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양정현,우상욱<SUP>1<.SUP>,Jung-Hyun Yang,and Sang-Uk Woo,<SUP>1<.SUP> 대한갑상선-내분비외과학회 2005 The Koreran journal of Endocrine Surgery Vol.5 No.1
Purpose: Multiple Endocrine Neoplasm (MEN) is a rare, complex and familial disease. There are MEN syndromes are inherited in an autosomal dominant fashion with high penetrance. The variations in the RET gene play an important role in the MEN syndromes. Recent advances in diagnosis, treatment and genetic study of patients with MEN in Korean are reviewed. Methods: There were 79 cases and 20 families with MEN syndromes in Korea which based on my experiences and 27 published papers. According to subtypes, there were classified and analyzed. Results: Mean age was 37.9⁑11.5 years old. Sex ratio was 1:2.6. There were 7 families and 23 cases with MEN type I in Korean. The clinical characteristics of MEN I in Korean are mostly not different from the previous reports except older age (mean=43.2 old-year) at diagnosis. The frequency of the MEN I germ-line mutation in Korean MEN I (80%) families was similar to those reported previously. There were 13 families and 52 cases with MEN type II A in Korean. Three-quarters (9/12) of the Korean patients with MEN IIa had RET mutations on codon 634 of exon 11 (4 patients, C634; 4 patients, C634Y; 1 patient, C634W), but a quarter (3/12) had mutations on codon 618 of exon 10 (2 patients, C618R; 1 patient, C618S). A small medullary carcinoma in a patient of MEN type II A family was detected by genetic mutation screening in SMC. MEN IIb was reported only 4 cases. A case showed a codon 918 mutation (M918T) at exon 16 of RET proto-oncogene. Conclusion: Multiple endocrine neoplasia is rare hereditary cancer syndromes expressing a variety of tumors. Withunderstanding of the molecular and clinical pathology of MEN syndromes, genetic screening is now feasible, and treatments have become more individualized based on genetic information of Korean. (Korean J Endocrine Surg 2005;5:1-6)
갑상선 유두암에서 BABA 내시경 갑상선 절제술에 대한 임상적 고찰
천보성,김재홍,박경식,배수연,정성일,유영범,양정현,윤여규<SUP>1<,SUP>,Bo Sung Cheon,Jae Hong Kim,Kyoung Sik Park,Soo Youn Bae,Sung Il Jung,Young Bum Yoo,Jung-Hyun Yang,and Yeo-Kyu Youn,<SUP>1<,SUP> 대한갑상선-내분비외과학회 2011 The Koreran journal of Endocrine Surgery Vol.11 No.3
Purpose: The aim of this study was to evaluate the operative feasibility and safety of endoscopic thyroidectomy via bilateral axillo breast approach (BABA) compared to conventional thyroidectomy in papillary thyroid carcinoma (PTC) patients. Methods: From July 2009 to November 2010, patients underwent BABA endoscopic thyroidectomy (ET group; n=41) or conventional open thyroidectomy (OT group; n=61) for PTC. Clinical and pathologic characteristics of patients, operation time, post-operative complications, cosmetic satisfaction and thyroglobulin (TG) level were analyzed retrospectively. Results: The mean age of the patients was 40.05±9.58 years (range 25∼61 years) and 46.21±13.68 years (range 19∼79 years) for the ET and OT group, respectively. The operative extent in the ET group did not include advanced thyroid cancer or lateral neck dissection. The size of the tumor was 0.78±0.59 cm (range 0.1∼3.00 cm) and 1.54 ±1.05 cm (range 0.3∼6.00 cm) for the ET and OT group, respectively. Extrathyroidal extension and number of retrieved lymph nodes were significantly higher in the OT group. Postoperative radioactive iodine ablation was performed on 25 patients (72.43%) in the ET group and 48 patients (78.69%) in the OT group. There was no abnormal uptake on radioactive iodine scans in the iodine-treated patients and no significant differences in postoperative off-T4 TG levels between the two groups. There were no significant differences in operative time, amount of drainage, postoperative hospitalization period, hypocalcemia, and vocal cord palsy between the two groups. Cosmetic results of ET group were rated as excellent in a 3-month postoperative questionnaire by 25 (72.43%) of 35 patients. Conclusion: Endoscopic thyroidectomy via the bilateral axillo breast approach can be a feasible and effective option for PTC in selected cases. (Korean J Endocrine Surg 2011;11:158-163)
한국인에서 다발성 내분비선종 증후군의 초기 증상 및 임상 양상
김기호,구민영,허성모,이세경,최준호,이정언,김지수,정재훈<SUP>1<.SUP>,남석진,양정현,김정한,Ki-Ho Kim,M.D.,Min-Young Koo,M.D.,Sung-Mo Hur,M.D.,Se-Kyung Lee,M.D.,Jun-Ho Choe,M.D.,Jeong Eon Lee,M.D.,Jee Soo Kim,M.D.,Jae-Hoon Chung,M.D.<SU 대한갑상선-내분비외과학회 2010 The Koreran journal of Endocrine Surgery Vol.10 No.4
Purpose: Multiple endocrine neoplasia (MEN) syndrome is an inherited, autosomal dominant disease that presents as a combination of several endocrine tumors. Early diagnosis of this syndrome is difficult, because of the nonspecific symptoms and signs. This study analyzed early manifestations and clinical characteristics in patients with MEN syndrome. Methods: Medical records were retrospectively reviewed and telephone interviews were conducted with 35 patients diagnosed as MEN syndrome at Samsung Medical Center from December 1994 to December 2009. Results: The 35 patients had been diagnosed as MEN1 (n=14), MEN2A (n=19) and MEN2B (n=2). The early manifestations of the 14 MEN1 patients were related with hyperparathyroidism (n=5), pituitary tumor (n=3), and pancreatic endocrine tumor (n=2). There were tumors of the parathyroid gland in all 14 patients, anterior pituitary in eight patients, and pancreatic islet cells in seven patients. Four cases were incidentally detected during the screening examination. Six cases harbored a MEN1 gene mutation. The twenty-one patients diagnosed with MEN2 comprised medullary thyroid cancer (n=20), adrenal pheochromocytoma (n=15), and hyperparathyroidism (n=4). The MTC-related symptoms in the 21 MEN2 patients included neck mass or discomfort in 12 patients and pheochromocytoma-related symptoms in seven patients. Two cases were detected through familial genetic screening test. The RET gene mutationwas detected in 19 cases. Conclusion: Early manifestations of MEN syndrome were very different between the types of MEN and the types of its presenting tumor. The early diagnosis and proper management of MEN requires awareness of the clinical characteristics of each expressed tumor and is influenced by genetic screening methods. (Korean J Endocrine Surg 2010; 10:266-275)
원발성 알도스테론증으로 부신 절제술 받은 환자의 수술 후 결과에 대한 분석
류재민,정승필,이정희,김지영,최민영,이세경,길원호,최준호,이정언,김지수,남석진,양정현<SUP>1<.SUP>,김정한,Jai-Min Ryu,M.D.,Seung-Pil Jung,M.D.,Jeong-Hee Lee,M.D.,Ji-Yeong Kim,M.D.,Min-Yeong Choi,M.D.,Se-Kyung Lee,M.D.,Won-Ho Kil,M.D.,Jun-Ho C 대한갑상선-내분비외과학회 2011 The Koreran journal of Endocrine Surgery Vol.11 No.4
Purpose: Primary aldosteronism (PA) is characterized by hypertension (HTN), hypokalemia, suppressed plasma renin activity, and inappropriate aldosterone secretion. The purpose of this study was to analyze postoperative results on blood pressure (BP), and to determine the factors associated with resolution of HTN after adrenalectomy for PA. Methods: One hundred eight patients (66 females and 42 males) with a mean age of 46 years underwent adrenalectomy for PA between January 1, 1996 and September 30, 2009. Their clinical characteristics and biochemical parameters were reviewed retrospectively. Results: All patients had HTN preoperatively and 20 patients (18.1%) had uncontrolled HTN. Hypokalemia was evident in 89.1% of patients, cardiovascular events in 4.5% and cerebrovascular events in 8.2%. There was a significant decrease in both systolic BP and diastolic BP postoperatively, as compared with that before operation. Median systolic BP decreased from 150 mmHg to 125 mmHg at the last follow-up (P<0.01), and median diastolic BP decreased from 93.5 mmHg to 81.5 mmHg (P<0.01). Sixty two (57.4%) patients were cured of HTN and did not require any hypertensive agent, and 38 (35.1%) patients had an improvement in BP control, whereas 9 (8.3%) patients had no change in BP. Univariate analysis showed that duration of HTN and more than two HTN treatment agents were independent factors predicting sustained hypertension after surgery. Conclusion: The duration of HTN and the severity of HTN are factors influencing persistence of HTN after operation for a PA. (Korean J Endocrine Surg 2011;11:276-282)
강보라,김지영,최준호,김지수,남석진,양정현1,김정한,Bo Ra Kang,Jiyoung Kim,Jun-Ho Choe,Jee Soo Kim,Seok Jin Nam,Jung-Hyun Yang1,Jung-Han Kim 대한갑상선-내분비외과학회 2013 The Koreran journal of Endocrine Surgery Vol.13 No.4
Purpose: Because the main drawback of bilateral adrenalectomy is permanent adrenal insufficiency and the subsequent risks of life-long steroid use, adrenal preserving partial adrenalectomy is being accepted as its alternative. The aim of this study is to investigate the indications for bilateral adrenalectomy and to assess the postoperative outcomes and steroid replacement according to operative methods. Methods: From May 1996 through July 2013, a total of 25 patients who underwent bilateral adrenalectomy in our institution were reviewed retrospectively. Surgical outcomes were compared between total and partial adrenalectomy, and postoperative steroid hormone replacement were examined according to the volume of remnant adrenal gland. Results: The median follow-up duration of 25 patients was 55.8 months. The most common indication for bilateral adrenalectomy was bilateral pheochromocytoma (n=16), which was associated with genetic mutation of RET or VHL gene in 11 cases. Cushing's syndrome (n=8) and hyperaldosteronism (n=1) were another indications of bilateral adrenalectomy. Total adrenalectomy was performed in 8 patients and adrenal preserving partial adrenalectomy in 17 patients. Among the 17 patients, only 5 patients needed adrenal hormone replacement and 2 patients had a recurrence at remnant adrenal tissue. Conclusion: Adrenal preserving partial adrenalectomy might be a better option for bilateral adrenal tumor than total adrenalectomy because it can reduce complications associated with adrenal insufficiency and recurrence of the disease is not common.