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21 - Hydroxylase 결핍 선천성 부신 증식증 가계에서 ACTH Stimulation Test
최철준(Cheul Joon Choi),주영실(Young Sil Ju),이기영(Gi Young Yi),김성운(Sung Woon Kim),양인명(In Myung Yang),김진우(Jin Woo Kim),김영설(Young Seoul Kim),최영길(Young Kil Choi),오보훈(Bo Hoon Oh) 대한내과학회 1987 대한내과학회지 Vol.33 No.2
N/A CAH (congenital adrenal hyperplasia) due to 21-hydroxylase (2l-OH) deficiency is a autosomal recessive disorder in which affected homozygotes present with virilization, hirsutism and short stature. For the detection of heterozygote carrier, we measured 17-hydroxy progesterone (17-OHP), progesterone, cortisol and aldosterone after ACTH stimulation to the patient, her family members and controls. The following results were obtained. 1) ACTH stimulation test was a goad test for the confirm diagnosis of homozygotes and the detection of heterozygote carriers in 21-OH deficiency CAH. 2) The patient and two siblings were homozygotes. The mother and one sibling were heterozygotes, and her father was suggested to be a heterozygote without biochemical expression. 3) 21-hydroxylase deficiency of zona glomerulosa was Not deficient in all homozygotes.
중증 비갑상선 질환에서 혈청 Angiotensin - Converting enzyme 활성도의 변화에 관한 연구
심재두(Jae Doo Shim),최성근(Seong Gun Choi),남연호(Yeun Ho Nam),김성운(Sung Woon Kim),양인명(In Myung Yang),김진우(Jin Woo Kim),김영설(Young Seoul Kim),최영길(Young Kil Choi) 대한내과학회 1987 대한내과학회지 Vol.33 No.2
N/A To examine the hypothesis that the serum ACE activity was decreased in proportion to decrement of the thyroid hormone concentration in patients with severe nonthyroidal illness, we measured serum T₃, T₄, free TTSH and serum ACE activity of 20 patients with nonthyroidal illness and 12 normal controls, 1) The mean serum T₃, concentration (57.6±25.1ng/dl) in patients with severe nonthyroidal illness was significantly different from that (100.9±10.5) of normal controls. (P<0.005). 2) The mean serum free T₃, concentration (1.39±0.79 pg/ml) in patients with severe nonthyroidal illness was signi fiantly different from that (2.47±0.50 pg/ml) of normal controls. (P <0.005). 3) There were no significant differences in the mean serum T₄, and TSH concentrations between two groups. 4) There was no significant difference in the mean serum ACE activity between patients with severe nonthyroidal illness (6.1±2.4 unit) and normal controls (6.9±2.2). 5) In 8 patients with severe nonthyroidal illness which could increase the serum ACE activity, the mean serum ACE activity was relatively high as compared to low mean serum free T₃, concentration and in other 6 patients with servere nonthyroidal illness, the decrease in serum ACE activity was associated with a parallel decrease in the serum T₃, concentration and there was significant correlation between serum free T, concentration and ACE activity (r=0.84). These dnta suggest that serum ACE activity can be an useful index of thyroid function in patients with severe nonthyroidal illness, but one should exclude the diseases which can increase serum ACE activity before interpretation.