목적: 결절성 경화증에서 뇌실막밑 거대세포 별아교세포종은 잘 알려져 있는 뇌 내 병변중의 하나로서, 서서히 증식하지만, 몬로공의 폐쇄를 유발하여 뇌압상승으로 인한 신경학적 합병증...
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https://www.riss.kr/link?id=A101737153
2015
-
516
학술저널
1-6(6쪽)
1
0
상세조회0
다운로드국문 초록 (Abstract)
목적: 결절성 경화증에서 뇌실막밑 거대세포 별아교세포종은 잘 알려져 있는 뇌 내 병변중의 하나로서, 서서히 증식하지만, 몬로공의 폐쇄를 유발하여 뇌압상승으로 인한 신경학적 합병증...
목적: 결절성 경화증에서 뇌실막밑 거대세포 별아교세포종은 잘 알려져 있는 뇌 내 병변중의 하나로서, 서서히 증식하지만, 몬로공의 폐쇄를 유발하여 뇌압상승으로 인한 신경학적 합병증을 유발하기도 한다. 최적요법은 수술적인 종양 절제술이지만, 합병증과 위험성으로 인하여 수술의 시기에 있어 많은 논란이 있어왔다. 최근 mTOR 억제제의 개발로 약물치료에 대한 연구도 이루어져 왔으나, 아직은 그 근거가 충분치 않다. 이에 세브란스 어린이 병원에서 치료받은 환자들을 대상으로 뇌실막밑 거대세포 별아교세포종의 치료 현황 및 수술 결과를 분석하여 치료에 도움이 되고자 한다.
방법: 본원에서 2007년부터 2014년까지 진단받은 161명의 결절성 경화증 환자들을 후향적으로 분석하였다.
결과: 17명의 뇌실막밑 거대세포 별아교세포종 환자가 있었으며 이 중 네 명에서 수술적인 절제술을 받았다. 네 명 중 뇌압상승 증상이 있었던 두 명의 환자에서는 신경학적 합병증이 발생하였고, 영구적인 뇌실 복강 션트를 삽입하였으나, 증상이 없었던 두 명의 환자에서는 종양의 전 절제술이 가능하였으며 신경학적인 합병증도 없었다.
결론: 이를 통하여 뇌실막밑 거대세포 별아교세포종의 치료로서 약물 치료 방법뿐 아니라 조기 수술적인 치료가 증상이 없는 환자에서도 반드시 고려되어야 한다고 판단된다.
다국어 초록 (Multilingual Abstract)
Purpose: Subependymal giant cell astrocytomas (SEGAs) are a well-known complication of tuberous sclerosis complex (TSC). Treatment experience has been accumulated for several years. The aim of this study was to review our experiences in treatment of S...
Purpose: Subependymal giant cell astrocytomas (SEGAs) are a well-known complication of tuberous sclerosis complex (TSC). Treatment experience has been accumulated for several years. The aim of this study was to review our experiences in treatment of SEGAs and prognosis.
Method: From March 2007 to March 2014, 161 TSC patients were diagnosed. Of 161 patients, 17 patients met diagnostic criteria of SEGAs. We reviewed clinical data of SEGA patients retrogradely.
Results: Among 17 SEGAs patients, five patients underwent surgical treatment. Of five patients who underwent surgery, including two patients who underwent gamma knife surgery before tumorectomy, four patients underwent tumorectomy, and one patient underwent shunt operation. Out of four patients who underwent surgery, two patients with increased intracranial pressure (IICP) symptoms before surgery showed worse neurological outcome than the other two patients without IICP symptoms.
Conclusion: Our experiences suggest that early surgical intervention should be considered as one of treatment modalities for asymptomatic SEGA patients.
참고문헌 (Reference)
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1 Tee AR, "Tuberous sclerosis complex-1 and-2 gene products function together to inhibit mammalian target of rapamycin(mTOR)-mediated downstream signaling" 99 : 13571-13576, 2002
2 Curatolo P, "Tuberous sclerosis" 372 : 657-668, 2008
3 Jiang T, "The diagnosis and treatment of subependymal giant cell astrocytoma combined with tuberous sclerosis" 27 : 55-62, 2011
4 de Ribaupierre S, "Subependymal giant-cell astrocytomas in pediatric tuberous sclerosis disease : when should we operate?" 60 : 83-89, 2007
5 Sinson G, "Subependymal giant cell astrocytomas in children" 20 : 233-239, 1994
6 Roth J, "Subependymal giant cell astrocytoma: diagnosis, screening, and treatment. recommendations from the international tuberous sclerosis complex consensus conference 2012" 49 : 439-444, 2013
7 Campen CJ, "Subependymal giant cell astrocytoma(SEGA)treatment update" 13 : 380-385, 2011
8 Cuccia V, "Subependymal giant cell astrocytoma in children with tuberous sclerosis" 19 : 232-243, 2003
9 Kumar R, "Subependymal giant cell astrocytoma : a report of five cases" 27 : 274-280, 2004
10 Franz DN, "Rapamycin causes regression of astrocytomas in tuberous sclerosis complex" 59 : 490-498, 2006
11 Sun P, "Outcomes of resecting subependymal giant cell astrocytoma(SEGA)among patients with SEGA-related tuberous sclerosis complex : A national claims database analysis" 28 : 657-663, 2012
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학령전기 보행 장애의 원인 중 양성 급성 소아기 근염의 의미
Intracranial Epidural Abscess and Pansinusitis in an Eleven Year Old boy
Fukuyama Congenital Muscular Dystrophy in a Young Infant
Clinical Characteristics and Risk Factors of Multiple Recurrent Febrile Seizures among Children
학술지 이력
연월일 | 이력구분 | 이력상세 | 등재구분 |
---|---|---|---|
2019 | 평가예정 | 신규평가 신청대상 (신규평가) | |
2018-12-01 | 평가 | 등재후보 탈락 (계속평가) | |
2016-01-01 | 평가 | 등재후보학술지 선정 (신규평가) | |
2012-01-01 | 평가 | 등재후보 탈락 (등재후보1차) | |
2010-01-01 | 평가 | 등재후보 1차 FAIL (등재후보1차) | |
2008-01-01 | 평가 | 등재후보학술지 선정 (신규평가) |
학술지 인용정보
기준연도 | WOS-KCI 통합IF(2년) | KCIF(2년) | KCIF(3년) |
---|---|---|---|
2016 | 0.17 | 0.17 | 0.17 |
KCIF(4년) | KCIF(5년) | 중심성지수(3년) | 즉시성지수 |
0.16 | 0.14 | 0.384 | 0.02 |