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1.
Korean Circulation Journal ; : 606-620, 2022.
Article in English | WPRIM | ID: wpr-938447

ABSTRACT

Background and Objectives@#Protein-losing enteropathy (PLE) is a devastating complication after the Fontan operation. This study aimed to investigate the clinical characteristics, treatment response, and outcomes of Fontan-associated PLE. @*Methods@#We reviewed the medical records of 38 patients with Fontan-associated PLE from 1992 to 2018 in 2 institutions in Korea. @*Results@#PLE occurred in 4.6% of the total 832 patients after the Fontan operation. After a mean period of 7.7 years after Fontan operation, PLE was diagnosed at a mean age of 11.6years. The mean follow-up period was 8.9 years. The survival rates were 81.6% at 5 years and 76.5% at 10 years. In the multivariate analysis, New York Heart Association Functional classification III or IV (p=0.002), low aortic oxygen saturation (<90%) (p=0.003), and ventricular dysfunction (p=0.032) at the time of PLE diagnosis were found as predictors of mortality. PLE was resolved in 10 of the 38 patients after treatment. Among medical managements, an initial heparin response was associated with survival (p=0.043). Heparin treatment resulted in resolution in 4 patients. We found no evidence on pulmonary vasodilator therapy alone. PLE was also resolved after surgical Fontan fenestration (2/6), aortopulmonary collateral ligation (1/1), and transplantation (1/1). @*Conclusions@#The survival rate of patients with Fontan-associated PLE has improved with the advancement of conservative care. Although there is no definitive method, some treatments led to the resolution of PLE in one-fourth of the patients. Further investigations are needed to develop the best prevention and therapeutic strategies for PLE.

2.
Korean Circulation Journal ; : 1017-1029, 2021.
Article in English | WPRIM | ID: wpr-917369

ABSTRACT

Background and Objectives@#While diuretics are sometimes used in atrial septal defect (ASD) treatment, their effect on ASD size reduction remains unclear. We aimed to evaluate the efficacy of diuretics in ASD size reduction in pediatric patients. @*Methods@#We retrospectively reviewed the medical records of patients with secundum ASD (size ≥10 mm), between 2005 and 2019. Patients were divided into two groups based on the diuretic administration. @*Results@#Of the 73 enrolled patients, 40 received diuretics. The initial age at ASD diagnosis (2.8±1.7 vs. 2.5±2.0 years, p=0.526) and follow-up duration (22.3±11.4 vs. 18.7±13.2 months, p=0.224) were not significantly different between the groups. The ASD diameter at the initial diagnosis (13.7±2.0 vs. 13.5±3.4 mm, p=0.761) and the indexed ASD diameter (25.5±5.9 vs. 26.9±10.3 mm/m2 , p=0.493) were also not significantly different between two groups. The ASD diameter significantly increased in the non-diuretic group during follow-up (0.0±2.9 vs. +2.6±2.0 mm, p<0.001). The indexed ASD diameter significantly decreased in the diuretic group during follow-up (−5.7±6.5 vs. +0.2±3.9 mm/m 2 , p<0.001). In the linear mixed model analysis, diuretic use was associated with ASD diameter decrease (p<0.001) and indexed ASD diameter reduction (p<0.001) over time. Device closure was more frequently performed in the diuretic (75.0%) than in the non-diuretic group (39.4%). @*Conclusions@#Patients receiving diuretics are less likely to undergo surgery. The diuretics administration may be associated with the use of smaller ASD devices for transcatheter treatment through ASD size reduction.

3.
Korean Circulation Journal ; : 360-372, 2021.
Article in English | WPRIM | ID: wpr-901583

ABSTRACT

Background and Objectives@#We reviewed the long-term outcomes after tetralogy of Fallot (TOF) repair with trans-annular incision; and evaluated the effectiveness of pulmonary valve replacement (PVR) on outcomes. @*Methods@#This was a retrospective review of clinical outcomes of 180 of 196 TOF patients who underwent total correction with trans-annular incision from 1991 to 1997 (PVR group: 81; non-PVR group: 99). @*Results@#The median age of the patients was 14.0 months (interquartile range [IQR], 10.7–19.8 months) at TOF repair. Ten in-hospital deaths (5.1%) occurred. During the followup, 81 patients underwent PVR at the median age of 13.5 years (IQR, 11.2–17.1 years). The patients in PVR group showed better outcomes than non-PVR group in overall survival rate (100% in PVR vs. 88.7% in non-PVR, p=0.007), in all adverse events (arrhythmia, neurologic complications, 95.5% in PVR vs. 74.6% in non-PVR, p=0.024) at 20 years. Age at TOF repair younger than 1 year (hazard ratio [HR], 2.265; p=0.01) and previous shunt history (HR, 2.195; p=0.008) were predictive for requiring PVR. During follow-up, 10 late deaths (5 sudden deaths) occurred in the non-PVR group, mainly due to ventricular arrhythmia and right ventricular failure; there was 1 late death (not a sudden death) in the PVR group. @*Conclusions@#Long-term survival after repair of TOF with trans-annular incision were acceptable. However, arrhythmias were frequently observed during 20 years of follow-up. The patient age <1 year at the time of TOF repair and shunt implantation prior to TOF repair were predictive factors for requiring PVR.

4.
Korean Circulation Journal ; : 360-372, 2021.
Article in English | WPRIM | ID: wpr-893879

ABSTRACT

Background and Objectives@#We reviewed the long-term outcomes after tetralogy of Fallot (TOF) repair with trans-annular incision; and evaluated the effectiveness of pulmonary valve replacement (PVR) on outcomes. @*Methods@#This was a retrospective review of clinical outcomes of 180 of 196 TOF patients who underwent total correction with trans-annular incision from 1991 to 1997 (PVR group: 81; non-PVR group: 99). @*Results@#The median age of the patients was 14.0 months (interquartile range [IQR], 10.7–19.8 months) at TOF repair. Ten in-hospital deaths (5.1%) occurred. During the followup, 81 patients underwent PVR at the median age of 13.5 years (IQR, 11.2–17.1 years). The patients in PVR group showed better outcomes than non-PVR group in overall survival rate (100% in PVR vs. 88.7% in non-PVR, p=0.007), in all adverse events (arrhythmia, neurologic complications, 95.5% in PVR vs. 74.6% in non-PVR, p=0.024) at 20 years. Age at TOF repair younger than 1 year (hazard ratio [HR], 2.265; p=0.01) and previous shunt history (HR, 2.195; p=0.008) were predictive for requiring PVR. During follow-up, 10 late deaths (5 sudden deaths) occurred in the non-PVR group, mainly due to ventricular arrhythmia and right ventricular failure; there was 1 late death (not a sudden death) in the PVR group. @*Conclusions@#Long-term survival after repair of TOF with trans-annular incision were acceptable. However, arrhythmias were frequently observed during 20 years of follow-up. The patient age <1 year at the time of TOF repair and shunt implantation prior to TOF repair were predictive factors for requiring PVR.

5.
Chonnam Medical Journal ; : 44-49, 2020.
Article in English | WPRIM | ID: wpr-787275

ABSTRACT

The aim of this study was to investigate the usefulness of a clinical screening test [the Korean Infant and Child Developmental Test (KICDT)] compared to language specific tests: the sequenced language scale for infant (SELSI) and the Preschool Receptive-Expressive language Scale (PRES) in children with delayed language development. A retrospective chart review was conducted on 615 children who visited the Department of Pediatrics at Chonbuk National University Hospital from January 2013 to December 2016. All patients were evaluated with KICDT as a clinical screening test and SELSI or PRES as a language specific test. Language Developmental Quotients (LDQs) from the KICDT were compared with the Receptive Language Quotient (RLQ) and expressive language quotient (ELQ) from the SELSI or PRES. The sensitivity, specificity and predictive values of LDQ of KICDT were calculated by comparing with SELSI/PRES. Language DQs from the KICDT were significantly correlated with the RLQ (r=0.706), ELQ (r=0.768), and total language quotient (TLQ) (r=0.766) from the SELSI/PRES (p<0.05). In cross tabulation, the patients belonging to the retardation groups in both KICDT and SELSI/PRES were 417 (67.8%). Otherwise, patients belonging to the normal group in KICDT but not in SELSI/PRES were 151 (24.6%). Sensitivity and specificity of LDQ of KICDT relative to SELSI/PRES were 72.3% and 92.2% respectively (p<0.05). Our data suggests that clinical screening tests alone, not cumbersome language specific tests, can determine language developmental delays in children.


Subject(s)
Child , Humans , Infant , Child Development , Language Development , Language Development Disorders , Language Tests , Mass Screening , Pediatrics , Retrospective Studies , Sensitivity and Specificity
6.
Journal of Korean Medical Science ; : e107-2020.
Article | WPRIM | ID: wpr-831494

ABSTRACT

Background@#Despite improvements in palliative care for critically ill children, the characteristics of end-of-life care for pediatric patients with advanced heart disease are not well-known. We investigated these characteristics among hospitalized children with advanced heart disease in a tertiary referral center in Korea. @*Methods@#We retrospectively reviewed the records of 136 patients with advanced heart disease who died in our pediatric department from January 2006 through December 2013. @*Results@#The median age of patients at death was 10.0 months (range 1 day–28.3 years). The median duration of the final hospitalization was 16.5 days (range 1–690 days). Most patients (94.1%) died in the intensive care unit and had received mechanical ventilation (89.7%) and inotropic agents (91.2%) within 24 hours of death. The parents of 74 patients (54.4%) had an end-of-life care discussion with their physician, and the length of stay of these patients in the intensive care unit and in hospital was longer. Of the 90 patients who had been hospitalized for 7 days or more, the parents of 54 patients (60%) had a documented end-of-life care discussion. The time interval from the end-of-life care discussion to death was 3 days or less for 25 patients. @*Conclusion@#Children dying of advanced heart disease receive intensive treatment at the end of life. Discussions regarding end-of-life issues are often postponed until immediately prior to death. A pediatric palliative care program must be implemented to improve the quality of death in pediatric patients with heart disease.

7.
International Journal of Arrhythmia ; : 18-2020.
Article in English | WPRIM | ID: wpr-898666

ABSTRACT

Background@#Radiofrequency catheter ablation (RFCA) has been accepted as an efficient therapy for tachycardia, and substantial improvement in the outcomes of RFCA in pediatric patients has been seen. However, there is not enough data on the clinical outcomes of RFCA for ventricular tachycardia (VT) in pediatric patients. The objective of this study was to elucidate the efficacy and safety of RFCA for VT in pediatric patients. @*Methods@#We performed a retrospective study involving 35 consecutive pediatric VT ablation procedures in 28 patients at a single institution. @*Results@#The median age at ablation was 14.0 years (range 6.9–19.2 years). There were 24 patients with a structurally normal heart and four patients with congenital heart disease (CHD). The left ventricular posteroseptal area was the most common site of VT origin (22/28, 78.6%). However, there was an unusual case of VT which involved a papillary muscle as its origin. Acute success was achieved in 30 (85.7%) of 35 procedures. The recurrence rate after successful RFCA was 14.2% (5/35) at a median follow-up of 6.7 years (range 1.0–16.7 years). There were five procedural failures due to the inability to induce VT for complete mapping. Ultimately, long-term success was achieved in 27 patients (96.4%) after repeated procedures and no major complications occurred. Long-term success was associated with VT inducibility (p = < 0.001). @*Conclusions@#Difficulty in inducing VT for precise mapping was a significant obstacle to successful RFCA. RFCA was identified as safe and effective therapy to eliminate VT in the selected pediatric VT patients.

8.
International Journal of Arrhythmia ; : 18-2020.
Article in English | WPRIM | ID: wpr-890962

ABSTRACT

Background@#Radiofrequency catheter ablation (RFCA) has been accepted as an efficient therapy for tachycardia, and substantial improvement in the outcomes of RFCA in pediatric patients has been seen. However, there is not enough data on the clinical outcomes of RFCA for ventricular tachycardia (VT) in pediatric patients. The objective of this study was to elucidate the efficacy and safety of RFCA for VT in pediatric patients. @*Methods@#We performed a retrospective study involving 35 consecutive pediatric VT ablation procedures in 28 patients at a single institution. @*Results@#The median age at ablation was 14.0 years (range 6.9–19.2 years). There were 24 patients with a structurally normal heart and four patients with congenital heart disease (CHD). The left ventricular posteroseptal area was the most common site of VT origin (22/28, 78.6%). However, there was an unusual case of VT which involved a papillary muscle as its origin. Acute success was achieved in 30 (85.7%) of 35 procedures. The recurrence rate after successful RFCA was 14.2% (5/35) at a median follow-up of 6.7 years (range 1.0–16.7 years). There were five procedural failures due to the inability to induce VT for complete mapping. Ultimately, long-term success was achieved in 27 patients (96.4%) after repeated procedures and no major complications occurred. Long-term success was associated with VT inducibility (p = < 0.001). @*Conclusions@#Difficulty in inducing VT for precise mapping was a significant obstacle to successful RFCA. RFCA was identified as safe and effective therapy to eliminate VT in the selected pediatric VT patients.

9.
Clinical Pediatric Hematology-Oncology ; : 60-65, 2019.
Article in English | WPRIM | ID: wpr-763503

ABSTRACT

Primary cardiac tumors are rare, with a prevalence of 0.001–0.2%. Among such tumors, cardiac hemangioendotheliomas are some of the most uncommon. In Korea, there have been no reports of hemangioendothelioma occurring in the heart of infants. We herein report a case of an infant that was admitted to our medical center and presented with cough and a runny nose. The initial diagnosis was acute bronchiolitis. Cardiomegaly was observed on chest radiography. Echocardiography revealed a tumor measuring 3.5×4.0 cm in the right atrium. The infant was transferred to a tertiary medical center for tumor excision. The excised lesion was 3.8×3×3.2 cm in size, and biopsy confirmed a diagnosis of hemangioendothelioma. In this case report, we describe our experience with a rare case involving cardiac tumor in an infant with an upper respiratory tract infection.


Subject(s)
Humans , Infant , Biopsy , Bronchiolitis , Cardiomegaly , Cough , Diagnosis , Echocardiography , Heart , Heart Atria , Heart Neoplasms , Hemangioendothelioma , Korea , Nose , Prevalence , Radiography , Respiratory Tract Infections , Thorax , Twins
11.
Allergy, Asthma & Respiratory Disease ; : 212-217, 2019.
Article in Korean | WPRIM | ID: wpr-762199

ABSTRACT

Eosinophilic granulomatosis with polyangiitis (EGPA, also known as the Churg-Strauss syndrome) is a disorder characterized by asthma, peripheral eosinophilia and systemic vasculitis. It rarely occurs in children, so that physicians may frequently mistake it for a simple uncontrolled asthma. Since a subsequent cardiac involvement is critical for the prognosis, it is important to suspect EGPA in children with severe, uncontrolled asthma. The cardiac manifestations in EGPA are variable from asymptomatic electrocardiogram abnormalities to pericarditis with pericardial effusion, myocarditis with cardiomyopathy, heart failure, and sudden cardiac death. Although delayed treatment may lead to fatal cardiac complications in EGPA, adequate immune suppression can reverse cardiac impairment. We report a 14-year-old girl with persistent asthma refractory to steroids who was eventually diagnosed with an anti-neutrophil cytoplasmic antibody-negative EGPA.


Subject(s)
Adolescent , Child , Female , Humans , Asthma , Cardiomyopathies , Churg-Strauss Syndrome , Cytoplasm , Death, Sudden, Cardiac , Electrocardiography , Eosinophilia , Eosinophils , Granulomatosis with Polyangiitis , Heart Failure , Heart , Myocarditis , Pericardial Effusion , Pericarditis , Prognosis , Steroids , Systemic Vasculitis
12.
International Journal of Arrhythmia ; : 126-144, 2018.
Article in Korean | WPRIM | ID: wpr-740051

ABSTRACT

Syncope is a very common symptom that occurs in all age groups, especially in adolescents and elderly people. The cause of syncope is very diverse, and patients with syncope visit various medical departments such as general medicine, cardiology, neurology, and emergency medicine. If we do not perform appropriate diagnostic tests based on detailed history of syncope, we may have some difficulty to identify the cause of syncope. Failure to identify the cause of syncope can lead to physical trauma due to recurrence of syncope or may increase the risk of cardiovascular events in the future. However, there is no Korean guidelines for the diagnosis and treatment of syncope yet. Considering these circumstances in Korea, we prepared writing and publishing committee for evaluation and management guidelines of syncope in the Korean Society for Holter and Noninvasive Electrocardiology (KSHNE) under the Korean Heart Rhythm Society (KHRS). In this guideline, we reviewed the Korean published literatures and European / American guidelines on syncope.


Subject(s)
Adolescent , Aged , Humans , Cardiology , Diagnosis , Diagnostic Tests, Routine , Emergency Medicine , Heart , Korea , Neurology , Recurrence , Syncope , Writing
13.
International Journal of Arrhythmia ; : 145-185, 2018.
Article in Korean | WPRIM | ID: wpr-740050

ABSTRACT

The general concept and initial approach to syncope patients has been mentioned in the general sections. This special sections have been described the characteristics, diagnosis, and treatment with patient education for the each syncope. It has been described in order of reflex syncope, orthostatic hypotension, postural orthostatic tachycardia syndrome (POTS), cardiac syncope, and unexplained syncope. Several clinical issues will have been dealt with in special issues. Neurological assessment is added when the patients were diagnosed with psychogenic pseudosyncope (PPS). Although many childhood syncope caused by reflex syncope, they are also presented as syncope caused by arrhythmic events in patients with congenital heart disease. In the elderly patients, syncope is because of not only a single cause of syncope but a combination of various conditions. In case of a syncope patient visiting the emergency department, a standardized systematic approach will be required to determine whether hospitalize the patient according to the risk of recurrence and the needs for the syncope management unit. We also mention recommendations on the limits of driving, exercising and social life style that are relevant to syncope in all patients. In this guideline, we reviewed the Korean published literatures and European/American guidelines on syncope. We, writing and publishing committee for evaluation and management guidelines of syncope in the Korean Society for Holter and Noninvasive Electrocardiography (KSHNE) under the Korean Heart Rhythm Society (KHRS) are very pleased to be able to publish this guideline. We also hope this guideline will be a good support to manage the syncope patients and a useful trigger for further research in Korea.


Subject(s)
Aged , Humans , Diagnosis , Electrocardiography , Emergency Service, Hospital , Heart , Heart Defects, Congenital , Hope , Hypotension, Orthostatic , Korea , Life Style , Patient Education as Topic , Postural Orthostatic Tachycardia Syndrome , Recurrence , Reflex , Syncope , Writing
14.
Annals of Laboratory Medicine ; : 54-58, 2018.
Article in English | WPRIM | ID: wpr-739103

ABSTRACT

Long QT syndrome (LQTS) is an inherited cardiac disease characterized by a prolonged heart rate-corrected QT (QTc) interval. We investigated the genetic causes in patients with prolonged QTc intervals who were negative for pathogenic variants in three major LQTS-related genes (KCNQ1, KCNH2, and SCN5A). Molecular genetic testing was performed using a panel including 13 LQTS-related genes and 67 additional genes implicated in other cardiac diseases. Overall, putative genetic causes of prolonged QTc interval were identified in three of the 30 patients (10%). Among the LQTS-related genes, we detected a previously reported pathogenic variant, CACNA1C c.1552C>T, responsible for cardiac-only Timothy syndrome. Among the genes related to other cardiac diseases, a likely pathogenic variant, RYR2 c.11995A>G, was identified in a patient with catecholaminergic polymorphic ventricular tachycardia. Another patient who developed dilated cardiomyopathy with prolonged QTc interval was found to carry a likely pathogenic variant, TAZ c.718G>A, associated with infantile dilated cardiomyopathy. Comprehensive screening of genetic variants using multigene panel sequencing enables detection of genetic variants with a possible involvement in QTc interval prolongation, thus uncovering unknown molecular mechanisms underlying LQTS.


Subject(s)
Humans , Cardiomyopathy, Dilated , Heart , Heart Diseases , Long QT Syndrome , Mass Screening , Molecular Biology , Ryanodine Receptor Calcium Release Channel , Tachycardia, Ventricular
15.
Korean Circulation Journal ; : 148-158, 2018.
Article in English | WPRIM | ID: wpr-738679

ABSTRACT

BACKGROUND AND OBJECTIVES: Multifocal atrial tachycardia (MAT), in general, has a favorable outcome. However, there are insufficient data regarding MAT in a pediatric population. This study sought to determine the clinical course of MAT and identify potential prognostic factors. METHODS: The medical records of MAT patients from 1997–2015 were reviewed. The arrhythmia control rate and factors for unfavorable outcomes were assessed and compared to those in the literature. RESULTS: Of the 33 included patients (19 boys and 14 girls), 27 were infants less than 1 year of age. The median age at diagnosis was 1.7 months (range, 0 day to 14 years). Fourteen (42%) patients had structural heart disease. Eight (24%) patients had lung disease and 6 (18%) had a syndromic diagnosis belonging to RASopathy. Two patients developed polymorphic ventricular tachycardia, in whom genetic analysis confirmed the presence of the RyR2 mutation several years later. MAT was controlled in 26 patients (84%) within 3.9 months (median; range, 16 days–18.4 years) using an average of 2.4 medications. There were 3 cases of cardiopulmonary mortality. The arrhythmia control rate was higher in the infant group (85%) than in the non-infant group (67%), although this trend was not statistically significant. There was a significantly lower rate of unfavorable outcomes in the idiopathic infant group (n=11) than in the other groups (p=0.008). Considering the findings of previous studies, the mortality rate was significantly higher in patients with structural heart disease than in patients without (21% vs. 5%, p=0.01). CONCLUSIONS: MAT usually affects infants and has a favorable prognosis, particularly in the idiopathic infant group. However, in the presence of other comorbidities, MAT may have a variable clinical course.


Subject(s)
Humans , Infant , Arrhythmias, Cardiac , Atrial Fibrillation , Atrial Flutter , Comorbidity , Diagnosis , Heart Diseases , Lung Diseases , Medical Records , Mortality , Prognosis , Ryanodine Receptor Calcium Release Channel , Tachycardia , Tachycardia, Supraventricular , Tachycardia, Ventricular
16.
Journal of Genetic Medicine ; : 20-23, 2018.
Article in English | WPRIM | ID: wpr-715205

ABSTRACT

Point mutations in the human cardiac homeobox gene NKX2.5 are associated with familial atrial septal defect (ASD), atrioventricular (AV) conduction disturbance, as well as sudden cardiac death. To date, more than 60 NKX2.5 mutations have been documented, but there are no reports in Korea. We are reporting the first Korean family with ASD and AV block associated with a novel mutation in the NKX2.5 coding region. A 9-year-old boy presented with a slow and irregular pulse, and was diagnosed with secundum ASD and first degree AV block. The boy's father, who had a history of ASD correction surgery, presented with second degree AV block and atrial fibrillation. The boy's brother was also found to have secundum ASD and first degree AV block. There were two sudden deaths in the family. Genetic testing revealed a novel mutation of NKX2.5 in all affected members of the family.


Subject(s)
Child , Humans , Male , Atrial Fibrillation , Atrioventricular Block , Clinical Coding , Death, Sudden , Death, Sudden, Cardiac , Fathers , Genes, Homeobox , Genetic Testing , Heart Septal Defects, Atrial , Korea , Point Mutation , Siblings
17.
Korean Circulation Journal ; : 148-158, 2018.
Article in English | WPRIM | ID: wpr-917125

ABSTRACT

BACKGROUND AND OBJECTIVES@#Multifocal atrial tachycardia (MAT), in general, has a favorable outcome. However, there are insufficient data regarding MAT in a pediatric population. This study sought to determine the clinical course of MAT and identify potential prognostic factors.@*METHODS@#The medical records of MAT patients from 1997–2015 were reviewed. The arrhythmia control rate and factors for unfavorable outcomes were assessed and compared to those in the literature.@*RESULTS@#Of the 33 included patients (19 boys and 14 girls), 27 were infants less than 1 year of age. The median age at diagnosis was 1.7 months (range, 0 day to 14 years). Fourteen (42%) patients had structural heart disease. Eight (24%) patients had lung disease and 6 (18%) had a syndromic diagnosis belonging to RASopathy. Two patients developed polymorphic ventricular tachycardia, in whom genetic analysis confirmed the presence of the RyR2 mutation several years later. MAT was controlled in 26 patients (84%) within 3.9 months (median; range, 16 days–18.4 years) using an average of 2.4 medications. There were 3 cases of cardiopulmonary mortality. The arrhythmia control rate was higher in the infant group (85%) than in the non-infant group (67%), although this trend was not statistically significant. There was a significantly lower rate of unfavorable outcomes in the idiopathic infant group (n=11) than in the other groups (p=0.008). Considering the findings of previous studies, the mortality rate was significantly higher in patients with structural heart disease than in patients without (21% vs. 5%, p=0.01).@*CONCLUSIONS@#MAT usually affects infants and has a favorable prognosis, particularly in the idiopathic infant group. However, in the presence of other comorbidities, MAT may have a variable clinical course.

18.
Korean Journal of Pediatrics ; : 189-195, 2017.
Article in English | WPRIM | ID: wpr-168677

ABSTRACT

PURPOSE: The purpose of this study was to investigate the effects of lamotrigine for the treatment of attention-deficit hyperactivity disorder (ADHD) symptoms in children with epilepsy. METHODS: Pediatric patients newly diagnosed with epilepsy (n=90 [61 boys and 29 girls]; mean age, 9.1±3.4 years) were enrolled. All patients were evaluated with the Korean ADHD rating scale (K-ARS)-IV before treatment with lamotrigine and after doses had been administered. The mean interval of ADHD testing was approximately 12.3 months. The initial dosage of lamotrigine was 1 mg/kg/day (maximum 25 mg/day for the first 2 weeks), and increased by 1 mg/kg every 2 weeks until titrated up to 7 mg/kg/day (or maximum 200 mg/day). RESULTS: The mean ADHD test score of the 90 subjects was 17.0±1.8 at baseline. It was slightly reduced to 15.6±1.7 after lamotrigine monotherapy (P >0.01). Prior to treatment, a total of 31 patients (34.4%) met the diagnostic criteria for ADHD according to Diagnostic and Statistical Manual of Mental Disorders, 4th Edition, Text Revision, Of these 31 patients, 27 (87.1%) had significantly improved ADHD scores with lamotrigine monotherapy (28.0±1.6 reduced to 18.1±2.6, P<0.001). Among these 27 patients, 25 (92.6%) showed normalized electroencephalogram (EEG) and 26 (96.3%) achieved total freedom from seizures within 12 months of the initiation of lamotrigine monotherapy. CONCLUSION: The results from our study show that lamotrigine had a positive effect in pediatric epilepsy patients by reducing ADHD symptoms, preventing seizures, and normalizing EEG. However, further research is required to determine whether lamotrigine is efficacious against ADHD symptoms independent of its effects on epileptic seizures.


Subject(s)
Child , Humans , Anticonvulsants , Diagnostic and Statistical Manual of Mental Disorders , Electroencephalography , Epilepsy , Freedom , Seizures
19.
The Journal of Korean Academy of Prosthodontics ; : 234-243, 2015.
Article in Korean | WPRIM | ID: wpr-39285

ABSTRACT

Developing of digital technique, it is possible to fabricate implant prostheses for edentulous area using intraoral 3-dimentional information throughout implant diagnosis and treatment process. It is being changed that from the method using CAD/CAM, producing prostheses by model scanning after conventional impression and model processing, to the method of fabricating implant provisional restorations and customized abutments by digital impression after connecting digital impression copings (scanbody) and implant fixtures without models. But, this digital method has not been actively used for implant prostheses not yet. Specially, it is short of intraoral digital impression cases for immediate provisional restorations of the maxillary anterior implants. The gingival contour impression of maxillary anterior area is very important for esthetic restorations. Accordingly, in this case report, the using a digital impression coping (scanbody) and digital impression by CEREC Omnicam (Sirona, Bensheim, Germany) or Trios (3shape, Copenhagen, Denmark) were introduced for immediate provisional restorations in 3 cases needed a single implant restoration in maxillary anterior area. The clinical results were satisfactory on the convenience and accuracy of digital impression technique and the good esthetics of final restorations.


Subject(s)
Diagnosis , Esthetics , Prostheses and Implants
20.
Korean Circulation Journal ; : 342-345, 2011.
Article in English | WPRIM | ID: wpr-148008

ABSTRACT

QT prolongation is a serious adverse drug effect, which is associated with an increased risk of Torsade de pointes and sudden death. Many drugs, including both cardiac and non-cardiac drugs, have been reported to cause prolongation of QT interval. Although meperidine has not been considered proarrhythmic, we present a unique case of a 16-year-old boy without an underlying cardiac disease, who developed polymorphic ventricular tachycardia, ventricular fibrillation and QT prolongation after an intravenous meperidine injection. He had no mutation in long QT syndrome genes (KCNQ1, KCNH2, and SCN5A), but single nucleotide polymorphisms were reported, including H558R in SCNA5A and K897T in KCNH2.


Subject(s)
Adolescent , Humans , Death, Sudden , Heart Diseases , Long QT Syndrome , Meperidine , Polymorphism, Single Nucleotide , Tachycardia, Ventricular , Torsades de Pointes , Ventricular Fibrillation
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