Acute acalculous cholecystitis (AAC) is an inflammation of the gallbladder in the absence of demonstrated stones. AAC is frequently associated with severe systemic inflammation. However, the exact etiology and pathogenesis of AAC still remain unclear. Acute infection with Epstein Barr virus (EBV) in childhood is usually aymptomatic, whereas it often presents as typical infectious mononucleosis symptoms such as fever, cervical lymphadenopathy, and hepatosplenomegaly. AAC may occur during the course of acute EBV infection, which is rarely encountered in the pediatric population. AAC complicating the course of a primary EBV infection is usually associated with a favorable outcome. Most of the patients recover without any surgical treatment. Therefore, the detection of EBV in AAC would be important for prediction of better prognosis. We describe the case of a 10-year-old child who presented with AAC during the course of primary EBV infection, the first in Korea, and review the relevant literature.
Acalculous Cholecystitis* ; Child ; Epstein-Barr Virus Infections ; Fever ; Gallbladder ; Herpesvirus 4, Human* ; Humans ; Infectious Mononucleosis ; Inflammation ; Korea ; Lymphatic Diseases ; Prognosis

We present a case of a 13-year-old boy with Gorham's disease involving the thoracic and lumbar spine, femur, and gastrointestinal (GI) tract, which was complicated by recurrent chylothorax and GI bleeding. The presenting symptoms were intermittent abdominal pain, back pain, and melena. Esophagogastroduodenoscopy and colonoscopy showed no abnormal lesions, but duodenal biopsy showed marked dilation of the lymphatics in the mucosa and submucosa, which revealed positive staining with a D2-40 immunohistochemical marker. In cases of GI bleeding with osteolysis, the expression of a D2-40 marker in the lymphatic endothelium of the GI tract may help to diagnose GI involvement in Gorham's disease. To the best of our knowledge, this is the first case report to pathologically demonstrate intestinal lymphatic malformation as a cause of GI bleeding in Gorham's disease.
Abdominal Pain ; Adolescent ; Back Pain ; Biopsy ; Child ; Chylothorax ; Colonoscopy ; Duodenum* ; Endoscopy, Digestive System ; Endothelium, Lymphatic ; Femur ; Gastrointestinal Tract* ; Hemorrhage ; Humans ; Male ; Melena ; Mucous Membrane ; Occult Blood ; Osteolysis ; Osteolysis, Essential ; Spine

PURPOSE: Neonatal late-onset hypocalcemia is defined as hypocalcemia developed after postnatal 3 days and associated with hypoparathyroidism, high phosphate diets and vitamin D deficiency. We experienced the increment of neonatal late onset hypocalcemia over 1 year. We tried to evaluate the relationship between late onset hypocalcemia and maternal hypovitaminosis D. METHODS: The medical records in the neonates with late-onset hypocalcemia during January 2007 to July 2008 were retrospectively reviewed. Among those patients, 17 paired sera of mothers and neonates had collected. The levels of 25-OH vitamin D (25OHD) and intact parathyroid hormone (iPTH) were measured and were compared with neonate and the mother. RESULTS: The mean gestational age was 38+1 weeks, and the mean body weight was 2,980 g. The onset time of hypocalcemia was 5.9 days of age. Most of them (88.2%) were feeding with formula and no one was only breast milk feeding. Of the 17 patients, 13 were born in spring or in winter. The median levels of calcium, phosphorus, alkaline phosphatase, iPTH and 25OHD were 7.0 mg/dL, 8.6 mg/dL, 191.0 U/L, 57.2 pg/mL and 24.0 ng/mL in neonates. The levels of 25OHD of 6 neonates were <20 ng/mL. A total of 16 mothers were considered vitamin D-deficient (<20 ng/mL), and vitamin D insufficient (20<25OHD<30 ng/mL). CONCLUSION: Neonatal late-onset hypocalcemia in our study seems to be influenced by maternal vitamin D deficiency and insufficiency. Sun tanning and vitamin D supplements from winter to spring would be helpful to prevent maternal vitamin D deficiency, one of the causes of neonatal late-onset hypocalcemia.
Alkaline Phosphatase ; Body Weight ; Calcium ; Diet ; Gestational Age ; Humans ; Hypocalcemia* ; Hypoparathyroidism ; Infant, Newborn ; Medical Records ; Milk, Human ; Mothers ; Parathyroid Hormone ; Phosphorus ; Retrospective Studies ; Solar System ; Tanning ; Triacetoneamine-N-Oxyl ; Vitamin D ; Vitamin D Deficiency ; Vitamins

PURPOSE: The spontaneous seroconversion rate of hepatitis B e antigen (HBeAg)-positive chronic hepatitis B (CHB) virus infection in children is lower than that in adults. However, few studies have investigated the rate of transition from the immune-tolerant to the early immune-clearance phase in children. METHODS: From February 2000 to August 2011, we enrolled 133 children aged <18 years who had visited the Department of Pediatrics, Kyungpook National University Hospital. All subjects were in the immune-tolerant phase of HBeAg-positive CHB virus infection. The estimated transition rate into the early immune-clearance phase was calculated using the Kaplan-Meier method. RESULTS: Among the 133 enrolled pediatric CHB virus infection patients in the HBeAg-positive immune-tolerant phase, only 21 children (15.8%) had converted to the early immune-clearance phase. The average age at entry into active hepatitis was 10.6+/-4.8 years. The incidence of transition from the immune-tolerant to the early immune-clearance phase in these children was 1.7 episodes/100 patient-years. When analyzed by age, the estimated transition rate was 4.6%, 7.1%, and 28.0% for patients aged <6, 6-12, >12 years, respectively. CONCLUSION: In children with CHB virus infection, the estimated rate of entry into the early immune-clearance phase was 28.0% for patients aged 12-18 years, which was significantly higher than that observed for children aged <12 years (11.7%; p=0.001).
Adult ; Child* ; Gyeongsangbuk-do ; Hepatitis ; Hepatitis B ; Hepatitis B virus ; Hepatitis B, Chronic* ; Hepatitis, Chronic* ; Humans ; Incidence ; Natural History ; Pediatrics ; Phase Transition

PURPOSE: Crigler-Najjar syndrome type II (CN-2) is characterized by moderate non-hemolytic unconjugated hyperbilirubinemia as a result of severe deficiency of bilirubin uridine diphosphate-glucuronosyltransferase (UGT1A1). The study investigated the mutation spectrum of UGT1A1 gene in Korean children with CN-2. METHODS: Five Korean CN-2 patients from five unrelated families and 50 healthy controls were enrolled. All five exons and flanking introns of the UGT1A1 gene were amplified by polymerase chain reaction (PCR) and the PCR products were directly sequenced. RESULTS: All children initially presented with neonatal jaundice and had persistent indirect hyperbilirubinemia. Homozygous p.Y486D was identified in all five patients. Three patients had an associated homozygous p.G71R and two a heterozygous p.G71R. The allele frequency of p.Y486D and p.G71R in healthy controls was 0 and 0.16, respectively. No significant difference in mean serum bilirubin levels was found between homozygous carriers of p.G71R and heterozygous carriers. CONCLUSION: The combination of homozygous p.Y486D and homozygous or heterozygous p.G71R is identified. The p.Y486D and p.G71R can be screened for the mutation analysis of UGT1A1 in Korean CN-2 patients.
Bilirubin ; Child ; Crigler-Najjar Syndrome* ; Exons ; Gene Frequency ; Humans ; Hyperbilirubinemia ; Infant, Newborn ; Introns ; Jaundice, Neonatal ; Polymerase Chain Reaction ; Uridine

PURPOSE: Infliximab (IFX) is considered safe and effective for the treatment of ulcerative colitis (UC) in both adults and children. The aim of this study was to evaluate the short- and long-term clinical course of IFX in Korean children with UC. METHODS: Pediatric patients with UC who had received IFX infusions between November 2007 and May 2013 at Samsung Medical Center were retrospectively investigated. The clinical efficacy of IFX treatment was evaluated at 8 weeks (short term) and 54 weeks (long term) after the initiation of IFX treatment using the Pediatric Ulcerative Colitis Activity Index (PUCAI). The degree of response to IFX treatment was defined as complete response (PUCAI score=0), partial response (decrement of PUCAI score> or =20 points), and non-response (decrement of PUCAI score <20 points). Adverse events associated with IFX treatment were also investigated. RESULTS: Eleven pediatric patients with moderate to severe UC had received IFX. The remission rate after IFX treatment was 46% (5/11) and 82% (9/11) at 8 weeks and 54 weeks after IFX treatment, respectively. All patients who were steroid-dependent before treatment with IFX achieved remission at 54 weeks and were able to stop treatment with corticosteroids, while all steroid-refractory patients failed to achieve remission at 54 weeks after treatment with IFX. CONCLUSION: Response to IFX treatment after 8 weeks may predict a favorable long-term response to IFX treatment in Korean pediatric UC patients.
Adrenal Cortex Hormones ; Adult ; Child ; Colitis, Ulcerative* ; Humans ; Inflammatory Bowel Diseases ; Korea ; Retrospective Studies ; Ulcer* ; Infliximab

Manometry is a noninvasive diagnostic tool for identifying motility dysfunction of the gastrointestinal tract. Despite the great technical advances in monitoring motility, performance of the study in pediatric patients has several limitations that should be considered during the procedure and interpretation of the test results. This article reviews the clinical applications of conventional esophageal and anorectal manometries in children by describing a technique for performing the test. This review will develop the uniformity required for the methods of performance, the parameters for measurement, and interpretation of test results that could be applied in pediatric clinical practice.
Child* ; Diagnosis ; Esophageal Motility Disorders ; Gastrointestinal Motility ; Gastrointestinal Tract ; Hirschsprung Disease ; Humans ; Manometry*

Esophageal pH monitoring is considered the gold standard for the diagnosis of gastroesophageal reflux disease because of the normal ranges across the pediatric age range. However, this method can only detect acid reflux. Multichannel intraluminal impedance-pH (MII-pH) monitoring has recently been used for the detection of bolus reflux in infants and children. This method allows for the detection of liquid, gas or mixed reflux in addition to acid, weakly acidic or weakly alkaline reflux. MII-pH monitoring can record the direction of flow and the height of reflux, which are useful parameters to identify an association between symptoms and reflux. However, the technique is limited by its high cost and the lack of normative data of MII-pH in the pediatric population. Despite certain limitations, MII-pH monitoring will become more common and gradually replace pH monitoring in the future, because pH monitoring is part of MII-pH.
Child* ; Diagnosis ; Esophageal pH Monitoring ; Gastroesophageal Reflux ; Humans ; Hydrogen-Ion Concentration* ; Infant ; Reference Values

The original version of this article, the second author name was erroneously misspelled.

Meckel diverticulum (MD) is one of the most common congenital gastrointestinal anomalies and occurs in 1.2-2% of the general population. MD usually presents with massive painless rectal bleeding, intestinal obstruction or inflammation in children and adults. Suppurative Meckel diverticulitis is uncommon in children. An experience is described of a 3-year-old girl with suppurative inflammation in a tip of MD. She complained of acute colicky abdominal pain, vomiting and periumbilical erythema. Laparoscopic surgery found a relatively long MD with necrotic and fluid-filled cystic end, which was attatched to abdominal wall caused by inflammation. Herein, we report an interesting and unusual case of a suppurative Meckel diverticulitis presenting as periumbilical cellulitis in a child. Because of its varied presentations, MD might always be considered as one of the differential diagonosis.
Abdominal Pain ; Abdominal Wall ; Adult ; Cellulitis* ; Child ; Child, Preschool* ; Diverticulitis ; Erythema ; Female ; Hemorrhage ; Humans ; Inflammation ; Intestinal Obstruction ; Laparoscopy ; Meckel Diverticulum ; Suppuration ; Vomiting