소아 특발성 중추성 요붕증의 임상경과, 내분비학적 소견 및 뇌자기공명 영상 소견의 경과 관찰

정윤하,정우영 인제대학교 2008 仁濟醫學 Vol.29 No.-

Idiopathic central diabetes insipidus (CDI) can be diagnosed when it occurs in the absence of a cause known to be responsible for diabetes insipidus. Some patients initially diagnosed as idiopathic CDI progressed to brain tumors. We reviewed clinical, endocrinological and brain MRI courses in patients with pediatric idiopathic central diabetes insipidus, to assess the predicting factors for progression to brain tumors. We reviewed the medical records of all patients who had documented cases of idiopathic CDI from 1988 to 2008, and studied clinical, endocrinological and brain MRI courses at diagnosis and during follow up. The patients include 6 males and 5 females. Their mean age at diagnosis was 10.0±4.5(4-19) years and mean follow up duration is 4.3±4.7(0.3-14) years. After mean follow up duration of 4.3±4.7(0.3-14) years, follow up Brain MRI showed brain tumor in 3(27%) patients and no interval change in 4(36%) patients with pituitary stalk thickening and in 3(27%) patients with loss of posterior pituitary signal. 4(27%) patients had growth hormone deficiency and 3(27%) patients had multiple pituitary hormone deficiency. Because some patients of idiopathic CDI progressed to brain tumor, we should follow up more carefully brain MRI and pituitary hormone deficiency.

Differential Ubiquitin Binding by the Acidic Loops of Ube2g1 and Ube2r1 Enzymes Distinguishes Their Lys-48-ubiquitylation Activities

Choi, Yun-Seok,Lee, Yun-Ju,Lee, Seo-Yeon,Shi, Lei,Ha, Jung-Hye,Cheong, Hae-Kap,Cheong, Chaejoon,Cohen, Robert E.,Ryu, Kyoung-Seok American Society for Biochemistry and Molecular Bi 2015 The Journal of biological chemistry Vol.290 No.4

<P>The ubiquitin E2 enzymes, Ube2g1 and Ube2r1, are able to synthesize Lys-48-linked polyubiquitins without an E3 ligase but how that is accomplished has been unclear. Although both E2s contain essential acidic loops, only Ube2r1 requires an additional C-terminal extension (184–196) for efficient Lys-48-ubiquitylation activity. The presence of Tyr-102 and Tyr-104 in the Ube2g1 acidic loop enhanced both ubiquitin binding and Lys-48-ubiquitylation and distinguished Ube2g1 from the otherwise similar truncated Ube2r1<SUP>1–183</SUP> (Ube2r1C). Replacement of Gln-105–Ser-106–Gly-107 in the acidic loop of Ube2r1C (Ube2r1C<SUP>YGY</SUP>) by the corresponding residues from Ube2g1 (Tyr-102–Gly-103–Tyr-104) increased Lys-48-ubiquitylation activity and ubiquitin binding. Two E2∼UB thioester mimics (oxyester and disulfide) were prepared to characterize the ubiquitin binding activity of the acidic loop. The oxyester but not the disulfide derivative was found to be a functional equivalent of the E2∼UB thioester. The ubiquitin moiety of the Ube2r1C<SUP>C93S</SUP>-[<SUP>15</SUP>N]UB<SUP>K48R</SUP> oxyester displayed two-state conformational exchange, whereas the Ube2r1C<SUP>C93S/YGY</SUP>-[<SUP>15</SUP>N]UB<SUP>K48R</SUP> oxyester showed predominantly one state. Together with NMR studies that compared UB<SUP>K48R</SUP> oxyesters of the wild-type and the acidic loop mutant (Y102G/Y104G) forms of Ube2g1, <I>in vitro</I> ubiquitylation assays with various mutation forms of the E2s revealed how the intramolecular interaction between the acidic loop and the attached donor ubiquitin regulates Lys-48-ubiquitylation activity.</P>

발 기형을 동반한 일측성 거대뇌증

정윤하 ( Yun Ha Cheong ),박은아 ( Eun Ah Park ),박규건 ( Kyu Keon Park ),전가원 ( Ga Won Jeon ),신종범 ( Jong Beom Sin ) 대한주산의학회 2008 Perinatology Vol.19 No.3

Hemimegalencephaly is a rare congenital malformation of the cortical development arising from abnormal proliferation of anomalous neuronal and glial cells. The characteristic clinical manifestations are macrocephaly, psycomotor retardation, intractable seizure and hemihypertrophy of face, body and extremities, but musculoskeletal deformities are reported only in case of epidermal nevus syndrome. We report a case of hemimegalencephaly that was associated with foot deformity, without symptoms and signs of epidermal nevus syndrome.

실패한 요추수술에 대한 재수술

하기용,김기원,장정호,원지윤 대한척추외과학회 1997 대한척추외과학회지 Vol.4 No.2

Study Design : A retrospective analysis was performed on 40 patients who had had previous lumbar spine surgeries. Objective : To determine what factors most influenced surgical outcome and to analyze results in a series of revision lumbar surgeries. Summary of Background Data : Satisfactory surgical outcome of the revision lumbar surgery range from 28% to 82% and are rarely comparable to primary surgery. Many factors predicting outcome from repeat lumbar surgery have been listed. Methods : Forty patients were analyzed who had had previous lumbar surgeries. The patients were classified into 5 groups according to diagnosis: 3 Infection, 5 instability, 8 nonunion, 14 HNP and 10 spinal stenosis. Of 40 patients, 33 patients(82.5%) underwent fusion with instrumentation for repeat surgery. Their clinical course was followed for a minimum of 1 year. The number of surgery on each patient was 1.3 times on an average. Results : Overall, 80% of patients had a satisfactory result. Obviously extruded or sequestrated HNP in MRI findings, complete block of contrast with severe radiculopathy and/or myelopathy in spinal stenosis, complete decompression, neurolysis and fusion with instrumentation, and longer than 6 month pain relief after previous surgery were correlated with satisfactory outcome. However, the number of previous operation, age, repair of pseudarthrosis, no abnormality at surgery and combined multiple degenerative joint disease were significantly correlated with poor surgical outcome. The most common complication during repeat surgery was dural tear in 5 cases(12.5%). Conclusions : Success rate of revision surgery was low as compared to primary operation. Therefore, erroneous diagnosis and faulty surgical technique understandably lead to failure, and precise attention to preoperative and intraoperative detail can minimize these sources of error.

복합유기용제 장기 노출자들의 골수충실도 평가를 위한 자기공명영상의 계량적 활용

정해관,최대섭,박건욱,윤환중,김정란,하경임,양승오,김양호 大韓産業醫學會 2002 대한직업환경의학회지 Vol.14 No.2

목 적 : 자기공명영상은 골수의 지방세포와 조혈세포간의 분퐁 차이를 신호강도 차이로 구분할 수 있어 골수 충성도에 대한 평가가 가능하다. 저자들은 유기용제에 장기간 노출된 조선업체 도장공 및 대조군을 대상으로 비참습적인 방법으로 골수 충실도를 평가하는 도구를 개발하기위하여 본 연구를 시행하였다. 방 법 : 1998녀 8월부터 2000년 1월까지 조선소 도장공 중 1개 대학병원에서 유기용제 장기간 사용으로 인한 골수검사 결과 저세포성 골수로 진단 받은 환자 6명(남자 5명, 여자 1명; 평균연령 환자군 46.5세)을 대상으로 말초혈액이 혈액학적 검사, 골수검사 및 요추부 자기공명영상을 실시하였다. 대조군으로는 동일 병원에서 요추부 자기공명영상을 촬영한 환자 132명(남 67명, 여자 65명, 연령 평균 42.5세, 범위 30-59세)을 선정하였다. 요추부 자기공명영상은 T1 및 T2 강조영상의 시상 단면에서 제 12 흉추부터 제 1 천추사이의 추체에서 각반드시 신호강도를 기록하였다. 기준 부위로는 동일 단면의 척추주위근 및 지방조직의 산호강도를 이용하여 각 부위에서 측정한 신호강도를 나누어 골수 신호강도지표를 구하였다. 결 과 : 환자군의 골수 충실도는 20.33-33.6 %이었고 혈액검사상 백혈구수는 2,100-7,600/㎣이었다. 환자군과 대조군 모두에서 각 지표는 제 12흉추에서 아래로 내려갈수록 점차 감소하였다가 제 4 요추 이후 다시 증가하는 양상을 보였다. 환자군과 대조군간의 골수 신호강도지표를 비교하였을 때 T1 강조영상에서 척추주위근을 기준으로 한 골수 신호강도지표가 환자군과 대조군간의 차이를 가장 잘 반영하였으며 환자군이 모든 추체에서 유의하게 높았다. (p<0.05, p<0.01). 대조군의 골수 신호강도지표는 T1 강조영상이 척추주위근을 기준으로 측정하였을 때 여성에서 연령 증가에 따라 모든 측정점에서 골수 신호강도지표는 증가하는 양상을 보였으며 30대와 50대간의 차이는 유의하였다. (p<0.05, p<0.01). 성별에 따른 골수 신호강도지표는 근육을 기준으로 한 골수 신호강도지표에서는 보이지 않았으나 비장을 기준으로 한 골수 신호강도지표는 남녀간에 유의한 차이를 보였다. (p<0.05). 다변량 선형회귀분석에서 연령 및 성별을 보정하였을 때 T1 강조영상의 근육을 기준으로 한 골수 신호강도지표는 환자군에서 대조군에 비하여 제 1 천추에서 0.364 더 높았다. 제 1 천추의 T1 근육을 기준으로 한 골수 신호강도지표의 연령 성별 평균+1 표준편차를 기준으로 하였을 때 6명 중 5명이 그 이상이 분포를 보여 두 군간의 차이가 가장 뚜렷하였다. 결 론 : 저자들은 자기공명영상을 이용하여 골수 충실도를 평가하기 위한 지표를 개발하였고 정상 참고치를 제시하였다. 골수 신호강도지표는 지방조직 함량, 골밀도함께 칼슘 등의 영향을 동시에 반영하지만 이러한 영향을 적절히 보정할 경우 고위험집단에서 골수 충실도를 평가하는데 활용할 수 있음을 확인하였다. 보다 좋은 지표의 개발을 위하여 충분한 표본수를 가진 저세포성 골수 환자집단을 대상으로 후속연구를 수행할 필요가 있다. Objectives : To develop a quantitative and noninvasive method of bone marrow cellularity evaluation in solvent exposed painters. Methods : Six painters (mean 46.5 years, 5 males and one female with hypocellular marrow, and 132 controls were examined using magnetic resonance imaging (MRI). A full examination of the peripheral blood and a bone marrow biopsy was done on each patient. Signal intensities were measured at the vertebral bodies from T12 to S1 on both the T1 and the T2-weighted image (T1WI and T2WI). Signal indices were calculated by dividing the signal indeces of the vertebral bodies by that of the paraspinal muscle and the subcutaneous fat in the same view. Results : The Bone marrow cellularities of the cases painters were between 20.3 % and 33.6 %. Signal indices based on the muscle at T1WI were greater in the cases of the painters compared to those of the controls (p<0.05, p<0.01). Signal indices based on the muscle at T1WI were significantly higher in older women compared with men (p<0.05). After adjusting for age and gender, the signal index of cases at S1 based on muscle of T1WI was higher than that of the controls by 0.364. Five of the six cases had a muscle signal index at S1 of T1WI higher than the mean + 1 standard deviation for the same age group and gender. Conclusions : MR signal indices are influenced by constitutional factors such as fat content. bone density, and the presence of other pathology. However, after adequate adjustment, it can be used as a useful indicator of bone marrow cellularity in a high-risk population.

Hebei Spirit Oil Spill Exposure and Subjective Symptoms in Residents Participating in Clean-Up Activities

Cheong, Hae-Kwan,Ha, Mi-Na,Lee, Jong-Seong,Kwon, Ho-Jang,Ha, Eun-Hee,Hong, Yun-Chul,Choi, Ye-Yong,Jeong, Woo-Chul,Hur, Jong-Il,Lee, Seung-Min,Kim, Eun-Jung,Im, Ho-Sub The Korean Society of Environmental Toxicology 2011 환경독성보건학회지 Vol.26 No.-

Objectives: This study was conducted to examine the relationship between crude oil exposure and physical symptoms among residents participating in clean-up work associated with the Hebei Spirit oil spill, 2007 in Korea. Methods: A total of 288 residents responded to a questionnaire regarding subjective physical symptoms, sociodemographic characteristics and clean-up activities that occurred between two and eight weeks after the accident. Additionally, the urine of 154 of the respondents was analyzed for metabolites of volatile organic compounds (VOCs) and polycyclic aromatic hydrocarbons (PAHs) and heavy metals. To compare the urinary levels of exposure biomarkers, the urine of 39 inland residents who were not directly exposed to the oil spill were analyzed. Results: Residents exposed to oil remnants through clean-up work showed associations between physical symptoms and the exposure levels defined in various ways, including days of work, degree of skin contamination, and levels of some urinary exposure biomarkers of VOCs, metabolites and metals, although no major abnormalities in urinary exposure biomarkers were observed. Conclusions: This study provides evidence of a relationship between crude oil exposure and acute human health effects and suggests the need for follow-up to evaluate the exposure status and long-term health effects of clean-up participants.

한ㆍ중 기생충 협력 사업 참관기

윤청하,Yun, Cheong-Ha 한국건강관리협회 1998 건강소식 Vol.22 No.12

Performance Evaluation of a RAM based Storage System NGS

Kang, Yun-Hee,Kung, Jae-Ha,Cheong, Seung-Kook The Korea Contents Association 2009 International Journal of Contents Vol.5 No.4

Recently high-speed memory array based on RAM, which is a type of solid-state drive (SSD), has been introduced to handle the input/output (I/O) bottleneck. But there are only a few performance studies on RAM based SSD storage with regard to diverse workloads. In this paper, we focus on the file system for RAM based memory array based NGS (Next Generation Storage) system which is running on Linux operating system. Then we perform benchmark tests on practical file systems including Ext3, ReiserFS, XFS. The result shows XFS significantly outperforms other file systems in tests that represent the storage and data requests typically made by enterprise applications in many aspects. The experiment is used to design the dedicated file system for NGS system. The results presented here can help enterprises improve their performance significantly.

AIDS 상담소 운영 및 내용분석

윤청하,Yun, Cheong-Ha 한국건강관리협회 1987 건강소식 Vol.11 No.8

한국건강관리협회의 국제협력사업 '한ㆍ라오스 기생충퇴치사업'

윤청하,Yun, Cheong-Ha 한국건강관리협회 2003 한국건강관리협회지 Vol.1 No.1

The NGO program of "intestinal parasite Control among primary Schoolchildren of Lao PDR", supported by KOICA and implemented by KAHP, has started in 1999 and will be executed until 2004. Under cooperation of the LAo Ministry of Health, WHO, Korean Embassy and KOCIA, for the last 4 years, KHIP has conducted parasitological survey throughout the country, supply of medical equipment and anti-helminthics and treatment of positive people. The results of the survey is as below : A total of 29,846 stool specimens were collected from primary schoolchildren from May 2000 to June 2002 and examined once with the cellphone thick smear. By species, the rate for Ascaris lumbricoides was 34.9%, hookworm 19.1%, Trichuris trihiura 25.8%, Opisthorchis iverrini 10.9%, Taenia spp. 0.6% and Hymenolepis spp. 0.1%. The northern mountainous regions showed a higher prevalence of soil-transmitted helminths. The regions along the Mekong River such as Khammuane, Saravane or Svannakhet Province showed a higher prevalence of fish-borne parasites.