RISS 학술연구정보서비스

검색
다국어 입력

http://chineseinput.net/에서 pinyin(병음)방식으로 중국어를 변환할 수 있습니다.

변환된 중국어를 복사하여 사용하시면 됩니다.

예시)
  • 中文 을 입력하시려면 zhongwen을 입력하시고 space를누르시면됩니다.
  • 北京 을 입력하시려면 beijing을 입력하시고 space를 누르시면 됩니다.
닫기
    인기검색어 순위 펼치기

    RISS 인기검색어

      검색결과 좁혀 보기

      선택해제
      • 좁혀본 항목 보기순서

        • 원문유무
        • 원문제공처
          펼치기
        • 등재정보
          펼치기
        • 학술지명
          펼치기
        • 주제분류
        • 발행연도
          펼치기
        • 작성언어
        • 저자
          펼치기

      오늘 본 자료

      • 오늘 본 자료가 없습니다.
      더보기
      • 무료
      • 기관 내 무료
      • 유료
      • 수수로부터 아플라톡신B₁ 정량분석에 관한 연구

        정향동,원옥남,구경림,류성렬 한국공업화학회 2003 응용화학 Vol.7 No.2

        Aflatoxin B₁was known to have the strongest poisonous character among Aflatoxn compounds. To search the production of Aflatoxin B₁according to the change of temperature and relative humidity, millet incubated with Aspergillus flavus KCCM 35078 was incubated at 20℃, 25℃, 30℃, 25℃ and at relative humidity 50%, 60%, 70% and for incubation time 72, 96, 120, 144, 168, 192 hours. Production of Aflatoxin B₁shows that the highest production appeared at 25℃ and its relative humidity 70%. The result that was quantitatively analyzed by using HPLC was 23.35ppm, and by using fluorescence detector was 23.50ppm Production of Aflatoxin B₁shows that the lowed production appeared at 20℃ and its relative humidity 50%. The result that was Quantitatively analyzed by using HPLC was 6,26ppm, and by using fluorescence detector was 6.33ppm The structure of Aflatoxin B₁was identify with authentic sample by using instrumental analysis methods of MS, H-NMR, FT-IR, respectively.

      • KCI등재후보

        소아 급성 시신경염

        구남향(Nam Hyang Koo),김연희(Yeun Hee Kim),김성환(Sung Hwan Kim) 대한소아신경학회 2004 대한소아신경학회지 Vol.12 No.2

        목적 : 급성 시신경염은 시신경 수초의 염증이나 탈수초화로 인해 발생하는 시신경 질환으로 소아 급성 시신경염은 임상 양상과 예후가 성인과 차이를 보이는 것으로 알려져 있다. 그러나 소아에서 발생한 급성 시신경염은 성인에 비해 발생빈도가 적어서 임상 증상, 스테로이드 치료 후 시력 예후, 시신경염의 재발 및 다발성 경화증으로 이행에 대한 체계적인 연구가 결여되어 있다. 성인에서 시행된 ONTT의 연구 결과는 그대로 소아에 적용하기에는 문제가 있기에 저자들은 소아에서 발생한 급성 시신경염의 원인, 임상 양상, 시력 예후 및 시신경염의 재발과 다발성 경화증으로의 이행 여부를 알아보고자 본 연구를 시행하였다. 방법 : 1995년 1월부터 2004년 4월까지 아주대학교병원 소아과에서 급성 시신경염으로 진단 받은 15세 이하 소아 환자 10명의 의무기록을 토대로 시신경염의 원인, 임상 양상 및 단기 시력 예후에 대해 후향적 조사를 하였다. 또한 고용량 methyl-prednisone으로 치료한 9명의 환자에서 퇴원 후 30개월 간 장기 시력 예후, 시신경염의 재발 및 다발성 경화증으로 이행 여부를 추적 관찰하였다. 결과 : 10명의 환아 중 남아 4명, 여아가 6명이었으며 평균 연령은 10세였다. 전체 환자 10명 중 8명에서 시신경염이 양쪽 눈에 발생하였고 나머지 2명은 한쪽 눈에만 발생하였으며 90%의 환자는 유두염 소견을 보였다. 소아 급성 시신경염의 원인은 특발성 시신경염이 50%로 가장 많았으며, 급성 파종성 뇌척수염 30%, 전부비동염과 시신경 척수염이 각각 10%이었다. 시신경 자기공명영상 검사를 시행한 10명 환자의 18안 중 15안에서 시신경비대 및 조영증강과 유두돌출 소견을 보였다. 시간유발전위 검사상 8명 환자 16안 중 12안(75%)에서 이상 소견이 관찰되었다. 이상 소견이 발견되었던 12안 중 6안은 P100파의 진폭이 약화되어 P100파를 측정할 수 없었고 나머지 6안은 P100파의 진폭은 정상이었으나 잠시가 지연되었다. 스테로이드 치료 1개월 후 추적 조사한 시각 유발전위 검사에서 P100파의 진폭은 모두 정상으로 회복되었으나 60%의 환자는 P100파의 잠시가 여전히 지연되어 있었다. 고용량 스테로이드 치료 후 8명 환자의 15안 중 14안에서 단기 시력이 정상으로 회복되었다. 고용량 스테로이드로 치료받은 9명의 환자 중 1명(11%)에서 시신경염이 재발하였으나 다발성 경화증으로 이행된 환자는 없었다. 결론 : 소아에서 발생한 급성 시신경염은 특발성 시신경염이 가장 흔한 원인이며 주로 양쪽 눈에 유두염으로 나타나는 특성이 있다. 본 연구는 다른 보고와 달리 고용량 스테로이드 치료 후 단기 시력 예후는 아주 좋았고, 치료 6개월 후 측정한 장기 시력 예후는 단기 시력 예후와 차이가 없었다. 대부분의 소아 시신경염 환자에서 시신경염 환자에서 시신경염이 재발하거나 다발성 경화증으로 이행되지 않아 예후는 양호하였으나 더 많은 환자를 대상으로 장기간 추적 관찰을 통한 전향적 연구가 필요한 것으로 생각된다. Purpose: Acute optic neuritis is an inflammatory optic neuropathy, and rarely occurs in children. Childhood-onset optic neuritis is different from adult-onset optic neuritis in terms of clinical and prognostic aspects. We investigated the clinical characteristics and visual prognosis related to the steroid therapy in children with acute optic neuritis. In addition, we evaluated the recurrence rate of acute optic neuritis and subsequent evolution into multiple sclerosis. Methods: The medical records of ten children, who were previously admitted for acute optic neuritis at the Department of Pediatrics of Ajou University Medical Center from January 1995 to April 2004, were retrospectively reviewed. Nine patients were treated with intravenous methylprednisone pulse therapy, 0.5-1.0 g/day, for 3-5 days followed by an oral prednisone therapy. To 30 months after discharge, we followed up a long-term visual prognosis, recurrence rate and subsequent evolution into multiple sclerosis. Results: There were 4 boys and 6 girls and the mean age of onset was 10 years. Bilateral optic neuritis was noticed in 8 children and 9 children presented with papillitis. Etiology of acute optic neuritis was idiopathic in 50% of patients. Of the remaining cases, it was related with acute disseminated encephalomyelitis(30%), sinusitis(10%), and neuromyelitis optica(10%). Orbital MRI showed optic nerve enhancement and/or enlargement and optic disc protrusion in 15 of the 18 examined eyes. Visual evoked potential (VEP) was abnormal in 12 of the 16 examined eyes; P100 response was totally attenuated in 6 eyes, and in the others the amplitude of P100 response was normal but the latency was delayed. On follow-up VEP at 1 month after steroid pulse therapy, the amplitude of P100 response was normalized in all, but the latency was still delayed in 60% of the patients. A short-term visual acuity after treatment with steroid was normally recovered in 14 of 15 involved eyes. Acute optic neuritis recurred in one of the nine patients who were treated with high dose methyl prednisone pulse therapy, but there was no subsequent development of multiple sclerosis during the follow up. Conclusions: The most common cause of acute optic neuritis in children is idiopathic, and presents as bilateral papillitis. On this study, a short-term visual prognosis after high dose methylprednisone pulse therapy is extremely good, but there was no significant difference in long-term visual prognosis at 6 months after treatment with steroid. Acute optic neuritis in children does not usually recur, and does not evolve into multiple sclerosis.

      • KCI등재

        형질세포종에서 병발된 강직성 척추염

        김용남 ( Yong Nam Kim ),이향이 ( Hyang Ee Lee ),이상훈 ( Sang Hoon Lee ),이연아 ( Yeon A Lee ),우두현 ( Doo Hyun Woo ),황보영 ( Young Hwangbo ),조경삼 ( Kyung Sam Cho ),박용구 ( Yong Koo Park ),양형인 ( Hyung In Yang ) 대한류마티스학회 2005 대한류마티스학회지 Vol.12 No.3

        Plasmacytomas are tumors composed of plasma cells of variable maturity, which are histologically identical to those seen in multiple myeloma. Ankylosing spondylitis is a chronic inflammatory disease, probably resulting from the interaction of a genetic predisposition involving HLA-B27 with an environmental event such as enteric bacterial infection. Multiple myeloma has been intermittently reported in patients with ankylosing spondylitis. It has been proposed that the protracted stimulation of immunocytes by inflammatory lesions on the mucosal surfaces of the gastrointestinal, respiratory tracts may be implicated in the pathogenesis of multiple myeloma in some patients. We observed a 23 year old male patient with a history of plasmacytoma who subsequently developed ankylosing spondylitis. He was diagnosed as plasmacytoma 4 years ago and took a radiation therapy. There was no previous report of ankylosing spondylitis following plasmacytoma. The relationship between two diseases is uncertain until now and further study should be needed.

      • SCOPUSKCI등재

        Peutz - Jeghers 증후군에서 병발한 대장 선암종 1예

        박경남(Kyung Nam Park),전규영(Kyu Young Jun),이성구(Sung Koo Lee),이종철(Jong Chul Rhee),박문향(Moon Hyang Park),이중달(Jung Dal Lee) 대한소화기학회 1985 대한소화기학회지 Vol.17 No.1

        N/A The Peutz-Jeghers syndrome, characterized by pigmentation of the skin and mucous membranes and gastrointestinal polyposis of hamartomatous variety, has been well documented. There have been conflicting views, however, as to the frequency with which malignant change occurs in the polyp. There are insufficient data to establish this syndrome as a precancerous condition but documented cases of gastrointestinal cancer arising in this syndrome have been reported with an incidence of 2~3%. A case of Peutz-Jeghers syndrome with two descrete invasive adenocarcinom as arising in colonic polyps is reported with pertinent literature review. The patient was a 42 year old male admitted with symptoms of intestinal obstruction. He was found to have numerous polyps of variable size throughout the entire length of the colon and in part in the small intestine. He also had pigmentation of oral mucosa. Histologically the polyps were mostly hamartomatous and some were transformed into adenomatous in type with considerable cellular atypia. There were two descrete invasive Duke C adenocarcinomas arising in polyps on colectomy specimen.

      • Decreased Circulating Klotho Levels in Patients Undergoing Dialysis and Relationship to Oxidative Stress and Inflammation

        Oh, Hyung Jung,Nam, Bo Young,Lee, Mi Jung,Kim, Chan Ho,Koo, Hyang Mo,Doh, Fa Mee,Han, Jae Hyun,Kim, Eun Jin,Han, Ji Suk,Park, Jung Tak,Yoo, Tae-Hyun,Kang, Shin-Wook,Han, Dae-Suk,Han, Seung Hyeok International Society for Peritoneal Dialysis 2015 Peritoneal dialysis international Vol.35 No.1

        <P>♦ <I>Introduction:</I> It has been reported that klotho deficiency is associated with oxidative stress and inflammation in experimental kidney disease models. Patients with endstage renal disease (ESRD) are particularly characterized by increased oxidative stress and inflammation. However, little is known about the relationship between these features and klotho in patients with ESRD.</P><P>♦ <I>Methods:</I> We conducted a single-center, cross-sectional study of 78 patients receiving peritoneal dialysis (PD). Serum concentrations of klotho, high-sensitivity C-reactive protein (hsCRP), interleukin-6 (IL-6), and 8-isoprostane were measured by enzyme-linked immunosorbent assay. To define factors independently associated with klotho, we determined Spearman’s correlation coefficients for between co-variates and conducted multiple linear regression analyses.</P><P>♦ <I>Results:</I> Patients were classified by median concentration of klotho. In patients with klotho levels > 329.6 pg/mL, serum 8-isoprostane and IL-6 levels were significantly higher than in those with klotho levels < 329.6 pg/mL. In correlation analyses, log 8-isoprostane (γ = –0.310, <I>p</I> = 0.006) and log IL-6 (γ = –0.343, <I>p</I> = 0.002) were inversely correlated with log klotho. After adjustment for age, gender, mean arterial pressure, log intact parathyroid hormone, and log IL-6, log 8-isoprostane was independently associated with log klotho (β = –0.158, <I>p</I> = 0.040). However, the significant relationship between klotho and IL-6 was not seen in an adjusted model.</P><P>♦ <I>Conclusions:</I> This study showed that circulating klotho levels were significantly associated with 8-isoprostane levels in patients undergoing PD, suggesting a potential link between klotho deficiency and enhanced oxidative stress in ESRD patients.</P>

      • KCI등재

        환경미화원의 작업별 근골격계질환 자각증상 특성과 상지의 인간공학적 평가

        명준포,이향기,김형렬,정혜선,정은희,남 웅,구정완 大韓産業醫學會 2008 대한직업환경의학회지 Vol.20 No.2

        목적: 본 연구는 서울 및 경기 일부 지역의 환경미화원 을 대상으로 신체부위별 근골격계 질환의 증상 호소율을 파악한 후 작업의 차이에 따른 증상호소의 차이를 확인하고,원인 작업을 평가하여 근골격계질환의 관련요인을 찾고자 한다. 방법: 서울 및 경기 일부 지역에 소재하는 환경미화업체 근로자 중 대상자 543명을 대상으로 분석을 시행하였다. 근골격계 질환의 증상호소율은 NIOSH의 근골격계 질환의 자각증상에 대한 기준을 사용하였다. 환경미화원의 작업에 대한 인간공학적인 평가는 RULA. REBA를 사용하여 분석하였다. 결과: 환경미화원의 72.2%가 근골격계질환의 자각증상 및 통증이 있다고 답하였다. 전체 대상자 중에서 상지의 경우 39.6%,허리는 30.0%, 다리는 27.4%가 경도 이상의 통증을 호소하였다. 거리환경미화원의 자각증상 기준별 양성자 비율이 지역환경미화원보다 허리의 '증상 기준 3’를 제외한 모든 영역에서 높게 나타났다. 그리고 거리환경미화원이 상지의‘증상기준 1’,하지의‘증상기준 2∼3’의 영역에서 지역환경미화원보다 통계적으로 유의하게 (p<0.05) 높은 비율을 나타냈다. NIOSH 기준 1∼3에 양성자로 해당하는 군을 종속변수로 작업 이외에 근골격제 질환에 영향을 미칠 것으로 판단되는 인자의 영향을 확인하고자 다변량 로지스틱 회귀분석을 실시하였다. 그 결과 연령이 증가할수록‘증상기준 1’에 대하여 1.018배(95% CI: O.993∼1.046) 근골격계질환의 자각 증상이 증가하였던 것으로 확인되었다. 10시간 이상 작업 할 경우‘증상기준 1’과 ‘증상기준 2’를 모두 만족시키는 경우가 2.165(95% CI: 1.156∼4.1311,2.187배(95% CI: 1.071∼4.65l) 통계적으로 유의하게 높게 나타났다. 지역환경미화원의 RULA,RULA 접수 중 상지의 subtotal A 접수, REBA,REBA 접수 중 상지 total A 점수는 모두 거리환경미화원의 점수에 비해 높게 나타났다. 결론: 거리환경미화원이 지역환경미화원에 비하여 인간 공학적인 평가점수가 낮음에도,거리환경미화원이 상대적으로 높은 반복 작업의 빈도와 10시간 이상 작업 등에 의해 상지에 대한 근골격계 질환의 자각증상 호소한다고 판단된다. 환경미화원의 근골격계질환에 대한 다양한 대책 들이 필요하다. Objectives: The aim of this study was to estimate musculo-skeletal symtoms of municipal sanitation workers and to evaluate working conditions of municipal sanitation workers to search for the factors related to musculo-skeletal symptoms. Methods: We conducted a descriptive cross-sectional survey. The study subjects were comprised of 543 municipal sanitation workers in some divisions of Seoul and the Gyeonggi province. Musculo-skeletal analysis was done by using the modified criteria of NIOSH musuclo-skeletal symptoms, RULA and, REBA. Statistical analysis was done by using the chi-square test, multiple logistic regression analysis. Results: 72.2% of the subjects complained musculo-skeletal symptoms (39.6% on the upperlimbs, 30.0% on the lumbar region, 27.4% on the legs). For the musculo-skeletal symptoms criteria (1∼3), street cleaners complained of more musculo-skeletal symptoms than the solid waste collectors, after classifying the jobs of sanitation workers. On logistic regression analysis of the musculo-skeletal "symptoms criterial" positive groups, age was related to the criteria positive (Prevalence odds ratio=1.018, 95% CI: 0.993-1.046). The worktime was significantly related to 'criteria positive 1 and, 2' (Prevalence odds ratio=2.165 (95% CI: 1.156-4.131), 2.187 times (95% CI: 1.071-4.651)). The RULA score, the subtotal A score of RULA, the REBA score and , the total AREBA score of the upperlimbs of solid waste collectors were higher than those of the street cleaners. Conclusions: In spite of the lower ergonomic evaluation score of the street cleaner, the street cleaners complained of more musculo-skeletal symptoms than did the solid waste collectors due to more frequent repetitive motions, a longer work time (over 10 hours) etc. It is suggested that the sanitation workers need to use the proper methods to avoid musculo-skeletal disease.

      • An Aqueous Extract of Herbal Medicine ALWPs Enhances Cognitive Performance and Inhibits LPS-Induced Neuroinflammation via FAK/NF-κB Signaling Pathways

        Lee, Ju-Young,Joo, Bitna,Nam, Jin Han,Nam, Hye Yeon,Lee, Wonil,Nam, Youngpyo,Seo, Yongtaek,Kang, Hye-Jin,Cho, Hyun-Ji,Jang, Young Pyo,Kim, Jeongyeon,We, Young-Man,Koo, Ja Wook,Hoe, Hyang-Sook Frontiers Media S.A. 2018 FRONTIERS IN AGING NEUROSCIENCE Vol.10 No.-

        <P>Recent studies have shown that Liuwei Dihuang pills (LWPs) can positively affect learning, memory and neurogenesis. However, the underlying molecular mechanisms are not understood. In the present study, we developed ALWPs, a mixture of <I>Antler</I> and LWPs, and investigated whether ALWPs can affect neuroinflammatory responses. We found that ALWPs (500 mg/ml) inhibited lipopolysaccharide (LPS)-induced proinflammatory cytokine IL-1β mRNA levels in BV2 microglial cells but not primary astrocytes. ALWPs significantly reduced LPS-induced cell-surface levels of TLR4 to alter neuroinflammation. An examination of the molecular mechanisms by which ALWPs regulate the LPS-induced proinflammatory response revealed that ALWPs significantly downregulated LPS-induced levels of FAK phosphorylation, suggesting that ALWPs modulate FAK signaling to alter LPS-induced IL-1β levels. In addition, treatment with ALWPs followed by LPS resulted in decreased levels of the transcription factor NF-κB in the nucleus compared with LPS alone. Moreover, ALWPs significantly suppressed LPS-induced BV2 microglial cell migration. To examine whether ALWPs modulate learning and memory <I>in vivo</I>, wild-type C57BL/6J mice were orally administered ALWPs (200 mg/kg) or PBS daily for 3 days, intraperitoneally injected (i.p.) with LPS (250 μg/kg) or PBS, and assessed in Y maze and NOR tests. We observed that oral administration of ALWPs to LPS-injected wild-type C57BL/6J mice significantly rescued short- and long-term memory. More importantly, oral administration of ALWPs to LPS-injected wild-type C57BL/6J mice significantly reduced microglial activation in the hippocampus and cortex. Taken together, our results suggest that ALWPs can suppress neuroinflammation-associated cognitive deficits and that ALWPs have potential as a drug for neuroinflammation/neurodegeneration-related diseases, including Alzheimer’s disease (AD).</P>

      • SCOPUSKCI등재

        Original Article : Impact of immunosuppressant therapy on early recurrence of hepatocellular carcinoma after liver transplantation

        ( Ju Yeun Lee ),( Yul Hee Kim ),( Nam Joon Yi ),( Hyang Sook Kim ),( Hye Suk Lee ),( Byung Koo Lee ),( Hye Young Kim ),( Young Rok Choi ),( Geun Hong ),( Kwang Woong Lee ),( Yung Suk Suh4 ) 대한간학회 2014 Clinical and Molecular Hepatology(대한간학회지) Vol.20 No.2

        Background/Aims: The most commonly used immunosuppressant therapy after liver transplantation (LT) is a combination of tacrolimus and steroid. Basiliximab induction has recently been introduced; however, the most appropriate immunosuppression for hepatocellular carcinoma (HCC) patients after LT is still debated. Methods: Ninety-three LT recipients with HCC who took tacrolimus and steroids as major immunosuppressants were included. Induction with basiliximab was implemented in 43 patients (46.2%). Mycophenolate mofetil (MMF) was added to reduce the tacrolimus dosage (n=28, 30.1%). The 1-year tacrolimus exposure level was 7.2 ± 1.3 ng/mL (mean ± SD). Results: The 1- and 3-year recurrence rates of HCC were 12.9% and 19.4%, respectively. Tacrolimus exposure, cumulative steroid dosages, and MMF dosages had no impact on HCC recurrence. Induction therapy with basiliximab, high alpha fetoprotein (AFP; >400 ng/mL) and protein induced by vitamin K absence/antagonist-II (PIVKA-II; >100 mAU/mL) levels, and microvascular invasion were significant risk factors for 1-year recurrence (P<0.05). High AFP and PIVKA-II levels, and positive 18fluoro-2-deoxy-d-glucose positron-emission tomography findings were significantly associated with 3-year recurrence (P<0.05). Conclusions: Induction therapy with basiliximab, a strong immunosuppressant, may have a negative impact with respect to early HCC recurrence (i.e., within 1 year) in high-risk patients. (Clin Mol Hepatol 2014;20:192-203)

      • KCI등재후보

        뽕잎 엽위별 이화학성분 함량 변화

        박장현(Jang Hyun Park),남승희(Seung Hee Nam),임순희(Sun Hee Yim),구희연(Hui Yeon Koo),김현진(Hyun Jin Kim),김윤경(Yoon Kyeong Kim),최향철(Hyang Choul Choi) 한국차학회 2013 한국차학회지 Vol.19 No.4

        Mulberry leaves contain the highest amount of total nitrogen, total amino acid, tannin, and vitamin C at the first leaf with 5.33%, 3,607 mg 100 g⁻¹, 5.91%, or 309 mg 100 g⁻¹, respectively. The caffeine content of mulberry tea was 1.95% at the first leaf and decreased with 1.86% at the fourth leaf but increased with 1.92% at the fifth leaf. Mulberry tea showed the highest amount of sucrose at the first leaf, fructose at the second leaf, sorbitol at the third leaf and glucose at the sixth leaf. The total phenolics, flavonoid and antioxidant activity of mulberry tea were the highest at the third leafwith 586 mg 100 g⁻¹, 813 mg 100 g⁻¹, and 34.4%, respectively. The NO scavenging activity was high at the first leafwith 8.54% but low at the sixth leaf with 3.32%. The color value of mulberry tea showed the high L value at the third leave and high a and b values at the first leaf. The sensory evaluation data showed that the third leaf tea received the highest score with 84.9 points whereas the sixth leave tea showedthe lowest score with 78.2 points.

      • KCI등재후보

        시상의 정맥성 뇌경색과 전신성 심부정맥 혈전증을 동반한 호모시스틴뇨증 1례

        강주형(Ju Hyung Kang),구남향(Nam Hyang Koo),김성환(Sung Hwan Kim),조재현(Jae Hyun Cho),김선용(Sun Yong Kim) 대한소아신경학회 2002 대한소아신경학회지 Vol.10 No.2

        저자들은 양측 시상의 정맥성 뇌경색과 전신성 심부정맥 혈전증이 동반된 호모시스틴뇨증 증례를 국내에서 처음 경험하였기에 이를 문헌 고찰과 함께 보고하며, 호모시스틴뇨증은 조기발견 및 치료로 혈관계 합병증을 줄일 수 있다는 점에서 소아를 포함한 청소년 연령에서 혈전색전증이 발생한 경우 항상 호모시스틴뇨증의 가능성을 고려하여야 한다는 점을 강조한다. Homocystinuria is an autosomal recessive inherited disorder of methionine metabolism. The most common cause of homocystinuria is cystathione-β-synthase deficiency, which has the characteristic clinical features such as ectopia lentis, Marfanoid skeletal changes, mental retardation, and vascular thromboembolic events such as deep vein thrombosis, cerebral infarction, pulmonary embolism, and myocardial infarction. The thromboembolic vascular events occur in 20-50% of untreated patients with homocystinuria at the age of 15, and could be associated with vasculopathy related mortality in 20% of untreated patients before the age of 30. Therefore, homocystinuria is one of the important cause of stroke in children and young adults. Only 2 cases of homocystinuria were reported in Korea; one without vasculopathy and the other with cerebral infarction. Homocystinuria complicated with systemic deep vein thrombosis is first reported in Korea. We report a 13 year old female with homocystinuria complicated with severe systemic deep vein thrombosis and venous infarction of both thalami due to thrombosis of vein of Galen and internal cerebral vein.

      연관 검색어 추천

      이 검색어로 많이 본 자료

      활용도 높은 자료

      해외이동버튼