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성장호르몬 결핍증 환아에서 성장호르몬 분비안자에 대한 성장호르몬 분비 양상
문형로,양세원,박병규 대한내분비학회 1990 Endocrinology and metabolism Vol.5 No.1
The authors observed the growth hormone (GH) response to pharmacologic agents (arginine-insulin, L-dopa) and growth hormone releasing hormone (GHRH) at Seoul National University Children's Hospital from September 1988 to August 1989. 42 patients included in the study were divided into 3 groups according to the peak plasma GH levels after pharmacologic stimulation test: complete GH deficiency group (25 patients), GH$lt;5 ng/ml; imcomplete GH deficiency group (6 patients), 5≤GH$lt;10 ng/ml; normal GH group (11 patients), GH≥10 ng/ml (GH levels of more than 10 ng/ml were measured from 8 patients). The peak plasma GH levels were measured from each group after GHRH stimulation test. The results were as follows: 1) The mean peak plasma levels of GH measured from complete and incomplete GH deficiency groups after GHRH stimulation test were higher than those after pharmacologic stimulation test, but were not different significantly in normal GH group. Following GHRH administration, peak plasma GH levels of more than 10 ng/mwere measured from 8 patients with idiopathic GH deficiency. Thus hypothalamic defect was suggested as the origin of their GH deficiency. 2) The serial mean plasma GH levels measured from normal GH group were persistently higher than those from complete and incomplete GH deficiency groups. But considerable overlapping of peak GH levels was observed among three groups. 3) The peak plasma GH levels after GHRH stimulation test were negatively correlated with age in complete GH deficiency and normal GH group. 4) The peak value of sequential mean plasma GH levels measured from incomplete GH deficiency group after GHRH stimulation test was reached earlier than that from complete GH deficiency group. In conclusion, GHRH stimulation test might be useful in the evaluation of readily releasable GH reservoir and speculation of putative mechanism of GH deficiency. But the discriminination of GH deficiency from normal GH status couldn't be made solely on the basis of the peak plasma GH levels after single GHRH stimulation test. Further study using different doses of GHRH is recommended for the evaluation of GH status (J. Kor Soc Endocrinol 5:14~22, 1990).
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Noonan 증후군에서 유발검사에 대한 성장호르몬의 분비반응
문형로,양세원 대한내분비학회 1989 Endocrinology and metabolism Vol.4 No.1
We observed the responses of growth hormone to the various provocative testes in 5 patients with Noonan's syndrome, and compared the results with that in 5 patients with various etiologies of short stature (control group), who showed normal responses to the various provocative tests. The observed results were as follows: 1) We observed normal GH responses to the provocative tests (L-dopa, arginine, insulin and GRF) in patients with Noonan's syndrome. We strongly suggest that the mechanism of short stature in Noonan's syndrome may not come from the deficiency of GH secretion. But to make sure that suggestion, study of 24 hours GH secretory patterns should be necessary. 2) The secretory patterns of GH to the provocative tests (L-dopa, arginine and insulin) in Noonan's syndrome were similar to that in control group. But the GH responses to GRF in Noonan's syndrome were significantly higher than those in control group (p$lt;0.05). This phenomenon remains to be clarified with further studies (Fig. 1, 2, 3, and 4). 3) The secretory patterns of other pituitary hormones except gonadotropin were normal. We can conclude with above findings that the endocrine abnormalities, especially in pituitary hormones may be manifested unusually as sporadic findings in Noonan's syndrome. (J Kor Soc Endocrinol 4:37-42, 1989)
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Turner 증후군 환자에서 Oxandrolone이 성장 속도에 미치는 영향
문형로,양세원 대한내분비학회 1986 Endocrinology and metabolism Vol.1 No.2
The growth velocity of patients with Turner's syndrome was studied during pre-treatment and treatment with Oxandrolone (2-oxa-17- alpha - methyldihydrotestosterone) in eight subjects. During treatment with Oxandrolone, height was measured every 3 months and bone age and height age were compared with chronological age. The results were as follows; Of eight patients, karyothping revealed 45, X in four, 45, X/46, XX in three and 46, X, I(Xq)in one. The mean age of subjects was 12.25 years(7-16 years).The mean duration before Oxandrolone therapy and the mean duration on Oxandrolone therapy was 4.25 months (1-18 months) and 33 months(13-52 months), respectively. The man growth velocity before Oxandrolone therapy was 0.12 cm/months, The mean growth velocity during Oxandrolone therapy was 0.53?0.25 cm/month in the first year, 0.43?0.10 cm/month in the second year, 0.21?0.16 cm/month in the third year and 0.22?0.14cm/month in the fourth year. These values in the growth velocity during treatment had no statistic signance compard with the growth velocity during pre-treatment. The difference between the chronological age and the bone age was minimal but the difference between the chronological age and the height age was noticeable. In one case, final adult height had been reached. Throughout the peroid of therapy, there were no untoward effect including excessive bone growth and musculinization.
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성장호르몬 결핍증 환아에서 국내에서 합성된 성장호르몬 ( DA - 3001 )의 성장 효과
문형로,김덕희,양세원,신재훈,이병철 대한내분비학회 1995 Endocrinology and metabolism Vol.10 No.4
Recently, methionyl-hGH was produced in the E. coil K-12, W3110 by recombinant DNA technology in Korea. In this paper, the clinical efficacy and immunogenicity of this GH were studied in 43 patients with growth hormone deficency. The subjects of this study were aged 4.3-18.5 years and each patient received GH 0.5-0.71U/kg week subcutaneously, 6-7 times a week for 1 year. During treatment, height, body weight and bone age were checked. Blood count, urinalysis, blood chemistry and thyroid hormonal concentrations were checked before and every 3 months. The measurement of IGF-1 was performed and assay of antibody against hGH was performed before and every 6 months. The height velocities significantly increased from 3.7+-3.0 cm/year to 11.0+-4.2 cm/year and 9.9+-3.2 cm/year at 6 and 12 months after GH therapy, respectively. The Height SDS were significantly improved after GH therapy with increasing ratio of bone age to chronological age from 0.60+-0.19 at pretreatment to 0.68+-0.16 at 6 month, 0.69+-0.16 at 12 month of therapy. The plasma IGF-1 levels significantly increased during treatment. Three out of 35 patients(8.3%) showed antibody against hGH after 1 year of treatment. Thoughout study, we could not observe any remarkable side effect with GH treatment. These results indicate that this E. coli derived methionyl recombinant growth hormone is effective in improving the index of linear growth in the children with growth hormone deficiency without significant side effect(J Kor Soc Endocrinol 10: 377-385, 1995).
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