• Journal of mucopolysaccharidosis and rare diseases
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• v.4 no.1
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• pp.21-25
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• 2018

Fabry disease is a hereditary lysosomal storage disorder caused by the reduction or absence of lysosomal enzyme alpha-galactosidase A and the accumulation of glycosphingolipids, such as globotriaosylceramide (Gb3), in various organs, including the heart. The prevention of cardiac involvement in Fabry disease can only be achieved by enzyme replacement therapy (ERT), and the method of assessing the efficacy of ERT should be confirmed. Changes in the electrocardiogram, such as the shortening of PQ interval, prolongation of QTc and repolarization abnormalities as well as left ventricular hypertrophy in voltage criteria, can be used to identify Fabry disease patients; however, the usefulness of electrocardiograms for evaluating the efficacy of ERT is limited. The assessment of left ventricular hypertrophy using echocardiography has been established to evaluate the efficacy of ERT during long-term period. A new technique involving speckled tracking method might be useful for detecting early cardiac dysfunction and identifying the effect of ERT for a relatively short period. The estimation of left ventricular hypertrophy using cardiac magnetic resonance (CMR) is also useful for assessing the efficacy of ERT. Identifying late gadolinium enhancement in CMR may affect the effectiveness of ERT, and the new technique of T1 mapping might be useful for monitoring the accumulation of Gb3 during ERT. Histopathology in cardiac biopsy specimens is another potentially useful method for identifying the accumulation of GB3; however, the use of histopathology to evaluate of the efficacy of ERT is limited because of the invasive nature of an endomyocardial biopsy.

• Journal of The Korean Society of Clinical Toxicology
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• v.12 no.1
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• pp.14-21
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• 2014

Purpose: The objective of this study was to develop a new scoring tool that is comprehensively applicable and predicts fatality within 24 h of intoxication. Methods: This was a cohort study conducted in two emergency medical centers from 2011 to 2012. We identified factors associated with severe/fatality. Through a discriminant analysis, we devised the aBIG (age, Base deficit, Infection, and Glasgow coma scale) score. To compare the ability of aBIG to predict intoxication severity with that of previous scoring systems such as APACHE II, MODS, SAPS IIe, and SOFA, we determined the receiver operating characteristic curves of each variable in predicting severe-to-fatal toxicity. Results: Compared with the mild/moderate toxicity group (n=211), the severe/fatal group (n=143) had higher incidences of metabolic acidosis, infection, serious mental change, QTc prolongation and hepato-renal failure. Age, base deficit, infection-WBC count, and Glasgow Coma Scale were independently associated with severe/fatal poisoning. These variables were combined into the poisoning "aBIG" score [$0.28{\times}$Age group+$0.38{\times}WBC$ count/$10^3+0.52{\times}$Base deficit+$0.64{\times}$(15-GCS)], which were each calculated to have an area under the curve of 0.904 (95% confidence interval: 0.868-0.933). The aBIG poisoning score had an equivalent level of severity predictability as APACHE II and a superior than MODS, SOFA, and SAPS IIe. Conclusion: We developed a simplified scoring system using the four variables of age, base deficit, infected leukocytosis, and GCS. The poisoning aBIG score was a simple method that could be performed rapidly on admission to evaluate severity of illness and predict fatal severity in patients with acute intoxications.

• Korean Journal of Biological Psychiatry
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• v.19 no.1
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• pp.38-44
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• 2012

Objectives : QT interval prolongation and dispersion known as indicators of an increased risk for ventricular arrhythmias and sudden death have been reported to be prolonged in patients with anorexia nervosa. The aims of this study were to compare conduction abnormalities in Korean patients with anorexia nervosa and bulimia nervosa, and to examine its relation with clinical and laboratory factors. Methods : We retrospectively examined 45 women with anorexia nervosa and 75 women with bulimia nervosa who were assessed by 12-lead electrocardiogram at baseline. QT interval and corrected QT interval, QT dispersion of the difference between the longest and shortest QT intervals, and abnormal U wave were measured for conduction abnormalities. Results : QT interval was significantly longer in patients with anorexia nervosa compared with those with bulimia nervosa. There were no differences in QTc (Corrected QT), QTd (QT dispersion) and abnormal U wave between patients with anorexia nervosa and those with bulimia nervosa. QTd was significantly correlated with the lowest ever lifetime body mass index ($kg/m^2$) as well as the serum amylase level in patients with anorexia nervosa. Conclusions : These results suggest some conduction abnormalities reported in patients with anorexia nervosa are also found in patients with bulimia nervosa. It appears that severity of weight loss and purging behavior could affect the cardiac arrhythmia in patients with eating disorders. Appropriate attention should be paid to cardiac involvement in patients with eating disorders.

• Journal of The Korean Society of Clinical Toxicology
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• v.8 no.2
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• pp.79-87
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• 2010

Purpose: The previous studies on $H_1$ antihistamine overdose have generally been limited to cases of acute doxylamine succinate (DS) poisoning, yet there have been some studies on diphenhydramine (DPH) overdosing. But many clinicians consider the two drugs to be very similar and to have similar ingredients. The purpose of this study was to clarify the toxicologic characteristics and clinical outcomes between DS and DPH poisoning/overdose. Methods: We reviewed the medical and intensive care records of the patients with acute DS or DPH poisoning and who admitted to our emergency department from January 2008 and April 2010. We collected patient information regarding the features of the poisoning and the clinical and demographic characteristics. The patients were assessed for the clinical outcomes, the GCS, the PSS (Poisoning Severity Score) and the SOFA (Sequential Organ Failure Assessment). Results: Fifty seven patients (45 cases of DS poisoning and 12 cases of DPH poisoning) were enrolled. Compared with the DS group, the DPH group had higher incidences of intubation, serious mental change, QTc prolongation and ECG conduction abnormality (p=0.041, <0.001, 0.014 and 0.044, respectively). The DPH group had a higher PSS and a longer ICU stay. The peak CPK time and the CPK normalization time were longer for the patients with rhabdomyolysis due to DS poisoning. Conclusion: Two common $H_1$ antihistamines, doxylamine and diphenhydramine, are in the same ethanolamine-structural class, but the toxico-clinical outcomes are different according to many aspects. Therefore, clinicians could take a careful approach for the differential diagnosis and management between DS and DPH poisoning.

• Clinical Psychopharmacology and Neuroscience
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• v.16 no.4
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• pp.505-507
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• 2018

Clozapine may be associated with cardiovascular adverse effects including QTc prolongation and, more rarely, with myocarditis and pericarditis. Although rare, these latter cardiovascular adverse effects may be life-threatening and must be immediately recognized and treated. Several cases of clozapine related-pericarditis have been described and often it has a subtle and insidious onset with symptoms that may be often misdiagnosed with psychiatric manifestations (e.g. anxiety, panic or somatization) leading to a delayed correct diagnosis with potential fatal consequences. In the present report we describe the case of a 27-year-old girl with schizoaffective disorder taking long acting aripiprazole and valproate who developed a sudden onset clozapine-related pericarditis during titration phase that resolved with immediate clozapine discontinuation and indomethacin administration. We underline the importance of an early diagnosis of clozapine-related pericarditis and the need to have monitoring protocols to prevent this potentially fatal adverse effect especially when polypharmacy is administered to patients taking clozapine.

• Clinical and Experimental Pediatrics
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• v.51 no.11
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• pp.1232-1235
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• 2008

Imipramine, a tricyclic antidepressant (TCA), is used for the treatment of non-polar depression and nocturnal enuresis in children in whom an organic pathology has been excluded, anxiety disorders, and neuropathic pain. Clinical toxicity following the treatment of TCAs, including imipramine, is well known. The anticholinergic effects initially present include a dry mouth, ileus, dilated pupils, urinary retention, and mild sinus tachycardia. The central nervous system toxicity includes delirium, agitation, restlessness, hallucinations, convulsions, and CNS depression or coma. However, the most life-threatening toxicity remains the development of cardiac dysrhythmias. Conduction delays such as QRS and corrected QT prolongation, wide QRS complex tachycardia, and the Brugada electrocardiographic pattern have been reported. Sodium bicarbonate decreases QRS widening and suppresses dysrhythmias by providing excess sodium to reverse the TCA-induced sodium-channel blockade and possibly by binding directly to the myocardium. There are no pediatric case reports on imipramine or other TCA associated toxicity in Korea. Here, we describe a patient who presented with convulsions, tachycardia with a wide QRS complex, a Brugada electrocardiographic pattern, and anuresis associated with an accidental overdose of imipramine and the outcome of treatment with sodium bicarbonate.

• Biomolecules & Therapeutics
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• v.23 no.4
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• pp.386-389
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• 2015

Sibutramine is an anorectic that has been banned since 2010 due to cardiovascular safety issues. However, counterfeit drugs or slimming products that include sibutramine are still available in the market. It has been reported that illegal sibutramine-contained pharmaceutical products induce cardiovascular crisis. However, the mechanism underlying sibutramine-induced cardiovascular adverse effect has not been fully evaluated yet. In this study, we performed cardiovascular safety pharmacology studies of sibutramine systemically using by hERG channel inhibition, action potential duration, and telemetry assays. Sibutramine inhibited hERG channel current of HEK293 cells with an $IC_{50}$ of $3.92{\mu}M$ in patch clamp assay and increased the heart rate and blood pressure ($76{\Delta}bpm$ in heart rate and $51{\Delta}mmHg$ in blood pressure) in beagle dogs at a dose of 30 mg/kg (per oral), while it shortened action potential duration (at $10{\mu}M$ and $30{\mu}M$, resulted in 15% and 29% decreases in $APD_{50}$, and 9% and 17% decreases in $APD_{90}$, respectively) in the Purkinje fibers of rabbits and had no effects on the QTc interval in beagle dogs. These results suggest that sibutramine has a considerable adverse effect on the cardiovascular system and may contribute to accurate drug safety regulation.

• Journal of The Korean Society of Clinical Toxicology
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• v.19 no.2
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• pp.83-92
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• 2021

Purpose: Glyphosate herbicide (GH) is a widely used herbicide and has been associated with significant mortality as poisoned cases increases. One of the reasons for high toxicity is thought to be toxic effect of its ingredient with glyphosate. This study was designed to determine differences in the clinical course with the salt-type contained in GH. Methods: This was a retrospective study conducted at a single hospital between January 2013 and December 2017. We enrolled GH-poisoned patients visited the emergency department. According to salt-type, patients were divided into 4 groups: isopropylamine (IPA), ammonium (Am), potassium (Po), and mixed salts (Mi) groups. The demographics, laboratory variables, complications, and their mortality were analyzed to determine clinical differences associated with each salt-type. Addtionally, we subdivided patients into survivor and non-survivor groups for investigating predictive factors for the mortality. Results: Total of 348 GH-poisoned patients were divided as follows: IPA 248, Am 41, Po 10, and Mi 49 patients. There was no difference in demographic or underlying disease history, but systolic blood pressure (SBP) was low in Po group. The ratio of intentional ingestion was higher in Po and Mi groups. Metabolic acidosis and relatively high lactate level were presented in Po group. As the primary outcome, the mortality rates were as follows: IPA, 26 (10.5%); Am, 2 (4.9%); Po, 1 (10%); and Mi, 1 (2%). There was no statistically significant difference in the mortality and the incidence of complications. Additionally, age, low SBP, low pH, corrected QT (QTc) prolongation, and respiratory failure requiring mechanical ventilation were analyzed as independent predictors for mortality in a regression analysis. Conclusion: There was no statistical difference in their complications and the mortality across the GH-salt groups in this study.