Predictive Factors for Valproate Treatment in Childhood Absence Epilepsy

PURPOSE: To determine the clinical and demographic factors associated with long-term remission of valproate(VPA) therapy in childhood absence epilepsy. METHODS: Fifty-six cases of childhood and juvenile absence epilepsy were identified by reviewing of Electroencephalographic records and medical charts. Thirty-six cases were initially treated with VPA. Factor associated with responsiveness were identified by uni- and mutivariate logistic regression. RESULTS: Twenty-seven patient achieved long-term remission(75%). Failure to achieve remission was more likely if the initial treatment of VPA had failed than if it was successful(53% versus 90.4%, P<0.02) was also associated with failure of long-term remission. Lamotrigine was more efficacious add-on drug than Ethosuximide(63.6% vs 25% P=0.04). CONCLUSION: Long-term seizure remission was related to the patient's initial response to VPA.

Study on the diagnostic utility of serum levels of insulin-like growth Factor-I and insulin-like growth factor binding protein-3 in growth hormone deficiency / 소아과

PURPOSE: This study aimed to determine the best cutoff line for insulin-like growth factor (IGF)-I and insulin-like growth factor binding protein (IGFBP)-3 to discriminate between growth hormone deficiency (GHD) patients and the control group. METHODS: Two hundred thirty subjects with normal controls (129 boys and 101 girls, aged 7-15 years), 14 patients with complete GHD (12 boys and 2 girls), and 17 patients with partial GHD (9 boys and 8 girls) were studied. IGF-I serum concentrations were measured by radioimmunoassay (RI), and IGFBP-3 concentrations were measured by immunoradiometric assay (IRMA). RESULTS: The receiver operating characteristic (ROC) plot analysis showed that the best IGF-I and IGFBP-3 cutoff line was at -1 standard deviation (SD). By comparing IGF-I serum levels of GHD children within 1 SD of normal control, we determined the sensitivity (S) (87.5-100%) and specificity (Sp) (80-84.6%) according to the age group. For IGFBP-3 , we determined the following values: S (58.7-85.7%) and Sp (79.2-85.5%). Eleven of 1 4 patients with complete GHD (78.5%) and 16 of 17 patients with partial GHD (94.1%) had IGF-I concentrations equal to or below -1 SD of the control group mean. Ten of 12 complete GHD children (83.3%) and 13 of 17 partial GHD children (76.5%) had IGFBP-3 concentrations equal or below -1 SD of the control group mean. CONCLUSION: We conclude that the measurement of IGF-I and IGFBP-3 concentrations might provide essential supplementary data in the diagnostic evaluation of patients with GHD. Our results support the need to use cutoff lines based on below -1 SD of the control.

A Case of Atypical Kawasaki Disease with Severe Neutropenia

Leukocytosis and neutrophilia is common during the acute phase of Kawasaki disease whereas leukopenia is not common and severe neutropenia is rare. Severe neutropenia is defined as absolute neutrophil count less than 500/mm3. There are only few publicatons reporting of atypical Kawasaki disease with severe neutropenia. We report a case of atypical Kawasaki disease with severe neutropenia.

Moyamoya Disease in Children and Adolescents

PURPOSE: Moyamoya disease is a chronic cerebrovascular illness characterized by bilateral stenoses or occlusions of the arteries around the circle of Willis with prominent collateral circulation. We studied 36 children and adolescents with Moyamoya disease to evaluate the clinical features and outcomes. METHODS: Records were reviewed of 36 pediatric patients admitted at the Busan Paik Hospital for Moyamoya disease between January 2000 and July 2007. The clinical records were reviewed in terms of the patient profiles, imaging findings, surgical techniques, and pathologic findings. RESULTS: Overall clinical features, responses to treatment and outcome were nearly same as those of other previous reports in Korea as well as the other countries. The mean age of onset was 8 years and 5 months and the ratio of male to female was 1:1.2. Ischemia was more often than infarction(5.6: 1). Half of the patients had conservative therapy and 17 cases(47%) were treated with EDAS. CONCLUSION: The clinical features of childhood Moyamoya disease are similar to the result from other studies in Korea.

Prolonged Survival of an Adult with Hydranencephaly

Hydranencephaly is a rare condition in which the brain's cerebral hemispheres are absent and replaced by sacs filled with cerebrospinal fluid. The prognosis for children with hydranencephaly is generally poor, so many children with this disorder die before the age of 1 year. We experienced a patient with hydranencephaly who showed prolonged survival until age of 22 years. To our limited knowledge, this case may be the longest survival of a patient with hydranencephaly in the world.

A Case of a Dieulafoy Lesion Treated usingCoil Embolization in a Child / 대한소아소화기영양학회지

A dieulafoy lesion, which is an unusual cause of gastrointestinal bleeding that can be fatal in children. Dieulafoy lesions are characterized by an abnormally large eroded submucosal artery that is commonly located in the lesser curvature of the proximal stomach. In most cases, permanent hemostasis is achieved by endoscopic epinephrine injection, however, some patients require other endoscopic treatment modalities, embolization or surgery. We report here a case of a Dieulafoy lesion in an 11-year-old boy who had recurrent bleeding from the lesion in the duodenal bulb after endoscopic epinephrine injection and surgical ligation, that was successfully treated using coil embolization.

A Case of a Dieulafoy Lesion Treated usingCoil Embolization in a Child / 대한소아소화기영양학회지

A dieulafoy lesion, which is an unusual cause of gastrointestinal bleeding that can be fatal in children. Dieulafoy lesions are characterized by an abnormally large eroded submucosal artery that is commonly located in the lesser curvature of the proximal stomach. In most cases, permanent hemostasis is achieved by endoscopic epinephrine injection, however, some patients require other endoscopic treatment modalities, embolization or surgery. We report here a case of a Dieulafoy lesion in an 11-year-old boy who had recurrent bleeding from the lesion in the duodenal bulb after endoscopic epinephrine injection and surgical ligation, that was successfully treated using coil embolization.

A Study for Incidence of Childhood Leukemia in Kyongnam Province, Korea / 대한소아혈액종양학회지

PURPOSE: Of the cancers in childhood, leukemia is the most frequent one. For the desirable control of childhood leukemia, the basic data for the incidence has a great importance. The authors made a report about the incidence of leukemia in childhood, which analyzed the data from 126 cases in Kyongnam province, Korea, during 1991~1995. METHODS: The data were obtained from 126 new cases of childhood leukemia who had been living in the Kyongnam province and were diagnosed at the 26 university hospitals or general hospitals in the Kyongnam area and other cities from 1991 to 1995. RESULTS: The age-and-sex adjusted annual incidence rate per 100,000 population during 1991~1995 varied from 1.82 to 2.86, and cumulative annual incidence rate was 2.41 (male 2.26 and female 2.57 respectively). Male to female sex ratio was 1:1 in total cases. By the major types of childhood leukemia, the cases were composed of acute lymphocytic leukemia 70.6%, acute myelocytic leukemia 26.9% and chronic myelocytic leukemia 2.5%. The cumulative annual incidence rate per 100,000 population (crude rate) during 1991~1995 were 2.77 in Ulsan city, 2.62 in Chinju city and 2.34 in the whole area of Kyongnam province. CONCLUSION: It was concluded that the age-and-sex adjusted annual incidence rate per 100,000 of childhood in Kyongnam province was 2.41, which was lower than that in Pusan city in the same period. And, there was no significant difference of the cumulative annual incidence rate between Ulsan area and Chinju area in the same period.

A Comparative Analysis of the Clinical and Pathological Features of IgA Nephropathy and Thin Glomerular Basement Membrane Disease

PURPOSE: IgA nephropathy(IgAN) and thin glomerular basement membrane disease(TGBMD) are common glomerular diseases that cause hematuria in childhood. IgAN has characteristics of IgA deposit as the sole or predominantly localized to the mesangium. Recently, it has been reported that thinning of glomerular basement membrane(GBM) is commonly accompanied with precipitation of electron dense deposits in IgAN. We performed this study to examine the frequency of thinning of GBM among children with IgAN and to analysis the correlation between urinary abnormalities and GBM thickness, and furthermore to conduct comparative analysis of the clinical and pathological features of IgAN and TGBMD. METHODS: This study summarizes data collected from Department of Pediatrics, Busan Paik Hospital, Inje Medical College. Data include 51 cases who were diagnosed as IgAN from 1995 to 2000, and 26 cases who were diagnosed as TGBMD from 1990 to 2000 by percutaneous renal biopsy. RESULTS: Males accounted for 29/51(56.9%) patients with IgAN and 8/26(30.8%) of those with TGBMD. The clinical and laboratory features between IgAN and TGBMD were significantly different regarding the incidence of proteinuria(IgAN vs TGBMD: 43.1% vs 3.8%, P=0.001), the incidence of co-appearance of proteinuria with hematuria (41.2% vs 3.8%, P=0.001), total amount of protein in 24 hours collected urine (808+/-188.5 mg vs 251+/-00.7 mg, P=0.001) and the incidence of proteinuria more than 1 gm in 24 hours collected urine (23.5% vs 3.8%, P=0.01). On the contrary, there were no significant differences in the levels of serum albumin, creatinine, BUN, and Ccr between two groups. The mean thickness of GBM in patients with IgAN was293.0+/-9.2 nm(139.7-461.9 nm) and 180.9+/-5.8 nm (110.5-229.5 nm) in patients with TGBMD. The mean GBM thickness revealed significantly thinner in TGBMD compared than those with IgAN (P=0.0001). The frequency of thickness being less than 250 nm was 37.4 +/-34.4% in IgAN and 93.0 +/-7.0% in TGBMD (P=0.0001). But there were no correlations between urinary abnormalities and GBM thickness in patients with IgAN. CONCLUSION: The thinning of GBM would be one of the common pathological findings in IgAN. Moreover, there is no significant correlations between urinary abnormalities and GBM thickness in patients with IgAN. However, patients with IgAN tend to have significantly higher possibilities of proteinuria, co-appearance of proteinuria with hematuria and higher total amount of protein in 24 hours collected urine compared those with TGBMD. These differences might be play an important role as progressive prognostic indicators in patients with IgAN.