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Effect of Bronchial Artery Embolization(BAE) in Management of Massive Hemoptysis (대량 객혈환자에서 기관지 동맥색전술의 효과)

  • Yeo, Dong-Seung;Lee, Suk-Young;Hyun, Dae-Seong;Lee, Sang-Haak;Kim, Seok-Chan;Choi, Young-Mee;Suh, Ji-Won;Ahn, Joong-Hyun;Song, So-Hyang;Kim, Chi-Hong;Moon, Hwa-Sik;Song, Jeong-Sup;Park, Sung-Hak;Kim, Ki-Tae
    • Tuberculosis and Respiratory Diseases
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    • v.46 no.1
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    • pp.53-64
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    • 1999
  • Background : Massive and untreated hemoptysis is associated with a mortality of greater than 50 percent. Since the bleeding is from a bronchial arterial source in the vast majority of patients, embolization of the bronchial arteries(BAE) has become an accepted treatment in the management of massive hemoptysis because it achieves immediate control of bleeding in 75 to 90 percent of the patients. Methods: Between 1990 and 1996, we treated 146 patients with hemoptysis by bronchial artery embolization. Catheters(4, 5, or 7F) and gelfoam, ivalon, and/or microcoil were used for embolization. Results: Pulmonary tuberculosis and related disorders were the most common underlying disease of hemoptysis(72.6%). Immediate success rate to control bleeding within 24hours was 95%, and recurrence rate was 24.7%. The recurrence rate occured within 6 months after embolization was 63.9%. Initial angiographic findings such as bilaterality, systemic-pulmonary artery shunt, neovascularity, aneurysm were not statistically correlated with rebleeding tendency(P>0.05). Among Initial radiographic findings, only pleural lesions were significantly correlated with rebleeding tendency(P<0.05). At additional bronchial artery angiograpy done due to rebleeding, recanalization of previous embolized arteries were 63.9%, and the presence of new feeding arteries were 16.7%, and 19.4% of patients with rebleeding showed both The complications such as fever, chest pain, headache, nausea and vomiting, arrhythmia, paralylytic ileus, transient sensory loss (lower extremities), hypotension, urination difficulty were noticed at 40 patients(27.4%). Conclusion: We conclude that bronchial artery embolization is relatively safe method achieving immediate control of massive hemoptysis. At initial angiographic findings, we could not find any predictive factors for subsequent rebleeding. It may warrant further study whether patients with pleural disease have definetely increased rebleeding tendency.

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Countermeasure and Outbreak Mechanism of Cochlodinium polykrikoides red tide 1. Environmental characteristics on outbreak and disappearanceof C. polykrikoides bloom (Cochlodinium polykrikoides 적조 발생기작과 대책 1. Cochlodinium polykrikoides 적조 발생과 소멸의 환경특성)

  • Park, Young-Tae;Kim, Young-Sug;Kim, Kui-Young;Park, Jong-Soo;Go, Woo-Jin;Jo, Yeong-Jo;Park, Seong-Yoon;Lee, Young-Sik
    • The Sea:JOURNAL OF THE KOREAN SOCIETY OF OCEANOGRAPHY
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    • v.6 no.4
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    • pp.259-264
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    • 2001
  • Typhoon and neap tide on Cochlodinium polykrikoides bloom and water temperature on disappearance of C. polykrikoides bloom were investigated to elucidate the outbreak mechanism of C. polykrikoides blooms at Naro and Namhae coastal area in South Sea of Korea. The first observation of C. polykrikoides blooms were observed when thermocline was disappeared by typhoon, tide, etc. The first blooms of C. polykrikoides were observed on neap tide or before one day from neap tide in 1996-1998 and 2000. However, thermocline was disappeared by typhoon in 1994 and 1999, the first blooms were observed early 12-30 day than 1996-1998 and 2000. The main reason of disappearance of C. polykrikoides blooms after typhoon on 1997-2000 seems to be other environmental change by typhoon rather than low water temperature. In the future, the first C. polykrikoides bloom will be appear around the first neap tide of latter part of August with breaking down of thermocline, but if the thermocline be collapsed by typhoon in July, the C. polykrikoides bloom will be appear at beginning of August. The outbreak of C. polykrikoides blooms will be explain as follows: The vegetative cells, which was germinated by environmental change or already exist in surface water at low level, input to the surface water, and then nutrients and trace metals which were suppled from out side of C. polykrikoides bloom area inflow to surface. The vegetative cells are growth by the nutrients and trace metals at suitable environmental conditions e.g. water temperature, salinity, and sufficient light.

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The Enhancement of Radiosensitivity by Celecoxib, Selective Cyclooxygenase-2 Inhibitor, on Human Cancer Cells Expressing Differential Levels of Cyclooxygenase-2 (선택적 Cyclooxygenase-2 억제제인 Celecoxib가 상이한 Cyclooxygenase-2 발현량을 가진 인간 암세포주들에 대하여 유도하는 방사선 감수성 증진 작용)

  • Pyo Hongryull;Shin You Keun;Kim Hyun Seok;Seong Jinsil;Suh Chang Ok;Kim Gwi Eon
    • Radiation Oncology Journal
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    • v.21 no.3
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    • pp.216-221
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    • 2003
  • Purpose: To investigate the modulation of radiosensitivity by celecoxib, a selective cyclooxygenase-2 (COX-2) inhibitor, on cancer cells over- and under-expressing COX-2. Materials and Methods: A clonogenic radiation survival analysis was performed on A549 human lung and MCF-7 human breast cancer cell lines incubated in both 1 and $10\%$ fetal bovine serum (FBS) containing media. The apoptosis in both cell lines was measured after treatment with radiation and/or celecoxib. Results: Celecoxib enhanced the radiation sensitivity of the A549 cells in the medium containing the $10\%$ FBS, with radiation enhancement ratios of 1.58 and 1.81 respectively, at surviving fractions of 0.1, with $30\muM\;and\;50\muM$ celecoxib. This enhanced radiosensitivity disappeared in the medium containing the $1\%$ FBS. Celecoxib did not change the radiation sensitivity of the MCF-7 cells in either media. The induction of apoptosis by celecoxib and radiation was not synergistic in either cell line. Conclwsion: Celecoxib, a selective COX-2 inhibitor, preferentially enhanced the effect of radiation on COX-2 over-expressing cancer cells compared to the cells with a low expression, and this effect disappeared on incubation of the cells during drug treatment in the medium with suboptimal serum concentration. Apoptosis did not appear to be the underlying mechanism of this radiation enhancement effect due to celecoxib on the A549 cells. These findings suggest radiosensitization by a selective COX-2 inhibitor is COX-2 dependent.

Changes of Amino Acids and Formation Mechanism of Flavor in Cooked Small Shrimps (새우 가열(加熱)중의 아미노산의 변화(變化) 및 향기성분(香氣成分) 생성기구(生成機構)에 관한 연구(硏究))

  • Choi, Sung-Hee;Lee, Byung-Ho
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.16 no.4
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    • pp.371-378
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    • 1987
  • Free and total amino acids of before and after the heating of the small shrimps were analysed quantitatively, in order to confirm the role of amino acids as important precursors of cooked odor components. Differences of free amino acids contents of the three samples were slightly reconized but free amino acids contents of all sample decreased as about half by heating, It seems that decreased amino acids participate In volatile components of the Small Shrimps. (Sergia lucens Hancen, Euphausia Surperba and Euphausia pacifica Hancen). The amino-carbonyl model reaction was carried out, in order to confirm formation mechanism of volatile compounds of the small shrimps during cooking. The model systems constituted by diluted solutions. of glucose and amino acids (proline, taurine and betaine) the most containing in small shrimps. The volatile odor concentrates of model system were obtained by simultaneous distillation and extraction with Nickerson's apparatus. The odor concentrates of model systems(I, III) had not small shrimp-like odor and main compounds were 1,4,5,6-tetrahydro-2-acetopyridine and 2-acetyl pyridine. In model system II, hetero ring nitrogen and sulfur compounds identified in small shrimps were not did.

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Cooperative Induction of HL-60 Cell Differentiation by Combined Treatment with Eugenol and 1α,25-Dihydroxyvitamin D3 (Eugenol과 1α,25-dihydroxyvitamin D3의 병합처리에 의한 HL-60 세포의 분화 유도)

  • Oh, Mi-Kyung;Park, Seon-Joo;Kim, Nam-Hoon;Cho, Jin-Kyung;Jin, Jong-Youl;Kim, In-Sook
    • Journal of Life Science
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    • v.17 no.9 s.89
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    • pp.1191-1196
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    • 2007
  • Eugenol (4-allyl-2-methoxyphenol) is a main component of essential oils obtained from various spices. Recent reports have shown that eugenol induces growth inhibition and apoptosis of malignant tumor cells. In this study, the stimulatory effect of eugenol on cell differentiation was investigated in HL-60 promyelocytic leukemia cells. When HL-60 cells were treated in combination with 150 ${\mu}M$ of eugenol and 3 nM of $1{\alpha},25-dihydroxyvitamin$ $D_{3}$, cell growth was slower than that of cells treated with eugenol or $1{\alpha},25-dihydroxyvitamin$ $D_{3}$ alone. Eugenol enhanced low dose of $1{\alpha,25-dihydroxyvitamin }$ $D_{3}-induced$ a $G_{0}/G_{1}$ phase arrest in cell cycle. Consistent with this, combined treatment of eugenol and $1{\alpha},25-dihydroxyvitamin$ $D_{3}$ cooperatively increased p27 level and decreased cyclin A, cdk 2 and cdk 4 levels, which are cell cycle regulators related to $G_{0}/G_{1}$ arrest. According to flow cytometric analysis, the expression of CD14 (monocytic differentiation marker) was more increased in the cells co-treated with eugenol and $1{\alpha},25-dihydroxyvitamin$ $D_{3}$. These results indicate that eugenol potentiates cell differentiation mediated by $1{\alpha},25-dihydroxyvitamin$ $D_{3}$ of suboptimal concentration. The differentiation-inducing property of eugenol maybe contributes to chemopreventive activity of cancer.

Applied-Mineralogical Characterization and Assessment of Some Domestic Bentonites (II): Mineralogical Characteristics, Surface Area, Rheological Properties, and Their Relationships (국내산 벤토나이트에 대한 응용광물학적 특성 평가 (II): 광물학적 특징, 체표면적 및 유변학적 특성과 그 연계성)

  • 노진환;유재영;최우진
    • Journal of the Mineralogical Society of Korea
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    • v.16 no.1
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    • pp.33-47
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    • 2003
  • Various applied-mineralogical characterization including measurements of surface area, size distribution, swelling index, and viscosity were done for some domestic bentonites in order to decipher the rheological properties and their controlling factors. The bentonites, which are Ca-type and relatively low-grade (rnontmorillonite contents: 30 ∼ 75 wt%), occur mostly as subhedral lamellas with the size range of 2 ∼ 4 $\mu\textrm{m}$. The size distribution of mineral fractions in bentonite suspension is dominant in the range of 10 ∼ 100 $\mu\textrm{m}$, and though rather complicated, exhibits roughly bimodal patterns. The feature is more conspicuous in the case of zeolitic bentonite. The bentonites have surface areas ranging 269 ∼ 735 $\m^2$/g, which are measured by EGME adsorption method. The EGME surface areas are nearly proportional to the rnontmorillonite contents, moisture contents, or total CEC. In the surface area measurements, zeolitic bentonites have slightly higher values than those zeolite- free types. The measured swelling index and viscosity of domestic bentonites are comparatively low in values. The swelling values of bentonites were measured to be 250∼500% at maximum by progressively mixing amounts of 2 ∼ 5 wt% Na$_2$CO$_3$, which varies depending on the contents of rnontmorillonite and other impurities, especially zeolite. Much amount of sodium carbonate is required for optimum swelling property of zeolitic bentonited which has usually strong Na- exchanged capacity. The bentonites, which are comparatively feldspar-rich and low in size and crystallinity, tend to be higher in viscosity values. Tn addition, the viscosity is largely higher in case of the bentonites with higher pH in suspension. However, the rheological properties of bentonites such as swelling index and viscosity do not show any obvious relationships with rnontmorillonite contents and mean particle size in suspension. In contrast, roughly speaking, the swelling index of bentonites is reversely proportional to the values of surface area which can be regarded as a collective physico-chemical parameter encompassing all the effects caused by mineral composition, surface charge, particle size, morphological farm, and etc. in bentonites. Thus, the rheological properties in bentonite suspension appear to be rather complicated characteristics which mainly depend on the flocculation of clay particles and the mode of particle association, i.e. quasicrystals, controlled by surface charge, morphology, size, and texture of rnon-tmorillonite, and which partly affected by the finer impurities such as zeolite.

A case of simultaneously identified glycogen storage disease and mucopolysaccharidosis (당원병과 뮤코다당체침착증이 동시에 발견된 증례 1예)

  • Lee, Ju Young;Shim, Jeong Ok;Yang, Hye Ran;Chang, Ju Young;Shin, Choong Ho;Ko, Jae Sung;Seo, Jeong Kee;Kim, Woo Sun;Kang, Gyeong Hoon;Song, Jeong Han;Kim, Jong Won
    • Clinical and Experimental Pediatrics
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    • v.51 no.6
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    • pp.650-654
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    • 2008
  • Glycogen storage disease (GSD) and mucopolysaccharidosis (MPS) are both independently inherited disorders. GSD is a member of a group of genetic disorders involving enzymes responsible for the synthesis and degradation of glycogen. GSD leads to abnormal tissue concentrations of glycogen, primarily in the liver, muscle, or both. MPS is a member of a group of inherited lysosomal storage diseases, which result from a deficiency in specific enzymatic activities and the accumulation of partially degraded acid mucopolysaccharides. A case of a 16-month-old boy who presented with hepatomegaly is reported. The liver was four finger-breadth-palpable. A laboratory study showed slightly increased serum AST and ALT levels. The liver biopsy showed microscopic features compatible with GSD. The liver glycogen content was 9.3% which was increased in comparison with the reference limit, but the glucose-6-phosphatase activity was within the normal limit. These findings suggested GSD other than type I. Bony abnormalities on skeletal radiographs, including an anterior beak and hook-shaped vertebrae, were seen. The mucopolysaccharide concentration in the urine was increased and the plasma iduronate sulfatase activity was low, which fulfilled the diagnosis criteria for Hunter syndrome (MPS type II). To the best of the authors' knowledge, this is the first case of GSD and Hunter syndrome being identified at the same time.

Fortschritte in der Diagnostik von $Eisenstoffwechselst\ddot{o}rungen$ (철대사장애(鐵代謝障碍)의 진단(診斷)에 관(關)한 최근동향(最近勳向))

  • Keiderling, Walter
    • The Korean Journal of Nuclear Medicine
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    • v.4 no.1
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    • pp.1-9
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    • 1970
  • 철대사(鐵代謝)에 관(關)한 연구(硏究)는 과거(過去) 30년(年)동안 새로운 검사방법(檢査方法)의 도입(導入)으로 눈부신 발전(發展)을이룩하였다. 1937년(年) Heilmeyer 등(等)에 의(依)하여 Ortho-phenanthrolin방법(方法)의 개발(開發)로 저색소성빈혈(低色素性貧血)의 원인(原因)이 구명(究明)되고 또한 이에 대(對)한 치료(治療)의 원칙(原則)이 세워졌다. 그 후 심(甚)한 감염(感染)이나 악성종양(惡性腫瘍)을 가진 환자(患者)者에서 관찰(觀察)되는 빈혈(貧血)에 대(對)해서 하나의 가설(假說)을 세워 이를 설명(說明)하려 하였는데 이는 곧 혈장(血漿)으로부터 철분(鐵分)이 신속(迅速)히 소실(消失)되어 망내계(網內系)나 병변(病變)이 있는 국소부위(局所部位)에 주(主)로 모여 들어 특수(特殊)한 방어기능(防禦機能)을 발휘(發揮)한다는것으로 연자(演者)는 방사성동위원소(放射性同位元素)를 이용(利用)하여 이 가설(假說)을 증명(證明)하였으며 이 연구(硏究)에는 또한 이문호교수(李文鎬敎授)가 Freiburg대학(大學) 유학중(留學中) 참여(參與)한 바 있다. 철대사(鐵代謝)를 파악(把握)하기 위(爲)해서 $^{59}Fe$가 흔히 사용(使用)되는데 이러한 방사성동위원소(放射性同位元素)를 이용(利用)함으로서 다음 사항(事項)들을 관찰(觀察)할 수 있었다. 즉(卽) 1. 소화장기(消化臟器)로 부터의 철흡수(鐵吸收) 2. 혈장(血漿)에서의 철(鐵)의 소실속도(消失速度) 3. 혈장내(血漿內)에서의 철교체율(鐵交替率) 4. 적혈구(赤血球)의 철이용(鐵利用) 5. 생체내(生體內)의 철분포(鐵分布) 6. 철배설(鐵排泄)의 정량적(定量的) 분석(分析) 또한 근년(近年)에는 특수(特殊)한 기능(機能)을 발휘(發揮)할 수 있는 동위원소(同位元素)를 이용(利用)하여 철흡수(鐵吸收) 및 대사이외(代謝以外)에도 적혈구(赤血球)의 수명(壽命)과 혈액량등(血液量等)을 측정(測定)하게 되었다. 경구적(經口的)으로 투여(投與)된 철(鐵)은 대부분(大部分) 십이지장(十二指腸)의 상부(上部)에서 흡수(吸收)되고 무기철(無機鐵)이 보다 쉽게 흡수(吸收)되어 가(價)의 상태(狀態)로 된다. 혈장(血漿)에서는 transferrin에 의(依)해서 철(鐵)이 운반(運搬)된다. 혈장철(血漿鐵)의 대부분(大部分)은 혈색소분해(血色素分解)에서 유래(由來)되며 이는 다시 혈색소(血色素)의 재생(再生)에 이용(利用)되는데 혈장내(血漿內) 철교체율(鐵交替率)은 방사성철(放射性鐵)을 이용(利用)하여 측정(測定)할수 있다 이와같이 방사성철(放射性鐵)을 이용(利用)하여 철대사과정(鐵代謝過程)을 숙지(熟知)함으르서 임상(臨床)에 응용(應用)하기에 이르렀으며 다음과 같은 질환(疾患)의 진단(診斷)에 특(特)히 큰 도움을 준다. A. 진성철결핍증(眞性鐵缺乏症) : 혁색소철(血色素鐵) 및 저장철(貯藏鐵)을 포함(包含)한 생체내(生體內) 전철분(全鐵分)의 부족(不足)된 상태(狀態)로서 실혈(朱血)에 의(依)한 것이 대부분(大部分)이다. 이 경우 철흡수(鐵吸收)는 증가(增加), 혈장철치(血漿鐵値)는 저하(低下), 철소실속도(鐵消失速度)는 증가(增加)되며 혈장철(血漿鐵) 교체율(交替率)은 항진(亢進) 혹(或)은 정상(正常)이다. B. 심(甚)한 염증성(炎症性) 질환(疾患) : 이 경우에도 혈장철치(血漿鐵値)의 저하(低下), 소실속도(消失速度)의 증가(增加), 교체율(交替率)은 정상(正常)보다 4배(倍)까지 증가(增加)할 수 있다. 골수(骨髓)에서 보다는 간(肝), 비(脾)와 같은 망내계(網內系)에 방사성철(放射性鐵)이 집결(集結)되는 것으로 보아 혈색소철(血色素鐵)보다는 저장철(貯藏鐵)이 관여(關與)되는 것이다. C. 원발성(原發性) 혈색소증(血色素症)(Idiopathic hemochromatosis) : 혈장철(血漿鐵)의 증가(增加)가 현저(顯著)하며 transferrin 농도(濃度)는 정상(正常)보다 낮으나 거의 대부분(大部分)의 철분(鐵分)으로 포화(飽和)된다. 철흡수(鐵吸收)는 증가(增加)되고 철소실속도(鐵消失速度)는 감소(減少) 되어 있으나 교체율(交替率)은 항진(亢進)되어 있다. 혈장철(血漿鐵)은 간(肝), 비(脾) 등(等)의 기관(器管)으로 저장집결(貯藏集結)되어 철저류(鐵貯溜)가 증대(增大)되므로 철이용증((鐵利用症)은 저하(低下)된다. D. 선천성(先天性) 무(無)$\ulcorner$트란스헤 린$\lrcorner$증(症)(Congenital atransferrinemia) : 방사성철(放射性鐵)을 이용(利用)한 진단방법(診斷方法)으로 Freiburg에서 7세(歲)의 소녀(少女)에서 발견(發見)한 증례(症例)인데 간(肝), 비(脾), 심(心)의 비대(肥大)가 임상적(臨床的)으로 인지(認知)되었고 중증(重症)의 철결핍상(鐵缺乏狀)을 검출(檢出)할 수 있었다. 철흡수율(鐵吸收率)의 상승(上昇), 혈장철치(血裝鐵値)의 감소(減少), 혈장철소실속도(血漿鐵消失速度)의 증가(增加), 혈장철교체율(血漿鐵交替率)의 상승(上昇) 및 적혈구(赤血球)에서의 철분이용율(鐵分利用率)의 저하(低下)를 ferrokinetic study에서 알 수 있었고 간(肝)에서 고도(高度)의 방사능(放射能)이 검출(檢出)되는 반면(反面), 비(脾)에서는 극소(極小), 골수(骨髓)에는 전(全)혀 방사능(放射能)이 들어가 있지 않았다. 이 증례(症例)와 같이 transferrin이 없으면 철분(鐵分)은 쉽게 조직(組織)으로 들어가 hemosiderin으로 저장(貯藏)되고 골수(骨髓)는 고도(高度)의 철결핍증(鐵缺乏症)을 나타내어 기관철침착증(器管鐵沈着症)과 철결핍성빈혈(鐵缺乏性貧血)이 동시(同時)에 나타나게 된다. 철대사장애면(鐵代謝障碍面)으로 보아 많은 미해결점(未解決點)이 남아 있으며 앞으로 자라나는 젊은 학도(學徒)들이 구명(究明)할 문제(間題)라고 믿는다.

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Psychosocial Risk Factors of Postpartum Depression (산후우울증의 심리사회적 위험요인)

  • Park, Si-Sung;Han, Kwi-Won
    • Korean Journal of Psychosomatic Medicine
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    • v.7 no.1
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    • pp.124-133
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    • 1999
  • Objective : Postpartum depression(PPD) was known to be caused by many factors including various psychosocial risk factors. This study was performed to identify the psychosocial risk factors for ppd, preliminarily in Korea. Methods : A group of 119 postpartum women, each of whom was at 6 to 8 weeks after delivery was identified at the time when they visited to the child health clinic or postnatal check-up clinic. The risk factors were surveyed by the self-reported questionnaire. The items of questionnaire were consisted of known risk factors in other studies and other possible stress-related factors. PPD was assessed by the Edinburgh Postnatal Depression Scale(EPDS) and the degree of postpartum depression was determined by its score. Results : 16 women(13.45%) in the high risk group were diagnosed as PPD among the 119 women. Risk factors including past experience of depressive symptoms and low level of marital satisfaction were founded more frequently in women in the high risk group than in the low risk group. The score of EPDS was significantly high in the group who experienced depressive symptoms in the past, anxiety or depression during pregnancy, stressful life event during the period of recent pregnancy and postpartum, and who had low level of marital satisfaction. There was a positive correlation between age and the score of EPDS. However, the postpartum depressive symptoms were not influenced by the level of education, job, retirement due to pregnancy and delivery, wanted or unwanted pregnancy, delivery method, feeding method, the hospitalization of infant, expected and real gender of infant. Conclusion : These results suggest that PPD is quite frequent at postpartum period. Various risk factors contribute to the development of PPD. If clinicians pay attention to the risk factors of PPD and give appropriate psychiatric intervention to the mothers during pregnancy and postpartum, it will be easy for the clinicians to recognize and treat PPD in the early stage.

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Clinical Features, Molecular Analysis, and Outcome of ERT in Korean Patients with Mucopolysaccharidosis Type VI (국내 6형 뮤코다당증의 임상 양상, 분자유전학적 특징 및 효소치료의 효과에 대한 고찰)

  • Wichajarn, Khunton;Kim, Jinsup;Yang, Aram;Sohn, Young Bae;Lee, Beom Hee;Yoo, Han-Wook;Cho, Sung Yoon;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.16 no.1
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    • pp.24-33
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    • 2016
  • Mucopolysaccharidosis type VI (MPS VI) is a rare disease caused by the mutation of ARSB with prevalence range from 1/5,000 in northeast Brazil to 1/2,057,529 births in Czech Republic. In Asia, there is only one published figure in Taiwan of about 1/833,000 births. The exact prevalence in the Korean population is unknown, but we estimated the incidence of MPS VI is about 0.03/100,000 live births. Enzyme replacement therapy (ERT) with recombinant human Arylsulfatase B (rhASB) is a modality for the treatment of MPS VI that reduces the excretion of urine glycosaminoglycan (GAG) and improves joint motion, pulmonary function, and endurance. We presented the clinical features, molecular analysis and outcome of ERT in three Korean MPS VI patients. All patients had the typical characteristic clinical features of MPS IV. Short stature, dysostosis multiplex, corneal opacity and valvular heart disease were found at first presentation, while restrictive lung disease and carpal tunnel syndrome developed later in all patients. Molecular analysis demonstrated novel missense and nonsense mutation in the patients, including p.Ile 67Ser, p.Gly328Arg, $p.Arg191^*$, p.Asp352Asn, and p.Gly17Asp. After ERT, urine GAG was decreased in all patients. Skeletal involvement, corneal opacity, heart valve abnormalities and pulmonary function were not improved with ERT, but it had a better outcome on regarding joint motion and endurance. One patient underwent allogeneic bone marrow transplantation (BMT) prior to ERT, but their clinical response was not improved much after BMT. This study demonstrates clinical phenotypes and molecular analysis of the severe form of MPS VI in Korean patients.

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