• 제목/요약/키워드: 인자 중요도

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A Case of Urologic Manifestation of IARS2-associated Leigh Syndrome (IARS2 유전자 연관 리 증후군(Leigh syndrome) 여아에서 방광기능장애 증례)

  • Hyunjoo Lee;Ji-Hoon Na;Young-Mock Lee
    • Journal of The Korean Society of Inherited Metabolic disease
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    • 제23권1호
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    • pp.25-30
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    • 2023
  • Leigh syndrome is a rare progressive neurodegenerative mitochondrial disorder with clinical and genetic heterogeneity. Recently, balletic IARS2 variants have been identified in a number of patients presenting broad clinical phenotypes from Leigh and West syndrome to a rare syndrome CAGSSS characterized by cataracts, growth hormone deficiency, sensory neuropathy, sensorineural hearing loss, and skeletal dysplasia syndrome (OMIM#616007). We describe a child with Korean Leigh syndrome with urologic manifestations resulting from a compound heterozygote mutation in IARS2. A 5-year-old girl visited the emergency room with a complaint of abdominal pain accompanied by abdominal distension. Abdominal-pelvic CT showed a markedly distended urinary bladder without definite obstructive lesions. She was diagnosed with neurogenic bladder dysfunction based on a urodynamic study. She had global delayed development due to neurologic regression after 6 months of age and a history of bilateral cataract surgery at the age of 2 years. Her brain magnetic resonance imaging showed symmetrically increased signal intensities in the bilateral putamen and caudate nuclei with diffuse cerebral atrophy. No gene variants were identified through whole-mitochondrial genome analysis. Whole exome sequencing was performed for diagnosis, and compound heterozygous pathogenic variants were identified in IARS2: c.2446C>T (p. Arg816Ter) and c.2450G>A (p. Arg817His). To the best of our knowledge, this is the first case report of bladder dysfunction manifestation in a patient with IARS2-related Leigh syndrome. Thus, it broadens the clinical and genetic spectrum of IARS2-associated diseases.

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Effect of Accelerated Storage on the Microstructure and Water Absorption Characteristics of Korean Adzuki Bean (Vigna angularis L.) Cultivar (팥의 가속화 저장에 따른 미세구조 및 수분흡수 특성)

  • Jieun Kwak;Seon-Min Oh;You-Geun Oh;Yu-Chan Choi;Hyun-Jin Park;Suk-Bo Song;Jeong-Heui Lee;Jeom-Sig Lee
    • KOREAN JOURNAL OF CROP SCIENCE
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    • 제68권3호
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    • pp.167-174
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    • 2023
  • This study investigated the microstructure and water absorption characteristics of the Korean adzuki bean (Vigna angularis L.) cultivar under accelerated storage. The germination rate, acid value, redness (a*), and yellowness (b*) values showed no significant differences after three months of storage compared to pre-storage under low temperatures (4℃). However, a statistically significant difference was observed under accelerated high temperatures (45℃). In particular, after storage for three months, the germination rate and acid value were 0% and 33.63 mg KOH/100g, respectively, under accelerated high temperatures. After storage for three months, the holes, hilum damage, and spaces between the seed coat and cotyledon shortened the time and speed of water absorption under accelerated high temperatures compared to that under low temperatures. Conversely, further research is required to investigate the reason for the low rate of parallel water absorption.

Evaluation of Applicability of Sea Ice Monitoring Using Random Forest Model Based on GOCI-II Images: A Study of Liaodong Bay 2021-2022 (GOCI-II 영상 기반 Random Forest 모델을 이용한 해빙 모니터링 적용 가능성 평가: 2021-2022년 랴오둥만을 대상으로)

  • Jinyeong Kim;Soyeong Jang;Jaeyeop Kwon;Tae-Ho Kim
    • Korean Journal of Remote Sensing
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    • 제39권6_2호
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    • pp.1651-1669
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    • 2023
  • Sea ice currently covers approximately 7% of the world's ocean area, primarily concentrated in polar and high-altitude regions, subject to seasonal and annual variations. It is very important to analyze the area and type classification of sea ice through time series monitoring because sea ice is formed in various types on a large spatial scale, and oil and gas exploration and other marine activities are rapidly increasing. Currently, research on the type and area of sea ice is being conducted based on high-resolution satellite images and field measurement data, but there is a limit to sea ice monitoring by acquiring field measurement data. High-resolution optical satellite images can visually detect and identify types of sea ice in a wide range and can compensate for gaps in sea ice monitoring using Geostationary Ocean Color Imager-II (GOCI-II), an ocean satellite with short time resolution. This study tried to find out the possibility of utilizing sea ice monitoring by training a rule-based machine learning model based on learning data produced using high-resolution optical satellite images and performing detection on GOCI-II images. Learning materials were extracted from Liaodong Bay in the Bohai Sea from 2021 to 2022, and a Random Forest (RF) model using GOCI-II was constructed to compare qualitative and quantitative with sea ice areas obtained from existing normalized difference snow index (NDSI) based and high-resolution satellite images. Unlike NDSI index-based results, which underestimated the sea ice area, this study detected relatively detailed sea ice areas and confirmed that sea ice can be classified by type, enabling sea ice monitoring. If the accuracy of the detection model is improved through the construction of continuous learning materials and influencing factors on sea ice formation in the future, it is expected that it can be used in the field of sea ice monitoring in high-altitude ocean areas.

Ameliorative Effects of Soybean Leaf Extract on Dexamethasone-Induced Muscle Atrophy in C2C12 Myotubes and a C57BL/6 Mouse Model (콩잎 추출물의 근위축 개선 효과)

  • Hye Young Choi;Young-Sool Hah;Yeong Ho Ji;Jun Young Ha;Hwan Hee Bae;Dong Yeol Lee;Won Min Jeong;Dong Kyu Jeong;Jun-Il Yoo;Sang Gon Kim
    • Journal of Life Science
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    • 제33권12호
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    • pp.1036-1045
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    • 2023
  • Sarcopenia, a condition characterized by the insidious loss of skeletal muscle mass and strength, represents a significant and growing healthcare challenge, impacting the mobility and quality of life of aging populations worldwide. This study investigated the therapeutic potential of soybean leaf extract (SL) for dexamethasone (Dexa)-induced muscle atrophy in vitro and in an in vivo model. In vitro experiments showed that SL significantly alleviated Dexa-induced atrophy in C2C12 myotube cells, as evidenced by preserved myotube morphology, density, and size. Moreover, SL treatment significantly reduced the mRNA and protein levels of muscle RING-finger protein-1 (MuRF1) and muscle atrophy F-box (MAFbx), key factors regulating muscle atrophy. In a Dexa-induced atrophy mouse model, SL administration significantly inhibited Dexa-induced weight loss and muscle wasting, preserving the mass of the gastrocnemius and tibialis anterior muscles. Furthermore, mice treated with SL exhibited significant improvements in muscle function compared to their counterparts suffering from Dexa-induced muscle atrophy, as evidenced by a notable increase in grip strength and extended endurance on treadmill tests. Moreover, SL suppressed the expression of muscle atrophy-related proteins in skeletal muscle, highlighting its protective role against Dexa-induced muscle atrophy. These results suggest that SL has potential as a natural treatment for muscle-wasting conditions, such as sarcopenia.

Research on Immune Responses Induced by Salmonella Typhimurium Infectionin CRIP1-Deficient Condition (CRIP1결손조건 하에서 Salmonella Typhimurium 감염에 의해 유도되는 면역반응에 관한 연구)

  • Dongju Seo;Se-Hui Lee;Sun Park;Hyeyun Kim;Jin-Young Yang
    • Journal of Life Science
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    • 제34권1호
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    • pp.48-58
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    • 2024
  • Salmonella is a common food-borne intracellular bacterial pathogen that has triggered significant public health concerns. Salmonella hosts' genetic factors play a pivotal role in determining their susceptibility to the pathogen. Cysteine-rich intestinal protein 1 (CRIP1), a member of LIM/double zinc finger protein family, is widely expressed in humans, such as in the lungs, spleen, and especially the gut. Recently, CRIP1 has been reported as a key marker of several immune disorders; however, the effect of CRIP1 on bacterial infection remains unknown. We aimed to elucidate the relationship between Salmonella infection and CRIP1 gene deficiency, as Salmonella spp. is known to invade the Peyer's patches of the small intestine, where CRIP1 is highly expressed. We found that CRIP1-deficient conditions could not alter the characteristics of bone marrow-derived myeloid cells in terms of phagocytosis on macrophages and the activation of costimulatory molecules on dendritic cells using ex vivo differentiation. Moreover, flow cytometry data showed comparable levels of MHCII+CD11b+CD11c+ dendritic cells and MHCII+F4/80+CD11b+ macrophages between WT and CRIP1 knockout (KO) mice. Interestingly, the basal population of monocytes in the spleen and neutrophils in MLNs is more abundant in a steady state of CRIP1 KO mice than WT mice. Here, we demonstrated that the CRIP1 genetic factor plays dispensable roles in host susceptibility to Salmonella Typhimurium infections and the activation of myeloid cells. In addition, differential immune cell populations without antigen exposure in CRIP1 KO mice suggest that the regulation of CRIP1 expression may be a novel immunotherapeutic approach to various infectious diseases.

Effects of the Type of Exchanged Ions and Carbon Precursors on Methane Adsorption Behavior in Zeolite Templated Carbons Synthesized Using Various Ion-Exchanged Faujasite Zeolites (이온교환된 Faujasite 제올라이트를 이용한 제올라이트 주형 탄소체 합성 시 이온 교환 금속과 탄소 전구체가 메탄 흡착 거동에 미치는 영향)

  • Ki Jun Kim;Churl-hee Cho;Dong-Woo Cho
    • Clean Technology
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    • 제30권2호
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    • pp.123-133
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    • 2024
  • Zeolite template carbon (ZTC) was synthesized as an adsorbent to remove low-concentration CH4 from the atmosphere. The synthesis of ZTC was performed using CH4 and C2H2 as carbon precursors and their impact on adsorption was investigated. ZTC was also synthesized using Y zeolite ion-exchanged with CaCl2 and LiCl as templates to investigate the effect of using metals in ion exchange. The comparison of the carbon precursors revealed that C2H2 had a higher carbon yield than CH4. The synthesized ZTC exhibited developed micropores due to carbon deposition deep inside the micropores of the zeolite template. The kinetic diameter of C2H2 (0.33 nm) is smaller than that of CH4 (0.38 nm), which allowed for its deposition. The study compared metal precursors used for ion exchange and confirmed that the CaCl2-based ZTC developed more micropores compared to the LiCl-based ZTC. The ion-exchanged Ca inhibited pore blocking by the carbon precursor, allowing it to enter the pores. The ability of synthesized ZTC to adsorb N2 and CH4 at 298 K was investigated. The results showed that CH4 had a higher overall adsorption amount than N2. The sample synthesized using C2H2 and CaY exhibited the highest N2 and CH4 adsorption capacity. However, the sample synthesized with CH4 had the highest CH4/N2 gas uptake ratio, which is a crucial factor in designing an adsorption process. The observed difference was likely caused by the underdevelopment of ultrafine pores that are associated with N2 adsorption. This resulted in a reduction of N2 adsorption, leading to an increase in CH4/N2 separation.

The Patterns of Change in Arterial Oxygen Saturation and Heart Rate and Their Related Factors during Voluntary Breath holding and Rebreathing (자발적 호흡정지 및 재개시 동맥혈 산소포화도와 심박수의 변동양상과 이에 영향을 미치는 인자)

  • Lim, Chae-Man;Kim, Woo-Sung;Choi, Kang-Hyun;Koh, Youn-Suck;Kim, Dong-Soon;Kim, Won-Dong
    • Tuberculosis and Respiratory Diseases
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    • 제41권4호
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    • pp.379-388
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    • 1994
  • Background : In sleep apnea syndrome, arterial oxygen saturation($SaO_2$) decreases at a variable rate and to a variable degree for a given apneic period from patient to patient, and various kinds of cardiac arrythmia are known to occur. Factors supposed to affect arterial oxygen desaturation during apnea are duration of apnea, lung voulume at which apnea occurs, and oxygen consumption rate of the subject. The lung serves as preferential oxygen source during apnea, and there have been many reports related with the influence of lung volume on $SaO_2$ during apnea, but there are few, if any, studies about the influence of oxygen consumption rate of an individual on $SaO_2$ during breath holding or about the profile of arterial oxygen resaturation after breathing resumed. Methods : To investigate the changes of $SaO_2$ and heart rate(HR) during breath holding(BH) and rebreathing(RB) and to evaluate the physiologic factors responsible for the changes, lung volume measurements, and arterial blood gas analyses were performed in 17 healthy subjects. Nasal airflow by thermistor, $SaO_2$ by pulse oxymeter and ECG tracing were recorded on Polygraph(TA 4000, Gould, U.S.A.) during voluntary BH & RB at total lung capacity(TLC), at functional residual capacity(FRC) and at residual volume(RV), respectively, for the study subjects. Each subject's basal metabolic rate(BMR) was assumed on Harris-Benedict equation. Results: The time needed for $SaO_2$ to drop 2% from the basal level during breath holding(T2%) were $70.1{\pm}14.2$ sec(mean${\pm}$standard deviation) at TLC, $44.0{\pm}11.6$ sec at FRC, and $33.2{\pm}11.1$ sec at RV(TLC vs. FRC, p<0.05; FRC vs. RV, p<0.05). On rebreathing after $SaO_2$ decreased 2%, further decrement in $SaO_2$ was observed and it was significantly greater at RV($4.3{\pm}2.1%$) than at TLC($1.4{\pm}1.0%$)(p<0.05) or at FRC($1.9{\pm}1.4%$)(p<0.05). The time required for $SaO_2$ to return to the basal level after RB(Tr) at TLC was not significantly different from those at FRC or at RV. T2% had no significant correlation either with lung volumes or with BMR respectively. On the other hand, T2% had significant correlation with TLC/BMR(r=0.693, p<0.01) and FRC/BMR (r=0.615, p<0.025) but not with RV/BMR(r=0.227, p>0.05). The differences between maximal and minimal HR(${\Delta}HR$) during the BH-RB manuever were $27.5{\pm}9.2/min$ at TLC, $26.4{\pm}14.0/min$ at RV, and $19.1{\pm}6.0/min$ at FRC which was significantly smaller than those at TLC(p<0.05) or at RV(p<0.05). The mean difference of 5 p-p intervals before and after RB were $0.8{\pm}0.10$ sec and $0.72{\pm}0.09$ sec at TLC(p<0.001), $0.82{\pm}0.11$ sec and $0.73{\pm}0.09$ sec at FRC(p<0.025), and $0.77{\pm}0.09$ sec and $0.72{\pm}0.09$ sec at RV(p<0.05). Conclusion Healthy subjects showed arterial desaturation of various rates and extent during breath holding at different lung volumes. When breath held at lung volume greater than FRC, the rate of arterial desaturation significantly correlated with lung volume/basal metabolic rate, but when breath held at RV, the rate of arterial desaturation did not correlate linearly with RV/BMR. Sinus arrythmias occurred during breath holding and rebreathing manuever irrespective of the size of the lung volume at which breath holding started, and the amount of change was smallest when breath held at FRC and the change in vagal tone induced by alteration in respiratory movement might be the major responsible factor for the sinus arrythmia.

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Matrix Metalloproteinase in Idiopathic Pulmonary Fibrosis (특발성 폐섬유화증환자의 기관지폐포세척액 및 폐포대식세포 배양액의 Matrix metalloproteinase의 변화)

  • Park, Joo-Hun;Shim, Tae-Sun;Lim, Chae-Man;Koh, Youn-Suck;Lee, Sang-Do;Kim, Woo-Sung;Kim, Won-Dong;Kim, Dong-Soon
    • Tuberculosis and Respiratory Diseases
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    • 제51권4호
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    • pp.303-314
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    • 2001
  • Background : Matrix metalioproteinase(MMP)-2 and MMP-9 have been known to play an important role in cell migration and the tissue remodeling process by type IV collagen lysis, a major component of the basement membrane. Intra-alveolar fibrosis, secondary to an injury to the basement membrane of the alveolar epithelial lining, is a major process in the pathogenesis of idiopathic pulmonary fibrosis(IPF). Therefore, MMP-2 and MMP-9 was hypothesized to play an important role in IPF pathogenesis. As a result, their level may reflect the activity or prognosis. Method : Forty one progressive IPF patients(age $59.82{\pm}1.73$ years, M:F=23:18), 16 patients with stable IPF for more than one year without therapy(age : $63.6{\pm}2.8$ years, M:F=13:3), and 7 normal controls were enrolled in this study. The MMP-2 and MMP-9 levels in the BAL fluid and alveolar macrophage conditioned media(AM-CM) were measured by zymography and the TIMP-1 level was measured by ELISA. Results : 1) The MMP-2 level in BALF was highest in the progressive IPF group ($1.36{\pm}0.28$) followed by the stable group ($0.46{\pm}0.13$) and the controls ($0.08{\pm}0.09$), which was statistically significant. The MMP-9 level of the IPF ($0.31{\pm}0.058$) and the stable group ($0.22{\pm}0.078$) were higher than that of the control group ($0.002{\pm}0.004$). In the AM-CM, only MMP-9 was detected, which was significantly higher in IPF group ($0.80{\pm}0.1O$) than in the control group($0.23{\pm}0.081$). The TIMP-1 level was also higher in both the IPF ($36.34{\pm}8.62\;{\mu}g/ml$) and stable group ($20.83{\pm}8.53\;{\mu}g/ml$) compared to the control group ($2.80{\pm}1.05\;{\mu}g/ml$) (p<0.05). 3) There was a correlation between the MMP-2 level in the BALF with the total cell number(r=0.298) and neutrophils(r=0.357) (p<0.05), and the MMP-9 level with the number of neutrophils (r=0.407) and lymphocytes (r=0.574)(p<0.05). The TIMP-1 level correlated with the total number of cell (r=0.338, p<0.05) and neutrophils(r=0.449, p=0.059). Conclusion : Both MMP and TIMP appear to play an important role in IPF pathogenesis, and their level may reflect the disease activity.

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A Study on Satisfaction and Evaluation of Students현 Spot-Practice in Department of Food and Nutrition (식품영양학과 학생들의 현장실습 만족도 및 평가도에 관한 연구)

  • 최미경;전예숙;홍원주;김순경;김동희;김애정;강명화;김미현
    • Journal of the Korean Society of Food Science and Nutrition
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    • 제33권2호
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    • pp.373-380
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    • 2004
  • The purpose of this study was to estimate satisfaction and evaluation degree of students' spot-practice in department of food and nutrition and to investigate relating factors to them for development of spot-practice Programme. The survey for analysis was conducted through the questionnaires to 229 students finished internship. Proportions of the students were 93.9% for senior, 93.9% for female. Proportions of spot-practice facilities were 38.9%, 46.7%, and 14.4% for school, business & industry, and hospital respectively. The major part of subjects (83.8%) took part in spot-practice during 2 weeks. The satisfaction scores to spot-practice were the lowest for pay, and the highest for a kind of spot-practice facilities. The evaluation scores were the lowest for 'I made a plan and prepared my work in advanced', and the highest for 'I did my best for doing my work'. The satisfaction score in hospital was significantly lower than those in school or business & industry. The satisfaction score in self-operated foodservice was significantly higher than that in contract managed one. However, there no significant differences in evaluation scores by foodsevice employment type. In the case of coincidence of area in spot-practice and residence, or facilities of spot-practice and job desired, the satisfaction and evaluation scores were significantly higher than those of others. The period of spot-practice was significantly positively correlated with satisfaction scores for atmosphere of spot-practice facilities, service and treatment for spot-practice student, while negatively correlated with evaluation score for 'I did my best for doing my work'. The spot-practice pay was significantly positively correlated with satisfaction and evaluation scores for various items. In conclusion, various factors are related to satisfaction and evaluation degree of spot-practice students, and consist concern and support from college St university, foodservice institution, and government for systematic spot-Practice are required.

The Changes of Plasma Catecholamines Concentration during Waking and Sleep in Obstructive Sleep Apnea Syndrome Patients with Systemic Hypertension (전신성 고혈압을 동반한 폐쇄성 수면 무호흡증후군 환자에서 각성시와 수면중의 혈장 Catecholamines 농도 변화)

  • Moon, Hwa Sik;Lo, Dae Guen;Choi, Young Mee;Kim, Young Kyoon;Kim, Kwan Hyoung;Song, Jeong Sup;Park, Sung Hak
    • Tuberculosis and Respiratory Diseases
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    • 제43권4호
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    • pp.600-612
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    • 1996
  • Background : Recent studies deported that untreated patients with obstructive sleep apnea syndrome had high long-term mortality rates, and cardiovascular complications of these patients clad a major effect on mortality. Several data indicates that obstructive sheep apnea syndrome contributes to the development of diurnal systemic hypertension. But the pathophysiological mechanism of the development of systemic hypertension in these patients is still uncertain. This study was performed to evaluate the possible role of sympathetic nervous system activity for the development of systemic hypertension in patients with obstructive sleep apnea syndrome. Method : 35 patients with obstructive sleep apnea syndrome(OSAS) and 13 Control subjects(control) were included in this study. 21 patients of OSAS were normotensives(OSAS-NBP), and 14 patients of OSAS were hypertensives(OSAS-HBP). Full night polysomnography was undertaken to all subjects. We measured plasma norepinephrine(NE) and epinephrine(EP) concentrations during waking and sleep, using high performance liquid chromatography, in all patients and control subjects. Results : In OSAS, OSAS-NBP and control, plasma NE and EP concentrations during sleep were lowed than during waking(p<0.01). But, in OSAS-HBP, these was no difference between during waking and sleep. Plasma NE concentrations during sleep of OSAS, OSAS-NBP and OSAS-HBP were higher than Control(p<0.05). In OSAS-HBP, daytime systolic blood pressure correlated with plasma NE concentration during sleep(r=0.7415, p<0.01), arid correlated inversely with mean arterial oxygen saturation(r=-0.6465, p<0.05) or arterial oxygen saturation nadir(r=-0.6) 14, p<0.05) during sleep. Conclusion : The sympathetic activity during sleep of obstructive sleep apnea syndrome patients was higher than control subjects. In obstructive sleep apnea syndrome patients with systemic hypertension, there was no diurnal variation of sympathetic activity, and there was correlation between daytime systolic blood pressure and sympathetic activity during sleep. These data suggests that chronic hyperactivity of sympathetic nervous system may contribute to the development of diurnal systemic hypertension in patients with obstructive sleep apnea syndrome.

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