The Patients with Hirschsprung's Disease Who Underwent Pull-Through at Age Less than 1 Year: Longitudinal Bowel Function.

BACKGROUND: Frequent stooling immediately after pull-through (PT), fecal soiling, and constipation are chronic complications of Hirschsprung's disease (HD). This study aimed to investigate the longitudinal outcomes in terms of bowel function of patients below the age of 1 year undergoing PT. METHODS: We retrospectively evaluated 396 patients who underwent PT for HD between September 1979 and March 2014. Stool frequency was analyzed up to 10 years of age, and soiling and constipation were analyzed up to 15 years of age. RESULTS: After resection of the aganglionic segment (AS), stool frequency decreased over time. Furthermore, stool frequency among the three groups was similar 4 years after PT. Among the patients with aganglionic bowel resection, those who underwent the Soave procedure (SP) had an increase (0.56/day) in stool frequency than those who underwent the Duhamel procedure (DP). The soiling severity according to the AS was similar after 5 years of age. More severe soiling was better associated with patients who underwent the SP than those who underwent the DP. The constipation severity increased gradually until around 5 years and declined thereafter. More severe constipation was better associated with the DP than with the SP. CONCLUSION: The result of the analysis of stool frequency and soiling in patients with HD indicated that shorter ASs resulted in fewer bowel movements and less severe soiling. However, with the increase in patient age, the differences became similar. Compared to the DP, the SP was associated with an increased frequency of bowel movements and soiling severity; however, the constipation severity was lower.

Analysis of Associated Anomalies in Anorectal Malformation: Major and Minor Anomalies.

BACKGROUND: Fifty to sixty percent of patients with anorectal malformation (ARM) have at least one associated anomaly (AA). We determined the incidence of AA with the subtypes of ARM classified in accordance with the Krickenbeck classification and analyzed differences in the incidence rates of major and minor AAs according to organ system. METHODS: From January 1999 to May 2017, we retrospectively analyzed congenital anomalies in patients who underwent an anoplasty for ARM at our institution. The AAs were divided into nine organ systems. To analyze the difference in the incidence of AAs, we calculated odds ratios (ORs) using cases of perineal fistula as the base group. RESULTS: Of the 460 patients, 256 (55.7%) were male, 299 (65%) had at least one anomaly, and 274 (59.6%) had major AAs. According to organ system, AAs were most common in the genitourinary (28%), cardiovascular (25%), and spinal/vertebral systems (22.6%). Major AA was most common in the cardiovascular (23%) and spinal/vertebral and genitourinary systems (19.3%). According to ARM subtype, AAs were common in the order of cloaca (93.9%), rectovaginal fistula (85.7%), and recto-bladder neck fistula (85%). For the incidence of AAs, cloaca (OR, 15.7) and recto-bladder neck fistula (OR, 5.74) showed significantly higher ORs. In the analysis of major AAs, the cloaca (OR, 19.77) showed the highest OR, followed by no fistula (OR, 4.78) and recto-bladder neck fistula (OR, 3.83). CONCLUSION: A considerable number of patients with ARM had AAs. Our data are useful for predicting AAs in patients with ARM.

Impact of hospital size on the outcomes of appendectomy in children: an analysis of a comprehensive nationwide pediatric dataset.

PURPOSES: The purpose of this study was to investigate the outcomes after appendectomy in children according to hospital size. METHODS: The records of 11,565 patients with the diagnosis-related group code for appendectomy were extracted from HIRA-Pediatric Patient Sample from 2012 to 2016. The number of hospital visits and the length of stay in hospital within 30 days after appendectomy were analyzed. RESULTS: Patients who were treated at large-sized hospitals were more likely to be younger, more likely to reside in metropolitan areas, and tended to receive laparoscopic surgery. The number of hospital visits within 30 days in patients managed by medium- and large-sized hospitals decreased in comparison to small-sized hospitals. The length of hospital stay in large-sized hospitals was decreased in comparison to small- and medium-sized hospitals. A subgroup analysis revealed that complicated appendectomy did not have a significant impact on the difference in the length of hospital stay between hospital sizes. CONCLUSION: The number of hospital visits and the length of hospital stay was higher in small-sized hospitals in comparison to large-sized hospitals. Appendectomy performed in the larger hospital showed better outcomes in pediatric patients. We recommend that pediatric surgical procedures be performed in large hospitals, and that proper incentives be given for procedures to be performed by pediatric specialists.

Predicting Survival of Congenital Diaphragmatic Hernia on the First Day of Life.

BACKGROUND: This study aimed to determine perinatal risk factors for 30-day mortality of congenital diaphragmatic hernia (CDH) patients and develop a prognostic index to predict 30-day mortality of CDH patients. Identifying risk factors that can prognosticate outcome is critical to obtain the best management practices for patients. METHODS: A retrospective study was performed for patients who were diagnosed with CDH from November 2000 to August 2016. A total of 10 prenatal risk factors and 14 postnatal risk factors were analyzed. All postnatal variables were measured within 24 h after birth. RESULTS: A total of 95 CDH patients were enrolled in this study, including 61 males and 34 females with mean gestational age of 38.86 ± 1.51 weeks. The overall 30-day survival rate was 63.2%. Multivariate analysis revealed that five factors (polyhydramnios, gestational age at diagnosis <25 weeks, observed-to-expected lung-to-head ratio ≤45, best oxygenation index in 24 h >11, and severity of tricuspid regurgitation ≥ mild) were independent predictors of 30-day mortality of CDH. Using these five factors, a perinatal prognostic index for 30-day mortality was developed. Four predictive models (poor, bad, good, and excellent) of the perinatal prognostic index were constructed, and external validation was performed. CONCLUSIONS: Awareness of risk factors is very important for predicting prognosis and managing patients. Five independent perinatal risk factors were identified in this study. A perinatal prognostic index was developed for 30-day mortality for patients with CDH. This index may be used to help manage CDH patients.

Comparison of Acute Abdominal Surgical Outcomes of Extremely-Low-Birth-Weight Neonates according to Differential Diagnosis.

BACKGROUND: Improvements in perinatal intensive care have improved survival of extremely-low-birth-weight (ELBW) neonates, although the risk of acute abdomen has increased. The differential diagnosis resulting in abdominal surgery can be categorized into necrotizing enterocolitis (NEC), spontaneous intestinal perforation (SIP), meconium-related ileus (MRI), and meconium non-related ileus (MNRI). The purpose of this study was to review our experience with abdominal surgery for ELBW neonates, and to evaluate characteristics and prognosis according to the differential diagnosis. METHODS: Medical records of ELBW neonates treated between 2003 and 2015 were retrospectively reviewed. RESULTS: Of 805 ELBW neonates, 65 (8.1%) received abdominal surgery. The numbers of cases by disease category were 29 for NEC, 18 for SIP, 13 for MRI, and 5 for MNRI. Ostoma formation was performed in 61 (93.8%) patients; primary anastomosis without ostoma was performed in 4 (6.2%). All patients without ostoma formation experienced re-perforation of the anastomosis site. Seven patients had 30-day postoperative mortality (6 had NEC). Long-term survival of the surgical and non-surgical groups was not statistically different. NEC was a poor prognostic factor for survival outcome (P = 0.033). CONCLUSION: Abdominal surgery for ELBW neonates is feasible. Ostoma formation can lead to reduced complications compared to primary anastomosis.

Analysis of Growth, Nutritional Status and Hospital Visitation Scores Associated with Reflux After Nissen Fundoplication in Neurologically Impaired Children with Gastroesophageal Reflux.

BACKGROUND: Neurologically impaired children (NIC) often experience swallowing difficulties and gastroesophageal reflux disease (GERD). Although these conditions could place children in a state of poor nutritional status and prevent them from thriving, there is insufficient research evaluating growth and nutritional status following fundoplication in these patients. METHOD: This is a retrospective study of patients who were neurologically impaired and underwent Nissen fundoplication between April 2001 and March 2015. Seventy-six patients were enrolled, and the follow-up period was 12 months or longer. Growth was measured by the change in body weight and height. Nutritional status was measured by the change in body mass index, serum albumin and protein level. RESULTS: Median age at operation was 1.85 years old, and median body weight was 10 kg. The respective Z scores for weight and height showed significant improvements after 1 year since the operation compared to 1 year within the operation (-2.42 ± 2.19 vs. -1.31 ± 1.96, P < 0.001) (-1.6 ± 2.16 vs. -1.05 ± 1.69, P = 0.002). The respective Z scores for body mass index, albumin and protein also showed improvements after 1 year since the operation compared to 1 year within the operation (-2.07 ± 2.99 vs. -0.89 ± 2.1, P < 0.001) (3.55 ± 0.48 vs. 3.86 ± 0.45, P < 0.001) (6.22 ± 0.76 vs. 6.65 ± 0.51, P < 0.001). Hospital visitation scores associated with reflux were significantly lower after the operation (4.1 ± 3.43 vs. 1.18 ± 1.67, P < 0.001). CONCLUSIONS: In summary, after Nissen fundoplication in NIC with GER, growth and nutritional status improved significantly. Also, hospital visitation scores associated with reflux decreased after the operation.

RNA-Seq for Gene Expression Profiling of Human Necrotizing Enterocolitis: a Pilot Study.

Necrotizing enterocolitis (NEC) characterized by inflammatory intestinal necrosis is a major cause of mortality and morbidity in newborns. Deep RNA sequencing (RNA-Seq) has recently emerged as a powerful technology enabling better quantification of gene expression than microarrays with a lower background signal. A total of 10 transcriptomes from 5 pairs of NEC lesions and adjacent normal tissues obtained from preterm infants with NEC were analyzed. As a result, a total of 65 genes (57 down-regulated and 8 up-regulated) revealed significantly different expression levels in the NEC lesion compared to the adjacent normal region, based on a significance at fold change ≥ 1.5 and P ≤ 0.05. The most significant gene, DPF3 (P < 0.001), has recently been reported to have differential expressions in colon segments. Our gene ontology analysis between NEC lesion and adjacent normal tissues showed that down-regulated genes were included in nervous system development with the most significance (P = 9.3 × 10⁻7; P(corr) = 0.0003). In further pathway analysis using Pathway Express based on the Kyoto Encyclopedia of Genes and Genomes (KEGG) database, genes involved in thyroid cancer and axon guidance were predicted to be associated with different expression (P(corr) = 0.008 and 0.020, respectively). Although further replications using a larger sample size and functional evaluations are needed, our results suggest that altered gene expression and the genes' involved functional pathways and categories may provide insight into NEC development and aid in future research.

Analysis of clinical features of lipoblastoma in children.

OBJECTIVE: A lipoblastoma is pathologically benign but often recurs. Due to its rarity, studies are scarce. The purpose of this study was to investigate the clinical characteristics of lipoblastoma occurring in children and to detect any correlations with the expression of Ki-67. PARTICIPANTS: From 1998 to 2010, 33 patients were diagnosed with lipoblastoma at Seoul National University Children's Hospital. METHODS: Ki-67 immunohistochemistry staining of the tumor tissue was performed. RESULTS: A total of 33 patients (64% males) were enrolled in the study, with a mean age of 28 month. Eleven and 22 lesions were deep and superficial, respectively. Complete excisions were performed for 30 patients, and three underwent incomplete excisions. Two patients who underwent incomplete excision subsequently underwent a second operation due to tumor regrowth, and one patient had a recurrence despite complete excision. There was no statistically significant correlation observed between the tumor size or recurrence and the expression of Ki-67. CONCLUSIONS: Lipoblastoma requires an accurate diagnosis and operative resection to alleviate the symptoms induced by its growth. Incompletely resected tumor may regrow; therefore, complete excision is the treatment of choice. Continuous follow-up is needed to monitor for recurrence of disease, even after a complete excision.

Use of spiral computed tomography volumetry for determining the operative approach in patients with Graves' disease.

BACKGROUND: The purposes of the present study were to assess (1) the correlation between the weight of the postoperative thyroid specimen and the spiral computed tomography (CT) volumetry results of the thyroid gland in patients with Graves' disease, and (2) the utility of CT volumetry for determining the operative approach. METHODS: From 2009 to 2010, a total of 56 patients with Graves' disease underwent total or subtotal thyroidectomy. An enhanced spiral CT was taken in all patients prior to the operation. From 2.5 mm-thick slices of the thyroid gland, the surface area was calculated to measure the volume of the thyroid gland. The glandular volume was compared to the weight of the postoperative thyroid specimen. RESULTS: A total of 42 and 14 patients underwent total and subtotal thyroidectomy, respectively. The mean weight of the postoperative thyroid specimen was 43.9 ± 33.4 g, and the mean volume obtained by CT volumetry was 44.2 ± 32.8 mL. A good correlation was observed between the weight of the postoperative thyroid specimen and the volume calculated by CT (r = 0.98, p < 0.001). When 100 mL was set as the higher cut-off value of the thyroid volume for minimally invasive thyroid surgery, the estimated blood loss showed a significant difference between the >100 mL and the ≤100 mL groups (608.3 ± 540.8 vs. 119.7 ± 110.4 mL; p = 0.036). CONCLUSIONS: Spiral CT volumetry may be used to measure the thyroid volume reliably in patients with Graves' disease. For cases in which surgery is indicated in patients with Graves' disease, CT volumetry provides useful information from which to determine the operative approach. One hundred milliliter or less of thyroid volume in CT volumetry is recommended to perform minimally invasive thyroid surgery.

Long term outcomes of pediatric liver transplantation according to age.

Liver transplantation (LT) has been the key therapy for end stage liver diseases. However, LT in infancy is still understudied. From 1992 to 2010, 152 children had undergone LT in Seoul National University Hospital. Operations were performed on 43 patients aged less than 12 months (Group A) and 109 patients aged over 12 months (Group B). The mean age of the recipients was 7 months in Group A and 74 months in Group B. The patients' survival rates and post-LT complications were analyzed. The mean Pediatric End-stage Liver Disease score was higher in Group A (21.8) than in Group B (13.4) (P = 0.049). Fulminant hepatitis was less common in Group A (4.8%) than in Group B (13.8%) (P = 0.021). The post-transplant lymphoproliferative disorder and portal vein complication were more common in Group A (14.0%, 18.6%) than in Group B (1.8%, 3.7%) (P = 0.005). However, the 1, 5, and 10 yr patient survival rates were 93%, 93%, and 93%, in Group A and 92%, 90%, and 88% in Group B (P = 0.212). The survival outcome of pediatric LT is excellent and similar regardless of age. LTs in infancy are not riskier than those of children.

Comparison of long-term outcome according to involved aganglionic segments of total colonic aganglionosis.

ABSTRACT: Total colonic aganglionosis (TCA) is a rare form of Hirschsprung disease, with more severe symptoms than rectosigmoid Hirschsprung disease. We aimed to evaluate the surgical outcomes according to the involved segments of TCA.Patients with aganglionosis extending from the anus to at least the ileocecal valve were included. The medical records of 33 TCA patients from 1981 to 2014 were reviewed. Three groups were analyzed based on the involved segment (jejunum, jejunoileal junction, and distal ileum).The median age at the pull-through operation was 6.2 (3.3-114) months. The median follow-up duration was 216 (21-411) months. Transition zone in the jejunum, jejunoileal junction, and distal ileum was identified in 3, 5, and 25 patients, respectively. The most common method of operation was Duhamel pull-through. Perianal excoriation and enterocolitis were the most common postoperative complications. The complication rates were 45% to 51% and not different among the groups. The defecation frequency normalized 3 years postoperatively, and body weight started to recover after 2 years irrespective of the involved segment.Therefore, close monitoring with proper management of defecation and body weight for at least 2 to 3 years postoperatively is required.

Incidence of necrotizing enterocolitis in South Korea from 2007 to 2017.

OBJECTIVES: The aim of this study was to investigate the trends of the incidence of and mortality from necrotizing enterocolitis (NEC) in South Korea. METHODS: Claim data were extracted with diagnosis code, including all stage of NEC. Kaplan-Meier curves for cumulative mortality rates are presented by birth weight (Bwt) and gestational age (GA). RESULT: The total number of NEC cases was 5840. The ratio of males to females was 1.2:1. There were 11.9 cases per 10,000 births. There were approximately 10 cases per 1000 preterm births and 50 per 1000 very preterm births. The mortality of patients born under 37 weeks decreased from 38.7% in 2007 to 20.9% in 2017. Male sex, lower GA, and lower Bwt were risk factors for mortality. CONCLUSIONS: NEC incidence and mortality have been decreasing over time in South Korea. Male sex, lower GA, and Bwt were risk factors for mortality.

Congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula: clinical and radiologic features.

BACKGROUND: Congenital esophageal stenosis (CES) can be associated with esophageal atresia/tracheoesophageal fistula (EA/TEF). Because there are a variety of degrees of obstruction and symptoms of CES, it is frequently difficult to make a pre- and post-operative diagnosis of the distal CES associated with EA/TEF. OBJECTIVE: To evaluate the clinical and radiologic features of congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula. MATERIALS AND METHODS: We retrospectively reviewed postoperative esophagograms and medical records of 187 children (107 boys, 80 girls) who had primary repair of EA/TEF from 1992 to 2009 at our institution. We evaluated the incidence of CES, clinical findings, radiologic features and management of CES in these children. RESULTS: CES was diagnosed in 22 of 187 EA/TEF children (12%); one child had double CES lesions, for a total of 23 lesions. Ten of those 22 children (45%) had presented with significant symptoms of esophageal obstruction. The diagnosis of CES was delayed in 10 children (45%) until 1-10 years of age. On esophagogram, CES (n = 23) was located in the distal esophagus (n = 20, 87%) or mid-esophagus (n = 3, 13%). The degree of stenosis was severe (n = 6, 26%), moderate (n = 10, 43%), or mild (n = 7, 30%). Eight children, including two with unsuccessful esophageal balloon dilatation of CES, were treated surgically. Histologic examination revealed tracheobronchial remnant (n = 7) or fibromuscular hyperplasia (n = 1). One child with surgically treated CES developed achalasia at the age of 3 years 9 months. CONCLUSION: Esophagography after EA/TEF repair should be performed with a high index of suspicion for the presence of distal CES, because the diagnosis and adequate management of CES can often be delayed.

Falciform ligament abscess after omphalitis: report of a case.

A falciform ligament abscess is a rare type of intra-abdominal abscess. A 2-yr-old male, who had omphalitis two months previously, presented with a fever and right upper quadrant abdominal pain. The ultrasound and CT scan showed an abdominal wall abscess located anterior to the liver, which was refractory to conservative management with percutaneous drainage and antibiotics. On the third recurrence, surgical exploration was performed and revealed an abscess arising from the falciform ligament; the falciform ligament was excised. A follow up ultrasound confirmed complete resolution of the abscess with no further recurrence.

Clinical features of children with Haddad syndrome: A single-center experience.

BACKGROUND/PURPOSE: Haddad syndrome (HS) is a very rare disease considered a form of neurocristopathy. It is characterized by a combination of congenital central hypoventilation syndrome (CCHS) and Hirschsprung's disease (HD). We report the clinical features and disease progression of HS to provide better care for HS patients by achieving an earlier diagnosis and optimal treatment. METHODS: Medical records of patients diagnosed with HS from 2005 to 2016 were retrospectively reviewed. Demographic data including gestational age, birth weight and height, and paired-like homeobox 2b (PHOX2B) gene mutation were collected. RESULTS: Seven males and three females were identified (mean gestational age 39.76 ±â€¯1.49 weeks, mean birth weight 3117.5 ±â€¯288.9 g). PHOX2B gene mutation was identified in all patients. Immediate ventilation care after birth was required in five patients due to poor respiration. The current median age of the children is 5.4 years (range, 1.8-10.1). Tracheostomy was performed in nine patients. Eight patients required sleep ventilation and two patients, 24-h continuous ventilation support. Six patients showed rectosigmoid aganglionosis and four patients exhibited total colonic aganglionosis, of these one had aganglionosis extended to the distal small bowel. Soiling was observed in seven patients (5 with laparoscopy-assisted transanal endorectal pull-through and 2 with Duhamel procedure) and one patient showed grade 2 constipation with Duhamel procedure. Six patients had developmental delay. All patients are alive. CONCLUSIONS: HS may require lifelong medical care. This study could be helpful to understand the clinical features of HS including associated abnormalities and disease progression. By assisting to understand the clinical features, we could provide better care for HS patients by achieving an earlier diagnosis and appropriate treatment. TYPE OF STUDY: Prognosis study. LEVEL OF EVIDENCE: Level IV.

Effect of sun exposure versus oral vitamin D supplementation on serum 25-hydroxyvitamin D concentrations in young adults: A randomized clinical trial.

BACKGROUND: Vitamin D inadequacy is associated with a wide range of diseases. However, optimal strategies to improve vitamin D status, especially in Asian populations, remain unclear. We tested the hypotheses that (1) relevant sun exposure or oral vitamin D supplementation would significantly increase serum 25-hydroxyvitamin D (25OHD) concentrations compared with placebo, (2) sun exposure and supplementary vitamin D would be similar in serum 25OHD increases, and (3) the two interventions may have different effects on cardio-metabolic markers. METHODS: In this 8-week randomized placebo-controlled clinical trial including vitamin D-deficient adults in Seoul (37 °N), Korea, changes in serum 25OHD concentrations were compared between the sun exposure (daily ≥20-30 min around noon, n = 50), oral vitamin D3 (500 IU/d, n = 50), and control (placebo, n = 50) groups. RESULTS: Both sun exposure and oral vitamin D3 effectively increased serum 25OHD concentrations. Compared with placebo, the between-group least-squares mean (LSM) differences in changes were 2.2 ng/mL (95% CI: 0.2, 4.2) in the sun exposure group and 8.5 ng/mL (6.5, 10.5) in the oral vitamin D3 group. Increases in serum 25OHD were greater with oral vitamin D3 than with sun exposure (LSM difference in changes = 6.3 ng/mL, 95% CI: 4.3, 8.3). More participants in the oral vitamin D3 group (54.2%) achieved serum 25OHD concentrations ≥20 ng/mL at week 8 than those in the sun exposure (12.2%) or control (4.3%) groups. Compliance with sun exposure advice was relatively low, and only those with adequate compliance had a significant increase in serum 25OHD. Changes in the cardio-metabolic markers were mostly insignificant in all groups. CONCLUSIONS: Enhanced sun exposure and 500 IU/d of oral vitamin D3 supplementation significantly increased serum 25OHD concentrations. However, our protocol for sun exposure was not as effective as 500 IU/d of oral vitamin D3 supplementation. This trial was registered at clinicaltrials.gov as NCT03310242.

The effect of Absolute Neutrophil Count (ANC) on early surgical site infection in Implanted Central Venous Catheter (ICVC).

PURPOSE: The aim of this study was to evaluate surgical site infection (SSI) rates related to implanted central venous catheters (ICVC) in pediatric hematology and oncology patients with respect to absolute neutrophil count (ANC) levels. PATIENTS AND METHODS: From January 2004 to December 2015, pediatric patients with ICVC insertion were investigated retrospectively. Patients were divided into four groups according to preoperative ANC levels and Granulocyte-colony stimulating factor (G-CSF) usage. Immediate and early surgical site infections were evaluated 7 and 30 days following surgery. RESULTS: In total, 1143 patients were enrolled. Patients were placed into 4 groups: 930 patients in group 1 with an ANC≥500/µL without G-CSF, 149 in group 2 with an ANC≥500/µL after G-CSF usage, 36 in group 3 with an ANC<500/µL without G-CSF, and 28 in group 4 with an ANC<500/µL even after G-CSF administration. Rates of immediate and early SSIs were not statistically different between groups. In the two-group analysis (group 1 and 2 vs. 3 and 4), the number of immediate and early SSIs were not also different, respectively. CONCLUSION: There was no correlation between ANC levels and immediate and early SSI occurrence after ICVC placement. LEVEL OF EVIDENCE: III.

Circulating microRNA expression profiling in young obese Korean women.

BACKGROUND/OBJECTIVES: This study investigates correlations between circulating microRNAs (miRNAs) and obesity-related parameters among young women (aged 20-30 years old) in Korea. SUBJECTS/METHODS: We analyzed TaqMan low density arrays (TLDAs) of circulating miRNAs in 9 lean (body mass index [BMI] < 25 kg/m2) and 15 obese (BMI > 25 kg/m2) women. We also performed gene ontology (GO) analyses of the biological functions of predicted miRNA target genes, and clustered the results using the database for annotation, visualization and integrated discovery. RESULTS: The TLDA cards contain 754 human miRNAs; of these, the levels of 8 circulating miRNAs significantly declined (> 2-fold) in obese subjects compared with those in lean subjects, including miR-1227, miR-144-5p, miR-192, miR-320, miR-320b, miR-484, miR-324-3p, and miR-378. Among them, miR-484 and miR-378 displayed the most significant inverse correlations with BMI (miR-484, r = -0.5484, P = 0.0056; miR-378, r = -0.5538, P = 0.0050) and visceral fat content (miR-484, r = -0.6141, P = 0.0014; miR-378, r = -0.6090, P = 0.0017). GO analysis indicated that genes targeted by miR-484 and miR-378 had major roles in carbohydrate and lipid metabolism. CONCLUSION: Our result showed the differentially expressed circulating miRNAs in obese subjects compared to lean subjects. Although the mechanistic study to reveal the causal role of miRNAs remains, these miRNAs may be novel biomarkers for obesity.

Experiences of Rectovaginal Fistula in Anorectal Malformation.

BACKGROUND: Rectovaginal fistulas (RVFs) are very rare malformations in females with anorectal malformations (ARMs). Here, we share the clinical features of RVF and report the long-term outcomes. METHODS: RVF patients were classified using a retrospective analysis of ARM patients who underwent operations at Seoul National University Hospital between January 1999 and May 2017. The Krickenbeck continence scoring system was used to evaluate bowel function 5 and 10 years after surgery. RESULTS: Of the total 460 ARM patients, 203 were female, 7 of whom were diagnosed with RVF. The median age and weight at the time of anorectoplasty were 292 days (range, 140-617) and 8.2 kg (range, 5.5-12), respectively. Six patients had associated anomalies and three patients underwent redo-anorectoplasty. Voluntary bowel movements were observed in 6 out of 7 patients at 5 and 10 years of age. Soiling was observed in all patients at the age of five years and in 6 out of 7 patients at the age of ten years. Constipation was observed in 6 out of 7 patients at both five and ten years of age. CONCLUSIONS: An RVF is a very rare malformation, accounting for 1.5% of total ARMs and 3.4% of ARMs in females. Long-term counseling, education, and guidance are needed for effective management of patients' bowel movements. TYPE OF STUDY: Prognosis study LEVEL OF EVIDENCE: Level IV.

Risk factors of meconium-related ileus in very low birth weight infants: patients-control study.

Very low birth weight (VLBW) neonates experience various problems, including meconium-related ileus (MRI). This study investigated the risk factors of MRI and surgical MRI in VLBW infants. VLBW neonates admitted to the Neonatal Intensive Care Unit of Seoul National University Children's Hospital from October 2002 to September 2016 were included in the study. The diagnostic criteria for MRI were a decreased frequency of defecation with intolerable feeding, vomiting, and increased gastric residue (>50%); meconium-filled bowel dilatation in an imaging study; and no evidence of necrotizing enteritis or spontaneous intestinal perforation. Medical MRIs and surgical MRIs were managed through conventional treatment and surgical intervention. Of 1543 neonates, 69 and 1474 were in the patient and control groups, respectively. The risk factors for MRI include low birth weight (BW), cesarean section delivery, fetal distress, maternal diabetes, maternal hypertension, and maternal steroid use. Low BW and fetal distress were independent risk factors for MRI. Compared to the medical MRI group (n = 44), the risk factors for surgical MRI (n = 25) included males, younger gestational age, low BW, and meconium located at the small bowel. Male gender and low BW were independent risk factors for surgical MRI. Low BW and fetal distress were independent risk factors for MRI and male gender and low BW were independent risk factors for surgical MRI. In VLBW neonates, careful attention to the risk factors for MRI could minimize or avoid surgical interventions.