급성림프구성백혈병에서 면역조직화학염색에 의한 p16 단백질 소실의 의의

진혜영,강경인,김선영,윤유숙,강준원,조덕연,권계철,박경덕 대한소아청소년과학회 2008 Clinical and Experimental Pediatrics (CEP) Vol.51 No.1

Purpose:p16 gene, mapped to the 9p21 chromosomal region, has emerged as a candidate tumor suppressor gene in human neoplasm. It is an inhibitor of cyclin-dependent kinase and inhibits Rb phosphorylation. In a variety of tumors including childhood acute lymphoblastic leukemia (ALL), deletion and/or mutation of the p16 gene has been found. Despite their high frequency, the prognostic importance of p16 alterations is still controversial in ALL and has been reported to be either unfavorable or similar to that of other patients. We studied the correlation between loss of p16 protein confirmed by immunohistochemical staining and clinical outcomes of patients diagnosed as ALL. Methods:We performed an immunohistochemical staining for p16 protein in 74 cases of bone marrow biopsy slide initially diagnosed as ALL between January 1998 and December 2006. We reviewed the clinical manifestations, laboratory findings, treatment outcomes retrospectively. Results:Of 74 slides, 12 were negative for p16 protein. Seven were males and 5 were females with a median age at diagnosis was 5.8 (1.3-18.8) years. Initial WBC were 17,225 (500-403,300)/µL. By immunologic surface marker analysis, 7 patients were early pre-B CALLA (+) and 5 patients were T-cell ALL. Two patients of intermediate risk group had relapsed and died. Three patients had family history of breast cancer. Four patients died and overall survival rates were 53.5±18.7%. Conclusion:Loss of p16 protein is supposed to be an independent risk factor of childhood ALL associated with poor outcomes. In clinical setting, the clinician must take into account p16 status, not only at the genomic but also at the protein level. Further clinical experience on thoroughly investigated cases will help a better understanding between p16 status and clinical outcomes. (Korean J Pediatr 2008;51:73-77) Purpose:p16 gene, mapped to the 9p21 chromosomal region, has emerged as a candidate tumor suppressor gene in human neoplasm. It is an inhibitor of cyclin-dependent kinase and inhibits Rb phosphorylation. In a variety of tumors including childhood acute lymphoblastic leukemia (ALL), deletion and/or mutation of the p16 gene has been found. Despite their high frequency, the prognostic importance of p16 alterations is still controversial in ALL and has been reported to be either unfavorable or similar to that of other patients. We studied the correlation between loss of p16 protein confirmed by immunohistochemical staining and clinical outcomes of patients diagnosed as ALL. Methods:We performed an immunohistochemical staining for p16 protein in 74 cases of bone marrow biopsy slide initially diagnosed as ALL between January 1998 and December 2006. We reviewed the clinical manifestations, laboratory findings, treatment outcomes retrospectively. Results:Of 74 slides, 12 were negative for p16 protein. Seven were males and 5 were females with a median age at diagnosis was 5.8 (1.3-18.8) years. Initial WBC were 17,225 (500-403,300)/µL. By immunologic surface marker analysis, 7 patients were early pre-B CALLA (+) and 5 patients were T-cell ALL. Two patients of intermediate risk group had relapsed and died. Three patients had family history of breast cancer. Four patients died and overall survival rates were 53.5±18.7%. Conclusion:Loss of p16 protein is supposed to be an independent risk factor of childhood ALL associated with poor outcomes. In clinical setting, the clinician must take into account p16 status, not only at the genomic but also at the protein level. Further clinical experience on thoroughly investigated cases will help a better understanding between p16 status and clinical outcomes. (Korean J Pediatr 2008;51:73-77)

Hepatic glycogenosis in a patient with poorly controlled type 1 diabetes mellitus

진혜영,강대영,최진호 대한소아청소년과학회 2009 Clinical and Experimental Pediatrics (CEP) Vol.52 No.11

Hepatomegaly and liver dysfunction might develop in patients with diabetes mellitus due to glycogen deposition or nonalcoholic steatohepatitis. We experienced a case of hepatic glycogenosis in a patient with type 1 diabetes mellitus who presented with recurrent hypoglycemia, suggesting impairment of glycogenolysis and gluconeogenesis. A 10-year-old girl with a 4-year history of type 1 diabetes mellitus was admitted because of recurrent hypoglycemia and abdominal pain in the right upper quadrant. She had Cushingoid features and hepatomegaly that extended 6 cm below the right costal margin. Laboratory data and radiologic examination revealed elevated liver enzyme levels due to fatty liver. Periodic acid-Schiff (PAS) staining revealed intense glycogen deposition in the cytoplasm of the hepatocytes and PAS reactivity was lost with diastase treatment. At 2 months after administration of glucagon injection and uncooked cornstarch between meals and at bedtime, the hypoglycemic episodes and liver dysfunction improved. It is important to distinguish hepatic glycogenosis from steatohepatitis, because it is possible to prevent excessive hepatic glycogen storage in hepatic glycogenosis cases by strictly controlling blood glucose level and by glucagon administration. To prevent severe hypoglycemic symptoms accompanied by hepatic glycogenosis, we suggest that uncooked cornstarch, which is effective in maintaining blood glucose level, can also be administered.

성인 터너증후군 환자에서 당뇨병과 고혈압 발생의 예측 인자에 관한 연구

진혜영,이범희,유한욱,최진호 대한소아내분비학회 2010 Annals of Pediatirc Endocrinology & Metabolism Vol.15 No.3

Purpose:The risk of cardiovascular diseases (CVD) including hypertension, artherosclerosis and endocrinologic diseases such as thyroid disease, obesity, diabetes mellitus are elevated in adult Turner syndrome (TS). Clinical and biochemical parameters of TS patients were analyzed to investigate the predictive factors of diabetes and hypertension. Methods:Twenty nine patients with TS were enrolled in this study. Clinical and biochemical parameters including height, weight, body mass index (BMI), blood pressure, waist and hip circumferences, lipid profile, insulin, fasting glucose, and HbA1c were retrospectively reviewed. Results:Seven (24.1%) out of 29 had either impaired fasting glucose (IFG) or diabetes, 3 (10.3%) had central obesity, 5 (17.2%) had hypertriglyceridemia, 4 (13.8%) had low HDL cholesterol, 6 (20.7%) had hypertension, and 3 (10.3%) out of 29 were consistent with the criteria of MS. The waist circumference was longer in patients with either IFG or diabetes (67.1±5.4 cm vs 79.4±8.3 cm, P=0.009). Insulin, homeostasis model assessment-insulin resistance (HOMA-IR), and adipokine levels were not significantly different according to the presence of either IFG or diabetes. Among TS patients, BMI was higher in patients with hypertension (26.2±5.7 kg/m2 vs 22.5±3.1 kg/m2, P=0.042). Total cholesterol exhibited a significant difference between patients with hypertension and those without hypertension (177.3±29.6 mg/dL vs 222.8±28.5 mg/dL, P=0.004). Conclusion:Adult TS patients should be carefully followed regarding the development of diabetes and hypertension in case of severe central obesity and dyslipidemia. 목 적:성인 터너증후군에서 갑상선 질환, 비만, 당뇨병과 같은 내분비 질환을 비롯하여 고혈압, 동맥경화증 같은 심혈관계 질환의 빈도가 높다. 본 연구는 터너증후군 환자의 임상적, 생화학적 지표를 분석하여 당뇨병 및 고혈압의 발생 가능성에 대한 예측 인자를 알아보고자 하였다. 방 법:서울아산병원에서 추적 중인 18세 이상의 터너증후군 29명을 대상으로 하였다. 의무기록을 통해 대상 환자들의 신체 계측치 등의 임상지표와 혈중 인슐린, 당화 혈색소, 혈중 지질 농도 등의 생화학적 지표들을 분석하였다. 결 과:환자군의 24.1%(7명)가 공복혈당장애(6명) 또는 당뇨병(1명)이 있었고 10.3%(3명)가 복부비만, 17.2%(5명)가 고중성지방혈증, 13.8%(4명)가 저밀도지단백콜레스테롤혈증, 20.7%(6명)가 고혈압이 있어, 10.3%(3명)가 대사증후군에 합당하였다. 공복혈당장애 또는 당뇨병이 있는 환자군에서 허리둘레가 유의하게 증가하여 있었다(67.1± 5.4 vs 79.4± 8.3, P=0.009). 혈중 인슐린, HOMA-IR, 렙틴과 아디포넥틴은 두 군간에 유의한 차이를 보이지 않았다. 환자군 중에서 혈압이 높은 군에서 체질량지수가 더 높았다(26.2± 5.7 vs 22.5± 3.1, P=0.042). TC, LDL-C, TG 농도 모두 혈압이 높은 군에서 높았으나, TC농도만 통계적으로 유의한 차이를 보였다(P=0.004, 0.070, 0.177). 결 론:터너증후군 환자들에서 복부 비만의 정도가 심한 경우 또는 비만의 정도가 심하고 혈중 콜레스테롤 수치가 높은 경우 공복혈당장애나 당뇨병 및 고혈압 같은 심혈관 질환에 대한 적극적인 감시, 예방 및 치료가 중요하겠다.

한·중 면세점 사업의 비교 연구

진혜영,장로,이상학 한국제도경제학회 2019 제도와 경제 Vol.13 No.3

면세점 사업은 출국자에게 보세판매장, 즉 면세점에서 면세 제품을 판매하는 사업이다. 소득증가 및 항공 요금의 인하 등에 힘입어 한국과 중국을 방문하는 관광객 및 한·중 양국의 해외여행객이 증가하 면서 양국 면세점의 매출은 빠르게 증가하고 있다. 본 논문은 한·중 양국의 면세점 사업의 현황과 제 도를 비교 분석하였다. 특히 한·중 양국의 면세점 매출의 증가 요인에 대해 ‘변이-할당 분석’을 실시 하고 이로부터 시사점을 도출하였다. 분석결과, 한·중 양국의 면세점 사업이 세계 및 아시아·태평양 지역보다 빠르게 성장하여 경쟁력 있는 산업임을 확인하였다. 또한 한·중 양국의 면세점 사업과 제도 를 비교하여 유사점과 차이점을 확인하고, 동반 성장이 가능한 정책을 제안하였다. 본 논문의 연구결과 는 한·중 양국의 면세점 사업의 지속적 발전을 위한 정책 설계에 기초자료로 활용될 수 있을 것이다. The duty-free shop business is a business that sells duty-free products at duty-free shops, which are bonded shops, to domestic residents and foreigners who travel to other countries. Thanks to the increase in income and spread of low-cost airlines, the number of tourists visiting Korea and China and the number of tourists in both Korea and China have increased rapidly, resulting in a rapid increase in sales at duty-free shops in both countries. This paper compares the duty-free shops of Korea and China. In particular, we conduct a shift-share analysis on the growth factors of duty-free shops in Korea and China, respectively, and draw implications from them. It is confirmed that Korean and Chinese duty-free businesses are more competitive than those in the rest of the world and Asia-Pacific region. In addition, this paper compares the duty-free policies of Korea and China to identify similarities and differences, and suggest policies for mutual growth of duty-free businesses. The results of this study can be used as a basis for policy development for sustainable development of duty-free business in Korea and China.

철모깍지벌레(Saissetia coffeae)에 대한 애홍점박이무당벌레(Chilocorus kuwanae)의 포식능력

진혜영,안태현,이봉우,전혜정,이준석,박종균,함은혜,Jin, Hye Young,Ahn, Tai Hyeon,Lee, Bong Woo,Jun, Hye Jeong,Lee, Jun Seok,Park, Jong Kyun,Ham, Eun Hye 한국응용곤충학회 2015 한국응용곤충학회지 Vol.54 No.4

This study, examined Chilocorus kuwanae for biological control of Saissetia coffeae. We measured basic developmental characteristics of C. kuwanae and its capability to prey on the second-instar larvae of S. coffeae by indoor breeding under the following conditions: $25^{\circ}C{\pm}2^{\circ}C$, humidity $70%{\pm}5%$, and day length 16L : 8D. The average daily consumption of the second-instar larvae of S. coffeae by C. kuwanae adults was approximately 77.5 larvae/day, which was significantly 2.8 and 2.9 times higher than consumption by Chrysoperla carnea and Cryptolaemus montrouzieri, respectively. During the nymphal period, C. kuwanae consumed a total of 659.0 second-instar larvae of S. coffeae. The interaction between C. kuwanae and the second-instar larvae of S. coffeae varied according to their densities, with the increase in C. kuwanae consumption rate noted to decelerate and gradually level off at the maximum, rsembling Holling's Type II functional response. Duration of egg to adult development varied from 21.1 days to nearly 27.9 days at temperatures of $25^{\circ}C{\pm}2^{\circ}C$. Hatchability, pupation rate, and adult emergence were greater than 72.3%, 77.8% and 83.3%, respectively.

한국의 민가정원 - 경상도 일대의 민가를 중심으로

진혜영,정미애,이경미,송수정,조민지,김현지,최유림,윤보민,안병주,이문규,심지연,이혜숙,진민령,정명석,이원호,이창훈,김동현,신현실 국립수목원 2019 한국의 민가정원 Vol.2019 No.0

A Basic Study on the Characteristics of the Modern Garden in Gunsan during the Opening Period - Focused on Sagawa House -

진혜영,신현실 한국전통조경학회 2020 한국전통조경학회지 Vol.영문 No.18

This study analyzed the process of forming and changing of the concession territory around Yeonghwa-dong in Gunsan to examine the formation process of the modern garden, the contents of the construction of Sagawa House, and found the following conclusions. First, the formation of Gunsan concession territory was made in 1899 with the signing of 『Concessions of Gunsan, Masan, and Seongjin』. Before the opening of the port, Gunsan was a small village that was used as a passageway for some merchants, but after the opening, Regulations for the Foreign Settlements were formed. As the concession territory was established, the city was formed under the leadership of the Japanese people. During the Japanese colonial period, industrial facilities were built in Gunsan urban area, which was expanded around the open port, as the port function was strengthened through the coastal reclamation work and the Gunsan urban area was expanded. However, with the withdrawal of Japanese landowners and their capital after Korea’s liberation from Japan, the development of the existing Gunsan urban area became stagnant, and modern gardens were distributed in the surrounding areas of the central area, which was formed as a concession territory at that time. Second, as a result of examining the construction process of garden for Sagawa House, the construction time of Sagawa House is not clear, but it is said that it was constructed when a Japanese ran a pawnshop in the Japanese colonial period. The garden is believed to have been built together at the time of the construction of the house, and the owner has been changed twice since then, but the garden has continued to look the same. Sagawa house is located in a densely populated Japanese residential area located behind the old city hall, which is believed to have been to Nagaya-style houses(terraced house) in the past. Third, Sagawa house today have gardens in front and back of Japanese-style wooden house on a rectangular site. The front garden facing the road has a semi-pyeongjeong-style alpine garden with a low level in the topography, and a stepping stone is placed to enter the house. Chōzhivachi is placed with evergreen trees, and a Japanese stone lantern and a stone pagoda are placed around the pond in front garden. A pyeongjeong-style garden centered on pine trees is built on a three-tiered flower bed built with natural stones in the courtyard behind the house. And the shrubs are planted in a row along the border of the layer, allowing visitors to view the courtyard along the corridor inside the house.

한국의 민가정원 - 전라도 일대의 민가를 중심으로

진혜영,이경미,진민령,심지연,정명석,이문규,최유림,강보미,이혜숙,이원호,이창훈,김동현,하태일,최지이,안준영,전다슬,신현실 국립수목원 2020 한국의 민가정원 Vol.2020 No.0

Transient neonatal diabetes mellitus with macroglossia diagnosed by methylation specific PCR (MS-PCR)

진혜영,최진호,김구환,유한욱 대한소아청소년과학회 2010 Clinical and Experimental Pediatrics (CEP) Vol.53 No.3

Transient neonatal diabetes mellitus (TNDM) has been associated with paternal uniparental isodisomy of chromosome 6, paternally inherited duplication of 6q24, or a methylation defect at a CpG island of the ZAC or HYMAI gene. We experienced a case of TNDM in which the patient presented with hyperglycemia, macroglossia, and intrauterine growth retardation, caused by a paternally derived HYMAI. An 18-day-old female infant was admitted to the hospital because of macroglossia and recurrent hyperglycemia. In addition to the macroglossia, she also presented with large fontanelles, micrognathia, and prominent eyes. Serum glucose levels were 200–300 mg/dL and they improved spontaneously 2 days after admission. To identify the presence of a maternal methylated allele, bisulfite-treated genomic DNA from peripheral blood was prepared and digested with BssHII after polymerase chain reaction (PCR) amplification with methylation-specific HYMAI primers. PCR and restriction fragment length polymorphism analysis showed that the patient had only the paternal origin of the HYMA1 gene. TNDM is associated with a methylation defect in chromosome 6, suggesting that an imprinted gene on chromosome 6 is responsible for this phenotype.

진성 성조숙증에서 GnRH agonist의 용량에 따른 최종예측 성인신장 개선 효과의 차이에 관한 연구

진혜영,최진호,유한욱 대한소아내분비학회 2010 Annals of Pediatirc Endocrinology & Metabolism Vol.15 No.2

Purpose:Depot gonadotropin releasing hormone agonist (GnRHa) has been widely used for the treatment of central precocious puberty (CPP). The effectiveness of GnRHa depends on potency, dosage, route of administration, duration of treatment, and compliance. There is a controversy about optimal doses of GnRHa. This study aimed to compare the efficacy of GnRHa on improvement of PAH in girls with CPP using two different dosages. Methods:Seventy-one girls with CPP were included in this study. The patients were categorized into two groups depending on the dosage of GnRHa; group 1 (80-100 μg/kg/4 wk) and group 2 (100-110 μg/kg/4 wk). The clinical parameters such as chronological age (CA), bone age (BA), duration of treatment, height, growth velocity, predicted adult height (PAH), and endocrine assay including GnRH stimulation test, luteinizing hormone, follicle stimulating hormone, and E2 at the start and the end of treatment were obtained to analyze the efficacy of GnRHa according to dosage. Results:CA and BA at the start of treatment were 8.2±1.26 yr and 10.7±0.61 yr, while CA and BA at the end of treatment were 10.7±0.61 yr and 11.7±0.50 yr, respectively. CA and BA before and after treatment, and duration of treatment were similar between two groups. BA/CA, Height SDS, growth velocity (cm/r) were decreased while PAH was significantly increased in both groups (P˂0.05). There were no significant differences inΔPAH,ΔBA/CA,ΔHeight SDS and growth velocity between two groups (P˃0.05). Conclusion:Higher dose (100-110 μg/kg/4 wk) of GnRHa group was not associated with higher PAH in girls with CPP, compared with the group with dose of 80-100 μg/kg/4 wk. Therefore, the results indicates that the dose of 80-100 μg/kg/4 wk is sufficient for treatment of CPP, necessitating further study with lesser dose to identify the optimal dose. 목 적:진성 성조숙증의 치료에서 GnRHa는 효과적이고 안전한 약제로 널리 이용되고 있다. GnRHa 치료의 효과는 약제의 역가, 용량, 투여 경로, 투여 기간 및 순응도에 따라 달라질 수 있는데 적정 용량에 대해서는 아직까지 논란이 되고 있다. 본 연구에서는 두 가지 다른 용량으로 치료받은 진성 성조숙증 여아에서 GnRH agonist가 최종성인예측신장에 미치는 치료 효과의 차이를 분석해 보고자 하였다. 방 법:총71명의 진성 성조숙증 여아를 대상으로 하여 의무기록을 후향적으로 분석하였다. GnRHa의 용량에 따라 Group 1 (80-100 μg/kg/4주) 37명(52.1%), Group 2 (100-110 μg/kg/4주) 34명(47.9%)으로 분류하였으며 치료 시작과 종료시의 역연령과 골연령, 신장, 치료 전후 1년간의 성장 속도, 예측성인신장, GnRH자극검사, FSH, LH, E2를 포함한 호르몬 검사 수치를 조사하여 GnRHa의 용량에 따른 최종성인예측신장 개선 효과를 분석하였다. 결 과:역연령과 골연령은 치료 전에 각각 8.2±1.26세, 10.7±0.61세였으며 치료 후에 각각 10.7±0.61세, 11.7±0.50세로 골연령/역연령의 비가 감소하였다. 치료 용량에 따른 두 군 간의 치료 전후의 역연령, 골연령, 치료 기간의 차이는 없었다. 두 군 모두에서 치료 후 골연령/역연령 비와 Height SDS, 성장 속도가 의미있게 감소하였으며 예측성인신장은 증가하였다(P˂0.05). ΔPAH, ΔBA/CA, ΔHeight SDS와 치료 후 성장속도는 두군 사이에 유의한 차이를 보이지 않았고 GnRHa의 용량에 따른 유의한 상관관계를 보이지 않았다(P˃0.05). 결 론:GnRHa 용량의 증가에 따른 예측 성인 신장의 증가는 보이지 않아 80-100 μg/kg의 용량이 진성 성조숙증의 치료에 적절한 것으로 사료된다. 향후 많은 수의 환자를 대상으로 사춘기의 진행을 억제하면서 사춘기 전의 성장 속도를 유지할 수 있는 최소 용량에 대한 연구가 필요하겠다.