• Clinical and Experimental Pediatrics
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• 제53권4호
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• pp.603-606
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• 2010

부신생물 변연계뇌염(paraneoplastic limbic encephalitis)은 신경계의 전이가 없이 종양의 원격 작용에 의해 발생하는 질환으로 소아에서는 드물게 보고되고 있다. 저자들은 사춘기 여아에서 발생한 성숙 난소기형종에 동반된 부신생물 변연뇌염을 경험하고 보고하고자 한다. 15세 여자 환자가 신경정신증상, 기억력 저하, 경련, 의식 저하를 주소로 내원하였다. 뇌척수액 검사, 뇌 MRI는 정상이었으나, 뇌 SPECT 검사에서 양측 측두엽의 저관류가 관찰되었다. 복부 초음파와 MRI에서 좌측 난소의 낭성 종양이 발견되었다. 종양의 수술적 제거 후 성숙 난소기형종으로 확인되었으며, 종양 제거 및 면역글로불린 정맥 투여로 완전한 인지 기능의 회복을 보였다.

• Clinical and Experimental Pediatrics
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• 제57권9호
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• pp.410-415
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• 2014

Purpose: Neurofibromatosis 1 (NF1) is an autosomal dominant condition caused by an NF1 gene mutation. NF1 is also a multisystem disorder that primarily affects the skin and nervous system. The goal of this study was to delineate the phenotypic characterization and assess the NF1 mutational spectrum in patients with NF1. Methods: A total of 42 patients, 14 females and 28 males, were enrolled in this study. Clinical manifestations and results of the genetic study were retrospectively reviewed. Results: Age of the patients at the time of NF1 diagnosis was $15.8{\pm}14.6$ years (range, 1-62 years). Twelve patients (28.6%) had a family history of NF1. Among the 42 patients, $Caf\acute{e}$-au-lait spots were shown in 42 (100%), neurofibroma in 31 (73.8%), freckling in 22 (52.4%), and Lisch nodules in seven (16.7%). The most common abnormal finding in the brain was hamartoma (20%). Mental retardation was observed in five patients (11.9%), seizures in one patient (2.4%), and plexiform neurofibromas (PNFs) in four patients (9.5%). One patient with PNFs died due to a malignant peripheral nerve sheath tumor in the chest cavity. Genetic analysis of seven patients identified six single base substitutions (three missense and three nonsense) and one small deletion. Among these mutations, five (71.4%) were novel (two missense mutations: p.Leu1773Pro, p.His1170Leu; two nonsense mutations: $p.Arg2517^*$, $p.Cys2371^*$; one small deletion: $p.Leu1944Phefs^*6$). Conclusion: The clinical characteristics of 42 Korean patients with NF1 were extremely variable and the mutations of the NF1 gene were genetically heterogeneous with a high mutation-detection rate.

• Clinical and Experimental Pediatrics
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• 제60권11호
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• pp.365-372
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• 2017

Purpose: The mechanism for the pathogenesis of adriamycin (ADR)-induced cardiomyopathy is not yet known. Different hypotheses include the production of free radicals, an interaction between ADR and nuclear components, and a disruption in cardiac-specific gene expression. Apoptosis has also been proposed as being involved in cardiac dysfunction. The purpose of this study was to determine if apoptosis might play a role in ADR-induced cardiomyopathy. Methods: Male Sprague-Dawley rats were separated into 2 groups: the control group (C group) and the experimental group (ADR 5 mg/wk for 3 weeks through intraperitoneal injections; A group). Echocardiographic images were obtained at week 3. Changes in caspase-3, B-cell leukemia/lymphoma (Bcl)-2, Bcl-2-associated X (Bax), interleukin (IL)-6, tumor necrosis $factor-{\alpha}$, brain natriuretic peptide (BNP), troponin I, collagen 1, and collagen 3 protein expression from the left ventricle tissues of C and A group rats were determined by Western blot. Results: Ascites and heart failure as well as left ventricular hypertrophy were noted in the A group. Ejection fraction and shortening fraction were significantly lower in the A group by echocardiography. The expression of caspase-3, Bax, IL-6, BNP, collagen 1, and collagen 3 were significantly higher in the A group as compared with the C group. Protein expression of Bcl-2 decreased significantly in the A group compared with the C group. Conclusion: ADR induced an upregulation of caspase-3, Bax, IL-6, and collagen, as well as a depression in Bcl-2. Thus, apoptosis and fibrosis may play an important role in ADR-induced cardiomyopathy.

• Radiation Oncology Journal
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• 제11권1호
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• pp.133-141
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• 1993

From Jan.1982 to Dec.1990, 77 patients with rectal cancer were treated with curative surgical resection followed by postoperative adjuvant irradiation alone or combined with chemotherapy at the Department of Radiation Oncology, Korea University Hospital (KUH). Fifty-four (54/77, $70.1{\%}$) patients underwent abdominoperineal resection , 20 (20/77, $26{\%}$) patients underwent low anterior resection, and 3 (3/77, $3.9{\%}$,) patients had wide excision only. Thirty-nine (39/77, $50.5{\%}$) received sequential chemotherapy (2 cycles to 12 cycles). The 5-year survival rate for the entire group was $43{\%}:\;78.2{\%}$ in B2+B3, $39.4{\%}$ in stage C1+C2+C3. Survival rates decreased with increasing penetration of the bowel wall by tumor and the presence of regional lymph node metastasis. Those patients survival who underwent an abdominoperineal resection also experienced a significant decrease in compared to low anterior resection ($23.1{\%}$ vs. $63.8{\%}$ in 5-year survival, p <0.05). Local failure occurred in 15 ($19.5{\%}$) out of the 77 patients overall, 1($5.3{\%}$) of 19 in stage B2+B3, and 14 ($24.1{\%}$,) of 58 in stage C1+C2+C3. Presacral area was most common site of local failufre (8/17, $47.1{\%}$). Distant failure occurred in 13 ($16.9{\%}$) of 77 patients. The most frequent site of distant failure was the lung followed by the liver, the bone, and the brain. Combined locoregional and distant failure occurred in 2 ($2.6{\%}$) of 77 patients. Pathological confirmation of perirectal fat and/or regional lymph node involvement resulted in a singificant decrease in survival and local control.

• 동의신경정신과학회지
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• 제12궈1호
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• pp.137-149
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• 2001

Cytokines are polypeptides which possess various biological properties affecting. host defense function and response to disease. Inflammatory cytokines, tumor necrosis $factor-{\alpha}$(TNF-${\alpha}$), interleukin(IL)-1 and IL-6 induce inflammation, fever, hypotension and pain when injected into animals or human subject. When glial cell cultures were prepared from neonatal mice or rats, astrocytes were reported to produce these inflammatory cytokines to viral infection, lipopolysaccharide(LPS), or cytokines. The purpose of this study was to investigate the regulatory effect of these cytokines secretion from primary cultures of rat astrocytes. Substance P(SP) can stimulate secretion of TNF-${\alpha}$ from astrocytes stimulated with LPS. Sesim-Tang significantly inhibited the TNF-${\alpha}$ secretion by astrocytes stimulated with SP and LPS. IL-1 has been shown to elevate TNF-${\alpha}$ secretion from LPS-stimulated astrocytes while having no effect on astrocytes in the absence of LPS. We therefore also investigated whether IL-1 mediated inhibition of TNF-${\alpha}$ secretion from primary astrocytes by Sesim-Tang. Treatment of Sesim-Tang to astrocytes stimulated with both LPS and SP decreased IL-1 secretion significantly. The secretion of TNF-${\alpha}$ by LPS and SP in astrocytes was progressively inhibited with increasing amount of IL-1 neutralizing antibody. Furthermore Sesim-Tang inhibited the IL-6 secretion by astrocytes stimulated with SP and LPS. The inhibitory effect of inflammatory cytokines by Sesim-Tang, observed in this study, might reflect an antiinflammatory activity and a reduction of various-type pains, fever etc. in the central nervous system.

• 생약학회지
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• 제43권4호
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• pp.316-322
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• 2012

Siegesbeckia Herba is known to have anti-oxidant, anti-inflammatory, anti-allergic and anti-tumor. The objective of this study is to explore the neuroprotective effect of Siegesbeckia Herba against glutamate-induced oxidative stress in mouse hippocampal HT22 cells. Siegesbeckia Herba 70% ethanol extract and solvent fractions have the potent neroprotective effects on glutamate-induced nerotoxicity by induced the expression of heme oxygenase (HO)-1 in the mouse hippocampal HT22 cells. Especially, ethyl acetate fraction showed higher protective effect. In HT22 cell, Siegesbeckia Herba ethyl acetate fraction makes the nuclear accumulation of Nrf2. Further, we found that treatment with c-JUN N-terminal kinase (JNK) inhibitor (SP600125) reduced Siegesbeckia Herba ethyl acetate fraction induced HO-1 expression and Siegesbeckia Herba ethyl acetate fraction also increased JNK phosphorylation. In conclusion, the ethyl acetate fraction of 70% ethanol extract of Siegesbeckia Herba significantly protect glutamate-induced oxidative damage by induction of HO-1 via Nrf2 and JNK pathway in mouse hippocampal HT22. Taken together these finding suggest that Siegesbeckia Herba ethyl acetate fraction good source for taking active compounds and may be a potential therapeutic for brain disorder by targeting the oxidative stress of neuronal cell.

• Radiation Oncology Journal
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• 제28권4호
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• pp.211-218
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• 2010

목적: 레티노인산(All-trans retinoic acid, ATRA)은 뇌종양 세포의 증식을 억제한다. 최근 ATRA에 의한 카타라제의 유도가 보고된 바 있다. 이에 저자들은 ATRA의한 카타라제의 유도가 ATRA의 세포증식 억제 효과와 관련이 있는지 알아보고자 하였다. 대상 및 방법: 36B10세포를 0~50${\mu}M$ ATRA로 24, 48시간 처리한 후 카타라제의 mRNA, 단백질 및 활성도를 측정하였다. 반응성산소종(reactive oxygen species, ROS)은 2',7'-dichlorofluorescin diacetate를 사용하여 측정하였고, 세포사는 clonogenic assay로 측정하였다. 결과: 카타라제의 mRNA, 단백질 및 활성도는 ATRA의 농도 및 처리 시간에 따라 증가하였다. ATRA에 의해 증가한 카타라제의 활성도는 3-amino-1,2,4-triazole (ATZ)에 의해 감소하였다. ATRA에 의해 증가한 ROS는 ATZ에 의해 감소하였다. ATRA에 의해 감소한 세포 생존분획은 ATZ에 의해 부분적으로 증가하였다. 결론: ATRA에 의해 유도된 카타라제는 ROS의 과생산과 관련 있으며 이는 36B10세포의 증식 억제를 가져온다.

• The Korean Journal of Pain
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• 제2권1호
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• pp.57-60
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• 1989

The Melkersson-Rosenthal (M-R) syndrome consists of a triad of (1) recurrent peripheral facial nerve paralysis which develops alternately on both sides of face, (2) non-inflammatory facial edema, and (3) fissuring of tongue. A 59 years old female patient developed the left facial palsy on September, 1988. Right facial palsy developed continuously 2 months later after the spontaneous remission of left facial palsy. On February, 1989, we have found out M-R syndrome which accompanied with migraine type of intermittent headache, and hypertension in one attack of cerebral stroke several years ago, there were no diabetes mellitus, pulmonary tuberculosis and brain tumor in clinical studies. Although the causes of this syndrome were not noted, we performed the stellate ganglion block and transcutaneous electrical nerve stimulation for treatment of the palsy, but the clinical effectiveness of these were not satisfactory.

• Advances in pediatric surgery
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• 제13권2호
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• pp.169-178
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• 2007

Teratomas arising from totipotential primitive germ cell are composed of 2 or 3 germ cell layers. We reviewed the records of 166 children who underwent the operation for teratoma from Jan, 1990 through April, 2007. There were 40 boys and 126 girls (average age $6.93{\pm}5.83$ years). Primary sites were ovary (n = 88), sacrococcygeum (n = 24), testis (n = 16), retroperitoneum (n = 16), mediastinum (n = 8), brain (n = 4), thymus (n = 3) and a single tumor involved the adrenal gland, neck, middle ear, oropharynx, stomach, pelvis, omentum, chest wall and scalp. Teratomas were mature in 141 patients, and immature in 25. Six patients who had ovarian immature teratomas grade 2 or 3 with peritoneal gliomatosis underwent adjuvant chemotherapy. During follow up period, 6 mature teratomas recurred at sacrococcygeal area (n = 1) and contralateral ovary (n = 5). Five patients of them underwent reoperation and diagnosed as a mature teratoma, but one who had underwent a left salpingooophrectomy with right ovary cystectomy at initial operation was observed carefully. Teratomas were dominant in female patients and developed at various organs. Complete excision was needed for good prognosis. In case of immature teratoma, complete excision and appropriate chemotherapy according to grading can contribute to favorable results.

• 대한두개안면성형외과학회지
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• 제17권2호
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• pp.68-76
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• 2016

Background: In scalp allotransplantation, the scalp from a brain-dead donor, including hair, is transferred to a recipient with scalp defects. Opinions differ on the appropriateness of scalp allotransplantation. In order to maintain graft function and cosmetic outcomes, scalp transplantation recipients would need to receive lifelong immunosuppression treatments. The risks of this immunosuppression have to be balanced against the fact that receiving a scalp allotransplant does not extend lifespan or restore a physical function. Therefore, the present study aimed to investigate risk acceptance and expectations regarding scalp allotransplantation in different populations. Methods: A questionnaire survey study was conducted. A total of 300 subjects participated; survey was conducted amongst the general public (n=100), kidney transplantation recipients (n=50), a group of patient who required scalp reconstruction due to tumor or trauma (n=50), and physicians (n=100). The survey was modified by using the Korean version of the Louisville instrument for transplantation questionnaire. Results: Risk acceptance and expectations for scalp transplantation varied widely across the groups. Kidney transplantation recipients revealed the highest risk acceptance and expectations, whereas the physicians were most resistant to the risks of scalp transplantation. Conclusion: Our study demonstrates that, in specific groups, scalp allotransplantation and the need for immunosuppression carries an acceptable risk despite the lack of lifeextending benefits. Our results suggest that scalp allotransplantation can be an acceptable alternative to existing scalp reconstruction surgeries in patients with pre-existing need for immunosuppression.