• Clinical and Experimental Pediatrics
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• 제46권10호
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• pp.996-1002
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• 2003

목 적 : Rett 증후군 환자에서는 일반인에서 보다 돌연사의 확률이 높은 것으로 알려져 있으며, 그 원인으로 자율신경계 기능 부전에 의한 치명적인 부정맥 같은 심장의 전기 생리적 불안정성이 가장 의심되고 있다. 본 연구는 Rett 증후군 환자에서 심박수 변이도와 교정 QT 간격을 측정하여 실제로 자율신경 활성도에 문제가 있는지의 여부를 확인하기 위하여 실시하였다. 방 법 : 분자유전학적 염색체검사나 임상적 진단기준에 의하여 진단된 Rett 증후군 환자 12례를 대상군으로, 나이가 비슷한 정상아 30례를 대조군으로 하여 이들에서의 자율신경 활성도인 심박수 변이도와 교정 QT 간격을 측정하여 비교하였다. 심박수 변이도는 24시간 보행 심전도를 기록한 후 디지털화 된 심전도를 자동적으로 분석하여 power spectrum을 만들고 그 중 주파수영역(고주파 성분, 저주파 성분, 극소저주파 성분, LF/HF 비율)과 시간 영역(평균 RR 간격, SDNN, rMSSD, pNN50), 교정 QT 간격을 측정하여 비교하였다. 비교는 세 그룹으로 나누어서 전체 Rett 증후군과 정상 대조군 사이, 전형적인 Rett 증후군과 정상 대조군 사이, 비전형적 Rett 증후군과 정상 대조군 사이에서 실시하였다. 결 과 : 1) Rett 증후군 환자에서는 대조군에서 보다 시간영역의 rMSSD이 통계적으로 의미있게 낮았고(P<0.005), LF/HF 비율이 의미있게 높았으며(P<0.005), 교정 QT 간격도 의미있게 연장되어(P<0.005) 있었다. 2) 전형적인 Rett 증후군 환자에서도 대조군에서 보다 rMSSD이 통계적으로 의미있게 낮았고(P<0.005), LF/HF 비율도 의미있게 높았으며(P<0.05), 교정 QT 간격도 의미있게 연장되어 있었다(P<0.001). 3) 비전형적 Rett 증후군에서도 마찬가지로 rMSSD이 통계적으로 의미있게 낮았고(P<0.05), LF/HF 비율도 의미있게 높았으며(P<0.001), 교정 QT간격도 의미있게 연장되어 있었다(P<0.05). 4) 이상의 결과에서 Rett 증후군에서는 전형적인 환자나 비전형적인 환자 모두 미주신경긴장의 변화와 호흡에 의해 민감한 영향을 받는 rMSSD의 저하가 있었고, 교감-부교감신경 균형성을 나타내는 LF/HF 비율의 증가가 있었으며, 자율신경계에 영향을 받는 교정 QT 간격이 연장되었던 것으로 보아, 교감-부교감신경의 균형성에 이상이 있는 것으로 사료된다. 결 론 : Rett 증후군 환자에서 자율신경 활성도의 지표인 심박수 변이도의 의미있는 저하와 교정 QT 간격의 의미있는 연장이 나타나는 것으로 미루어 보아, 이 질환에서 자주 동반되는 돌연사가 일어나는 원인 중의 하나로 이러한 교감-부교감신경의 불균형을 의심할 수 있겠다.

• Journal of Yeungnam Medical Science
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• 제26권2호
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• pp.148-155
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• 2009

Brugada syndrome is characterized by an ECG pattern of right bundle branch block and ST segment elevation in the right precordial leads ($V_1-V_3$) without structural heart disease. It is also characterized by sudden cardiac death that's caused by ventricular fibrillation. This is a familial syndrome with an autosomal dominant inheritance pattern and it may be considerably more common in Southeast Asia. Many factors during anesthesia can precipitate malignant dysrrhythmia in these patients, so careful choice of anesthetics is required. We experienced a case of Brugada syndrome in a 59-year-old male patient who was under general anesthesia for trans-sphenoidal surgery to treat a pituitary adenoma, and the patient was diagnosed as having Brugada syndrome without any untoward cardiovascular events.

• Korean Circulation Journal
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• 제53권4호
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• pp.239-250
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• 2023

Background and Objectives: Brugada syndrome (BrS) is an inherited arrhythmia syndrome that presents as sudden cardiac death (SCD) without structural heart disease. One of the mechanisms of SCD has been suggested to be related to the uneven dispersion of transient outward potassium current (I_to) channels between the epicardium and endocardium, thus inducing ventricular tachyarrhythmia. Artemisinin is widely used as an antimalarial drug. Its antiarrhythmic effect, which includes suppression of I_to channels, has been previously reported. We investigated the effect of artemisinin on the suppression of electrocardiographic manifestations in a canine experimental model of BrS. Methods: Transmural pseudo-electrocardiograms and epicardial/endocardial transmembrane action potentials (APs) were recorded from coronary-perfused canine right ventricular wedge preparations (n=8). To mimic the BrS phenotypes, acetylcholine (3 μM), calcium channel blocker verapamil (1 μM), and I_to agonist NS5806 (6-10 μM) were used. Artemisinin (100-150 μM) was then perfused to ameliorate the ventricular tachyarrhythmia in the BrS models. Results: The provocation agents induced prominent J waves in all the models on the pseudo-electrocardiograms. The epicardial AP dome was attenuated. Ventricular tachyarrhythmia was induced in six out of 8 preparations. Artemisinin suppressed ventricular tachyarrhythmia in all 6 of these preparations and recovered the AP dome of the right ventricular epicardium in all preparations (n=8). J wave areas and epicardial notch indexes were also significantly decreased after artemisinin perfusion. Conclusions: Our findings suggest that artemisinin has an antiarrhythmic effect on wedge preparation models of BrS. It might work by inhibition of potassium channels including I_to channels, subsequently suppressing ventricular tachycardia/ventricular fibrillation.

• Journal of Dental Anesthesia and Pain Medicine
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• 제16권1호
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• pp.67-71
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• 2016

Eisenmenger syndrome (ES) is characterized by pulmonary arterial hypertension and right-to-left shunting. The signs and symptoms of ES include cyanosis, shortness of breath, fatigue, hemoptysis, and sudden death. In patients with ES, it is important that the systemic and pulmonary circulations be properly distributed and maintained. General dental treatment is not known to be particularly dangerous. To control pain and anxiety, local anesthetics without epinephrine are usually recommended. However, in cases of difficulty of cooperation, general anesthesia for dental treatment makes the condition worse. In the present case, intravenous deep sedation with propofol and remifentanil was administered for behavioral management during dental treatment successfully.

• The Korean Journal of Pain
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• 제13권1호
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• pp.115-118
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• 2000

Stellate ganglion block is most commonly used in pain clinic because it is an easy procedure and it has broad indications reported that Angina pectoris, tachyarrhythmia and long QT syndrome (LQTS) are indicated. LQTS is a disorder of the abnormalities of cardiac sympathetic innervation and of myocardial repolarization. LQTS is characterized by marked prolongation of the QT interval, often manifestating as syncope, seizures, or sudden death due to polymorphic ventricular tachyarrhythmia known as torsades de pointes. Treatment of symptomatic patients usually begin with beta blocker. The elective treatment of LQTS patients unresponsive to beta blocker is the left cardiac sympathetic denervation. We report a case of LQTS patient who had received stellate ganglion block.

• Journal of Yeungnam Medical Science
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• 제25권1호
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• pp.78-83
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• 2008

Lesch-Nyhan syndrome is an inborn error of purine metabolism resulting from hypoxanthine-guanine-phosphoribosyltransferase (HGPRT) deficiency and leading to excess purine production and uric acid over-production. It is a very rare X-linked recessive disorder, characterized by movement disorder, cognitive deficits, and self-injurious behavior. However, because of the high incidence of calculi, patients may present for surgery of urinary tract, and have increased risk of difficult intubation, aspiration pneumonia, renal insufficiency or sudden death. We report the case of a 5-year-old boy with Lesch-Nyhan syndrome who underwent successive extracorporeal shockwave lithotripsy under general anesthesia.

• 대한의용생체공학회:의공학회지
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• 제36권2호
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• pp.48-53
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• 2015

Sudden infant death syndrome(SIDS) continues to be general cause of infant death. Also, apnea is supposed to be one of the main risk factor of SIDS. Therefore, Infant's respiratory monitoring and real-time apnea detection is very important to prevent SIDS. In this study, we proposed a non-contact home monitoring system for infant's respiration using Doppler radar in order to prevent SIDS. The respiration data were acquired from a commercialized baby simulator(Simbaby$^{TM}$) using a Doppler radar. To evaluate a performance of the proposed system, the simulator was placed in a supine and prone position and the chest belt was used simultaneously as a reference signal. As a result, correlation coefficients between respiration rates of Doppler radar and the chest belt in each position were 0.95(p < 0.001) and 0.98(p < 0.001), respectively. The averages of difference were $-0.29{\pm}5.21(mean{\pm}1.96{\cdot}$ standard deviation) in supine and $-0.12{\pm}3.05$ in prone from Bland-Altman analysis. The results indicated an excellent performance in detecting apnea with a sensitivity of 100% and a positive predictive value of 100% in each posture respectively. These results demonstrated that a proposed Doppler radar system is suitable for non-contact respiratory monitoring in order to prevent SIDS of infant.

• Clinical and Experimental Pediatrics
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• 제45권9호
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• pp.1065-1074
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• 2002

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.

• 한국환경보건학회지
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• 제34권5호
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• pp.361-368
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• 2008

Objective to assess whether exposure to air pollutants is associated with postneonatal infant death, using a timeseries methodology, between 1999 and 2003 in Seoul, Korea.. Methods We investigated the short-term effects of air pollution for 548,725 live births during the study period. The daily count of postneonatal infant deaths from all causes and from SIDS (sudden infant death syndrome) by birth order was analyzed by a Generalized Additive Poisson model, with controlling for the effects of seasonal trends, air temperature, relative humidity, barometric pressure, and day of the week as covariates. Results During the study period, we observed 699 deaths from all causes and 47 deaths from SIDS. We did not find any significant associations between daily mortality and ambient levels of air pollutants except for CO and $NO_2$. The estimated relative risk of postneonatal infant death from all causes was 1.17 (95% CI=1.04-1.32) and 1.16 (95% CI=1.03-1.29) by IQR (interquartile range) for CO and $NO_2$ respectively. Also, we observed no clear trend of the mortality effects of air pollution by birth orders. Conclusion In conclusion, our findings suggest that air pollution, in general, influenced adversely postneonatal infant death from all-cause and SIDS although it was not statistically significant. This study may support that the rationale.

• 대한의용생체공학회:학술대회논문집
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• 대한의용생체공학회 1997년도 추계학술대회
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• pp.232-235
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• 1997

Apnea refers to episode of apnea (or not breathing) lasting more than 10 seconds that occur while sleeping. These episodes, whitch can occur hundreds of times per night, may transiently awaken resulting in fragmentation of sleep. Although the precise cause of Sudden Infant Death Syndrome(SIDS) are still unclear, there is evidence to suggest that hypoxaemia may be a contributory actor. Transcutaneous oxygen monitor can be used, but it is very difficult to use or baby stayed at home. In this reason, monitors whitch is easy or deal with are reqiured. In 1972, Steinschieder reported that two of the five infants noted to have apnea lasting or more than 20 seconds later died of SIDS episode, he also suggested that home monitoring or neonates should be used or managing apnea at home. Transthoracic electrical impedance technique is used or acquiring respiration waveform and detecting episode of apnea state. Transthoracic electrical impedance measurements have been made from the human trunk over the frequency range 9.6KHz to 614KHz. We conclude that application of impedance technique or detecting apnea state is proper or neonates.