BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease. Despite of proper treatment and improving treatment regimens, HLH patients still show a fatal prognosis. Therefore the evaluation of prognostic factor is important and there are many studies about hyperbilirubinemia as a prognostic factor in HLH. So we studied the prognostic value of hyperbilirubinemia in HLH children. METHODS: A retrospective analysis was performed about 33 patients who were diagnosed with HLH at Pusan National University Hospital and Yangsan Pusan University Hospital between January 2000 to December 2012. We reviewed the clinical characteristics, laboratory findings, and results of treatment to identify hyperbilirubinemia as a prognostic factor in HLH patients. RESULTS: The median age of patients at diagnosis was 32 months. Most of patients presented with fever, pale appearance, abdominal pain and jaundice. Forty-eight point five percentage of patients showed normal serum bilirubiln level (<2.0 mg/dL) and 51.5% showed hyperbilirubinemia (> or =2.0 mg/dL). In normal serum bilirubin group, 1 patient (6.3%) was relapsed and 1 patient (5.9%) was relapsed in hyperbilirubinemia group. In the hyperbilirubinemia group, the mortality was higher than the normal bilirubin group but, there was no statistical significance. CONCLUSION: As a prognostic factor serum bilirubin at diagnosis in HLH patients, there was no significant correlation between hyperbilirubinemia and poor outcome. But, our study has a limitation that the number of patients is too small and almost showed good prognosis.
Abdominal Pain ; Bilirubin* ; Busan ; Child ; Diagnosis ; Fever ; Fibrinogen ; Gyeongsangnam-do ; Humans ; Hyperbilirubinemia ; Jaundice ; Lymphohistiocytosis, Hemophagocytic* ; Mortality ; Prognosis ; Retrospective Studies

Diffusion tensor imaging (DTI) has a diagnostic value in cervical myelopathy. The usefulness of diffusion metrics as a potential prognostic biomarker was assessed in this review. Various diffusion metrics, such as fractional anisotropy, mean diffusivity, apparent diffusion coefficient, and radial diffusivity, were significant in correlation with functional recovery after surgery. Changes in diffusion metrics not only at the compression level, but also above and below the compression level were confirmed. However, it was difficult to confirm the results owing to the diversity of studies. Further efforts to make a consensus on spinal cord DTI are needed.
Anisotropy ; Consensus ; Diffusion Tensor Imaging ; Diffusion ; Recovery of Function ; Spinal Cord ; Spinal Cord Diseases

Cantrell's syndrome is a rare congenital anomaly, consists of five malformations. There are the midline abdominal wall defect, the defect of sterna lower part, an agenesis of the anterior part of the diaphragm, an absence of the diaphragmatic pericardium, and the congenital intracardiac problem. We diagnosed Cantrell's syndrome with left ventricular diverticulum in a female neonate with no perinatal problems. She experienced an one-staged operation and discharged with no complications. We report a case with brief review of the literature.
Abdominal Wall ; Diaphragm ; Diverticulum* ; Female ; Humans ; Infant, Newborn* ; Pericardium

Fetal alcohol syndrome is characterized by distinctive facial appearance, prenatal onset growth deficiency, an increased frequency of development and mental retardation, cardiac anomaly and genitourinary anomaly. Complete abstinence during pregnancy is recommended, since alcohol consumption in each trimester has been associated with abnormalities. We experienced a case of fetal alcohol syndrome in a 11-month-old female infant. Her mother had drunk as much as 500cc of alcohol every day from 1st trimester to 3rd trimester of pregnancy. The child was small for gestational age, other distinctive features were microcephaly, thin upper lip, short palpebral fissure, hypertelorism, low nasal bridge, hypoplasia of philtrum, dysplastic ear, developmental and mental retardation, mild pulmonary stenosis and ovarian cysts. On the basis of maternal history and clinical features mentioned above, we diagnosed the case as fetal alcohol syndrome.
Alcohol Drinking ; Child ; Ear ; Female ; Fetal Alcohol Spectrum Disorders* ; Gestational Age ; Humans ; Hypertelorism ; Infant ; Intellectual Disability ; Lip ; Microcephaly ; Mothers ; Ovarian Cysts ; Pregnancy ; Pulmonary Valve Stenosis

Fetal alcohol syndrome is characterized by distinctive facial appearance, prenatal onset growth deficiency, an increased frequency of development and mental retardation, cardiac anomaly and genitourinary anomaly. Complete abstinence during pregnancy is recommended, since alcohol consumption in each trimester has been associated with abnormalities. We experienced a case of fetal alcohol syndrome in a 11-month-old female infant. Her mother had drunk as much as 500cc of alcohol every day from 1st trimester to 3rd trimester of pregnancy. The child was small for gestational age, other distinctive features were microcephaly, thin upper lip, short palpebral fissure, hypertelorism, low nasal bridge, hypoplasia of philtrum, dysplastic ear, developmental and mental retardation, mild pulmonary stenosis and ovarian cysts. On the basis of maternal history and clinical features mentioned above, we diagnosed the case as fetal alcohol syndrome.
Alcohol Drinking ; Child ; Ear ; Female ; Fetal Alcohol Spectrum Disorders* ; Gestational Age ; Humans ; Hypertelorism ; Infant ; Intellectual Disability ; Lip ; Microcephaly ; Mothers ; Ovarian Cysts ; Pregnancy ; Pulmonary Valve Stenosis

Acute vanishing bile duct syndrome, a rare but rapidly progressive destruction of the intrahepatic bile ducts with unknown pathogenesis, is most often a drug- or toxin-related. Toxic epidermal necrolysis is a serious dermatologic condition and a potentially life threatening disease, which is drug or infection induced. Ibuprofen associated acute vanishing bile duct syndrome and toxic epidermal necrolysis have not been reported previously in infants. We report a 7-month-old infant with ibuprofen associated toxic epidermal necrolysis, followed by severe and rapidly progressive vanishing bile duct syndrome. She recovered totally with supportive care.
Female ; Humans ; Ibuprofen/*adverse effects ; Infant ; Stevens-Johnson Syndrome/*diagnosis/*etiology

PURPOSE: Gastroesophageal reflux in infant is a physiological process. However, surgery is performed in high risk infants with severe gastroesophageal reflux disease (GERD) when medical management fails. This study focuses on efficacy and safety of Nissen fundoplication for GERD in infants under age 12 months. METHODS: This study was a retrospective case analysis of 11 neonates and infants under 12 months of age who underwent Nissen fundoplication following a failure of medical treatment between June 2010 and June 2013 at Pusan National University Children's Hospital. The records were reviewed to determine the effect of fundoplication on symptoms and post-operative complications. RESULTS: A total of 11 infants consist of four males and seven females. Mean birth weight was 2,305.5+/-558.6 g (1,390-3,130 g). They had some underlying disease, which are not related with GERD such as congenital heart disease (54.5%), prematurity (45.5%), neurologic disease (18.2%), respiratory disease (18.2%), and other gastrointestinal disease. Mean body weight at surgery was 3,803.6+/-1,864.9 g (1,938.7-5,668.5 g). Mean age at operation was 99.9+/-107.6 days (17-276 days). Duration from operation to full enteral feeding was 10.9 days. Symptoms related GERD disappeared in all patients including one who got reoperation. One infant died of congenital heart disease unrelated to surgery. There were no complications related to fundoplication. CONCLUSION: Fundoplication is effective and safe treatment in the neonates and infants with severe GERD.
Birth Weight ; Body Weight ; Busan ; Enteral Nutrition ; Female ; Fundoplication* ; Gastroesophageal Reflux* ; Gastrointestinal Diseases ; Heart Defects, Congenital ; Humans ; Infant* ; Infant, Newborn* ; Male ; Physiological Processes ; Reoperation ; Retrospective Studies

We report a case of septic arthritis due to H. influenzae associated with atypical Kawasaki disease. A 2-year-old boy was admitted to our orthopedic department due to limping gait of the right leg. CT finding of right hip joint revealed fluid collection. He was diagnosed as transient synovitis and septic arthritis. He was treated with antibiotics, but no incision nor drainage was performed. During the antibiotics therapy, limping gait subsided, but high fever, skin rash and lip fissure developed. Echocardiographic finding showed both coronary artery aneurysms. We diagnosed the boy's condition as atypical Kawasaki disease, and treated it with intravenous immunoglobulin(IVIG) and aspirin. Even, with the use of IVIG, fever was persistent. A second IVIG was tried, but fever did not subside. An MRI of right hip joint was followed up. The finding was consistent with septic arthritis associated with osteomyelitis of the right femur head. An incision and drainage was performed on the right hip joint, and the pus culture revealed beta-lactamase negative H. influenzae.
Aneurysm ; Anti-Bacterial Agents ; Arthritis, Infectious* ; Aspirin ; beta-Lactamases ; Child, Preschool ; Coronary Vessels ; Drainage ; Echocardiography ; Exanthema ; Femur Head ; Fever ; Gait ; Haemophilus influenzae* ; Haemophilus* ; Hip Joint ; Humans ; Immunoglobulins, Intravenous ; Influenza, Human ; Leg ; Lip ; Magnetic Resonance Imaging ; Male ; Mucocutaneous Lymph Node Syndrome* ; Orthopedics ; Osteomyelitis ; Suppuration ; Synovitis

PURPOSE: This study aimed to determine the prevalence of immediate-type food allergy (FA) among schoolchildren in Korea. METHODS: A nationwide, cross-sectional study was performed in September 2015. A stratified random sample of 50,000 participants was selected from children and adolescents aged 6-7 years (n=17,500), 9-10 years (n=17,500), 12-13 years (n=7,500), and 15-16 years (n=7,500). Parents were asked to complete a questionnaire on the detailed history of immediate-type FA. RESULTS: A total of 32,001 (64.0%) responded to the questionnaire survey, and 29,842 children (59.7%) were analyzed after adjusting for missing data. The number of the cases in each age group was 9,671 (6-7 years), 9,756 (9-10 years), 5,169 (12-13 years), and 5,246 (15-16 years). The prevalence of lifetime perceived FA was 15.82%. The prevalence of current immediate-type FA was 4.06% in total, with 3.15% in 6- to 7-year-olds, 4.51% in 9- to 10-year-olds, 4.01% in 12- to 13-year-olds, and 4.49% in 15- to 16-year-olds. Among individual food items, peanut (0.22%) was the most frequent causative food, followed by hen's egg (0.21%), cow's milk (0.18%), and buckwheat (0.13%). Among the food groups, fruits (1.41%), crustaceans (0.84%), tree nuts (0.32%), and fish (0.32%) were the most common offending foods. The prevalence of food-induced anaphylaxis was 0.97%. The most frequent causative food of anaphylaxis was peanut (0.08%), followed by cow's milk (0.07%), buckwheat (0.06%), and hen's egg (0.06%), while fruits (0.28%), crustaceans (0.18%), tree nuts (0.12%), and fish (0.09%) were the most commonly responsible food groups. CONCLUSIONS: The prevalence of current immediate-type FA and food-induced anaphylaxis in Korean schoolchildren in 2015 was 4.06% and 0.97%, respectively. Peanuts, cow's milk, hen's egg, fruits, crustaceans, and tree nuts are common allergens.
Adolescent ; Allergens ; Anaphylaxis ; Arachis ; Child ; Cross-Sectional Studies ; Fagopyrum ; Food Hypersensitivity* ; Fruit ; Humans ; Korea ; Milk ; Nuts ; Ovum ; Parents ; Prevalence* ; Trees

PURPOSE: The aim of this study was to evaluate the details of the clinical characteristics and food exposure types at the first symptom onset in immediate-type cow's milk allergy (CMA) and egg white allergy (EWA) in Korean children. METHODS: This study included children with immediate-type CMA (n=288) or EWA (n=233) with symptom onset time of 2 hours or less, who visited Samsung Medical Center, Ajou University Hospital, and Soonchunhyang University Seoul Hospital between September 2014 and August 2015. The details of clinical features and food exposure types at the first symptom onset were evaluated by retrospective medical record review using a standardized case report form. RESULTS: The median ages of first symptom onset were 10 months in CMA and 12 months in EWA. The most common types of exposure at the first symptom in CMA were formula milk (29.5%) and milk (29.5%), followed by cheese (17.7%) and yogurt (14.2%). The most common type of exposure in EWA was boiled eggs (35.6%), followed by rice/porridge/soup containing eggs (27.5%), pan-fried eggs (17.6%), and baked goods (9.9%). Cutaneous symptoms were most common in both CMA and EWA, and anaphylaxis was noticed in 36.1% and 30.3%, respectively. Baked goods containing milk or eggs also induced anaphylaxis. The symptom onset time was less than 30 minutes in the majority of patients and the most common place of occurrence was home in both CMA and EWA. CONCLUSION: This study provides comprehensive information on CMA and EWA, and therefore helps clinicians diagnose and guide appropriate food restriction in children with CMA and EWA.
Anaphylaxis ; Cheese ; Child* ; Egg Hypersensitivity ; Egg White* ; Eggs ; Humans ; Hypersensitivity* ; Hypersensitivity, Immediate ; Medical Records ; Milk Hypersensitivity ; Milk* ; Ovum* ; Retrospective Studies ; Seoul ; Yogurt