• 대한자기공명의과학회:학술대회논문집
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• 대한자기공명의과학회 2003년도 제8차 학술대회 초록집
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• pp.99-99
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• 2003

To exhibit our clinical experience of diffusion-weighted (DW) MR imaging for various brain pathologies and to determine its role in characterizing brain pathologies in children. DW images in 177 children (M：F＝96：81, mean age, 4.7 years) with various brain pathologies were retrospectively collected over past 3 years. DW images (b value： 1000 s/mm) were reviewed along with corresponding apparent diffusion coefficient (ADC) maps. Brain pathologies included cystic or solid brain tumor (n = 55), cerebral infarct (n = 32), cerebritis with or without brain abscess (n = 21), metabolic or toxic brain disorder (n = 19), demyelinating disease (n = 16), hypoxic-ischemic encephalopathy (n = 16), intracerebral hemorrhage including traumatic brain lesion (n = 15), and posterior reversible leukoencephalopathy (n = 3). We reviewed whether DW images and ADCmaps contribute to further characterization of brain pathologies by defining a chronological age of lesions, the presence of cytotoxic edema in lesions, and the nature of cystic lesions.

• Journal of Medicine and Life Science
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• 제16권3호
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• pp.55-59
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• 2019

Cerebral vessels are functionally and structurally specialized to provide adequate blood flow to brain which shows high metabolic rates. Cerebral hemorrhage or ischemic infarction due to cerebrovascular injury or occlusion can cause the immediate brain damage, and if not treated rapidly, can lead to serious or permanent brain damages, and sometimes life-threatening. Unlike these popular cerebrovascular diseases, there are diseases caused by genetic problems. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is one of them. CADASIL does not show the high incidence, but it is considered to be significantly affected by regional obstructiveness such as islands and therefore, to be an important genetic disease in Jeju. This paper aims to summarize the possibility of animal model research that can provide preclinical data for CADASIL disease research and to evaluate its applicability in future research plans.

• Clinical and Experimental Pediatrics
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• 제46권11호
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• pp.1112-1117
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• 2003

목 적 : 소아의 악성 종양에 대한 새로운 치료법의 개발로 환아의 생존 기간이 증가하는 추세에 있으나 이로 인한 신경계 합병증의 발생도 증가하고 있다. 저자들은 항암 치료를 시행한 소아 종양 환아에서 나타난 중추 신경계 합병증에 대한 임상 양상을 알아보고자 하였다. 방 법 : 1995년 1월부터 2002년 11월까지 동산병원 소아과에서 항암치료를 시행한 환아 중 신경계 합병증이 동반되었던 15례의 환아를 대상으로 그 임상상을 후향적으로 검토하였다. 결 과 : 전체 15례 중에서 방사선학적 검사로 원인 질환을 밝힌 경우는 모두 13례로 백질뇌병증이 7례, 무기질화 미세혈관병증이 4례, 뇌경색이 3례, 뇌출혈이 1례, 저산소성 허혈성 뇌증이 1례, 2가지 이상의 합병증을 함께 나타낸 경우는 2례였으며, 혈액학적 방사선학적 검사상에서 이상 소견이 없이 MTX의 부작용으로 생각되는 경련성 질환이 2례였다. 뇌출혈과 뇌경색이 나타낸 경우는 예후가 나빴으며 전해질 불균형으로 인한 저산소성 허혈성 뇌증에서는 원인 교정 후 후유증 없이 회복되었다. 결 론 : 소아 종양 환아에서 치료 중 발생한 신경계 합병증은 질환의 예후에 큰 영향을 미치고 신경인지 발달에 장애를 남기므로 이들 합병증에 대한 예방, 진단 및 즉각적인 치료와 추적관찰이 필요할 것이다.

• Asian Pacific Journal of Cancer Prevention
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• 제15권14호
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• pp.5945-5950
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• 2014

Objectives: To retrospectively review the safety and clinical efficacy of bevacizumab concomitant with chemotherapy in Chinese patients with advanced non-squamous non-small cell lung cancer (NSNSCLC). Methods: Clinical data for 79 patients with NSNSCLC who received bevacizumab concomitant with chemotherapy in Chinese PLA General Hospital from April 28th 2009 to May 5th 2013 were retrospectively reviewed to analyze the clinical efficacy including disease control rate (DCR), overall response rate (ORR), progression-free survival (PFS), overall survival (OS), the Eastern Cooperative Oncology Group (ECOG) score and the safety. Results: The Eastern Cooperative Oncology Group (ECOG) score was 0-2. By the final cutoff date (June 9, 2013), 54 (68.4%) patients had disease progression and 37 (46.8%) died. The ORR was 32.9% and the DCR was 83.5%. The ORR of the first-, second-, and third- or later-line treatments were 51.4%, 25.0% and 12.5%, while the DCR were 94.3%, 80.0% and 70.8%, respectively. The median OS (mOS) and PFS (mPFS) were 13.5 and 5.83 months, respectively. The mOS of patients with the first-, second-, and third- or later- line treatments were 16.2, 10.9 and 8.30 months, while the mPFS were 7.27, 5.90 and 5.17 months, respectively. Chemotherapy-related adverse events included myelosuppression, vomiting, hepatic dysfunction and renal dysfunction, while the common serious bevacizumab-related adverse events were thromboembolic problems, gastrointestinal perforation and reversible posterior leukoencephalopathy syndrome, which could be well managed. Conclusions: Bevacizumab concomitant with chemotherapy is effective and the related toxicity can be well tolerated in Chinese patients with NSNSCLC.

• 대한응급의학회지
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• 제29권5호
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• pp.519-528
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• 2018

Objective: Delayed neuropsychiatric sequelae (DNS) following carbon monoxide (CO) poisoning, which may result from a demyelinating leukoencephalopathy, is a disease with a poor prognosis. This study examined the factors affecting the long-term prognosis of DNS and the efficacy of hyperbaric oxygen therapy (HBOT) in patients with DNS. Methods: This retrospective study included 84 patients with DNS following CO poisoning from January 2013 to June 2016. HBOT was given to 24 patients. The patients were divided into an improvement group and non-improvement group based on their clinical condition on a telephone interview at intervals between 3 months and 3 years after the onset of DNS. The improvement group was defined as having Cerebral Performance Category (CPC) scores in their daily life that improve to 1 or 2 grade. Results: Of the 594 patients, DNS were found in 18.2%, and 70.2% (59 of 84) of the patients with DNS improved. The prognostic factors for the improvement of DNS were an age of 45 years or less (odds ratio [OR], 12.068; 95% confidence interval [CI], 2.393-60.858; P<0.005), CPC score of 1 or 2 group at the time of DNS onset (OR, 12.361; 95% CI, 3.161-48.330; P<0.005), and a lucid interval longer than 20 days (OR, 5.164; 95% CI, 1.393-19.141; P<0.01). HBOT was not associated with the improvement of DNS in CO poisoning (OR, 0.467; 95% CI, 0.172-1.269; P>0.1). Conclusion: Patients aged less than 45 years, low grade CPC score of 1 and 2, and lucid interval longer than 20 days are more likely to have a good prognosis. On the other hand, HBOT failed to produce a benefit for DNS patients.

• Journal of Medicine and Life Science
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• 제17권3호
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• pp.65-73
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• 2020

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a single-gene disease of the cerebral small blood vessels caused by mutations in the NOTCH3 gene on chromosome 19. Although CADASIL was known as a rare disease, recent research has suggested that the NOTCH variants could be found frequently even in the general population. The main clinical features included recurrent stroke, migraine, psychiatric symptoms, and progressive cognitive decline. On brain magnetic resonance imaging, patients with CADASIL showed multifocal white matter hyperintensity lesions, lacunar infarcts, microbleeds, and brain atrophy. Among them, lacunar infarcts and brain atrophy are important in predicting the clinical outcomes of patients with CADASIL. In the Jeju National University Hospital, we have diagnosed 213 CADASIL patients from 2004 to 2020. Most NOTCH3 mutations were located in exon 11 (94.4%), and p.Arg544Cys was the most common mutation. The mean age at diagnosis was 61.0±12.8 years. The most common presenting symptoms were ischemic stroke (24.4%), followed by cognitive impairment(15.0%), headache (8.9%), and dizziness(8.0%). Although the exact prevalence of CADASIL in Jeju is still unknown, the disease prevalence could be as high as 1% of the population considering the prevalence reported in Taiwan. Therefore, it is necessary to discover efficient biomarkers and genetic tests that can accurately screen and diagnose patients suspected of having CADASIL in this region. Ultimately, it is urgent to explore the exact pathogenesis of the disease to identify leading substances of treatment potential, and for this, multi-disciplinary research through active support from the Jeju provincial government as well as the national government is essential.

• Journal of Korean Neurosurgical Society
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• 제30권3호
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• pp.334-341
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• 2001

Objective : We have currently changed treatment strategies to methotrexate(MTX)-based preirradiation chemotherapy with subsequent planned radiation for the initial therapy of primary central nervous system lymphoma (PCNSL). The aim of this study was to evaluate the results of treating PCNSL with chemotherapy plus radiotherapy (CRT) or radiotherapy(RT) alone. Method and Material : This study involved 10 females and 3 males patients with a mean age of 54.2 years. All patients underwent surgery, open(8 cases) or stereotactic biopsy(5 cases) for histological diagnosis. Eleven tumors were diffuse large B-cell lymphomas. Tumor volume change in the follow-up images and survival time were evaluated in patients treated with CRT and RT alone. In the beginning, two patients received ProMACE-Cytabom chemotherapeutic regimen, but did not complete the course and died of progressive tumor 8 and 9 months after diagnosis, respectively. One patient died at 6 months before chemotherapy. These three were excluded from the survival analysis. Five patients(RT group) completed full courses of cranial irradiation with or without boost. For the current combined modality treatment, high-dose MTXbased chemotherapy(systemic and intrathecal MTX, IV vincristine, and oral procarbazine) followed by whole brain irrdiation to 45Gy to tumor was introduced in 5 patients of CRT group. Result : A complete response was achieved in three of five who received RT only and in all of five who received CRT. All patients in CRT groups are in disease free status at a mean 23 months following therapy. The RT group patients refused any additional salvage therapy at tumor relapse and survived at mean 20 months from diagnosis. The Karnofsky performance status improved in eight of ten patients with treatment. The treatment toxicity included leukoencephalopathy in RT group and severe leukopenia, transient hepatitis, avascular necrosis of femoral head, hearing loss, and amenorrhea in CRT group, respectively. Conclusion : The combined modality therapy of MTX-based chemotherapy plus radiotherapy for PCNSL may enhance tumor response and improve patient survival. The patients who received CRT should be carefully followed up because of the higher risk of treatment-induced late neurotoxicity.

• Childhood Kidney Diseases
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• 제17권2호
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• pp.149-153
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• 2013

응고성 미세혈관병증은 빠른 진단이 예후에 중요한 인자이나, 현재의 진단 기준에 따라서는 thrombotic thrombocytopenic purpura, haemolytic uremic syndrome 외의 비전형적인 응고성 미세혈관병증의 진단이 늦어짐에 따라 나쁜 예후를 초래하게 되는 경우가 많다고 보고되어 있다. 본 저자들은 시행한 혈액 검사상 용혈의 증거가 없는 빈혈, 혈소판 감소증 그리고 급성 신부전을 보인 소아 환아에서 신조직 검사를 통해 비특이적 응고성 미세혈관병증을 진단받은 1증례를 보고하고자 한다. 14세 여자 환아는 3주간 지속된 발열, 구역과 전신 부종을 주소로 본원으로 전원되었다. 내원하여 시행한 혈액 검사상 빈혈과 혈소판 감소증을 보였으나, 용혈의 증거는 없었으며, 혈정 크레아티닌이 증가되어 있었다. 내원 이후 급성 신부전과 발열은 지속적으로 진행되었으며, 소변 검사상 단백뇨가 발생하였다. 환아는 내원 40일경 신고혈압과 동반된 전신 경련이 5분간 있어 뇌 자기 공명 영상을 촬영하였으며, 가역성 후백질 뇌병증 증후군의 양상을 보여 항 경련제 투여를 시작하였다. 이후 지속되는 혈소판 감소증 및 발열은 고용량 스테로이드 치료를 진행한 후 호전되었으나, 급성 신부전 및 단백뇨가 지속되어 신장 조직 검사를 진행하였으며, 검사 결과상 혈전성 미세혈관병증의 소견을 보였다. 이와 같이 조직검사상에서는 응고성 미세혈관병증을 보이나 전형적인 응고성 미세혈관병증의 혈액학적인 진단 기준이 충족되지 않는 비특이적 형태의 응고성 혈관병증의 효과적인 진단을 위하여 보체 기전이나 ADAMTS 13와 같은 유전자 범위의 보다 활발한 연구를 통한 효과적인 진단 기준의 마련이 되어야 할 것으로 보인다.

• 대한영상의학회지
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• 제84권3호
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• pp.736-744
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• 2023

성인형 알렉산더병은 운동 실조, 경련성 마비 또는 뇌간 징후를 나타내는 드문 유전 질환이다. 성인형 알렉산더병의 진단은 흔히 자기공명영상 검사 소견을 통해 내려진다. 이에 저자들은 glial fibrillary acidic protein (이하 GFAP) 유전자 변이 분석을 통해 확진된 37세와 61세 성인형 알렉산더병 환자의 특징적인 자기공명영상 소견과 추적 검사상 변화 사례에 대해 보고하고자 한다. 자기공명영상에서 환자들은 전형적인 뇌간 위축과 백질 이상 소견을 보였다. 특징적인 자기공명영상을 바탕으로 추정진단을 내렸으며 이후 GFAP 유전자 변이 분석을 통해 확진되었다. 또한, 추적 관찰 검사에서 연수와 상부 경부 척수 위축의 진행을 보였다. 이러한 성인형 알렉산더병의 특징적인 자기공명영상 소견에 대한 이해를 바탕으로, 감별진단을 위한 GFAP 유전자 검사 시행 결정에 도움이 될 수 있을 것이다.