PURPOSE: A number of drugs are capable of changing body weight as a side effect. A number of neurotransmitter systems acting in several hypothalamic nuclei are pivotal to the storage regulation of body fat. Leptin is a protein encoded by the ob gene that is expressed in adipocyte. It regulates eating behavior by activating the action to the satiety center in the hypothalmus. The purpose of this study is to investigate the changes of serum leptin in children with epilepsy. METHODS: Serum leptin levels were measured in 43 epileptic patients (30 males, 13 females) receiving valproate or carbamazepine by radioimmunoassay. Thirty patients (19 males, 11 females, 10.0+/-5.0 years of age) were treated with valproate (VPA group) and 13 patients (11 males, 2 females, 9.1+/-4.0 years of age) were treated with carbamazepine (CBZ group). Obesity index and body mass index were calculated before and during anticonvulsant medications. RESULTS: 1) Body mass indices were significantly increased after VPA or CBZ medication; from 17.24+/-2.74 to 18.47+/-2.60 in VPA group, from 16.77+/-1.69 to 17.43+/-3.01 in CBZ group. 2) Obesity indices were increased without statistical significance after medication in both group. 3) Serum leptin levels were significantly higher in VPA group (4.54+/-4.77ng/ml) than CBZ group (2.47+/-2.26ng/ml). CONCLUSION: Body weight gain after VPA medication in susceptible individual might be related to a certain mechanism that elevates serum leptin level.
Adipocytes ; Adipose Tissue ; Body Mass Index ; Body Weight ; Carbamazepine ; Child* ; Epilepsy ; Feeding Behavior ; Female ; Humans ; Leptin* ; Male ; Neurotransmitter Agents ; Obesity ; Radioimmunoassay ; Valproic Acid ; Weight Gain

Kabuki make-up syndrome (KMS) was firstly reported in 1981 by Niikawa, et al. and Kuroki et al. in a total of ten unrelated Japanese children with characteristic array of multiple congenital anomalies and mental retardation. The name reflects the resemblance between the facial features of patients and the actors of Kabuki, one of the most famous traditional performing arts in Japan. The syndrome is characterized by mental and developmental retardations and peculiar facial features including long palpebral fissures with eversion of the lateral portion of lower eyelid and arching of eyebrows. In addition, dermatoglyphic and skeletal abnormalities are commonly associated. In Japan, the syndrome appears to have an incidence of about 1 : 32,000 newborns. Outside of Japan, a growing number of patients have been recognized. However, this syndrome has been reported only a few cases in Korea. We report a boy diagnosed by clinical features with a brief review of the literature.
Asian Continental Ancestry Group ; Child ; Dermatoglyphics ; Eyebrows ; Eyelids ; Humans ; Incidence ; Infant, Newborn ; Intellectual Disability ; Japan ; Korea ; Male

PURPOSE: To evaluate the efficacy of interferon alpha therapy with or without prednisolone in children with chronic hepatitis B. METHODS: Twenty-eight children (22 boys, 6 girls, mean age 130 months) had seropositive results for HBsAg, HBeAg and HBV DNA; 11 had chronic persistent hepatitis and 17 had chronic active hepatitis. The patients were divided into two groups depending upon their inflammatory activity on liver biopsy, pretreatment serum ALT levels and HBV DNA levels. Fourteen children (group 1: chronic active hepatitis, ALT > or = 100 IU/L and HBV DNA < or = 100 pg/300 microliter) received interferon alpha 2a 5 MU/m2 of body surface three times weekly for 6 months. Fourteen children (group 2: chronic persistent hepatitis or chronic active hepatitis with ALT < 100 IU/L or HBV DNA > 100 pg/300 microliter) received prednisolone in decreasing daily doses of 60 mg/m2, 40 mg/m2, and 20 mg/m2, each for 2 weeks, followed after 2 weeks by interferon alpha 2a on the same schedule. At the end of therapy, 3 end points were analyzed: HBeAg seroconversion, serum ALT normalization rate and clearance of serum HBV DNA. RESULTS: At the end of treatment, HBe antigen-to antibody seroconversion was higher but not more significant in group 1 than group 2 (71.4% vs. 50.0%). Only one patient in group 2 who lost HBeAg, also cleared HBsAg. ALT normalization was similar in both groups (64.3% in group 1 vs. 55.6% in group 2). Clearance of serum HBV DNA was observed in 78.6% of patients in group 1 and 64.3% in group 2, but no significant differences. Complete response was similarly achieved in both groups (57.1% in group 1 vs. 50.0% in group 2). Interferon alpha therapy with prednisolone priming was well tolerated and all children finished therapy. CONCLUSION: The combined therapy with prednisolone followed by interferon alpha may be safe and effective in inducing a serological and biochemical remission of the disease in approximately 50% of children with chronic hepatitis B and with a high level of viral replication and less active disease. However, a controlled study should be performed to confirm these results.
Appointments and Schedules ; Biopsy ; Child* ; DNA ; Female ; Hepatitis B e Antigens ; Hepatitis B Surface Antigens ; Hepatitis B, Chronic* ; Hepatitis, Chronic* ; Humans ; Interferon-alpha* ; Interferons ; Liver ; Prednisolone*

PURPOSE: Dendropanax morifera Leveille(DML) exhibits diverse biological and pharmacological activities, including anti-oxidative effect, anti-cancer activity, hepatoprotection, immunological stimulation, and bone regeneration. As part of the identification for novel functions of DML, we investigated the therapeutic effects of DML on diabetes induced by streptozotocine (STZ) treatment. METHODS: First, the four extracts including the water extract of leaf (DLW), the ethanol extract of leaf (DLE), the water extract of stem (DSW), and the ethanol extract of stem (DSE) were collected from the leaf and stem of DML using a hot water and ethanol solvent. Alterations in body weight, glucose concentration, insulin level, and pancreatic islet structure were investigated in diabetic mice after treatment with extracts of DML for 2 weeks. RESULTS: Among four extracts, the highest level of total polyphenols and total flavonoids was detected in DLW, while the lowest level of these was measured in DSE. The radical scavenging activity was also higher in DLW than in the other three extracts at the concentration of 25-100 microg/mL, although this activity was maintained at a constant level in all groups at the concentration of 500 microg/mL. Based on the results of anti-oxidant activity, DLW and DLE were selected for examination of anti-diabetic effects in a diabetes model. Body weight was gradually decreased in all STZ treated groups compared with the No treated group. However, four STZ/DML treated groups maintained a high level of body weight during 7-14 days, while the STZ/vehicle treated group showed a gradual decrease of body weight during the same period. Also, a significant decrease or increase in the concentration of glucose and insulin in the blood of the diabetes model was detected in a subset of groups, although the highest increase was detected in the STZ/DLE-200 treated group. In addition, the histological structure of pancreatic islet was significantly recovered after treatment with DLW and DLE. CONCLUSION: These results suggest that DLW and DLE may contribute to attenuation of clinical symptoms of diabetes as well as prevent the destruction of pancreatic beta-cells in STZ-induced diabetes mice.
Animals ; Body Weight ; Bone Regeneration ; Ethanol* ; Flavonoids ; Glucose ; Immunization ; Insulin ; Islets of Langerhans ; Mice ; Polyphenols ; Streptozocin ; Water

PURPOSE: Fanconi anemia(FA) is a rare autosomal recessive disorder characterized by progressive bone marrow failure and congenital malformations. Patients with FA have aplastic anemia(> 90%), leukemia(10~15%), myelodysplasia(5%) and liver(5%) and other tumors(5%). In the International FA Registry study myelodysplasia in FA patients was detected at a median of 13 years. Presentation of FA with myelodysplasia in an infant should be extremely rare. CASE: A 3-month-old infant presented with anemia and poor feeding. The initial hemogram showed: hemoglobin, 4.6 g/dL; MCV, 104.1 fL/pg; white cell count, 4,300/microL; neutrophils, 450/microL; platelets, 23,000/microL. The bone marrow was normocellular, with findings of macrocytic anemia and dyserythropoiesis, and less than 5% of myeloid blasts, compatible with myelodysplastic syndrome(refractory anemia). The patient had multiple cafe-au-lait spots, hypopigmented nevi, broad nasal bridge, micrognathia, and thumb and toe anomalies. FA was confirmed by chromosomal hypersensitivity to diepoxybutane and mitomicin C. Supportive treatment with oxymetholone and prednisolone failed to improve hematologic and clinical findings. The patient succumbed to sepsis, pneumonia and meningitis due to Pseudomonas aeruginosa at 20 month of age. Clonal cytogenetic anomalies were not found. CONCLUSION: We reported here a rare case of FA presenting with myelodysplasia at the age of 3 month.
Anemia ; Anemia, Macrocytic ; Bone Marrow ; Cafe-au-Lait Spots ; Cell Count ; Cytogenetics ; Fanconi Anemia* ; Humans ; Hypersensitivity ; Infant* ; Meningitis ; Myelodysplastic Syndromes* ; Neutrophils ; Nevus ; Oxymetholone ; Pneumonia ; Prednisolone ; Pseudomonas aeruginosa ; Sepsis ; Thumb ; Toes

Urachal anomalies are very rare. The umbilicus and the structures which lead to its formation rarely cause trouble except in patients with an error. Embryologically, the urachus represents the vestigial remnant of the allantois which, in the fetus, communicates with the cloaca. The patent urachus results from failure of luminal closure of the urachus Herein a case of congenital patent urachus reported with a brief review of literatures.
Allantois ; Cloaca ; Fetus ; Humans ; Phenobarbital ; Umbilicus ; Urachus*

PURPOSE: Myelodysplastic syndrome (MDS) in children needs to be elucidated in terms of clinical characteristics, natural history, the most effective treatment and prognostic factors, as the disease is very rare and its definition and classification has not reached a consensus by many physician. This study was aimed to describe the characteristics and the disease courses of Korean children with MDS, and to analyze the usefulness of prognostic scoring systems in the prediction of transformation to acute myelogenous leukemia (AML) and overall survival among subgroups. MATERIALS AND METHODS: Fourteen children with MDS seen at Chonnam University Hospital and additional 59 patients identified by the review of Korean literature were evaluated to define clinical characteristics and disease courses. Kaplan-Meier (K-M) probability of leukemic transformation and overall survival were plotted. FAB subtypes, subgroups by Boumemouth Scoring System (BSS), and International Prognostic Scoring System (IPSS) risk groups were compared to predict transformation to AML and overall survival. RESULTS: The median age of 14 patients was 36.5 months. The sex ratio was 3.7:1 (M: F). The frequency of FAB subtypes in Korea was similar to that of other countries except for higher proportion of RA (37%). K-M 3-yr probability of AML transformation and survival for Korean patients were 54.7%, and 49.8%, respectively. Although FAB system, BMS and IPSS were all capable of discriminating subgroups in the prediction of AML transformation and survival, they did not reach the significant level possibly due to small number of patients assigned to each subgroup. CONCLUSION: The clinical characteristics of Korean children with MDS were not different from those of other countries. This study showed the high rate of AML transformation and poor survival in children with MDS.
Child ; Classification ; Consensus ; Humans ; Jeollanam-do ; Korea ; Leukemia, Myeloid, Acute ; Myelodysplastic Syndromes* ; Natural History ; Prognosis ; Sex Ratio

Patients with severe cerebral palsy (CP) are susceptible to bone fractures due to low bone mineral density, deformity from contracture and developmental disability. We experienced a 12-year-old spastic CP female who sustained recurrent fracture of the right femur. The first episode occurred when she had been moved passively by another child in residential care. She underwent operative treatment with plate fixation, but seventeen days after operation, spasticity of legs aggravated and refracture happened at the fixation site. She underwent re-operative fixation, but at three days after the second operation, she sustained another fracture at the top of the plate, for which she underwent the third operation. She was then referred to our department for scissoring patterns of the lower extremities. We performed bilateral obturator nerve block which relieved patient's spasticity. We think that her recurrent fracture may be related with ignored risk factor of refracture such as uncontrolled spasticity.
Bone Density ; Cerebral Palsy ; Child ; Congenital Abnormalities ; Contracture ; Developmental Disabilities ; Female ; Femur ; Fractures, Bone ; Humans ; Leg ; Lower Extremity ; Muscle Spasticity ; Obturator Nerve ; Risk Factors

BACKGROUND: Group B streptococci (GBS) are major cause of meningitis and septicemia in neonates and pregnant women, but the importance in non-pregnant adults has not been clearly defined. METHODS: Medical records of all patients with group B streptococcal bacteremia from 1988 to 1997 at Asan Medical Center were reviewed. We compared the clinical and laboratory findings of non-pregnant adults to those of neonates. RESULTS: In a 8-year period there were 41 patients with GBS bacteremia. Thirteen (31.7%) patients were neonates (mean age 14.0+/-11.5 day) and 28 (68.3%) were non-pregnant adults (mean age 52.8+/-13.3 year). Community-acquired infections were 2 cases (15.4%) in the neonates and 7 cases (25.0%) in the non-pregnant adults. In the non-pregnant adults, the most common clinical diagnosis was bacteremia without identified source (15 cases, 53.6%). The others were bone or joint infection (6), urinary tract infection (4), pneumonia (2), skin infection (2), peritonitis (2), and meningitis (1). GBS bacteremia was more common in old age (50 years, 20 cases, 71.4%), the presence of diabetes mellitus (10), solid tumors (10) and liver cirrhosis (10). The mortality rate in non-pregnant adults was 35.7% (10 cases), accounting for 10.7% (3) of deaths related to GBS. In the neonates, early onset infection were 5 cases (38.5%) and late onset infection were 8 (61.5%). The presumed portal of entries were bacteremia without identified focus (5 cases, 38.5%), and meningitis (8, 61.5%). The mortality rate in the neonates was 23.1% (3 cases) and 7.1% (1) related to GBS bacteremia. CONCLUSION: GBS bacteremia is a serious problem not only in the neonates and pregnant women but also in the non-pregnant adults, especially those who are elderly patients with significant underlying diseases.
Adult* ; Aged ; Bacteremia* ; Chungcheongnam-do ; Community-Acquired Infections ; Diabetes Mellitus ; Diagnosis ; Female ; Humans ; Infant, Newborn* ; Joints ; Liver Cirrhosis ; Medical Records ; Meningitis ; Mortality ; Peritonitis ; Pneumonia ; Pregnant Women ; Sepsis ; Skin ; Urinary Tract Infections

Although stress-induced cardiomyopathy (SCMP) is a reversible disease and the prognosis is usually excellent, several complications can occur and can result in fatal adverse events. The formation of left ventricular (LV) thrombus is one of these critical complications of SCMP. This report describes a case of SCMP complicated by formation of a LV thrombus that became increasingly mobile as LV contractility recovered, and for which surgical removal was performed. Here, we report a case of SCMP complicated by LV thrombus and review the literature regarding this topic.
Cardiomyopathies* ; Echocardiography ; Humans ; Prognosis ; Takotsubo Cardiomyopathy ; Thrombosis*