Idiopathic nephrotic syndrome (INS) in children is characterized by massive proteinuria and hypoalbuminemia. Minimal change nephrotic syndrome (MCNS) is the most common form of INS in children. The pathogenesis of MCNS still remains unclear, however, several hypotheses have been recently proposed. For several decades, MCNS has been considered a T-cell disorder, which causes the impairment of the glomerular filtration barrier with the release of different circulating factors. Increased levels of several cytokines are also suggested. Recently, a "two-hit" theory was proposed that included the induction of CD80 (B7-1) and regulatory T-cell (Treg) dysfunction, with or without impaired autoregulatory functions of the podocyte. In contrast to the well-established involvement of T cells, the role of B cells has not been clearly identified. However, B-cell biology has recently gained more attention, because rituximab (a monoclonal antibody directed against CD20-bearing cells) demonstrated a very good therapeutic response in the treatment of childhood and adult MCNS. Here, we discuss recent insights into the pathogenesis of MCNS in children.
Adult ; B-Lymphocytes ; Biology ; Child ; Cytokines ; Glomerular Filtration Barrier ; Humans ; Hypoalbuminemia ; Nephrosis, Lipoid* ; Nephrotic Syndrome ; Podocytes ; Proteinuria ; Rituximab ; T-Lymphocytes

BACKGROUND: The purpose of this retrospective study was to develop a two- and three-dimensional analysis of the airway using cone-beam computed tomography (CBCT) and to determine whether the airway space would be changed in mandibular prognathism after bimaxillary surgery involving maxillary posterior impaction. METHODS: Patients requiring orthognathic surgery from 2012 to 2014 were recruited for this study. CBCT scans were obtained at three points: preoperatively (T0), immediate postoperatively (T1), and after 6 months postoperatively (T2). The nasopharynx, oropharynx, and hypopharynx were measured on the CBCT scan for each patient in a repeatable manner. With the midsagittal plane, linear measurements in the middle of each were obtained. For the CBCT, volumetric measurements of each and total airway were obtained. RESULTS: A total of 22 consecutive patients (11 men and 11 women) were included in the present study. The total volume was significantly reduced (p < .001). However, the change of the diameter and volume of the nasopharynx was not statistically significant (p = .160, p = .137, respectively). In the oropharynx, the change of both the diameter and volume showed statistical significance between preoperatively and immediate postoperatively (p < .001, p = .001, respectively) and also preoperatively and after 6 months postoperatively (p = .001, p = .010, respectively). In the hypopharynx, the change of both the diameter and volume showed statistical significance between preoperatively and immediate postoperatively (p = .001, p < .001, respectively) and also preoperatively and after 6 months postoperatively (p = .001, p < .001, respectively). CONCLUSIONS: The bimaxillary surgery involving maxillary posterior impaction can reduce the volume of airway in the patients of mandibular prognathism. Although total airway volume was reduced significantly, the changes in the volume and diameter of the nasopharynx were not statistically significant. The maxillary posterior impaction affects on the nasopharyngeal airway minimally.
Airway Management ; Cone-Beam Computed Tomography ; Humans ; Hypopharynx ; Male ; Nasopharynx ; Oropharynx ; Orthognathic Surgery ; Prognathism* ; Retrospective Studies

BACKGROUND: The Le Fort I osteotomy is one of the most widely used and useful procedure to correct the dentofacial deformities of the midface. The changes of the maxilla position affect to overlying soft tissue including the nasal structure. Postoperative nasal septum deviation is a rare and unpredicted outcome after the surgery. There are only a few reports reporting the management of this complication. CASE PRESENTATION: In our department, three cases of the postoperative nasal septum deviation after the Le Fort I osteotomy had been experienced. Via limited intraoral circumvestibular incision, anterior maxilla, the nasal floor, and the anterior aspect of the septum were exposed. The cartilaginous part of the nasal septum was resected and repositioned to the midline and the anterior nasal spine was recontoured. Alar cinch suture performed again to prevent the sides of nostrils from flaring outwards. After the procedure, nasal septum deviation was corrected and the esthetic outcomes were favorable. CONCLUSION: Careful extubation, intraoperative management of nasal septum, and meticulous examination of pre-existing nasal septum deviation is important to avoid postoperative nasal septum deviation. If it existed after the maxillary osteotomy, septum repositioning technique of the current report can successfully correct the postoperative septal deviation.
Dentofacial Deformities ; Maxilla ; Maxillary Osteotomy ; Nasal Septum ; Osteotomy* ; Spine ; Sutures

Neonatal hydrocolpos is a rare condition that involves fluid accumulation in the vagina. On diagnostic imaging, the dilated vagina, along with the compressed uterus, can simulate a mature cystic teratoma with a mural nodule. Herein, we report the case of a newborn girl with congenital hydrocolpos that was caused by an imperforate hymen; the hydrocolpos mimicking a mature cystic teratoma on abdominal ultrasonography and magnetic resonance imaging. Any newborn girl with a pelvic cystic mass should be suspected as having a congenital vaginal obstruction manifesting as hydrocolpos or hydrometrocolpos. Thorough examination of the external genitalia, as well as imaging of the uterus and vagina, enables correct diagnosis and optimal treatment.
Diagnosis ; Diagnostic Imaging ; Female ; Genitalia ; Humans ; Hydrocolpos* ; Hymen ; Infant ; Infant, Newborn ; Magnetic Resonance Imaging ; Pelvis* ; Teratoma* ; Ultrasonography ; Uterus ; Vagina

The incidence of symptomatic thrombosis of umbilical arterial catheterization is 1-3%. Therapeutic options may include: using heparin or low molecular weight heparin, using a thrombolytic agent, or surgical thrombectomy. However, there are insufficient data to recommend any one treatment over the others. Recently, enoxaparin, a low molecular weight heparin, has emerged as a drug of choice for the treatment of neonatal thrombosis due to pharmacologic stability over unfractionated heparin or thrombolytic agents. We report a case of successful treatment of aortic thrombosis after umbilical arterial catheterization with enoxaparin in preterm infants.
Catheterization* ; Catheters* ; Enoxaparin* ; Fibrinolytic Agents ; Heparin ; Heparin, Low-Molecular-Weight ; Humans ; Incidence ; Infant, Newborn ; Infant, Premature* ; Thrombectomy ; Thrombosis* ; Umbilical Cord*

PURPOSE: Parenteral nutrition (PN) not only provides nutritional support but also plays a crucial role in the treatment of children with intestinal failure. The aim of this study was to evaluate the clinical significance and clinical outcomes of long-term PN. METHODS: Retrospective cohort study was conducted using the medical records of patients treated at Seoul National University Children's Hospital. This study included 19 patients who received PN for over six months. Most patients received home PN. RESULTS: The indications for PN included short bowel syndrome, chronic intestinal pseudo-obstruction, and intractable diarrhea of infancy. The median age of PN initiation was 1.3 years, and the median treatment duration was 2.9 years. Two patients were weaned from PN; 14 continued to receive PN with enteral feedings; and 3 patients died. The overall survival rates at 2 and 5 years were 93.3% and 84.0%, respectively. The incidence of catheter-related bloodstream infections was 2.7/1,000 catheter-days and was associated with younger age at PN initiation and lower initial height Z-score. Six patients developed catheter-related central vein thrombosis, with an incidence of 0.25/1,000 catheter-days. Eleven patients experienced PN-associated liver disease (PNALD), and one patient underwent multi-visceral transplant. The patients with PNALD exhibited lower final heights and body weight Z-scores. All patients experienced micronutrient deficiencies transiently while receiving PN. CONCLUSION: PN is an important and safe treatment for pediatric intestinal failure. PNALD was linked to final anthropometric poor outcomes. Micronutrient deficiencies were common. Anthropometric measurements and micronutrient levels must be monitored for successful PN completion.
Body Weight ; Catheter-Related Infections ; Child ; Cholestasis ; Cohort Studies ; Diarrhea ; Humans ; Incidence ; Intestinal Pseudo-Obstruction ; Liver Diseases ; Medical Records ; Micronutrients ; Nutritional Support ; Parenteral Nutrition ; Retrospective Studies ; Seoul ; Short Bowel Syndrome ; Survival Rate ; Thrombosis ; Veins

PURPOSE: Each ethnic group has a unique life style, including diets. Life style affects bowel movement. The aim of this study is to describe the results of colon transit time (CTT) tests in Korean children who had chronic functional constipation based on highly refined data. METHODS: One hundred ninety (86 males) out of 415 children who performed a CTT test under the diagnosis of chronic constipation according to Rome III criteria at Konkuk University Medical Center from January 2006 through March 2015 were enrolled in this study. Two hundreds twenty-five children were excluded on the basis of CTT test result, defecation diary, and clinical setting. Shapiro-Wilk and Mann-Whitney U, and chi-square tests were used for statistical analysis. RESULTS: The median value and interquartile range (IQR) of CTT was 54 (37.5) hours in Encopresis group, and those in non-encopresis group was 40.2 (27.9) hours (p<0.001). The frequency of subtype between non-encopresis group and encopresis was statistically significant (p=0.002). The non-encopresis group (n=154, 81.1%) was divided into normal transit subgroup (n=84, 54.5%; median value and IQR of CTT=26.4 [9.6] hours), outlet obstruction subgroup (n=18, 11.7%; 62.4 [15.6] hours), and slow transit subgroup (n=52, 33.8%; 54.6 [21.0] hours]. The encopresis group (n=36, 18.9%) was divided into normal transit subgroup (n=8, 22.2%; median value and IQR of CTT=32.4 [9.9] hours), outlet obstruction subgroup (n=8, 22.2%; 67.8 [34.8] hours), and slow transit subgroup (n=20, 55.6%; 59.4 [62.7]hours). CONCLUSION: This study provided the basic pattern and value of the CTT test in Korean children with chronic constipation.
Academic Medical Centers ; Child* ; Colon* ; Constipation* ; Defecation ; Diagnosis ; Diet ; Encopresis ; Ethnic Groups ; Humans ; Korea ; Life Style

PURPOSE: The aim of our study was to establish a safe and convenient diagnostic method for acute gastrointestinal (GI) graft-versus-host disease (GVHD) in children by determining the sensitivity and negative predictive values of upper and lower endoscopic biopsies for children suspected of GI GVHD. METHODS: Patients suspected of GI GVHD who received endoscopic evaluation within 100 days after stem cell transplantation and endoscopies between January 2012 and March 2014 in Seoul National University Children's Hospital were included in our study. RESULTS: Fifteen patients with a total of 20 endoscopic procedures were included in our study. Sensitivity at the esophagus, stomach, and duodenum were 22.2%, 30.0%, and 80.0%, respectively. Negative predictive values at the esophagus, stomach, and duodenum were 22.2%, 30.0%, and 60.0%, respectively. Overall sensitivity and negative predictive values of upper endoscopic biopsy for GVHD were 77.8% and 50.0%, respectively. Overall sensitivity and negative predictive values of lower endoscopic biopsy for GVHD were 88.9% and 66.7%, respectively. CONCLUSION: We recommend flexible sigmoidoscopy as a safe and accurate diagnostic tool for GVHD, similar to other studies reported previously. However, if there is no evidence of GVHD on sigmoidoscopy with high index of suspicion of GI bleeding, full colonoscopy and upper endoscopy should be considered.
Biopsy ; Child ; Colonoscopy ; Duodenum ; Endoscopy* ; Esophagus ; Graft vs Host Disease* ; Hemorrhage ; Humans ; Seoul ; Sigmoidoscopy* ; Stem Cell Transplantation ; Stomach

PURPOSE: Foreign body (FB) ingestion is common in children, and button battery (BB) ingestion has been increasing in recent years. This study was to identify factors related to outcomes of FB ingestion, particularly BBs in the stomach. We evaluated whether the current recommendations are appropriate and aimed to suggest indications for endoscopic removal of BB in the stomach in young children. METHODS: We investigated patient age, shape, size, location of FBs, spontaneous passage time and resulting complications among 76 children. We observed types, size, location of BB and outcomes, and analyzed their associations with complications. RESULTS: Coins and BB were the two most common FBs. Their shapes and sizes were not associated with the spontaneous passage time. Size, spontaneous passage time, and age were also not associated with any specific complications. For BB ingestion, all 5 cases with lithium batteries (≥1.5 cm, 3 V) presented moderate to major complications in the esophagus and stomach without any symptoms, even when the batteries were in the stomach and beyond the duodenum, while no complications were noted in 7 cases with alkaline batteries (<1.5 cm, 1.5 V) (p=0.001). All endoscopies were conducted within 24 hours after ingestion. CONCLUSION: The type and voltage of the battery should be considered when determining whether endoscopy is required to remove a BB in the stomach. For lithium battery ingestion in young children, urgent endoscopic removal might be important in order to prevent complications, even if the child is asymptomatic and the battery is smaller than 2 cm.
Child* ; Duodenum ; Eating* ; Endoscopy ; Esophagus ; Foreign Bodies* ; Humans ; Lithium ; Numismatics ; Stomach*

PURPOSE: This study was performed to evaluate the quality of sleep in snoring obese children without obstructive sleep apnea (OSA); and to study the possible relationship between sleep interruption and gastroesophageal reflux (GER) in snoring obese children. METHODS: Study subjects included 13 snoring obese children who were referred to our sleep lab for possible sleep-disordered breathing. Patients underwent multichannel intraluminal impedance and esophageal pH monitoring with simultaneous polysomnography. Exclusion criteria included history of fundoplication, cystic fibrosis, and infants under the age of 2 years. Significant association between arousals and awakenings with previous reflux were defined by symptom-association probability using 2-minute intervals. RESULTS: Sleep efficiency ranged from 67-97% (median 81%). A total of 111 reflux episodes (90% acidic) were detected during sleep, but there were more episodes per hour during awake periods after sleep onset than during sleep (median 2.3 vs. 0.6, p=0.04). There were 279 total awakenings during the sleep study; 56 (20.1%) of them in 9 patients (69.2%) were preceded by reflux episodes (55 acid, 1 non-acid). In 5 patients (38.5%), awakenings were significantly associated with reflux. CONCLUSION: The data suggest that acid GER causes sleep interruptions in obese children who have symptoms of snoring or restless sleep and without evidence of OSA.
Arousal ; Child* ; Cystic Fibrosis ; Electric Impedance ; Esophageal pH Monitoring ; Fundoplication ; Gastroesophageal Reflux* ; Humans ; Infant ; Polysomnography ; Sleep Apnea Syndromes ; Sleep Apnea, Obstructive ; Snoring*