• Journal of Korean Neurosurgical Society
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• 제59권5호
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• pp.512-517
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• 2016

Objective : The pathophysiology of idiopathic Chiari malformation (CM) type 1 is disturbance of free cerebrospinal fluid (CSF) flow and restoration of normal CSF flow is the mainstay of treatment. Additional migration of the medulla oblongata in pediatric patients is referred to as CM type 1.5, but its significance in adult patients is unknown. This study is to compare surgical outcomes of adult idiopathic CM type 1.5 with that of type 1. Methods : Thirty-eight consecutive adult patients (M : F=11 : 27; median, 33.5; range, 18-63) with syringomyelia due to idiopathic CM type 1 were reviewed. Migration of the medulla oblongata was noted in 13 patients. The modified McCormick scale (MMS) was used to evaluate functional status before and one year after surgery. All patients underwent foramen magnum decompression and duroplasty. Factors related to radiological success (${\geq}50%$ decrease in the diameter of the syrinx) were investigated. The follow-up period was $72.7{\pm}55.6$ months. Results : Preoperative functional status were MMS I in 11 patients and MMS II in 14 of CM type 1 and MMS I in 8 and II in 5 of CM type 1.5. Of patients with MMS II, 5/14 patients in group A and 3/5 patients in group B showed improvement and there was no case of deterioration. Radiological success was achieved in 32 (84%) patients and restoration of the cisterna magna (p=0.01; OR, 46.5) was the only significant factor. Conclusion : Migration of the medulla oblongata did not make a difference in the surgical outcome when the cisterna magna was restored.

• Molecules and Cells
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• 제46권12호
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• pp.746-756
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• 2023

A recent study revealed that the loss of Deup1 expression does not affect either centriole amplification or multicilia formation. Therefore, the deuterosome per se is not a platform for amplification of centrioles. In this study, we examine whether gain-of-function of Deup1 affects the development of multiciliated ependymal cells. Our time-lapse study reveals that deuterosomes with an average diameter of 300 nm have two different fates during ependymal differentiation. In the first instance, deuterosomes are scattered and gradually disappear as cells become multiciliated. In the second instance, deuterosomes self-organize into a larger aggregate, called a deuterosome cluster (DC). Unlike scattered deuterosomes, DCs possess centriole components primarily within their large structure. A characteristic of DC-containing cells is that they tend to become primary ciliated rather than multiciliated. Our in utero electroporation study shows that DCs in ependymal tissue are mostly observed at early postnatal stages, but are scarce at late postnatal stages, suggesting the presence of DC antagonists within the differentiating cells. Importantly, from our bead flow assay, ectopic expression of Deup1 significantly impairs cerebrospinal fluid flow. Furthermore, we show that expression of mouse Deup1 in Xenopus embryos has an inhibitory effect on differentiation of multiciliated cells in the epidermis. Taken together, we conclude that the DC formation of Deup1 in multiciliated cells inhibits production of multiple centrioles.

• Journal of Korean Neurosurgical Society
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• 제29권5호
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• pp.609-614
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• 2000

Objective : A retrograde clinical study was undertaken to determine the effect of opening lamina terminalis on the development of hydrocephalus after aneurysmal subarachnoid hemorrhage(SAH). We compared the incidence ratios of the development of hydrocephalus with and without opening lamina terminalis during operation after aneurysmal SAH. Patients and Methods : From Oct. 1996 to Sep. 1998, we performed 138 aneurysm surgery for 122 patients. In 98 cases, the lamina terminalis was opened to make direct cerebrospinal fluid flow from the third ventricle to subarachnoid space for prevention of delayed hydrocephalus. We compared the incidence of hydrocephalus after opening lamina terminalis to those without opened lamina terminalis. Results : In 95 cases, the hydrocephalus was noticed in 2 cases(2.2%). It is significantly lower in the group with opening lamina terminalis than the group without opening lamina terminalis(about 10%). Conclusion : It is simple and easy procedure to open the lamina terminalis during aneurysm surgery. With this maneuver, we could reduce the incidence of hydrocephalus after aneurysmal spontaneous SAH. Thus, it is considered that the opening lamina terminalis is one of the effective methods for preventing the development of hydrocephalus after aneurysmal spontaneous SAH.

• 대한약리학회지
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• 제31권1호
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• pp.27-37
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• 1995

본 연구에서는 콜린성 기전에 반응하여 분비되는 내피 의존성 이완물질(endothelium-derived relaxing factor, EDRF)나 nitric oxide(NO)가 마취 흰쥐의 뇌혈류 자가조절기전에 관여할 가능성을 관찰하였다. Acetylcholine($10^{-9}-10^{-6}M$)을 포함한 mock 뇌척수액(CSF)을 관류시 뇌연막 동맥은 농도에 의존하여 이완반응 나타내었고(평균; $19.3{\pm}1.7{\mu}m$, n=36), 이러한 이완반응은 $N{\omega}$-nitro-L-arginine(L-NNA, $10^{-5}M$)에 의해서 억제되었을 뿐 아니라 methylene blue($10^{-6}M$)나 oxyhemoglobin($10^{-6}M$)에 의하여도 억제되었다. 한편 이러한 acethlcholine에 의한 뇌연막동맥의 이완반응을 매게하는 무스카린 수용체는 무스카린 수용체 길항제의 봉쇄효과를 관찰한 실험에서 $M_1$과 $M_3$ 아형으로 생각되었다. L-Arginine을 함유한 mock CSF로 관류시 일어나는 일시적인 혈관이완반응은 NY 83583 ($10^{-5}M$)에 강력히 억제되었으나 L-NNA ($10^{-5}M$)에 의해서는 억제되지 아니하였다. 한편 acetylcholine과 L-arginine에 의한 혈관이완반응은 ATP-sensitive $K^+$ 통로 봉쇄제인 glibenclamide에 의해 유의하게 봉쇄되었다. 나아가 뇌연막동맥의 직경 변화를 동맥압의 변화에 대하여 검정한 결과 혈관이완과 혈관수축의 희귀 직선의 경사도는 $10^{-5}M$ L-NNA의 전처치에 의하여 영향을 받지 아니하였으나, $3{\times}10^{-6}M$ glibenclamide에 의해 유의하게 감소되었다. 이러한 결과로 보아 혈압하강에 대해 쥐의 뇌연막동맥에 나타나는 혈관이완반응은 EDRF(NO)에 의해 매개되지 않는다고 사료된다.

• Pediatric Infection and Vaccine
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• 제27권2호
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• pp.102-110
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• 2020

목적: 이번연구의목적은 human parechovirus (HPeV)로유발된패혈증의증을보였던 3개월미만의영아를대상으로임상적 특징 및 경과를 조사하는 것이다. 방법: 2018년 7월 1일부터 8월 31일까지 패혈증 의증으로 입원한 3개월 미만 영아들의 의무기록을 확인하였다. 환자들은 뇌척수액을 이용하여 역전사 중합효소 연쇄반응 검사를 시행하고 결과에 따라 HPeV 감염군(1군, 11명), 장바이러스 감염군(2군, 13명), 바이러스가 검출되지 않은 군(3군, 15명)으로 구분하였다. 결과: 1군은 빈맥, 빈호흡, 무호흡, 저혈압, 피부 변화를 보이는 환자가 2, 3군 보다 많았다(P<0.05). 또한 1군은 혈중 총 백혈구수가 다른 군에 비해 낮았다(5,622±2,355/μL, 9,397±2,282/μL and 12,312±7,452/μL, P=0.005). 뇌척수액 백혈구 수도 1군은 2, 3군에 비하여 낮았다 (0.9±1.7/μL, 85.1±163.6/μL, and 3.7±6.9/μL, P=0.06). 치료에서도 1군은 2, 3군 보다 승압제 보조(36.6% vs. 0% and 6.6%), 기계 환기 적용(18.1% vs. 0% and 0%)과 고유량 산소 사용(45.4% vs. 15.3% and 6.6%)이 더 많았다. 모든 환자들은 합병증 없이 회복 되었다. 결론: HPeV 감염은 심각한 임상 경과를 보이고, 3개월 미만의 영아에서 패혈증 유사 증후군의 원인이 될 수 있다. 따라서, HPeV 감염 여부를 조기에 진단하고 적절한 치료를 시작하는 것이 필요하다.