatric Nutrition Day (pNday)" survey was conducted among 872 hospitalized children (504 boys, 368 girls; 686 medical, 186 surgical) from 23 hospitals in South Korea. Malnutrition risk was screened using the Pediatric Yorkhill Malnutrition Score (PYMS) and the Screening Tool Risk on Nutritional status and Growth. Nutritional status was assessed by z-scores of weight-for-age for underweight, weight-for-height for wasting, and height-for-age for stunting as well as laboratory tests.

Results: At admission, of the 872 hospitalized children, 17.2% were underweight, and the prevalence of wasting and stunting was 20.2% and 17.3%, respectively. During hospitalization till pNday, 10.8% and 19.6% experienced weight loss and decreased oral intake, respectively. During the aforementioned period, fasting was more prevalent in surgical patients (7.5%) than in medical patients (1.6%) ( < 0.001). According to the PYMS, 34.3% and 30% of the children at admission and on pNday, respectively, had a high-risk of malnutrition, requiring consultation with the nutritional support team (NST). However, only 4% were actually referred to the NST during hospitalization.

Conclusions: Malnutrition was prevalent at admission and during hospitalization in pediatric patients, with many children experiencing weight loss and poor oral intake. To improve the nutritional status of hospitalized children, it is important to screen and identify all children at risk of malnutrition and refer malnourished patients to the multidisciplinary NST for proper nutritional interventions.
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http://dx.doi.org/10.4162/nrp.2021.15.2.213DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8007407PMC
April 2021

Factors associated with hiatal hernia in neurologically impaired children.

Neurogastroenterol Motil 2021 Apr 10:e14158. Epub 2021 Apr 10.

Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea.

Background: Hiatal hernia is clinically important because it impairs the protective mechanism that prevents gastroesophageal reflux-induced injury. Diagnosing hiatal hernia is more important in neurologically impaired children because hiatal hernia-induced gastroesophageal reflux often causes severe complications such as aspiration pneumonia or malnutrition. We aimed to evaluate the patient characteristics and early predictors of hiatal hernia in neurologically impaired children.

Methods: We retrospectively investigated 97 neurologically impaired children who underwent esophagogastroduodenoscopy and upper gastrointestinal series between March 2004 and June 2019. Demographic and clinical characteristics, as well as endoscopic and radiological findings, were statistically analyzed.

Results: Of the 97 children recruited, 22 (22.7%) had hiatal hernia. When comparing the non-hiatal hernia group with the hiatal hernia group, neurological disease longer than 6 months (odds ratio 10.9, 95% confidence interval 1.2-96.5), wasting (odds ratio 4.6, 95% confidence interval 1.3-16.3), enteral tube feeding (odds ratio 9.2, 95% confidence interval 1.6-53.0), and history of aspiration pneumonia (odds ratio 6.5, 95% confidence interval 1.2-34.5) were identified as early predictors of hiatal hernia.

Conclusions: Timely identification of predictors of developing hiatal hernia in neurologically impaired children is important for early diagnostic confirmation to initiate optimal medical or surgical treatment of hiatal hernia to avoid serious complications such as aspiration pneumonia and malnutrition.
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http://dx.doi.org/10.1111/nmo.14158DOI Listing
April 2021

Usefulness of Endoscopic Hill Grade in Evaluating Children Suspected of Having Gastroesophageal Reflux Disease.

J Neurogastroenterol Motil 2021 Apr;27(2):191-197

Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Gyeonggi-do, Korea.

Background/aims: This study aim to evaluate the relationship between the Hill grade confirmed by esophagogastroduodenoscopy (EGD) and the degree of gastroesophageal reflux (GER) by 24-hour multichannel intraluminal impedance-pH monitoring (MII-pH) in children suspected of having gastroesophageal reflux disease (GERD).

Methods: A retrospective review of 105 children and adolescents who underwent EGD and MII-pH for the evaluation of GERD from March 2013 to July 2019 was performed. Clinical features and results of EGD and 24-hour MII-pH were collected and statistically analyzed.

Results: Hill grades 1, 2, 3, and 4 were identified using EGD in 56 (53.3%), 22 (22.0%), 16 (15.2%), and 11 (10.5%) patients, respectively. As the Hill grade increased, the proportion of neurological diseases ( < 0.001) and endoscopic erosive esophagitis ( < 0.001) increased significantly. The acid exposure index, bolus exposure index, number of reflux episodes, and number of GER reaching proximal extent on MII-pH increased significantly as the endoscopic Hill grade increased (all < 0.001). Linear regression analysis revealed an increase in the Hill grade by 1 increased the acid exposure index by 2.0%, bolus exposure index by 0.7%, number of reflux episodes by 18.9 episodes, and the number of GER reaching the proximal esophagus increased by 10.5 episodes on average (all < 0.001).

Conclusions: Hill grade on EGD was associated with GER in children. Estimating the degree of GER by applying Hill grade in the retroflexion view may be useful in practice when evaluating children suspected with GERD.
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http://dx.doi.org/10.5056/jnm20033DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8026377PMC
April 2021

Particulate matter causes skin barrier dysfunction.

JCI Insight 2021 Mar 8;6(5). Epub 2021 Mar 8.

Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.

The molecular mechanisms that underlie the detrimental effects of particulate matter (PM) on skin barrier function are poorly understood. In this study, the effects of PM2.5 on filaggrin (FLG) and skin barrier function were investigated in vitro and in vivo. The levels of FLG degradation products, including pyrrolidone carboxylic acid, urocanic acid (UCA), and cis/trans-UCA, were significantly decreased in skin tape stripping samples of study subjects when they moved from Denver, an area with low PM2.5, to Seoul, an area with high PM2.5 count. Experimentally, PM2.5 collected in Seoul inhibited FLG, loricrin, keratin-1, desmocollin-1, and corneodesmosin but did not modulate involucrin or claudin-1 in keratinocyte cultures. Moreover, FLG protein expression was inhibited in human skin equivalents and murine skin treated with PM2.5. We demonstrate that this process was mediated by PM2.5-induced TNF-α and was aryl hydrocarbon receptor dependent. PM2.5 exposure compromised skin barrier function, resulting in increased transepidermal water loss, and enhanced the penetration of FITC-dextran in organotypic and mouse skin. PM2.5-induced TNF-α caused FLG deficiency in the skin and subsequently induced skin barrier dysfunction. Compromised skin barrier due to PM2.5 exposure may contribute to the development and the exacerbation of allergic diseases such as atopic dermatitis.
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http://dx.doi.org/10.1172/jci.insight.145185DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8021104PMC
March 2021

Eosinophilic gastroenteritis as a cause of non-Helicobacter pylori, non-gastrotoxic drug ulcers in children.

BMC Gastroenterol 2020 Aug 20;20(1):280. Epub 2020 Aug 20.

Department of Pediatrics, Seoul National University Bundang Hospital, Seoul National University, 82, Gumi-ro 173 Beon-gil, Bundang-gu, Seongnam-si, Gyeonggi-do, 13620, South Korea.

Background: While Helicobacter pylori (H. pylori) ulcers has declined recently, H. pylori-negative and/or gastrotoxic drug-negative peptic ulcers (HNGN-PU) has increased. This study aimed to analyze the etiology of peptic ulcers in children and the differences in clinical, laboratory, endoscopic, and histopathologic findings of peptic ulcers according to etiology, including eosinophilic gastroenteritis (EoGE).

Methods: In total, 255 children (157 boys and 98 girls) with peptic ulcers were recruited. The subjects were categorized into 5 groups according to the etiology of the ulcer: 1) H. pylori infection (n = 51); 2) gastrotoxic drugs (n = 18); 3) idiopathic (n = 144); 4) systemic disease (n = 23); 5) EoGE (n = 19). Clinical data were reviewed and analyzed retrospectively.

Results: Age at diagnosis, ulcer recurrence, atopic dermatitis history, white blood cell count, blood eosinophil count, platelet count, serum albumin level, iron level, erythrocyte sedimentation rate, and C-reactive protein level differed significantly among the 5 groups (all p < 0.05). Regarding endoscopic findings, multiple ulcers and gastric mucosal nodularity differed among the 5 groups (all p < 0.05). When comparing the EoGE ulcer group with the others, EoGE group revealed older ages (p = 0.022), higher rates of ulcer recurrence (p = 0.018), atopic dermatitis history (p = 0.001), and both blood and tissue eosinophilia (both p = 0.001).

Conclusions: EoGE ulcers constituted 10.2% of HNGN-PU in pediatric patients. In children with HNGN-PU, peripheral eosinophilia, ulcer recurrence, and atopic dermatitis history might imply EoGE, necessitating thorough investigation of tissue eosinophils during endoscopic biopsy.

Trial Registration: A total of 255 children was retrospectively registered between between July 2003 and April 2017.
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http://dx.doi.org/10.1186/s12876-020-01416-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7439514PMC
August 2020

Nationwide Population-Based Epidemiologic Study on Childhood Intussusception in South Korea: Emphasis on Treatment and Outcomes.

Pediatr Gastroenterol Hepatol Nutr 2020 Jul 3;23(4):329-345. Epub 2020 Jul 3.

Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea.

Purpose: This was a nationwide population-based study conducted to investigate the epidemiology, treatment, disease outcomes, and associated factors of childhood intussusception in South Korea.

Methods: Data from the Korean National Health Insurance Service database on all patients <18 years old diagnosed with intussusception from 2007 to 2017 were analyzed.

Results: A total of 34,688 cases were identified among 30,444 patients. The overall incidence was 28.3/100,000 person-years with a male predominance. Most cases (83.1%) occurred in children <3 years old, with an annual incidence of 195.2, 200.1, and 118.6 cases per 100,000 children in their first, second, and third year of life, respectively. The median age at the first occurrence was 18.7 months, and it was higher in boys than in girls. The post-discharge recurrence rate was 10.6% (3,226/30,444) and the in-hospital recurrence rate was 6.1% (1,842/30,444). The total recurrence rate (post-discharge recurrence and/or in-hospital recurrence) was 15.0% (4,580/30,444). Enema reduction was successful in 90.0% of cases. Enema reduction was more successful in girls than in boys. A total of 3,296 (10.8%) patients underwent 3,481 surgeries, including 735 (21.1%) laparoscopic surgeries. Post-discharge recurrence and surgery were significantly affected by age, sex, and hospital type. Mortality was noted in nine cases (0.03%).

Conclusion: Our study provides accurate epidemiologic data on the treatment and outcomes of intussusception through complete enumeration during an 11-year-period.
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http://dx.doi.org/10.5223/pghn.2020.23.4.329DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7354872PMC
July 2020

Comparison of loss of response between anti-tumor necrosis factor alone and combined use with immunomodulators in patients with inflammatory bowel disease.

Korean J Intern Med 2021 03 25;36(Suppl 1):S9-S17. Epub 2020 Jun 25.

Department of Internal Medicine and Liver Research Institute, Seoul National University College of Medicine, Seoul, Korea.

Background/aims: Combination therapy with immunomodulators (IMMs) was proposed as a strategy to prevent the development of loss of response (LOR) to anti-tumor necrosis factor (TNF) for patients with inflammatory bowel disease (IBD). However, the effect is unclear in patients already exposed to IMMs. The aim of this study was to evaluate whether combination therapy with IMMs is superior to monotherapy for prevention of LOR to anti-TNF.

Methods: This was a retrospective study of patients in Seoul National University Bundang Hospital with IBD between January 2009 and October 2018. LOR was defined as clinical deterioration after maintenance of anti-TNF for at least 6 months. We investigated the difference in incidence of LOR to anti-TNF between the monotherapy and combination groups. We additionally assessed factors affecting LOR development to anti-TNF.

Results: A total of 116 patients with IBD were included in this study (monotherapy 61 patients; combination 55 patients). Overall, LOR to anti-TNF occurred in 31 patients during the follow-up period. The combination of an anti-TNF agent and IMM showed no significant difference in the incidence of LOR compared to anti-TNF agent monotherapy (hazard ratio [HR], 1.64; 95% confidence interval [CI], 0.786 to 3.148; p = 0.182). Female sex was significantly associated with the development of LOR to anti-TNF (HR, 3.032; 95% CI, 1.467 to 6.268; p = 0.003).

Conclusion: Anti-TNF and IMM combination therapy did not prove efficacious in preventing the development of LOR in IBD patients. Female sex was associated with the development of LOR to anti-TNF; further studies are required to confirm these results.
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http://dx.doi.org/10.3904/kjim.2019.279DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8009168PMC
March 2021

Effect of nutritional supplement formula on catch-up growth in young children with nonorganic faltering growth: a prospective multicenter study.

Nutr Res Pract 2020 Jun 31;14(3):230-241. Epub 2020 Mar 31.

Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam 13620, Korea.

Background/objectives: Inadequate nutrition in infants and young children affects physical growth and neurocognitive development. Therefore, early nutritional intervention is important to promote catch-up growth in young children with faltering growth. The aim of this study was to evaluate the effect of nutritional supplementation with a pediatric concentrated and balanced nutritional supplement formula on promoting growth and improving nutritional status in children with nonorganic faltering growth.

Subjects/methods: Children aged 12-36 months whose body weight-for-age was < 5 percentile on the Korean Growth Charts were enrolled. Children born premature or having organic diseases were excluded. Children were instructed to consume 400 mL of formula per day in addition to their regular diet for 6 months. Pediatricians and dietitians educated the parents and examined the subjects every 2 months. Anthropometric parameters were measured at baseline and at 2, 4, and 6 months, and laboratory tests were done at baseline and 6 months. The good consumption group included children who consumed ≥ 60% of the recommended dose of formula.

Results: Total 82 children completed the 6-month intervention. At baseline, there were no significant differences in all variables between the good consumption and poor consumption groups. Weight and weight z-scores were significantly improved in the good consumption group compared to the poor consumption group at the end of the intervention ( = 0.009, respectively). The good consumption group showed a significant trend for gaining weight ( < 0.05) and weight z-score ( < 0.05) compared to the poor consumption group during 6 months of formula intake. The concentration of blood urea nitrogen was significantly increased in the good consumption group ( = 0.001).

Conclusions: Nutritional supplementation with a concentrated and balanced pediatric nutritional formula along with dietary education might be an effective approach to promote catch-up growth in children with nonorganic faltering growth.
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http://dx.doi.org/10.4162/nrp.2020.14.3.230DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7263897PMC
June 2020

A Nomogram for Predicting Non-Alcoholic Fatty Liver Disease in Obese Children.

Pediatr Gastroenterol Hepatol Nutr 2020 May 8;23(3):276-285. Epub 2020 May 8.

Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea.

Purpose: Non-alcoholic fatty liver disease (NAFLD) ranges in severity from simple steatosis to steatohepatitis. Early detection of NAFLD is important for preventing the disease from progressing to become an irreversible end-stage liver disease. We developed a nomogram that allows for non-invasive screening for NAFLD in obese children.

Methods: Anthropometric and laboratory data of 180 patients from our pediatric obesity clinic were collected. Diagnoses of NAFLD were based on abdominal ultrasonographic findings. The nomogram was constructed using predictors from a multivariate analysis of NAFLD risk factors.

Results: The subjects were divided into non-NAFLD (n=67) and NAFLD groups (n=113). Factors, including sex, body mass index, abdominal circumference, blood pressure, insulin resistance, and levels of aspartate aminotransferase, alanine aminotransferase (ALT), γ-glutamyl transpeptidase (γGT), uric acid, triglycerides, and insulin, were significantly different between the two groups (all <0.05) as determined using homeostatis model assessment of insulin resistance (HOMA-IR). In our multivariate logistic regression analysis, elevated serum ALT, γGT, and triglyceride levels were significantly related to NAFLD development. The nomogram was established using γGT, uric acid, triglycerides, HOMA-IR, and ALT as predictors of NAFLD probability.

Conclusion: The newly developed nomogram may help predict NAFLD risk in obese children. The nomogram may also allow for early NAFLD diagnosis without the need for invasive liver biopsy or expensive liver imaging, and may also allow clinicians to intervene early to prevent the progression of NAFLD to become a more advanced liver disease.
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http://dx.doi.org/10.5223/pghn.2020.23.3.276DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7231740PMC
May 2020

Nationwide Multicenter Study of Eosinophilic Esophagitis in Korean Children.

Pediatr Gastroenterol Hepatol Nutr 2020 May 8;23(3):231-242. Epub 2020 May 8.

Department of Pediatrics, Seoul National University Bundang Hospital, Sungnam, Korea.

Purpose: In East Asian countries, there are only a few epidemiologic studies of eosinophilic esophagitis (EoE) and no studies in children. We investigated the incidence and compared the clinical characteristics of EoE and eosinophilic gastroenteritis involving the esophagus (EGEIE) in Korean children.

Methods: A total of 910 children, who had symptoms of esophageal dysfunction, from 10 hospitals in Korea were included. EoE was diagnosed according to diagnostic guidelines and EGEIE was diagnosed when there were >15 eosinophils in the esophagus per high power field (HPF) and >20 eosinophils per HPF deposited in the stomach and duodenum with abnormal endoscopic findings.

Results: Of the 910 subjects, 14 (1.5%) were diagnosed with EoE and 12 (1.3%) were diagnosed with EGEIE. Vomiting was the most common symptom in 57.1% and 66.7% of patients with EoE and EGEIE, respectively. Only diarrhea was significantly different between EoE and EGEIE (=0.033). In total, 61.5% of patients had allergic diseases. Exudates were the most common endoscopic findings in EoE and there were no esophageal strictures in both groups. The median age of patients with normal endoscopic findings was significantly younger at 3.2 years, compared to the median age of 11.1 years in those with abnormal endoscopic findings (=0.004).

Conclusion: The incidence of EoE in Korean children was lower than that of Western countries, while the incidence of EGEIE was similar to EoE. There were no clinical differences except for diarrhea and no differences in endoscopic findings between EoE and EGEIE.
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http://dx.doi.org/10.5223/pghn.2020.23.3.231DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7231742PMC
May 2020

Fecal Calprotectin as a Useful Non-Invasive Screening Marker for Eosinophilic Gastrointestinal Disorder in Korean Children.

J Korean Med Sci 2020 May 4;35(17):e120. Epub 2020 May 4.

Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea.

Background: Eosinophilic gastrointestinal disorder (EoGID) is an emerging disease condition in Korean children, but its diagnosis requires invasive endoscopic biopsies. Fecal calprotectin (FCal) is a noninvasive biomarker for intestinal inflammation to differentiate organic gastrointestinal diseases from functional abdominal pain disorder. This study aimed to evaluate the diagnostic accuracy of FCal and to determine the optimal cutoff to differentiate EoGID from functional abdominal pain disorder.

Methods: A total of 253 children (122 boys, 131 girls; mean age 12.2 ± 3.6, range 2.9-17.8 years) who underwent endoscopy with biopsies for chronic gastrointestinal symptoms were recruited, except for 38 children diagnosed with inflammatory bowel disease, and divided into EoGID (n = 67) and functional abdominal pain disorder (n = 186). FCal, white blood cell (WBC) counts, eosinophil counts, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) levels were measured in all subjects at initial diagnosis.

Results: FCal levels weakly correlated with WBC ( = 0.127, = 0.044) and CRP ( = 0.126, = 0.040) but not with ESR and eosinophil count. FCal levels were significantly higher in the EoGID group than in the functional abdominal pain disorder group (mean 179.5 ± 242.9 mg/kg vs. 44.3 ± 68.1 mg/kg; < 0.001), while WBC, ESR, CRP, and eosinophil count did not differ between the two groups (all > 0.05). An optimal cutoff of FCal 73.2 mg/kg distinguished EoGID from functional abdominal pain disorder with a sensitivity of 50.7% and a specificity of 84.6%.

Conclusion: FCal is a useful and reliable noninvasive marker for differentiating EoGID from functional abdominal pain disorder in Korean children with chronic gastrointestinal symptoms when optimal cutoffs are applied.
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http://dx.doi.org/10.3346/jkms.2020.35.e120DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7200180PMC
May 2020

Updates on bone health in children with gastrointestinal diseases.

Authors:
Hye Ran Yang

Ann Pediatr Endocrinol Metab 2020 Mar 31;25(1):10-14. Epub 2020 Mar 31.

Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea.

Chronic gastrointestinal diseases such as inflammatory bowel disease, malabsorption syndromes (e.g., intestinal lymphangiectasia, celiac disease, congenital chloride diarrhea, cystic fibrosis), and postsubtotal gastrectomy state or short-bowel syndrome after extensive bowel resection are related to poor bone health in pediatric patients due to increased risks of low bone mineral density, osteoporosis, and fractures. The pathophysiology of abnormal bone health in pediatric gastrointestinal diseases may present from inflammation to malabsorption. In children with chronic gastrointestinal diseases at high risk of poor bone health, routine evaluation using dual-energy X-ray absorptiometry and appropriate prevention or treatment strategies are needed.
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http://dx.doi.org/10.6065/apem.2020.25.1.10DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7136502PMC
March 2020

Development of Hepatocellular Carcinoma in Patients with Glycogen Storage Disease: a Single Center Retrospective Study.

J Korean Med Sci 2020 Jan 6;35(1):e5. Epub 2020 Jan 6.

Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea.

Background: Glycogen storage disease (GSD) is an inherited disorder leading to abnormal glucose metabolism and glycogen accumulation, and is associated with various complications including hepatic adenoma and hepatocellular carcinoma. The aim of this study was to analyze the risk factors for hepatic adenoma and its malignant change, and the hepatocellular carcinoma-free survival rate in patients with GSD who developed adenoma.

Methods: A total of 72 patients with GSD who were enrolled from March 1982 to September 2013 at Seoul National University Children's Hospital were retrospectively analyzed, and the median follow-up period was 19.2 years.

Results: Thirty-two patients (44.4%) developed hepatic adenoma at an age range of 7.9-26.3 years (median, 14.3 years). Among the 32 patients with hepatic adenoma, 4 patients (12.5%) developed hepatocellular carcinoma on an average interval of 6.7 years between the diagnosis of adenoma and the development of hepatocellular carcinoma. GSD type I and portacaval shunt operation were found to be the risk factors for hepatic adenoma development. The hepatocellular carcinoma-free survival rate at 10 years from adenoma development was 82%.

Conclusion: The present study found that portacaval shunt operation increases the risk of development of hepatic adenoma in GSD patients, especially in GSD type I. The hepatic adenoma in GSD patients has a potential of malignant transformation, which should be keep in mind in follow-up process of the disease.
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http://dx.doi.org/10.3346/jkms.2020.35.e5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6942134PMC
January 2020

Association of Variants With Crohn's Disease in Korean Children.

Front Pediatr 2019 19;7:472. Epub 2019 Nov 19.

Department of Pediatrics, Seoul National University College of Medicine, Seoul, South Korea.

The interleukin 23 receptor gene () is strongly associated with Crohn's disease (CD). It is unknown whether genetic variations in determine susceptibility for pediatric CD in Asian populations. Here, we investigated the association between variants and CD in Korean children. Four single nucleotide polymorphisms (SNPs) of [rs76418789 (G149R), rs1004819, rs7517847, and rs1495965] were genotyped in 141 children with CD and 150 controls using DNA direct sequencing. The risk allele and genotype frequencies were compared between patients and controls. The association between clinical phenotypes and genotypes of patients was also analyzed. Two SNPs, rs76418789 (G149R), and rs1495965, were associated with CD in Korean pediatric patients as defense and risk loci, respectively. The odds ratio (OR) for rs76418789 (G149R) and rs1495965 was 0.409 (95% confidence interval [CI], 0.177-0.944; = 0.031) and 1.484 (95% CI, 1.070-2.059; = 0.018), respectively. Patients with the homozygous G allele of rs1495965 showed higher CD risk than those with other genotypes (GG vs. AA: OR, 2.256; 95% CI, 1.136-4.478; = 0.019; GG vs. GA+AA: OR, 2.000; 95% CI, 1.175-3.404; = 0.010). Additionally, they were more likely to have relatively invasive disease behavior of stenosis and/or penetration than simple inflammation (OR, 2.297; 95% CI, 1.065-4.950; = 0.032). This is the first study reporting variants in Asian pediatric patients with CD. was significantly associated with Korean pediatric CD, and the rs1495965 may influence the clinical features of CD in Korean children.
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http://dx.doi.org/10.3389/fped.2019.00472DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6878822PMC
November 2019

Comparison of four nutritional screening tools for Korean hospitalized children.

Nutr Res Pract 2019 Oct 20;13(5):410-414. Epub 2019 Sep 20.

Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Seoul National University College of Medicine, Gyeonggi 13620, Korea.

Background/objectives: Several nutritional screening tools were recently developed to screen the risk of malnutrition in hospitalized children, but have not been validated in Asia. We compared four nutritional screening tools for pediatric patients in evaluating nutritional risks in newly hospitalized children.

Subjects/methods: Medical records of newly admitted pediatric patients between June 2016 and May 2017 at two tertiary hospitals were reviewed. Initial information by nurses and hospital records by doctors on baseline demographic, clinical, and anthropometric data at admission were collected in all subjects. Nutritional risks were evaluated using four nutritional screening tools including the pediatric nutritional risk score (PNRS), the screening tool for the assessment of malnutrition in pediatrics (STAMP), the paediatric Yorkhill malnutrition score (PYMS), and the screening tools for risk of nutritional status and growth (STRONGkids).

Results: A total of 559 patients (310 boys and 249 girls, mean age 6.3 ± 5.5 years) were recruited. Patients in medical and surgical departments were 469 (83.9%) and 90 (16.1%), respectively. The prevalence of patients at risk of malnutrition were 31.1% for low risk, 52.2% for medium risk, and 16.6% for high risk by PNRS; 11.4%, 39.7%, and 48.8% by STAMP; 26.5%, 25.4%, and 48.1% by PYMS; and 35.6%, 58.9%, and 5.5% by STRONGkids. PNRS versus STRONGkids and STAMP versus PYMS showed moderate agreement ( = 0.566 and = 0.495, respectively). PYMS and STAMP revealed a relatively high sensitivity of 87.8% and 77.6% for wasting.

Conclusion: Different nutritional screening tools revealed considerably different results in evaluating nutritional risks in newly hospitalized children. Since pediatric patients are at risk of malnutrition at admission and during hospitalization, screening tools should be applied properly according to the situation of each hospital.
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http://dx.doi.org/10.4162/nrp.2019.13.5.410DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6760980PMC
October 2019

Wilson Disease Comorbid with Hereditary Sensory Autonomic Neuropathy Type IV and Gitelman Syndrome.

Pediatr Gastroenterol Hepatol Nutr 2019 Jul 18;22(4):392-399. Epub 2019 Jun 18.

Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea.

Wilson disease a rare autosomal recessive inherited disorder of copper metabolism, is characterized by excessive deposition of copper in the liver, brain, and other tissues. Wilson disease is often fatal if it is not recognized early and treated when it is symptomatic. Gitelman syndrome is also an autosomal recessive kidney disorder characterized by low blood levels of potassium and magnesium, decreased excretion of calcium in the urine, and elevated blood pH. Hereditary sensory autonomic neuropathy type IV (HSAN-IV), a very rare condition that presents in infancy, is characterized by anhidrosis, absence of pain sensation, and self-mutilation. It is usually accompanied by developmental delay and mental retardation. We report a case of Wilson disease manifested as fulminant hepatitis, acute pancreatitis, and acute kidney injury in a 15-year-old boy comorbid with HSAN-IV and Gitelman syndrome. Such concurrence of three genetic diseases is an extremely rare case.
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http://dx.doi.org/10.5223/pghn.2019.22.4.392DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6629596PMC
July 2019

A prospective study on changes in body composition and fat percentage during the first year of cancer treatment in children.

Nutr Res Pract 2019 Jun 13;13(3):214-221. Epub 2019 Mar 13.

Department of Pediatrics, Seoul National University Bundang Hospital, Seoul National University College of Medicine, 82 Gumi-ro 173 Beon-gil, Bundang-gu, Seongnam, Gyeonggi 13620, Korea.

Backgrounds/objectives: Cancer treatment may lead to significant body composition changes and affect growth and disease outcomes in pediatric cancer patients. This prospective study aimed to evaluate short- and long-term body compositions changes focused on body fat during the first year of cancer treatment in children.

Subjects/methods: A prospective study was conducted in 30 pediatric cancer patients (19 hematologic malignancies and 11 solid tumors) and 30 age- and sex-matched healthy controls. Anthropometric measurements and body composition analysis using whole body dual energy X-ray absorptiometry were performed at baseline and 1, 6, and 12 month(s) of cancer treatment. Kruskal-Wallis tests, Wilcoxon paired tests, and generalized estimation equation (GEE) were applied for statistical analysis.

Results: At baseline, no differences in weight, height, body mass index, abdominal circumferences, body fat, and fat-free mass were observed between 30 controls and 30 pediatric cancer patients. Total fat mass ( < 0.001) and body fat percentage ( = 0.002) increased significantly during the first month, but no changes were observed from 1 to 12 months; however, no changes in the total mass were observed during the first year of cancer treatment. Meanwhile, the total fat-free mass decreased during the first month ( = 0.008) and recovered between 6 and 12 months of follow-up ( < 0.001). According to GEE analysis, there was a significant upward trend in body fat percentage during the first year, especially the first month, of cancer treatment in children with hematologic malignancies, but not in those with solid tumors.

Conclusions: Our results indicate that cancer treatment is related to significant body composition changes and rapid body fat gain, particularly during the first month after initiating cancer treatment, in children with hematologic malignancies. Therefore, individualized dietary strategies to prevent excessive fat gain are needed in pediatric cancer patients for better outcomes.
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http://dx.doi.org/10.4162/nrp.2019.13.3.214DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6548708PMC
June 2019

Association Between Vitamin D Deficiency and Suspected Nonalcoholic Fatty Liver Disease in an Adolescent Population.

Pediatr Gastroenterol Hepatol Nutr 2019 May 16;22(3):233-241. Epub 2019 Apr 16.

Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea.

Purpose: Vitamin D deficiency is a condition widespread throughout the world. Recent studies have suggested that vitamin D deficiency was associated with obesity and metabolic syndrome. The purpose of the study was to examine the relationship between vitamin D deficiency and nonalcoholic fatty liver disease (NAFLD) in adolescents.

Methods: The data were obtained from the Korean National Health and Nutrition Examination Survey from 2008-2014. A total of 3,878 adolescents were included in the study. Vitamin D deficiency was defined as a 25-hydroxyvitamin D concentration <20 ng/mL and suspected NAFLD was defined as an alanine transaminase concentration >30 U/L.

Results: Vitamin D deficiency was noted in 78.9% of the studied population. Age, body mass index, waist circumference, and blood pressure, glucose, cholesterol, and triglyceride levels were significantly higher in adolescents with suspected NAFLD than in adolescents without suspected NAFLD, while the mean vitamin D level was significantly lower in adolescents with suspected NAFLD. The multivariate-adjusted odds of suspected NAFLD were higher with increased age, male gender, obesity, and metabolic syndrome. Individuals with vitamin D deficiency were at higher risk of suspected NAFLD (odds ratio, 1.77; 95% confidence interval, 1.07-2.95) after adjusting for age, gender, obesity, and metabolic syndrome.

Conclusion: Vitamin D deficiency was associated with suspected NAFLD, independent of obesity and metabolic syndrome, in adolescents.
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http://dx.doi.org/10.5223/pghn.2019.22.3.233DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6506433PMC
May 2019

Clinical Practice Guideline for the Diagnosis and Treatment of Pediatric Obesity: Recommendations from the Committee on Pediatric Obesity of the Korean Society of Pediatric Gastroenterology Hepatology and Nutrition.

Pediatr Gastroenterol Hepatol Nutr 2019 Jan 10;22(1):1-27. Epub 2019 Jan 10.

Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea.

The Committee on Pediatric Obesity of the Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition newly developed the first Korean Guideline on the Diagnosis and Treatment of Obesity in Children and Adolescents to deliver an evidence-based systematic approach to childhood obesity in South Korea. The following areas were systematically reviewed, especially on the basis of all available references published in South Korea and worldwide, and new guidelines were established in each area with the strength of recommendations based on the levels of evidence: 1) definition and diagnosis of overweight and obesity in children and adolescents; 2) principles of treatment of pediatric obesity; 3) behavioral interventions for children and adolescents with obesity, including diet, exercise, lifestyle, and mental health; 4) pharmacotherapy; and 5) bariatric surgery.
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http://dx.doi.org/10.5223/pghn.2019.22.1.1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6333581PMC
January 2019

What should we do to prevent and manage obesity in children and adolescents?

Authors:
Hye Ran Yang

Korean J Pediatr 2019 Jan 11;62(1):1-2. Epub 2019 Jan 11.

Department of Pediatrics, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea.

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http://dx.doi.org/10.3345/kjp.2018.07374DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6351799PMC
January 2019

Clinical practice guideline for the diagnosis and treatment of pediatric obesity: recommendations from the Committee on Pediatric Obesity of the Korean Society of Pediatric Gastroenterology Hepatology and Nutrition.

Korean J Pediatr 2019 Jan 27;62(1):3-21. Epub 2018 Dec 27.

Department of Pediatrics, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea.

The Committee on Pediatric Obesity of the Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition newly developed the first Korean Guideline on the Diagnosis and Treatment of Obesity in Children and Adolescents to deliver an evidence-based systematic approach to childhood obesity in South Korea. The following areas were systematically reviewed, especially on the basis of all available references published in South Korea and worldwide, and new guidelines were established in each area with the strength of recommendations based on the levels of evidence: (1) definition and diagnosis of overweight and obesity in children and adolescents; (2) principles of treatment of pediatric obesity; (3) behavioral interventions for children and adolescents with obesity, including diet, exercise, lifestyle, and mental health; (4) pharmacotherapy; and (5) bariatric surgery.
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http://dx.doi.org/10.3345/kjp.2018.07360DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6351800PMC
January 2019

EBV VCA IgM and cytomegalovirus IgM dual positivity is a false positive finding related to age and hepatic involvement of primary Epstein-Barr virus infection in children.

Medicine (Baltimore) 2018 Sep;97(38):e12380

Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam Seoul National University College of Medicine, Seoul, Korea.

Primary Epstein-Barr virus (EBV) infection is common in childhood, and dual positivity of serum EBV IgM and cytomegalovirus (CMV) IgM antibodies occur in some cases. This study aimed to evaluate the cause of EBV and CMV IgM dual positivity to determine whether it represents a false-positive finding or a true coinfection.A total of 494 children diagnosed with primary EBV infection, manifesting as infectious mononucleosis, were recruited. The diagnosis was based on positive EBV viral capsid antigen (VCA) IgM antibodies, and serum CMV IgM antibodies and liver enzymes were also evaluated in 149 subjects.Of 149 children with primary EBV infection, 40 (26.8%) had serum EBV VCA IgM and CMV IgM dual positivity. However, true CMV infection was confirmed only in 1 child of 40 (2.5%) who was positive for both serum CMV Ag and urine CMV polymerase chain reaction (PCR) and negative for serum CMV IgG antibody. Among the children with primary EBV infection, the rate of dual positivity was higher in infants and lower in adolescents (P = .013). Liver enzymes were more significantly elevated in children with dual positivity than in those with negative results for CMV IgM antibodies (P = .026), which correlated with the serum EBV and CMV IgM titers.Serum EBV and CMV IgM dual positivity are more prevalent in children with primary EBV infection than what was previously reported. Our results indicate that serum EBV and CMV IgM dual positivity represents a false-positive finding, as opposed to an actual CMV coinfection, possibly due to antigenic cross-reactivity.
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http://dx.doi.org/10.1097/MD.0000000000012380DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6160097PMC
September 2018

Characterization of Detailed Audiological Features of Cytomegalovirus Infection: A Composite Cohort Study from Groups with Distinct Demographics.

Biomed Res Int 2018 30;2018:7087586. Epub 2018 Aug 30.

Department of Otolaryngology, Seoul National University Hospital, Seoul National University, College of Medicine, Seoul 03080, Republic of Korea.

Congenital cytomegalovirus (cCMV) infection is a common congenital infection that causes sensorineural hearing loss (SNHL). Despite its substantial impact on public health and cost burden, epidemiology and clinical features of CMV-related SNHL have never been reported in the Korean populations. This study investigated the detailed audiologic phenotypes of cCMV infection to see if a specific SNHL pattern is associated with a particular clinical setting. A total of 38 patients with cCMV infection were studied retrospectively. Patients were classified into three groups with distinct demographics: clinically driven diagnosis (n=17), routine newborn CMV screening according to the NICU protocols (n=10), or referral to ENT for cochlear implant (CI) (n=11). The incidence of cCMV infection was 3.6%, showing 33.3% of SNHL among cCMV patients, 38% of asymmetric hearing loss, 29% of late-onset hearing loss, and diverse severity spectrum in patients with CMV-related SNHL. CI recipients with CMV-related SNHL showed a significantly improved speech perception. Surprisingly, in 36.4 % of CI implantees, initial audiological manifestation was significant asymmetry of hearing thresholds between both ears, with better ear retaining significant residual hearing up to 50dB. CMV turns out to be a significant etiology of SNHL, first to date reported in the Korean pediatric population. Analysis of audiologic phenotypes showed a very wide spectrum of SNHL and favorable CI outcomes in case of profound deafness. Especially for the patients with asymmetric hearing loss, close surveillance of hearing should be warranted and CI could be considered on the worse side first, based on the observation of rapid progression to profound deafness of better side.
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http://dx.doi.org/10.1155/2018/7087586DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6136484PMC
January 2019

Quantitative Analysis of Distribution of the Gastrointestinal Tract Eosinophils in Childhood Functional Abdominal Pain Disorders.

J Neurogastroenterol Motil 2018 Oct;24(4):614-627

Department of Pathology, Seoul National University Bundang Hospital, Seongnam, Gyeonggi-do, Korea.

Background/aims: Although functional abdominal pain disorders (FAPDs) are common in children, the accurate pathogenesis of FAPDs is not known yet. Micro-inflammation, particularly tissue eosinophilia of gastrointestinal (GI) tract, has been suggested as the pathophysiology observed in several GI disorders. We aimed to evaluate eosinophilic infiltration throughout the entire GI tract in children with FAPDs, compared to those with inflammatory bowel diseases (IBD) and to normal reference values.

Methods: We included 56 children with FAPDs, 52 children with Crohn's disease, and 23 children with ulcerative colitis. All subjects underwent esophagogastroduodenoscopic and colonoscopic examination with biopsies. Tissue eosinophil counts were assessed in 10 regions throughout the GI tract.

Results: Eosinophil counts of the gastric antrum, duodenum, terminal ileum, cecum, and ascending colon were significantly higher in children with FAPDs compared to normal reference values. Eosinophil counts of the stomach and the entire colon were observed to be significantly higher in children with IBD than in those with FAPDs. Even after selecting macroscopically uninvolved GI segments on endoscopy in children with IBD, eosinophil counts of the gastric body, cecum, descending colon, sigmoid colon, and the rectum were also significantly higher in children with IBD than those with FAPDs.

Conclusions: Significantly high eosinophil counts of the stomach and colon were observed in the order of IBD, followed by FAPDs, and normal controls, regardless of endoscopically detected macroscopic IBD lesions in children. This suggests some contribution of GI tract eosinophils in the intrinsic pathogenesis of FAPDs in children.
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http://dx.doi.org/10.5056/jnm18050DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6175563PMC
October 2018

Can We Estimate Quality of Life in Pediatric Inflammatory Bowel Disease Patients? An Asian Multicenter Study.

J Pediatr Gastroenterol Nutr 2019 01;68(1):45-49

Pediatric Gastroenterology, Hepatology and Nutrition, Yonsei University College of Medicine, Severance Pediatric IBD Research Group, Severance Children's Hospital, Seoul.

Objectives: Inflammatory bowel disease (IBD) is a chronic lifelong condition and is related to poor quality of life (QoL). The aim of this study was to evaluate the QoL of Asian pediatric patients with IBD and to determine the clinical factors that can influence QoL.

Methods: Children and adolescents aged 9 to 18 years diagnosed with IBD were enrolled from 7 hospitals. The patients completed the IMPACT-III questionnaire, and clinical data were collected. The results of the questionnaire and the correlation with clinical data were analyzed.

Results: A total of 208 patients (Crohn disease: n = 166; ulcerative colitis: n = 42) were enrolled. There was no definite QoL difference according to the Paris classification. Female sex (-5.92 ± 2.97, P = 0.0347) and active disease status (-10.79 ± 3.11, P = 0.0006) were significantly associated with poor QoL. Extreme body weight z score and older age at diagnosis were also associated with worse QoL.

Conclusions: Various clinical factors may affect the QoL in patients with IBD, but determining the overall QoL of patients using only these clinical factors is difficult. Therefore, regular direct measurements of QoL are necessary to better understand patients with IBD.
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http://dx.doi.org/10.1097/MPG.0000000000002119DOI Listing
January 2019

Effects of oral zinc supplementation on zinc status and catch-up growth during the first 2 years of life in children with non-organic failure to thrive born preterm and at term.

Pediatr Neonatol 2019 04 25;60(2):201-209. Epub 2018 Jun 25.

Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, South Korea; Seoul National University College of Medicine, Seoul, South Korea. Electronic address:

Background: We aimed to analyze the effect of oral zinc supplementation on serum insulin-like growth factor-1 (IGF-1) levels and catch-up growth in infants with non-organic failure to thrive (NOFTT) who were born preterm as compared to those born at term.

Methods: Totally, 105 NOFTT infants aged 2 years or less were enrolled and divided into two groups according to gestational age at birth. Oral zinc sulfate was administered for 6 months to 49/66 children born at term, and 21/39 children born preterm. Serum zinc, IGF-1, weight, and height were measured at baseline and at 6 months.

Results: There were no differences in baseline serum zinc levels between the two groups. In preterm NOFTT infants, zinc supplementation significantly increased serum zinc levels compared to those in the non-supplementation group (Δ zinc 10.3 ± 26.4 μg/dL vs. -8.8 ± 23.7 μg/dL, p = 0.018), but it did not significantly change serum IGF-1 levels or weight- and height for age Z-scores. In NOFTT infants born at term who received zinc supplementation, serum zinc levels, IGF-1, weight for age Z-score, and height for age Z-score increased at 6 months (p = 0.001, p = 0.014, p = 0.049, and p = 0.029, respectively), but this increase was not significantly greater than in the non-supplementation group. Only the increase in serum zinc levels was significant after 6 months (Δ zinc 16.8 ± 32.0 μg/dL vs. -10.0 ± 22.6 μg/dL, p = 0.002).

Conclusion: Zinc supplementation in NOFTT infants improves serum zinc status, regardless of gestational age at birth. Zinc supplementation in NOFTT infants born at term may improve serum IGF-1 levels and growth, but it does not in NOFTT infants born preterm. Overall nutritional support rather than supplementation of a single nutrient may be more effective for catch-up growth in NOFTT infants born preterm.
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http://dx.doi.org/10.1016/j.pedneo.2018.06.006DOI Listing
April 2019

Multiplex Ligation-dependent Probe Amplification Analysis Subsequent to Direct DNA Full Sequencing for Identifying Mutations and Phenotype Correlations in Children with Wilson Disease.

J Korean Med Sci 2018 Jun 16;33(26):e177. Epub 2018 May 16.

Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea.

Background: Mutations in cause Wilson disease (WD). However, direct DNA full sequencing cannot detect all mutations in patients with WD. Multiplex ligation-dependent probe amplification (MLPA) analysis is reportedly useful in increasing the diagnostic yield in other genetic disorders with large deletions or insertions. The aim of this study was to evaluate whether the detection rate of mutations can be increased by using MLPA.

Methods: We enrolled 114 children with WD from 104 unrelated families based on biochemical tests and direct DNA full sequencing. The patients with one or zero mutant allele were investigated using MLPA. We analyzed phenotypic correlations.

Results: Total allele frequency by full sequencing was 87.5%. Full sequencing revealed two mutant alleles in 80 of 104 unrelated children. One mutant allele was detected in 22 children, and no mutations were found in two children. Novel mutations including small deletions with frameshift mutations were identified by DNA sequencing. MLPA revealed no gross deletion or duplication in 24 children with one or zero mutant alleles. The number of detected mutations was not associated with hepatic manifestation, age of onset, Kayser-Fleischer ring, ceruloplasmin, and urinary Cu concentrations.

Conclusion: MLPA showed a limited role to increase the mutation detection rate in children who do not receive a definite genetic diagnosis of WD through DNA full sequencing. This finding suggests that large deletions or duplications might be extremely rare in WD. Further development is needed to improve the genetic diagnosis of WD.
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http://dx.doi.org/10.3346/jkms.2018.33.e177DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6010744PMC
June 2018

What We Know about Henoch-Schönlein Purpura in Children up to Date?

Authors:
Hye Ran Yang

J Korean Med Sci 2018 06 7;33(25):e199. Epub 2018 Jun 7.

Division of Pediatric Gastroenterology and Hepatology, Department of Pediatrics, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea.

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http://dx.doi.org/10.3346/jkms.2018.33.e199DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6000601PMC
June 2018

Current status of nutritional support for hospitalized children: a nationwide hospital-based survey in South Korea.

Nutr Res Pract 2018 Jun 19;12(3):215-221. Epub 2018 Apr 19.

Department of Pediatrics, Seoul National University Bundang Hospital, Seoul National University College of Medicine, 82 Gumi-ro, Bundang-gu, Seongnam, Gyeonggi 13620, Korea.

Background/objectives: The prevalence of malnutrition among hospitalized children ranges between 12% and 24%. Although the consequences of hospital malnutrition are enormous, it is often unrecognized and untreated. The aim of this study was to identify the current status of in-hospital nutrition support for children in South Korea by carrying out a nationwide hospital-based survey.

Subjects/methods: Out of 345 general and tertiary hospitals in South Korea, a total of 53 institutes with pediatric gastroenterologists and more than 10 pediatric inpatients were selected. A questionnaire was developed by the nutrition committee of the Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition. The questionnaires were sent to pediatric gastroenterologists in each hospital. Survey was performed by e-mails.

Results: Forty hospitals (75.5%) responded to the survey; 23 of them were tertiary hospitals, and 17 of them were general hospitals. Only 21 hospitals (52.5%) had all the required nutritional support personnel (including pediatrician, nutritionist, pharmacist, and nurse) assigned to pediatric patients. Routine nutritional screening was performed in 22 (55.0%) hospitals on admission, which was lower than that in adult patients (65.8%). Nutritional screening tools varied among hospitals; 33 of 40 (82.5%) hospitals used their own screening tools. The most frequently used nutritional assessment parameters were weight, height, hemoglobin, and serum albumin levels. In our nationwide hospital-based survey, the most frequently reported main barriers of nutritional support in hospitals were lack of manpower and excessive workload, followed by insufficient knowledge and experience.

Conclusions: Although this nationwide hospital-based survey targeted general and tertiary hospitals with pediatric gastroenterologists, manpower and medical resources for nutritional support were still insufficient for hospitalized children, and nutritional screening was not routinely performed in many hospitals. More attention to hospital malnutrition and additional national policies for nutritional support in hospitals are required to ensure appropriate nutritional management of hospitalized pediatric patients.
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http://dx.doi.org/10.4162/nrp.2018.12.3.215DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5974067PMC
June 2018