BACKGROUND AND PURPOSE: Laser induced prostatectomy(LIP) has recently been considered as safe alternative to conventional transurethral resection of the prostate(TURP) in the surgical treatment of BPH. However, the value of LIP remains incompletely defined. We herein have performed a prospective study to compare TURP and LIP in treatment efficacy, safety and costs to define the value of LIP. MATERIALS AND METHODS: 113 patients with BPH who were candidates of TURP were randomized to undergo TURP or LIP and were adequately followed up for more than 1 year. There were no significant differences in preoperative clinical characteristics between 55 patients who underwent TURP and 58 patients who underwent LIP. For the LIP procedure, Nd:YAG was used in 42 patients and diode laser in 16 patients, respectively. 37 patients were treated by contact LIP only, and 21 with hybrid procedures of contact LIP and noncontact LIP using side firing laser fiber or interstitial laser fiber. Seven patients underwent LIP under local anesthesia at the outpatient department. RESULTS: International prostate symptom score(IPSS) and peak urinary flow rate(Qmax) were significantly improved at 3 months, 6 months, and 1 year after LIP as well as after TURP. There were no significant difference between TURP group(85.4%) and LIP group(87.9%) in treatment success rate as defined by improvement of IPSS and Qmax as well as patient's content for the surgical outcome. Nine(16.4%) and two(3.6%) of the patients who underwent TURP and none of the patients who LIP underwent developed ignificant bleeding and electrolyte imbalance, respectively. There were no significant difference in postoperative incidence of retrograde ejaculation, infection and urethral stricture between the two groups. Compared to TURP, the LIP procedure required significantly shorter hospitalization(6.8 vs 4.5 days) and catheterization(4.1 vs 2.6 days, all p<0.0l). There was no significant difference in total treatment cost between the two groups. CONCLUSIONS: LIP may be comparable to TURP in terms of short term treatment efficacy and cost effectiveness. LIP may be better than TURP in terms of safety and shortened hospitalization and catheterization. Further studies are necessary on long-term outcomes of LIP."
Anesthesia, Local ; Catheterization ; Catheters ; Cost-Benefit Analysis ; Ejaculation ; Fires ; Health Care Costs ; Hemorrhage ; Hospitalization ; Humans ; Incidence ; Lasers, Semiconductor ; Lip ; Male ; Outpatients ; Prospective Studies ; Prostate* ; Prostatectomy* ; Transurethral Resection of Prostate ; Treatment Outcome ; Urethral Stricture

The incidence of ectopic pregnancy has been increasing for many years and ectopic pregnancy is still a leading cause of maternal death although its mortality is reduced significantly with improved early diagnosis and treatment. Intraligamentous pregnancy, a subset of ectopic pregnancy, is one of the most unusual accident so that the diagnosis of intraligamentous pregnancy is hardly ever made prior to laparotomy. We experienced intraligamentous pregnancy with hemoperitoneum. So we presented a case with brief review of literature.
Diagnosis ; Early Diagnosis ; Female ; Hemoperitoneum ; Hemorrhage* ; Incidence ; Laparotomy ; Maternal Death ; Mortality ; Pregnancy* ; Pregnancy, Ectopic

The incidence of ectopic pregnancy has been increasing for many years and ectopic pregnancy is still a leading cause of maternal death although its mortality is reduced significantly with improved early diagnosis and treatment. Intraligamentous pregnancy, a subset of ectopic pregnancy, is one of the most unusual accident so that the diagnosis of intraligamentous pregnancy is hardly ever made prior to laparotomy. We experienced intraligamentous pregnancy with hemoperitoneum. So we presented a case with brief review of literature.
Diagnosis ; Early Diagnosis ; Female ; Hemoperitoneum ; Hemorrhage* ; Incidence ; Laparotomy ; Maternal Death ; Mortality ; Pregnancy* ; Pregnancy, Ectopic

Acute giant coronary aneurysm after Kawasaki disease (KD) is a catastrophic complication that can be fatal and very difficult to manage. However, no fixed consensus has been reached for the management of super-giant coronary aneurysms in the acute setting. Here, we report the successful management of young children with super-giant coronary aneurysms after KD. Based on our experience, hemodynamic stabilization to prevent further coronary dilation or rupture and strict anticoagulation to avoid thrombus formation are mandatory in the management of this condition.
Child ; Consensus ; Coronary Aneurysm* ; Coronary Artery Disease ; Coronary Thrombosis ; Hemodynamics ; Humans ; Mucocutaneous Lymph Node Syndrome* ; Rupture ; Thrombosis

PURPOSE: The use of implantable cardioverter defibrillators (ICDs) to prevent sudden cardiac death is increasing in children and adolescents. This study investigated the use of ICDs in children with congenital heart disease. METHODS: This retrospective study was conducted on the clinical characteristics and effectiveness of ICD implantation at the department of pediatrics of a single tertiary center between 2007 and 2011. RESULTS: Fifteen patients underwent ICD implantation. Their mean age at the time of implantation was 14.5+/-5.4 years (range, 2 to 22 years). The follow-up duration was 28.9+/-20.4 months. The cause of ICD implantation was cardiac arrest in 7, sustained ventricular tachycardia in 6, and syncope in 2 patients. The underlying disorders were as follows: ionic channelopathy in 6 patients (long QT type 3 in 4, catecholaminergic polymorphic ventricular tachycardia [CPVT] in 1, and J wave syndrome in 1), cardiomyopathy in 5 patients, and postoperative congenital heart disease in 4 patients. ICD coils were implanted in the pericardial space in 2 children (ages 2 and 6 years). Five patients received appropriate ICD shock therapy, and 2 patients received inappropriate shocks due to supraventricular tachycardia. During follow-up, 2 patients required lead dysfunction-related revision. One patient with CPVT suffered from an ICD storm that was resolved using sympathetic denervation surgery. CONCLUSION: The overall ICD outcome was acceptable in most pediatric patients. Early diagnosis and timely ICD implantation are recommended for preventing sudden death in high-risk children and patients with congenital heart disease.
Adolescent ; Cardiomyopathies ; Channelopathies ; Child ; Convulsive Therapy ; Death, Sudden ; Death, Sudden, Cardiac ; Defibrillators ; Defibrillators, Implantable ; Early Diagnosis ; Follow-Up Studies ; Heart ; Heart Arrest ; Heart Defects, Congenital ; Heart Diseases ; Humans ; Korea ; Long QT Syndrome ; Pediatrics ; Retrospective Studies ; Shock ; Sympathectomy ; Syncope ; Tachycardia, Supraventricular ; Tachycardia, Ventricular

Since the first descriptions of Brugada as a new clinical entity defined by sudden cardiac death in patients with typical electrocardiogram (ECG) patterns, Brugada syndrome (BS) has been increasingly diagnosed. This syndrome is known as a disease that is inherited via an autosomal dominant trait, and the SCN5A mutation has been found in 20-25% of BS patients. Because BS primarily manifests in adulthood, little information is available on BS during childhood. Although there have been several reports on adult BS in Korea, pediatric BS has not been reported. Herein, we report on childhood BS in two families. One infantile BS patient and his family had a novel SCN5A mutation (c.4035G>T, p.W1345C, heterozygote) in domain III of the sodium channel.
Adult ; Brugada Syndrome ; Death, Sudden, Cardiac ; Electrocardiography ; Humans ; Korea ; Sodium Channels ; Tachycardia, Ventricular

Timothy syndrome, long QT syndrome type 8, is highly malignant with ventricular tachyarrhythmia. A 30-month-old boy had sudden cardiac arrest during anesthesia induction before plastic surgery for bilateral cutaneous syndactyly. After successful resuscitation, prolonged QT interval (QTc, 0.58-0.60 sec) and T-wave alternans were found in his electrocardiogram. Starting beta-blocker to prevent further tachycardia and collapse event, then there were no more arrhythmic events. The genes KCNQ1, KCNH2, KCNE1 and 2, and SCN5A were negative for long QT syndrome. The mutation p.Gly406Arg was confirmed in CACNA1C, which maintains L-type calcium channel depolarization in the heart and other systems.
Anesthesia/*adverse effects ; Calcium Channels, L-Type/*genetics ; Death, Sudden, Cardiac/*etiology ; Electroencephalography ; Humans ; Infant ; Long QT Syndrome/*genetics ; Magnetic Resonance Imaging ; Male ; Methyl Ethers/adverse effects ; Nitric Oxide/adverse effects ; Polymorphism, Single Nucleotide ; Sequence Analysis, DNA ; Surgery, Plastic ; Syndactyly/diagnosis/*genetics/surgery

A 14-month-old boy was transferred because of dilated and hypertrophied left ventricle, neutropenia, and developmental delay. After checking computed tomographic angiography with contrast-dye, the patient showed acute exacerbation and finally died from multi-organ failure despite intensive cares. From genetic analysis, we revealed that the patient had Barth syndrome and found a novel hemizygous frame shift mutation in his TAZ gene, c.227delC (p.Pro76LeufsX7), which was inherited from his mother. Herein, we report a patient with Barth syndrome who had a novel mutation in TAZ gene and experienced unexpected acute exacerbation after contrast dye injection for computed tomographic angiography.
Acidosis/etiology ; Acute Disease ; Adolescent ; Barth Syndrome/diagnosis/*genetics ; Contrast Media/adverse effects/*diagnostic use ; Frameshift Mutation ; Heart Failure/etiology ; Homozygote ; Humans ; Male ; Mutation ; Pedigree ; Sequence Analysis, DNA ; Tomography, X-Ray Computed ; Transcription Factors/*genetics

PURPOSE: Kabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center. METHODS: A retrospective analysis was conducted for a total of 13 patients with Kabuki syndrome. RESULTS: The median age at diagnosis of was 5.9 years (range, 9 days to 11 years and 8 months). All patients showed the characteristic facial dysmorphisms and congenital anomalies in multiple organs, and the diagnosis was delayed by 5.9 years (range, 9 days to 11 years and 5 months) after the first visit. Noncardiac anomalies were found in 84% of patients, and congenital heart diseases were found in 9 patients (69%). All 9 patients exhibited left-side heart anomalies, including hypoplastic left heart syndrome in 3, coarctation of the aorta in 4, aortic valve stenosis in 1, and mitral valve stenosis in 1. None had right-side heart disease or isolated septal defects. Genetic testing in 10 patients revealed 9 novel MLL2 mutations. All 11 patients who were available for follow-up exhibited developmental delays during the median 4 years (range, 9 days to 11 years 11 months) of follow-up. The leading cause of death was hypoplastic left heart syndrome. CONCLUSION: Pediatric cardiologist should recognize Kabuki syndrome and the high prevalence of left heart anomalies with Kabuki syndrome. Genetic testing can be helpful for early diagnosis and counseling.
Abnormalities, Multiple ; Aortic Coarctation ; Aortic Valve Stenosis ; Cause of Death ; Counseling ; Diagnosis ; Early Diagnosis ; Follow-Up Studies ; Genetic Testing ; Heart Defects, Congenital ; Heart Diseases ; Heart* ; Humans ; Hypoplastic Left Heart Syndrome ; Intellectual Disability ; Mitral Valve Stenosis ; Prevalence ; Retrospective Studies

The long QT syndrome (LQTS) is a rare hereditary disorder in which affected individuals have a possibility of ventricular tachyarrhythmia and sudden cardiac death. We investigated 62 LQTS (QTc > or = 0.47 sec) and 19 family members whose genetic study revealed mutation of LQT gene. In the proband group, the modes of presentation were ECG abnormality (38.7%), aborted cardiac arrest (24.2%), and syncope or seizure (19.4%). Median age of initial symptom development was 10.5 yr. Genetic studies were performed in 61; and mutations were found in 40 cases (KCNQ1 in 19, KCNH2 in 10, SCN5A in 7, KCNJ2 in 3, and CACNA1C in 1). In the family group, the penetrance of LQT gene mutation was 57.9%. QTc was longer as patients had the history of syncope (P = 0.001), ventricular tachycardia (P = 0.017) and aborted arrest (P = 0.010). QTc longer than 0.508 sec could be a cut-off value for major cardiac events (sensitivity 0.806, specificity 0.600). Beta-blocker was frequently applied for treatment and had significant effects on reducing QTc (P = 0.007). Implantable cardioverter defibrillators were applied in 6 patients. Congenital LQTS is a potentially lethal disease. It shows various genetic mutations with low penetrance in Korean patients.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Asian Continental Ancestry Group/genetics ; Calcium Channels/genetics ; Child ; Child, Preschool ; Electrocardiography ; Heart Arrest/genetics/pathology ; Humans ; Infant ; KCNQ1 Potassium Channel/genetics ; KCNQ2 Potassium Channel/genetics ; Long QT Syndrome/*diagnosis/*genetics ; Middle Aged ; Mutation/*genetics ; NAV1.5 Voltage-Gated Sodium Channel/genetics ; Penetrance ; Potassium Channels, Inwardly Rectifying/genetics ; Republic of Korea ; Risk Factors ; Seizures/genetics/pathology ; Young Adult