Benign recurrent intrahepatic cholestasis (BRIC) is a rare autosomal recessive inherited disorder characterized by multiple recurrent episodes of severe cholestatic jaundice without obstruction of extrahepatic bile duct. We present the case of a 7-year-old boy with BRIC confirmed by mutation analysis in the ATP8B1 gene and typical clinical manifestation. Despite inheritance of BRIC, we detected a mutation on only one allele. To our knowledge, this is the first report of BRIC with a confirmed single heterozygote novel mutation in the ATP8B1 gene in Korea.
Alleles ; Bile Ducts, Extrahepatic ; Child ; Cholestasis, Intrahepatic ; Heterozygote ; Humans ; Jaundice, Obstructive ; Korea ; Wills

Down syndrome is a rare cause of neonatal cholestasis. Neonatal cholestasis in a patient with Down syndrome is usually associated with severe liver diseases, such as neonatal hemochromatosis, myeloproliferative disorder and intrahepatic bile duct paucity. We experienced a case of idiopathic neonatal cholestasis in a patient with Down syndrome, which resolved spontaneously.
Bile Ducts, Intrahepatic ; Cholestasis ; Down Syndrome ; Hemochromatosis ; Humans ; Infant, Newborn ; Liver Diseases ; Myeloproliferative Disorders

Toxic hepatitis is a rare but devastating disease in children. Herbs are widely used in oriental medicine to treat various symptoms in Korea, however, several herbs have been reported to induce liver injury. We report a case of toxic hepatitis induced by Hovenia dulcis in a 3-year-old boy. He complained of nausea, abdominal discomfort, and jaundice. The patient had consumed water boiled with hovenia dulcis for about 1 year prior to presentation. A diagnosis of toxic hepatitis was made based on his history, laboratory data, viral markers, ultrasonography, and biopsied liver tissue. We administered supportive management for acute fulminant hepatitis but his symptoms and liver function progressed. He was transferred to another hospital for further evaluation and consideration for liver transplantation. Because acute liver failure due to herbs or dietary supplement taken for a long time is often fetal, it is important to make early diagnosis and stop taking the drug as soon as drug induced liver injury is suspected.
Biomarkers ; Child ; Dietary Supplements ; Drug-Induced Liver Injury ; Early Diagnosis ; Hepatitis ; Humans ; Jaundice ; Korea ; Liver ; Liver Failure, Acute ; Liver Transplantation ; Medicine, East Asian Traditional ; Nausea ; Preschool Child ; Water

Gastric ulcers are rare in children and are typically seen in cases of Helicobacter pylori (H. pylori) infection, non-steroidal anti-inflammatory drugs (NSAIDs) use, and critical illnesses such as sepsis. The risk of a bleeding ulcer due to use of NSAIDs is dependent on the dose, duration, and the individual NSAIDs, but the bleeding may occur soon after the initiation of NSAID therapy. An experience is described of a 16-month-old infant with a bleeding gastric ulcer after taking the usual dosage of ibuprofen for 3 days. The infant was also successfully treated with endoscopic hemostasis. Even a small amount of ibuprofen may be associated with bleeding gastric ulcers in infant.
Anti-Inflammatory Agents, Non-Steroidal ; Child ; Critical Illness ; Helicobacter pylori ; Hemorrhage ; Hemostasis, Endoscopic ; Humans ; Ibuprofen ; Infant ; Sepsis ; Stomach Ulcer ; Ulcer

Esophageal hiatal hernia is the hernia of a part of or the whole of stomach to posterior mediastinum through esophageal hiatus. Esophageal hiatal hernia can be classified as sliding hiatal hernia (type I), paraesophageal (type II), combined sliding and paraesophageal (type III), and complex paraesophageal (type IV). Type III and IV are clinically classified as paraesophageal hernia. The authors by chance found cystic mass filled with air in the lower lobe of the right lung during the treatment of mycoplasma pneumonia of 10 month-old patient. It was found to be paraesophageal hernia on the chest computed tomography and treated with the operation. As complex paraesophageal hernia is not usual among infants, the authors report it here with literature review.
Hernia ; Hernia, Hiatal ; Humans ; Infant ; Lung ; Mediastinum ; Mycoplasma ; Pneumonia ; Pneumonia, Mycoplasma ; Stomach ; Thorax

PURPOSE: The aims of this study was to compare and evaluate the clinical characteristics, laboratory data, and prognosis for infants under age 1 year with CMV hepatitis and those with viral hepatitis of unknown etiology. METHODS: A retrospective study was conducted of infants under age 1 year who were admitted with acute hepatitis. The exclusion criteria consisted of: autoimmune, genetic, metabolic, toxic, HAV, HBV, HCV, toxoplasma, rubella, herpes simplex, and Epstein-Barr virus. The 30 patients included were divided into two groups based on markers for CMV (IgM anti-CMV, CMV PCR in urine, CMV culture in urine). RESULTS: The median age of patients (n=15) was 2.8 months. No other organ involvement was detected in any patient. Peak serum total bilirubin levels (n=4) ranged from 2.6 to 6.7 mg/dL. Peak serum ALT levels ranged from 51 to 1,581 IU/L. The duration of ALT elevation ranged from 1.5 weeks to 26 weeks (median 9 weeks). All had recovered in full without ganciclovir; there were no cases of hearing loss. The median age of controls (n=15) was 2.5 months. Peak serum total bilirubin levels (n=4) ranged from 1.6 to 9.1 mg/dL. Peak serum ALT levels ranged from 26 to 1,794 IU/L. No significant differences were observed between both groups regarding the peak serum ALT levels, peak serum total bilirubin levels, duration of hyperbilirubinemia and ALT elevation. CONCLUSION: Although it was not possible to differentiate congenital infection with perinatal infection in this study, the prognosis of patients with CMV hepatitis without other organ involvement was good without ganciclovir treatment.
Bilirubin ; Cytomegalovirus ; Cytomegalovirus Infections ; Ganciclovir ; Hearing Loss ; Hepatitis ; Herpes Simplex ; Herpesvirus 4, Human ; Humans ; Hyperbilirubinemia ; Infant ; Methylmethacrylates ; Polymerase Chain Reaction ; Polystyrenes ; Prognosis ; Retrospective Studies ; Rubella ; Toxoplasma

PURPOSE: Commercial enzyme-linked immunosorbent assay (ELISA) kits have been considered less reliable for children than for adults. The aim of this study was to compare four ELISA kits and in-house immunoblotting based on the analysis of anti-H. pylori-IgG antibody reactivity. METHODS: A total of 399 serum samples were collected at the GNU Hospital during 1998-1999. All sera were tested using ELISA and immunoblotting. Statistically significant differences were determined by the chi2 test. RESULTS: The overall seropositivity rates using GAP IgG, Genedia IgG, HM-CAP, Pyloriset EIA-G, and immunoblotting were 13.0%, 25.1%, 18.3%, 15.8%, and 62.9%, respectively. Immunoblotting showed a higher seropositivity rate than did all four ELISA kits in all age groups. Genedia IgG had the highest seropositivity among the ELISA kits. The seropositivity rate for children aged 13 to 18 months was lowest, and that of children aged 15 years was highest (90.0%). The seropositivity rate for children aged 7 months to 5 years was significantly lower than that for children aged 6 to 15 years among the four ELISA kits (p<0.0001) and immunoblotting (p=0.02). CONCLUSION: Immunoblotting is the most sensitive test for detection of anti-Helicobacter pylori IgG antibodies among the serological tests in this study. These results emphasize the need for standardization when commercial ELISA tests are used in different nations or in young age groups. Immunoblotting could be a suitable noninvasive assay for serodiagnosis and seroepidemiologic study of H. pylori infection in Korean children.
Adult ; Aged ; Antibodies ; Child ; Enzyme-Linked Immunosorbent Assay ; Helicobacter ; Helicobacter pylori ; Humans ; Immunoblotting ; Immunoglobulin G ; Seroepidemiologic Studies ; Serologic Tests

Pediatric patients in hospital are at risk of malnutrition at admission and even during their hospitalization. Although the concept of nutritional support team (NST) was introduced to hospitals for optimal nutritional care since 1960s and the benefits of pediatric NST have been proven by many studies and reports in terms of patient clinical outcome and cost saving, the pediatric NST is not widespread yet. The pediatric NST composed of pediatricians, dieticians, pharmacist, and nutrition support nurses as core members dedicated to nutritional care in children should be independent of central NST or other disciplines, but closely cooperate with other teams in hospitals. There is no doubt that a multidisciplinary NST is an effective way to provide appropriate nutritional support to an individual patient. Therefore, the implementation of the pediatric NST in hospitals should be recommended to provide optimum nutritional support including enteral tube feeding and parenteral nutrition and to assess pediatric patients at risk of malnutrition.
Child ; Cost Savings ; Enteral Nutrition ; Hospitalization ; Humans ; Malnutrition ; Nutritional Support ; Parenteral Nutrition ; Pharmacists

With a markedly increased prevalence of obesity, non-alcoholic fatty liver disease (NAFLD) now becomes the most common cause of chronic liver disease in both adults and children. The etiology and pathogenesis of NAFLD are multifactorial and remain incompletely understood. According to the "two-hit" theory, inflammatory cytokines and adipokines are activated by oxidative stress and they are involved in insulin resistance, necroinflammatory steatohepatitis and fibrosis. This review discusses the latest updates on the role of some of important inflammatory adipokines and cytokines in the pathogenesis of NAFLD with an emphasis on their potential therapeutic implications.
Adipokines ; Adult ; Child ; Cytokines ; Fatty Liver ; Fibrosis ; Humans ; Inflammation ; Insulin Resistance ; Liver Diseases ; Obesity ; Oxidative Stress ; Prevalence

Development of antiviral resistance to lamivudine is the most important factor for the treatment failure. It is necessary to establish proper guidelines to overcome drug resistance for children with chronic hepatitis B. Primary treatment with lamivudine should be considered if patients are in immune-clearance phase and have persistently elevated ALT levels more than twice the upper limit of normal value. Before initiating the therapy, careful consideration of the patient's status is required to exclude abnormal liver function tests due to other causes. The treatment option should be carefully decided to suppress the viral replication effectively. To obtain good compliance, clinicians should educate patients and their parents. Appropriate monitoring for virologic breakthrough and genotypic resistance is important in deciding to change the treatment plan. Sequential monotherapy should be avoided and a combination of drugs in other categories is recommended. New antiviral agents, such as entecavir and tenofovir, which have high potency and high genetic barrier, are soon expected to be available for use with children.
Adenine ; Antiviral Agents ; Child ; Compliance ; Drug Resistance ; Guanine ; Hepatitis B, Chronic ; Hepatitis, Chronic ; Humans ; Lamivudine ; Liver Function Tests ; Organophosphonates ; Parents ; Reference Values ; Treatment Failure ; Tenofovir