A Clinical Study of Pediatric Myelodysplastic Syndrome: Application of International Prognostic Scoring System and the Review of the Korean Literature / 대한암학회지

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.

Serum Leptin Levels in Epileptic Children with Anticonvulsant Medication / 대한소아신경학회지

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.