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신우종,문여옥,윤혜란,동은실,안영민,Shin, Woo Jong,Moon, Yeo Ok,Yoon, Hye Ran,Dong, Eun Sil,Ahn, Young Min 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.3
출생 이후 머리둘레가 커지는 1개월 남아에게서 뇌초음파 검사상 지주막하 공간이 확장되어 있고 백질의 음영이 증가 되어 있었고 뇌 MRI 소견은 실비우스열의 확장과 양측 대뇌, 소뇌 피질과 백질이 T1 강조 영상에서 저신호 강도와 T2 강조영상에서 고신호 강도를 보였으며 경피생검으로 얻은 섬유아세포 배양에서 glutaryl-CoA 탈수소효소의 활성도가 전혀 없어 GA1을 확진하게 되었다. 대두증이 있는 신생아나 영아에서 전측두엽 위축의 신경방사선학적 징후가 있으면 우선적으로 GA1을 의심해 보아야 한다. 이후 특수분유인 Glutatex 분유의 수유와 카르니틴, 리보플라빈 보충요법을 시행하여 대사성 위기나 급성 뇌증의 위기 없이 양호한 발달을 하고 있는 환아를 경험하였기에 보고하는 바이다. Glutaric aciduria type 1(GA1) is an autosomal recessive disorder of the lysine, hydroxylysine and tryptophan metabolism caused by the deficiency of mitochondrial glutaryl-CoA dehydrogenase. This disease is characterized by macrocephaly at birth or shortly after birth and various neurologic symptoms. Between the first weeks and the 4-5th year of life, intercurrent illness such as viral infections, gastroenteritis, or even routine immunizations can trigger acute encephalopathy, causing injury to caudate nucleus and putamen. But intellectual functions are well preserved until late in the disease course. We report a one-month-old male infant with macrocephaly and hypotonia. In brain MRI, there was frontotemporal atrophy(widening of sylvian cistern). In metabolic investigation, there were high glutarylcarnitine level in tandem mass spectrometry and high glutarate in urine organic acid analysis, GA1 was confirmed by absent glutaryl-CoA dehydrogenase activity in fibroblast culture. He was managed with lysine free milk and carnitine and riboflavin. He developed well without a metabolic crisis. If there is macrocephaly in an infant with neuroradiologic sign of frontotemporal atrophy, GA1 should have a high priority in the differential diagnosis. Because current therapy can prevent brain degeneration in more than 90% of affected infants who are treated prospectively, recognition of this disorder before the brain has been injured is essential for treatment.
경막외 카테터의 고정방법과 수술후 카테터의 위치 변화와의 관계
신우종(Woo Jong Shin),염종훈(Jong Hoon Yeom),김희 대한통증학회 1997 The Korean Journal of Pain Vol.10 No.1
N/A Background: Patients mover more as their post operative pain decrease. With the increase in movement there will be a tendency for the epidural catheter to migrate out of its original position. We studied 2 methods of flxation of the epidural catheter and the changes in position as related to patient movement. Methods: Patients were divided into two groups. Patients in Group A had their epidural catheter formed with a circular loop at the (skin) exit site then directed over the right shoulder. Group B had the epidural catheter flxed with Fixomull on the exit site without forming a circular loop. At the end of the operation, 3 mg of epidural morphine was injected via indwelling epidural catheter for postop- erative pain control. Epidural catheter depth was measured 24 hours later. Results: The overall rate of migration of epidural catheter was 61.9% In Group A, number of patients whose catheter migrated over 0.5 cm was 23(69.9%) with l4 inward migration and 9 outward migration. Group B had 16(53%) patients catheters migrate over 0.5 cm, with 2 patients having inward migration and 14 outward migration. Conclusions: Although the rates of migration of epidural catheter were similar for both groups, the number of inner migration of catheter, which could result serious complications, was significantly lower in Group B than Group A. Based on our results we recommend the epidural catheter be fixed without a circular loop.
실험연구 : Midazolam이 가토의 대뇌동맥 평활근세포의 외향성 K+ 전류에 미치는 영향
신영철 ( Young Chul Shin ),심재항 ( Jae Hang Shim ),전우재 ( Woo Jae Jeon ),조상윤 ( Sang Yoon Cho ),신우종 ( Woo Jong Shin ),김경헌 ( Kyoung Hun Kim ),염종훈 ( Jong Hoon Yeom ) 대한마취과학회 2007 Korean Journal of Anesthesiology Vol.52 No.1
Background: Midazolam has a direct relaxing effect on vascular smooth muscle, but the mechanisms that this agent produces muscle relaxation are not fully understood. The current study was performed to identify the effects of the midazolam on K+ channels current in rabbit cerebral arterial smooth muscle cells. Methods: Whole cell patch-clamp recording technique was used to evaluate the effects of midazolam (0.1 to 100μM) on outward K+ channel currents in dispersed rabbit cerebral arterial smooth muscle cells. Results: Outward K+ currents of rabbit cerebral artery smooth muscle cells were voltage-dependent. Midazolam (10, 100μM) tested significantly inhibited outward K+ currents in a dose-dependent manner and half-blocking concentration (IC50) was 15.94μm at 60 mV. Conclusions: Midazolam inhibit outward K+ currents of rabbit cerebral arterial smooth muscle cells. Further study will be needed to determine the effect of midazolam on calcium channel current because it is unclear if the inhibitory effect of midazolam on outward K+ current induces vasoconstriction. (Korean J Anesthesiol 2007; 52: 67~71)
염종훈 ( Jong Hoon Yeom ),신우종 ( Woo Jong Shin ),김유정 ( Yu Jung Kim ),심재항 ( Jae Hang Shim ),전우재 ( Woo Jae Jeon ),조상윤 ( Sang Yun Cho ),김경헌 ( Kyoung Hun Kim ) 대한마취과학회 2009 Korean Journal of Anesthesiology Vol.56 No.5
Background: We hypothesized that pressure control ventilation allows a more even distribution in the lung and better maintenance of the mean airway pressure than is achieved with volume control ventilation. We try to compare the effect of pressure control ventilation (PC) with that of volume control ventilation without an end-inspiratory pause (VC) during one-lung ventilation (OLV) in an anesthetized, paralyzed patient for performing thoracopic bullectomy of the lung. Methods: We ventilated 20 patients with VC and PC after the insertion of a thoracoscope in continual order for, at least for 15 minutes, for each, VC and PC procedure. At the end of VC and PC, the respiratory mechanics, gasometrics, and hemodynamic parameters were measured and collected. Results: We found no significant differences between VC and PC except for the peak inspiratory airway pressure (PIP), the mean airway pressure and the arterial oxygen partial pressure (PaO2). The PIP was significantly decreased from 27.0±6.0 cmH2O (VC) to 21.8±5.4 cmH2O (PC). The mean airway pressure was significantly increased from 8.6±1.6 cmH2O (VC) to 9.4±2.0 cmH2O (PC), and the PaO2 was significantly increased from 252.9±97.3 mmHg (VC) to 285.2±103.8 mmHg (PC). Conclusions: If PC allows mechanical ventilation with the same tidal volume and respiratory rate as VC during OLV, then PC significantly increases the PaO2 but this is not clinically significant, and the PC significantly decreases the PIP, which induces barotrauma or volutrauma when the PIP is excessively high. (Korean J Anesthesiol 2009;56:492~6)
문여옥,신우종,신윤정,동은실,안영민,Moon, Yeo Ok,Shin, Woo Jong,Shin, Youn Jeong,Dong, Eun Sil,Ahn, Young Min 대한소아청소년과학회 2002 Clinical and Experimental Pediatrics (CEP) Vol.45 No.7
저자들은 출생시 빠른 골연령, 특이한 얼굴모습, 정신운동지체와 성장부전을 보인 Marshall-Smith 증후군 정상 만삭아 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다. Marshall-Smith syndrome is characterized by a triad of facial dysmorphism, failure to thrive and accelerated osseous maturation. We report a one-month-old male infant with of this rare syndrome, with laryngeal anomalies who died at 6 months of age with pneumonia. This is the first case of Marshall-Smith syndrome in Korea.
영아 돌연사에 대한 고찰 (13 부검례를 포함한 34례 분석)
문여옥,최희경,허정아,신우종,김명아,이성용,장성희,동은실,김종재,안영민,지제근,Moon, Yeo Ok,Choi, Hee Kyoung,Her, Jeoung-A,Shin, Woo Jong,Kim, Myoung-A,Lee, Seong Yong,Jang, Seong Hee,Dong, Eun Sil,Kim, Chong Jae,Ahn, Young Min,Chi, Je 대한소아청소년과학회 2002 Clinical and Experimental Pediatrics (CEP) Vol.45 No.9
목 적 : 영아 돌연사에 있어서 그 역학적 특성을 알아보고 사인을 밝히는데 병력에 대한 자세한 검토, 사망 당시 상황에 대한 조사, 그리고 부검 및 대사 이상 검사 등 사후 검사의 중요성에 대하여 알아보고자 하였다. 방 법 : 1987년 2월부터 2001년 12월까지 14년 8개월간 갑작스런 사망으로 응급실을 방문한 1세 미만의 영아 34례를 대상으로 하여 이들의 임상기록을 후향적으로 조사하여 이들의 역학적 특성을 조사하였다. 과거 병력에 대한 검토, 사망 당시 상황에 대한 조사 및 부검을 실시한 13례의 기록과 2000년 이후 시행한 혈액, 소변, 담즙 및 간조직에서의 사후 아실카르니틴 및 유기산 분석 검사를 통하여 사망의 원인을 분석하였다. 결 과 : 1) 성별 및 연령별 분포 : 총 34례 중 남아 18명(52.9%), 여아 16명(47.1%)으로 남아에서 많이 발생하였고 연령별 분포는 생후 6개월 이하가 31례 (88.2%)로 가장 많은 분포를 차지하였다. 2) 사망시각은 오전 6-12시에 18례(52.9%)로 가장 많은 분포를 보였다. 3) 계절적 분포는 겨울에 가장 많이 사망하였고 13례(38.2%)였다. 4) 사망 발견시 수면 자세를 알 수 있었던 15례 중 복와위가 9례, 앙와위는 6례였다. 5) 병력과 사망 당시 상황으로도 죽음의 원인을 설명할 수 없는 경우가 23례였고 이중 부검을 실시한 13례 중 특이한 병리 소견을 발견할 수 없어 SIDS로 진단을 내린 경우가 7례였고 6례는 부검을 통하여 사망의 원인을 밝힐 수 있었다. 부검을 통해 밝혀진 사인으로는 심내막 섬유 탄성증 1례, 췌도 세포증 1례, 간의 지방 변화로 지방산 대사 이상을 의심할 수 있었던 2례, 경막하 및 경막외 출혈 1례, 기관지폐렴이 1례였다. 선천성 대사 이상 질환은 부검상 총 3례에서 의심되었으나 당시에는 대사 이상 검사가 시행되지 못하였고 2000년부터 시행한 대사 이상 검사에서는 모두 정상 소견이었다. 결 론 : 자세한 병력과 사망 상황에 대한 조사와 함께 부검은 사망의 원인을 밝히고 영아 돌연사 증후군을 진단하는데 필수적이라고 할 수 있으며 앞으로 우리나라에서도 부검의 중요성을 인지하고 이를 적극적으로 시행하여야겠고 지방산 대사 이상을 포함하는 대사 이상 검사도 아울러 적극적으로 시행하여야 할 것이다. Purpose : The purpose of this study is to analyze the epidemiologic characteristics of sudden unexpected death in infancy and to evaluate the importance of postmortem autopsy. Methods : We reviewed, retrospectively, medical records of 34 infants admitted to Kangnam General Hospital from January 1987 to December 2001 because of sudden unexpected death. We investigated the cause of death through medical history, death scene examination, autopsy findings, acylcarnitine and organic acid analysis. Results : Among the total 34 infants, 18 were male(52.9%) and 16 were female(47.1%). Thirty infants(88%) were below the six months of age. Winter was the most affected season(38.2%). Eighteen infants(52.9%) died between 6 and 12AM. The prone sleeping position was observed more frequently than the supine position at death; nine cases in the prone position, six cases in the supine position. The cause of death of 23 cases could not be found by only history and death scene examination. Autopsy was done in 13 cases. Seven cases of them were thought to be SIDS. In six cases, we explained the cause of death with autosy findings. They were an endocardial fibroelastosis, a nesidioblastosis, a subdural hematoma, a bronchopneumonia and two fatty changes of liver. Metabolic screening tests performed in three cases to rule out metabolic disorder since 2000 were all normal. Conclusion : We concluded that autopsy and metabolic screening test should be performed to find out the cause of death in sudden unexpected death in infancy.