PURPOSE: Inadequate calorie intake is one of the most important causes of nonorganic failure to thrive (NOFTT) and is thought to lead to multiple micronutrient deficiencies. However, there have been few studies on NOFTT and micronutrients. The aim of this study was to evaluate the micronutrient status of children with NOFTT. METHODS: We conducted a retrospective cohort study in 161 children (106 with NOFTT and 55 health controls) at a single institution. Data on weight for age, height for age, body mass index, and biochemical parameters, indicating the children's nutritional and micronutrient status were reviewed via electronic medical records, and the two groups were compared. RESULTS: Except inorganic phosphate levels, no statistically significant differences were seen in the laboratory findings indicating the children's nutritional and micronutrient status; notably, the inorganic phosphate levels were within the normal range in both groups. We then compared the severe NOFTT (weight for age below the first percentile) and control groups; however, no statistically significant differences were seen for any of the measured parameters. CONCLUSION: Most children with NOFTT in this study had normal micronutrient levels and other laboratory findings. Therefore, element deficiencies should not be considered a natural consequence of NOFTT or in healthy children. Close monitoring and additional evaluations are needed.
Body Mass Index ; Child ; Cohort Studies ; Electronic Health Records ; Failure to Thrive ; Humans ; Micronutrients ; Reference Values ; Retrospective Studies

Purpose: The objective of this study was to identify the significance of 24-hour postreduction ultrasonography (US) in pediatric patients with intussusception. Methods: A total of 229 patients with intussusception who were treated with saline reduction at Severance Children’s Hospital between January 2014 and September 2020 were retrospectively reviewed. The 229 patients with successful saline reduction were divided into two groups: a recurrence at 24 hours group (R, n=41) and a non-recurrence group (NR, n=188). The full patient sample was divided into two groups: follow-up US (FU) or no followup US (NFU); the recurrence group was divided into follow-up (R-FU) and non-follow-up (R-NFU) subgroups, and stratified analyses were performed. Results: There were no significant differences in age, sex, laboratory findings, symptoms, and sonographic findings between the NR and R groups. In the R group, 24 patients underwent follow-up US, and 17 patients did not. Specific sonographic findings were statistically significant in the R-FU group compared to the R-NFU group (p=0.002). The R-FU group had fewer admissions (p=0.012) and longer mean hospitalization times (p<0.001) than the R-NFU group. The NFU group had a 12.2% recurrence rate, while the R-FU group recurrence rate was 25.8% (p=0.0099), suggesting that the omission of some recurrent events and follow-up US was a significant variable in the recurrence of intussusception. The median time to recurrence was 21 hours which supports the 24-hour follow-up protocol. Conclusion Twenty-four-hour follow-up US was shown to be valuable for detecting early recurrence of intussusception.

Purpose: Liver transplantation (LT) is the only curative treatment for acute liver failure (ALF) and acute-on-chronic liver failure (ACLF). In high-volume therapeutic plasma exchange (HV-TPE), extracorporeal liver support filters accumulate toxins and improve the coagulation factor by replacing them. In this study, we aimed to evaluate the effectiveness of HV-TPE in pediatric patients with ALF and ACLF. Methods: We reviewed the records of children waiting for LT at Severance Hospital who underwent HV-TPE between 2017 and 2021. Aspartate aminotransferase (AST), alanine aminotransferase (ALT), total and direct bilirubin (TB and DB), gamma-glutamyl transferase (GGT), ammonia, and coagulation parameter-international normalized ratio (INR) were all measured before and after HV-TPE to analyze the liver function. The statistical analysis was performed using IBM SPSS Statistics for Windows, version 26.0 (IBM Co., Armonk, NY, USA). Results: Nine patients underwent HV-TPE with standard medical therapy while waiting for LT. One had neonatal hemochromatosis, four had biliary atresia, and the other four had ALF of unknown etiology. Significant decreases in AST, ALT, TB, DB, GGT, and INR were noted after performing HV-TPE (930.38–331.75 IU/L, 282.62–63.00 IU/L, 11.75–5.59 mg/dL, 8.10– 3.66 mg/dL, 205.62–51.75 IU/L, and 3.57–1.50, respectively, p<0.05). All patients underwent LT, and two expired due to acute complications. Conclusion HV-TPE could remove accumulated toxins and improve coagulation. Therefore, we conclude that HV-TPE can be regarded as a representative bridging therapy before LT.

Pyoderma gangrenosum (PG) is a rare, non-infectious, neutrophilic dermatosis characterized by painful ulcers with indistinct borders and peripheral erythema. The diagnosis of PG requires the exclusion of other causes of similar appearing skin manifestations, including vasculitis and infections. The pathogenesis of PG is not clear; however, dysregulation of the immune system has been suggested in previous studies. More than half of the PG patients have underlying diseases; the most common being inflammatory bowel disease (IBD). The progression of PG in IBD patients is seen after the onset of IBD, usually during its exacerbation. On the other hand, PG may follow a course independent of the intestinal disease. We present a case of an 18-year-old young male with PG that presented before being diagnosed with ulcerative colitis as an associated condition. He had a painful ulcerative lesion on his right shin with no previous gastrointestinal symptoms. This case suggests that investigating for underlying disorders is essential in PG patients despite the lack of symptoms other than the skin lesions.

BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) has become prevalent worldwide in the last decade. However, the recent prevalence of NAFLD in adolescents has not yet been investigated in Korea. METHODS: Data were obtained from 1,416 participants aged 10–18 years from the Korea National Health and Nutrition Examination Survey conducted in 2010 and 2015. Systolic blood pressure (SBP), diastolic blood pressure (DBP), height, weight, waist circumference (WC), body mass index (BMI), fasting glucose, total cholesterol, high-density lipoprotein (HDL), aspartate aminotransferase (AST), alanine aminotransferase (ALT) level, waist-to-height ratio (WHtR), and pediatric NAFLD fibrosis index (PNFI) were analyzed. RESULTS: SBP, weight, WC, BMI, WHtR, and total cholesterol level were significantly higher in 2015 than in 2010. Prevalence of NAFLD (BMI ≥ 85th percentile plus ALT > 30 U/L for boys and ALT > 19 U/L for girls) were 4.7% in 2010 and 5.9% in 2015 (P = 0.360). Using various cutoffs for the ALT level (> 40, > 30, > 25.8 U/L for boys and >22.1 U/L for girls) NAFLD prevalence rates were 3.0%, 4.1%, and 5.5% in 2010; 2.9%, 5.0%, and 7.1% in 2015, respectively (P = 0.899, 0.469, and 0.289). Boys had a higher SBP, DBP, height, weight, WC, BMI, WHtR, fasting glucose, total cholesterol, ALT, and lower HDL level than girls. The probability of liver fibrosis using the PNFI varies between 21.3% and 24.5% among NAFLD participants (P < 0.001). CONCLUSION: The Korean society needs to quickly control the increasing prevalence of NAFLD in adolescents and reduce its complications.
Adolescent* ; Alanine Transaminase ; Aspartate Aminotransferases ; Blood Pressure ; Body Mass Index ; Cholesterol ; Fasting ; Female ; Fibrosis ; Glucose ; Humans ; Korea ; Lipoproteins ; Liver Cirrhosis ; Non-alcoholic Fatty Liver Disease* ; Nutrition Surveys ; Prevalence* ; Waist Circumference

PURPOSE: The efficacy of exclusive enteral nutrition (EEN) with a polymeric diet has not been confirmed in Korean pediatric patients with Crohn's disease (CD). This study aimed to compare the effectiveness of EEN with a specific polymeric diet (Encover®) and corticosteroids (CSs) for the induction of remission in Korean pediatric CD patients. METHODS: We retrospectively compared data from 51 pediatric CD patients who underwent induction therapy with EEN (n=19) or CSs (n=32) at Severance Children's Hospital or Incheon St. Mary's Hospital in Korea. The primary endpoint of this study was the rate of clinical remission, defined as a Pediatric Crohn's Disease Activity Index (PCDAI) score < 10, after 8 weeks of induction treatment. Clinical, laboratory, and growth data at post-induction as well as their changes from baseline were also compared between groups. RESULTS: After 8 weeks of induction therapy, clinical remission rates were 78.9% (15/19) and 65.6% (21/32) in the EEN and CS groups, respectively (p=0.313). No significant differences in PCDAI scores, laboratory variables, and growth parameters were noted between the two groups at post-induction. However, significant changes in albumin levels at post-induction were observed in the EEN group compared to the CS group (p=0.038). CONCLUSION: Our results suggest that the effectiveness of EEN with a polymeric diet and CSs for induction therapy did not differ in Korean pediatric CD patients. EEN with a polymeric diet is a good first-line treatment option for the induction of remission in these patients.
Adrenal Cortex Hormones ; Child ; Crohn Disease* ; Diet* ; Enteral Nutrition* ; Humans ; Incheon ; Korea ; Polymers* ; Remission Induction ; Retrospective Studies

PURPOSE: Several studies have investigated the association between acute gastroenteritis (AGE) and regional temperatures. We aimed to investigate the correlation between the occurrence of AGE and regional temperature in Korean children. METHODS: We reviewed the clinical characteristics of children aged 18 years or younger with AGE, defined according to the International Statistical Classification of Diseases, 10th Revision codes, who visited a Korean tertiary hospital emergency department (ED) between 2006 and 2016, and compared the children who were discharged from the ED and those who were hospitalized. Data on temperature in Seoul, Korea during the period were obtained from the Korea Meteorological Administration. Subsequently, we assessed correlations between the monthly mean numbers of the children with AGE and monthly mean temperature using Pearson's correlation analysis.
Child ; Child, Hospitalized ; Chills ; Classification ; Cough ; Emergencies ; Emergency Medicine ; Emergency Service, Hospital ; Epidemiology ; Fever ; Gastroenteritis ; Hospitalization ; Humans ; Korea ; Resource Allocation ; Seoul ; Sputum ; Tertiary Care Centers ; Vomiting ; Weather

Mitochondria play essential role in eukaryotic cells including in the oxidative phosphorylation and generation of adenosine triphosphate via the electron-transport chain. Therefore, defects in mitochondrial DNA (mtDNA) can result in mitochondrial dysfunction which leads to various mitochondrial disorders that may present with various neurologic and non-neurologic manifestations. Mutations in the nuclear gene polymerase gamma (POLG) are associated with mtDNA depletions, and Alpers-Huttenlocher syndrome is one of the most severe manifestations of POLG mutation characterized by the clinical triad of intractable seizures, psychomotor regression, and liver failure. The hepatic manifestation usually occurs late in the disease's course, but in some references, hepatitis was reportedly the first manifestation. Liver transplantation was considered contraindicated in Alpers-Huttenlocher syndrome due to its poor prognosis. We acknowledged a patient with the first manifestation of the disease being hepatic failure who eventually underwent liver transplantation, and whose neurological outcome improved after cocktail therapy.
Adenosine Triphosphate ; Diffuse Cerebral Sclerosis of Schilder* ; DNA, Mitochondrial ; Eukaryotic Cells ; Hepatitis ; Humans ; Liver Failure* ; Liver Transplantation* ; Liver* ; Mitochondria ; Mitochondrial Diseases ; Oxidative Phosphorylation ; Prognosis ; Seizures

Mitochondria play essential role in eukaryotic cells including in the oxidative phosphorylation and generation of adenosine triphosphate via the electron-transport chain. Therefore, defects in mitochondrial DNA (mtDNA) can result in mitochondrial dysfunction which leads to various mitochondrial disorders that may present with various neurologic and non-neurologic manifestations. Mutations in the nuclear gene polymerase gamma (POLG) are associated with mtDNA depletions, and Alpers-Huttenlocher syndrome is one of the most severe manifestations of POLG mutation characterized by the clinical triad of intractable seizures, psychomotor regression, and liver failure. The hepatic manifestation usually occurs late in the disease's course, but in some references, hepatitis was reportedly the first manifestation. Liver transplantation was considered contraindicated in Alpers-Huttenlocher syndrome due to its poor prognosis. We acknowledged a patient with the first manifestation of the disease being hepatic failure who eventually underwent liver transplantation, and whose neurological outcome improved after cocktail therapy.
Adenosine Triphosphate ; Diffuse Cerebral Sclerosis of Schilder* ; DNA, Mitochondrial ; Eukaryotic Cells ; Hepatitis ; Humans ; Liver Failure* ; Liver Transplantation* ; Liver* ; Mitochondria ; Mitochondrial Diseases ; Oxidative Phosphorylation ; Prognosis ; Seizures

BACKGROUND: It has been known that vitamin D level (serum 25[OH]D) has correlation with inflammatory bowel disease (IBD). The purpose of this study is to investigate changes of serum 25[OH]D in pediatric IBD patients according to the disease activity. METHODS: A total of 96 children and adolescent with IBD were enrolled in this retrospective study. Serologic inflammatory markers and clinical disease activity scores of the patients were collected, and their correlations with serum 25[OH]D were analyzed. Seasonal variations of serum 25[OH]D were also investigated both in active disease state and remission state. RESULTS: Of the 96 patients, 41 (43%) were women and patients with a vitamin D deficiency (< 20 ng/mL) at diagnosis were 77 (80.2%). There was no significant difference between Crohn's disease and ulcerative colitis for serum 25[OH]D. Serum 25[OH]D was higher in remission group than in active disease group (12.4 [8.8–29] ng/mL vs. 17.9 [12.3–34.4] ng/mL; P < 0.001) and the difference was more significant than other micronutrients. There was no significant difference in serum 25[OH]D concentration between patients with ileal involvement and patients without ileal involvement. There were seasonal variations in the active phase, but there was no significant difference by season in the remission phase. CONCLUSION: Serum 25[OH]D is inversely correlated with disease activity in IBD. Monitoring and supplementation is required especially for active disease status and in winter and spring season.
Adolescent ; Child ; Colitis, Ulcerative ; Crohn Disease ; Diagnosis ; Female ; Humans ; Inflammatory Bowel Diseases ; Micronutrients ; Retrospective Studies ; Seasons ; Vitamin D Deficiency ; Vitamin D ; Vitamins