The Effect of Growth Hormone Therapy on Bone Mineral Density in Young Adults with Childhood-onset Growth Hormone Deficiency / 대한소아내분비학회지

PURPOSE: Growth hormone (GH) has multiple beneficial effects in addition to its promotion of linear growth. Therefore adults with GH deficiency (GHD) have abnormal body composition, altered lipid metabolism, increased cardiovascular disease, and decreased bone mineral density (BMD). We evaluated the effect of GH therapy on BMD in young adults with childhood-onset GHD. METHODS: 17 childhood-onset GHD adults (10 male, 7 female, mean age 24.5+/-5.5 yr) with or without continuous GH treatment after final height were studied. All subjects divided two groups; GH-treated group (n=6) and GH-untreated group (n=11). BMD in lumbar spine and proximal femur was measured by dual energy X-ray absorptiometry. RESULTS: The mean serum level of IGF-I concentration in the GH-untreated group was lower than in the GH-treated group (88.4+/-55.9 ng/mL vs. 358.7+/-196.8 ng/mL, P<0.05). The BMD of lumbar spine in the GH-treated group and GH-untreated group was 1.02+/-0.13 g/cm2 and 0.82+/-0.09 g/cm2 and the BMD of femur was 1.15+/-0.14 g/cm2 and 0.82+/-0.10 g/cm2 respectively. The BMD of the GH-treated group was significantly higher than the GH-untreated group (P<0.05). CONCLUSION: These findings support the need of continuous GH treatment after completion of growth and careful evaluation of BMD in adult patients with childhood-onset GHD.

Brain-derived Neurotrophic Factor(BDNF) SNP 6265 Polymorphisms in Febrile Seizure and GEFS+

PURPOSE: Febrile seizure(FS) is one of the most common neurological conditions during childhood, but the pathogenesis of FS remains ambiguous. Various studies have shown that brain-derived neurotrophic factor(BDNF) increased neuronal excitability. In this study, to determine whether the polymorphisms of SNP 6265 within the gene encoding BDNF are associated with susceptibility to FS, the frequencies of the polymorphisms were investigated in children with FS and control subjects. In addition, we analyzed the SNP 6265 polymorphisms in Generalized epilepsy with febrile seizures plus (GEFS+) that hasn't been studied as yet in Korea. METHODS: A total of 79 children selected throughout a collaborative study of Catholic Child Neurology Research Group were divided into three groups: (1) FS(n=30); (2) GEFS+ (n=19); (3) control subjects(n=30). Genotypes and allelic frequencies for the polymorphisms of SNP 6265 located at nucleotide 196 was analyzed and compared among the groups. RESULTS: In this study, proportions for A homozygote, A/G heterozygote and G homozygote for BDNF were as follows: in FS, 46.7%, 36.7% and 16.7%, in GEFS+, 26.3%, 47.4% and 26.3% and in control subjects, 60.0%, 16.7% and 23.3%. The allele A and G frequencies for BDNF in FS were 65.0% and 35.0%, in GEFS+ were 50% and 50%, and in control subjects were 68.3% and 31.7%. However, these differences in genotype proportions and allele frequencies among three groups were not significant. CONCLUSION: These results suggest that genomic variations of BDNF might not be the susceptibility factor for FS and GEFS+ in Korean population.

The Clinical Manifestations and the Effect on Prognosis of Seizures during Acute Lymphoblastic Leukemia Treatment in Children

PURPOSE: This study was designed to assess the clinical manifestations and the effect on prognosis of seizures in children with acute lymphoblastic leukemia(ALL). METHODS: The study group consisted of 20 patients(10 males and 10 females) who experienced seizures out of the 198 pediatric ALL patients(117 males and 81 females) who were diagnosed and treated at the Department of Pediatrics, St. Mary's Hospital, the Catholic University of Korea. RESULTS: The overall incidence of seizure developing after the diagnosis of ALL was 10.1% and in 11 patients(5.6%), seizure recurred one or more times. An average of 6.5+/-3.6 months(range 0-42 months) elapsed between the beginning of treatment and the new onset of seizures. In 13 patients(65%), the first seizure occurred during the induction or re-induction chemotherapy phase. Seizure type was partial seizure in 11 patients(55%), and generalized seizure in 9 patients(45%). In 15 patients(88.2%), brain imaging study showed abnormal findings and in 18 patients(90%), EEG revealed abnormal findings. 11 patients were diagnosed with epilepsy and were treated with long-term anticonvulsants. In these patients, 4 patients(36.4%) had no seizure recurrence, but 2 patients(18.2%) showed no response to anticonvulsants. The 5-year survival rate of the patients experiencing seizures was 47%, while the rate was 78% for those did not experience seizures (P<0.001). CONCLUSION: Seizures in pediatric patients with ALL was closely related to the 5-year survival rate. Therefore, these patients require early careful observation, evaluation and intensive care. Also, further studies such as ways to diminish the side effects of antileukemic agents are necessary to reduce the risk of seizure.