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Graves 병에서 항갑상선제 장기치료후 예후인자로서의 TSH 수용체 항체와 TRH 자극시험
임성희(Sung Hee Ihm),조보연(Bo Youn Cho),이홍규(Hong Kue Lee),고창순(Chang Soon Koh),민헌기(Hun Ki Min),이문호(Mun Ho Lee) 대한내과학회 1987 대한내과학회지 Vol.33 No.1
N/A To evaluate the prognostic value of TRH receptor antibodies in Graves' disease and to compare it with TRH stimulation test, TBII activities were measured by radioreceptor assay in 59 patients with Graves' disease during and after 13 months of antithyroid drug therapy and TRH stimulation test was performed in 37 patients in 3 months after drug withdrawal. Within 12 months after the discontinuation of the drugs, 33 patients out of 59 patients (56%) relapsed. Out of 27 patients whose TBII were postive at the time of drug withdrawal 22 patients (8l.5%) relapsed. However in 32 patients whose TBII returned to normal range at the time of durg withdrawal, 21 patients (65.6%) remained in remission for 12 months and 11 patients (34.4%) relapsed. In 14 patients who showed no response to TRH stimulation, 11 patients (78.6%) relapsed. However in 23 patients who showed normal response to TRH stimulation, 19 patients (82.6%) remained in remission and only 4 patients (17.4%) relapsed. In patients who had positive TBII at the time of drug withdrawal and showed no respons to TRH stimulation, 5 patients (83.3%) relapsed, In contrast, 19 patients who had negative TBII at the time of drug withdrawal and normal response to TRH stimulation, 16 patients (84,2%) remained in remission for 12 months. There were no significant differences in predictive values for relapse among TBII positive group, TRH non-responsive group and both abnormal group. These results suggest that 1) positive TBII activities at the end of longterm antithyroid drug treatment is a useful indicator predicting subsequent relapse, and 2) prognostic value of the TBII activities seems to be of the same order as the TRH stimulation test in predicting relapse.
임성희(Sung Hee Ihm),유재명(Jae Myung Yoo),홍성관(Sung Gwan Hong),최문기(Moon Gi Choi),유형준(Hyung Joon Yoo),박성우(Sung Woo Park),윤병수(Byung Soo Yoon) 대한내과학회 1995 대한내과학회지 Vol.49 No.5
N/A Objectives: Thyroxine-binding glogulin (TBG) is a major thyroid hormone transport protein in serum. Its complete deficiency is one of inherited TBG abnormalities that transmit on X-chromosome. Two kinds of a nucleotide substitution or deletion have been shown to cause this hereditary condition in caucasians. In Japanese, a nucleotide deletion at codon 352 of the TBG gene has been shown to be a common cause of complete TBG deficiency. This study was performed to determine the presence of this mutaion in Korean patients with complete TBG deficiency. Methods: The genomic DNAs of 4patients from 2families (2males and 2females) and of 5control subjects were amplified by the polymerase chain reaction with normal (N) and mutant (M) allele specific anti-sense primers and common (C) sense pri- mer. Results: DNA samples from five control subjects were amplified only v;ith a primer pair of C and N. DNAs from two males with phenotype of complete TBG deficiency were amplified only with a primer pair of C and M. DNAs from two females with phenotype of partial TBG deficiency were amplified with both primer pairs. Thus, two male and two female patients were shown to have the mutation as hemizygotes and heterozygotes, respectively. Conclusion: It is concluded that a nucleotide deletion at codon 352 of the THG gene resulting in complete THG deficiency is present among Korean patients. Further study on the prevalence of this mutation in Korean patients with complete TRG deficiency is nesessary.
성현주(Hyun Ju Sung),임인기(In Ki Rim),천진옥(Jin Ok Chun),임성희(Sung Hee Ihm),최문기(Moon Ki Choi),유형준(Hyung Joon Yoo),박성우(Sung Woo Park),장연복(Young Bok Chang),강성원(Sung Won Kang) 대한내과학회 1994 대한내과학회지 Vol.47 No.3
N/A Postpartum thyroid dysfunction is a syndrome of thyroid dysfunction that occurs in the first year after delivery. Presenting clinically as either painless hypothyroidism or thyrotoxicosis, it usually resolves spontaneously. At the time postpartum thyroid dysfunction occurs, the relationship between thyroid antibodies and postpartum thyroid dysfunction is even stronger. The close association of these antibodies with postpartum thyroid dysfunction makes the detection of these antibodies an attractive method for screening large number of pregnant or postpartum women at risk for this disease. Of the 250 women screened on the second postpartum day, 22 women (8.8%) had positive results for serum antimicrosomal antibody. Among the normal controls, 16% had positive results. 18 of these 22 seropositive patients were followed for at least 6 months postpartum. 6 patients (2.4%) developed goiter, and 5 patients (2.0%) had biochemical thyroid dysfunction. All 5 patients developed hypothyroidism without thyrotoxic phase. The disease was mild and transient in two patients. But in remaining 3 patients, goiters and symptoms of hypothyroidism persisted and were treated. The titers of antimicrosomal antibody in women who developed the disease, increased progressively in all patients. Therefore, serum antimicrosomal antibody test after delivery will be useful as a screening test to detect thyroid dysfunction.
이상무 ( Sang Mu Lee ),홍은경 ( Eun Gyoung Hong ),이성진 ( Seong Jin Lee ),정인경 ( In Kyung Jeong ),김현구 ( Hyeon Kyu Kim ),최철수 ( Cheol Soo Choi ),유재명 ( Jae Myung Yu ),김두만 ( Doo Man Kim ),임성희 ( Sung Hee Ihm ),최문기 대한내과학회 2003 대한내과학회지 Vol.64 No.1
연구배경 : 원발성 부갑상선 기능저하증은 드물게 발생하는 질환으로 저칼슘혈증에 수반된 다양한 임상증상 및 징후가 발생한다. 대부분 젊은 나이에 발생하고 임상증상의 발현이 늦어 진단시 신경학적 이상소견을 동반하는 경우가 많고, 자가항체와 연관된 자가면역성 다선 증후군과 동반되어 발생할 수 있다. 따라서 본 연구에서는 원발성 부갑상선기능저하증의 진단시 주증상, 특이 이학적 소견 등의 빈도를 조사하고 질환 발생 연령·성별·가족력 및 임상적 특이 소견과 경과를 Background : Idiopathic hypoparathyroidism is a relatively rare disease characterized by hypocalcemia and hyperphosphatemia due to parathyroid hormone deficiency of unknown cause. It usually develop at young age and various clinical symptoms and signs acc
증례 : 내분비-대사 ; 당뇨병성 케톤산증과 동반된 편무도증 1예
김현성 ( Hyun Sung Kim ),이서희 ( Seo Hee Lee ),강준구 ( Jun Goo Kang ),김철식 ( Chul Sik Kim ),임성희 ( Sung Hee Ihm ),유형준 ( Hyung Joon Yoo ),이성진 ( Seong Jin Lee ) 대한내과학회 2010 대한내과학회지 Vol.79 No.1
저자들은 당뇨병의 과거력이 없으면서 건강하게 지내던 중 제2형 당뇨병, 당뇨병성 케톤산증, 급성 신부전과 동반된 편무도증이 발병한 환자에서 충분한 수액공급과 인슐린 투여 등으로 집중 치료한 결과 편무도증이 소실된 증례를 국내에서 처음으로 경험하였기에 문헌고찰과 함께 보고하는 바이다. Hemiballism-hemichorea (HB-HC) is defined as continuous, non-patterned, and involuntary movement involving one side of the body and is often associated with endocrine diseases such as diabetes mellitus (DM) and hyperthyroidism. In Korea, 16 cases of HB-HC associated with non-ketotic hyperglycemia have been reported. Here, we report the first known case of HB-HC associated with type 2 DM and diabetic ketoacidosis (DKA). A 32-year-old man visited our hospital complaining of hemichoreoballistic movement. The patient had no history of DM. In a neurological examination and laboratory tests, HB-HC associated with type 2 DM and DKA was diagnosed. The patient was immediately treated with intravenous hydration and insulin therapy, and completely recovered. DKA and non-ketotic hyperglycemia should be considered as a possible cause of HB-HC, although these are very rare. In HB-HC associated with DKA, strict blood glucose control is needed. (Korean J Med 79:62-66, 2010)
유리지방산이 원형질막 Na+ , K+ - ATPase 에 미치는 효과에 대한 알부민의 역할
이병두(Byoung Doo Rhee),임성희(Sung Hee Ihm),장학철(Hak Chul Jang),박경수(Kyong Soo Park),김성연(Seong Yeon Kim),조보연(Bo Youn Cho),이홍규(Hong Kyu Lee),고창순(Chang Soon Koh),민헌기(Hun Ki Min) 대한내과학회 1988 대한내과학회지 Vol.35 No.1
N/A There is increasing evidence that free fatty acids (FFA) inhibit the activity of plasma membrane Na+, K+-ATPase in vitro and thereby could be a control agent of metabolism. However, in several recent studies, physiological conclusion were drawn from the effect of FFA in systems in which albumin was absent. To explore the role of albumin in modulating the effect of FFA on the activity of this enzyme, the activities plasma membrane Na+, K+-ATPase were measured in the presence of various concentrations of FFA and/or albumin. 1) The activities of Na+, K+-ATPase in the presence of 4% bovine serum albumin (BSA) were inhibited by oleic and linoleic acid at concentrations ranging from 0.05 to 2.0mM. 2) The inhibitory effects of oleic acid at each concentration on the activities of Na+, K+-ATPase were increased as the concentration of BSA was decreased from 4% to 0%. 3) The activities of Na+, K+-ATPase were not inhibited when the FFA to albumin molar ratio was less than 1.0 but were inhibited to less than 50% when the ratio exceeded 3.5. The above results shows that, in vitro, the activities of plasma membrane Na+, K+-ATPase are inhibited by unsaturated FFA and albumin has a protective role on this inhibition. This suggests that in vivo unsaturated FFA could be a regulator of cellular metabolism by inhibition of Na+, K+-ATPase under the pathologic condition in which the FFA to albumin molar ratio is increased markedly.
그레이브스병 환자에서 항갑상선제가 지질과산화에 미치는 영향
정승화(Seung Hwa Jung),최용석(Yong Suk Choi),홍성표(Sung Pyo Hong),임성희(Sung Hee Ihm),최문기(Moon Gi Choi),유형준(Hyung Joon Yoo),박성우(Sung Woo Park) 대한내과학회 1995 대한내과학회지 Vol.48 No.5
N/A Objectives: The development of a significant increase in metabolic rate and total oxygen consumption leads to an enhancement in oxygen stress, which results in an increased lipid peroxidation. The study was undertaken to evaluate: 1) the lipid peroxidative stress of subjects with hyperthyroidism related to changes in metabolic activity, 2) the influence of the antithyroid agent, propylthiouracil on the oxidative stress status of Graves disease patients. Methods: We studied thyroid function indices(T3, free T4 TSH) and lipid peroxidation in Graves disease patients, before and after treatment with propylthiouracil for three months. Results: It is revealed that red blood cell membrane lipid peroxidation is significantly correlated with thyroid function indices(T3 r=0.676, p<0.01, free T4 r=0.683, <0.05), before PTU treatment and lipid peroxidation is diminished in parallel with attainment of euthyroid state of thyroid activity after propylthiouracil administration, after PTU treatment. Conclusion: It is shown that lipid peroxidation that is attributed to oxygen consumption and oxidative stress are significantly correlated with the state of thyroid function in Graves' disease patients.
증례 : 내분비-대사 ; 갑상선 기능저하증을 동반한 가족성 저칼슘뇨성 고칼슘혈증 1예
김은지 ( Eun Ji Kim ),박소영 ( So Young Park ),강준구 ( Jun Goo Kang ),김철식 ( Chul Sik Kim ),임성희 ( Sung Hee Ihm ),유형준 ( Hyung Joon Yoo ),이성진 ( Seong Jin Lee ) 대한내과학회 2011 대한내과학회지 Vol.80 No.6
저자들은 우연히 발견된 유전적 소인을 보이는 경도의 무증상 고칼슘혈증, 저칼슘뇨증, 낮은 뇨중 칼슘-크레아티닌비, 정상이거나 약간 상승된 부갑상선호르몬 수치, 정상 알칼리성 인산분해효소 수치 소견들과 칼슘감지수용체 유전자의 21번째 아미노산 돌연변이(G21R)와 990번째 아미노산 단일염기다형성(R990G) 소견들이 확인된 자가면역성 갑상선염과 갑상선 기능저하증을 동반한 가족성 저칼슘뇨성 고칼슘혈증 증례를 국내외에서 처음으로 경험하였기에 문헌고찰과 함께 보고하는 바이다. Familial hypocalciuric hypercalcemia (FHH) is a genetic disorder characterized by mild asymptomatic hypercalcemia, hypocalciuria, a decreased urinary calcium-creatinine ratio, and normal or elevated parathyroid hormone levels. FHH is caused by an inactivating heterozygous mutation of the calcium-sensing receptor (CaSR) gene. A 62-year-old woman was referred to our center because of inadequate glucose control. Her son had hypercalcemia. Biochemical and endocrine studies showed hypercalcemia, hypocalciuria, a decreased urinary calcium-creatinine ratio, normal parathyroid hormone levels, normal PTH-related peptide levels, decreased free T4, increased TSH, and thyroid antibody positivity. Direct sequencing analyses revealed a heterozygous mutation of G21R at exon 2 and a single nucleotide polymorphism of R990G at exon 7 of the CaSR gene. Here, we report a case of FHH associated with a heterozygous mutation and a single nucleotide polymorphism of the CaSR gene, which coexisted with autoimmune thyroiditis and hypothyroidism. To our knowledge, this is the first such case reported. (Korean J Med 2011;80:718-722)
증례 : 내분비-대사 ; 갑상선호르몬수용체 돌연변이가 동반된 갑상선호르몬저항증후군 1예
임수경 ( Soo Kyung Lim ),김성열 ( Seong Yeol Kim ),강준구 ( Jun Goo Kang ),김철식 ( Chul Sik Kim ),임성희 ( Sung Hee Ihm ),유형준 ( Hyung Joon Yoo ),이성진 ( Seong Jin Lee ) 대한내과학회 2011 대한내과학회지 Vol.80 No.2S
저자들은 갑상선호르몬 투여 전후 갑상선자극호르몬분비 호르몬 자극검사, α-아단위 측정, 뇌하수체자기공명영상, 갑상선호르몬수용체 β 유전자검사로 진단한 갑상선호르몬수용체 β 유전자 돌연변이(R438H)가 확인된 갑상선호르몬저 항증후군 증례를 국내에서 처음으로 경험하였기에 문헌고찰과 함께 보고하는 바이다. In thyroid hormone resistance syndrome (THR) TSH levels are normal or elevated despite thyroid hormone levels being elevated. THR is distinguished from TSH-producing pituitary adenoma by TRH stimulation and α-subunit tests, thyroid hormone receptor (TR) β gene analysis, and sellar MRI. A 24-year old man with diffuse goiter visited our hospital complaining of fatigue, heat intolerance, palpitation, and weight loss. He had elevated total T3 and free T4 levels, but normal TSH levels. Serum TSH levels during TRH stimulation tests performed before and after T3 suppression showed normal and non-suppressible responses, respectively. The serum basal α-subunit test result was normal. A TR β gene R438H mutation was identified, and a pituitary mass with cystic change was identified by sellar MRI. We report a case of THR with a mutation (R438H) in the TR β gene, the first case of its kind in Korea. (Korean J Med 2011;80:S220-S226)