• Clinical and Experimental Pediatrics
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• v.46 no.10
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• pp.996-1002
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• 2003

Purpose : In Rett syndrome patients, the incidence of sudden death is greater than that of the general population, and cardiac electrical instability including fatal cardiac arrhythmia is a main suspected cause. In this study, we are going to find out the possible cause of the higher risk of sudden death in Rett patients by the evaluation of heart rate variability, a marker of cardiac autonomic activity and corrected QT intervals. Methods : Diagnosis of Rett syndrome was made by molecular genetic study of Rett syndrome (MECP2 gene) or clinical diagnostic criteria of Rett syndrome. Heart rate variability and corrected QT intervals were measured by 24 h-Holter study in 12 Rett patients, and in 30 age-matched healthy children with chief complaints of chest pain or suspected heart murmurs. The were compared with the normal age-matched control. Results : Patients with total Rett syndrome, classic Rett syndrome, and Rett variants had significantly lower heart rate variability(especially rMSSD)(P<0.05) and longer corrected QT intervals than age-matched healthy children(P<0.05). Sympathovagal balance expressed by the ratio of high to low frequency(LF/HF ratio) also showed statistically significant differences between the three groups considered(P<0.05). Conclusion : A significant reduction of heart rate variability, a marker of autonomic disarray, suggests a possible explanation of cardiac dysfunction in sudden death associated with Rett syndrome.

• Journal of Yeungnam Medical Science
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• v.26 no.2
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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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• v.53 no.4
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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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• v.16 no.1
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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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• v.13 no.1
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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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• v.25 no.1
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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.

• Journal of Biomedical Engineering Research
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• v.36 no.2
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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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• v.45 no.9
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• pp.1065-1074
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• 2002

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.

• Journal of Environmental Health Sciences
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• v.34 no.5
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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.

• Proceedings of the KOSOMBE Conference
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• v.1997 no.11
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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.