Comparison of Outcomes after Device Closure and Medication Alone in Patients with Patent Foramen Ovale and Cryptogenic Stroke in Korean Population

PURPOSE: To compare the effectiveness of device closure and medical therapy in prevention of recurrent embolic event in the Korean population with cryptogenic stroke and patent foramen ovale (PFO). MATERIALS AND METHODS: Consecutive 164 patients (men: 126 patients, mean age: 48.1 years, closure group: 72 patients, medical group: 92 patients) were enrolled. The primary end point was a composite of death, stroke, transient ischemic attack (TIA), or peripheral embolism. RESULTS: Baseline characteristics were similar in the two groups, except age, which was higher in the medical group (45.3±9.8 vs. 50.2±6.1, p<0.0001), and risk of paradoxical embolism score, which was higher in the closure group (6.2±1.6 vs. 5.7±1.3, p=0.026). On echocardiography, large right-to-left shunt (81.9% vs. 63.0%, p=0.009) and shunt at rest/septal hypermobility (61.1% vs. 23.9%, p<0.0001) were more common in the closure group. The device was successfully implanted in 71 (98.6%) patients. The primary end point occurred in 2 patients (2 TIA, 2.8%) in the closure group and in 2 (1 death, 1 stroke, 2.2%) in the medical group. Event-free survival rate did not differ between the two groups. CONCLUSION: Compared to medical therapy, device closure of PFO in patients with cryptogenic stroke did not show difference in reduction of recurrent embolic events in the real world's setting. However, considering high risk of echocardiographic findings in the closure group, further investigation of the role of PFO closure in the Asian population is needed.

Non-Responders to Intravenous Immunoglobulin and Coronary Artery Dilatation in Kawasaki Disease: Predictive Parameters in Korean Children

BACKGROUND AND OBJECTIVES: In Kawasaki disease (KD), high dose intravenous immunoglobulin (IVIG) significantly lowers the coronary complications. However, some patients either do not respond to initial therapy or develop coronary complications. We aimed to identify the predictive factors for unresponsiveness to initial IVIG therapy and coronary artery dilatation (CAD; defined by Z-score≥2.5) in the acute phase and convalescent phase. SUBJECTS AND METHODS: A retrospective review was conducted of 703 patients with KD, admitted to Gachon University Gil Medical Center between January 2005 and June 2013. The patients were divided into two groups-IVIG responders vs. non-responders-based on the IVIG treatments, and presence of fever after treatment. Further, these groups were divided into two subgroups based on their CAD. RESULTS: Among the 703 patients with KD, the rate of non-responders to initial IVIG was 16.8%. Serum total bilirubin, platelet count, and neutrophil proportion were independent predictive parameters of unresponsiveness (p<0.05). CAD was found in 234 patients (33.3%) in the acute phase, and in 32 patients (4.6%) in the convalescent phase. Male gender, fever duration, serum C-reactive protein, and white blood cell count were related to CAD (p<0.05). CAD was detected more frequently in non-responders than in the responders (47.5% vs. 31.5%, p=0.001). Kobayashi, Egami, and Sano scoring systems applied to our study population reflected low sensitivities (28.0-33.9%). CONCLUSION: Several independent parameters were related to unresponsiveness to the initial IVIG or CAD. These parameters might be helpful in establishing more focused and careful monitoring of high-risk KD patients in Korea.

Prevalence of BRAF and NRAS Mutations and a Comparative Analysis in Primary and Metastatic Melanoma of Korean Patients / 이화의대지

OBJECTIVES: The aim of this study is to verify the status and the clinical significance of BRAF and NRAS mutations in patients of one of the university hospitals in Korea. METHODS: Polymerase chain reaction (PCR) amplification and direct sequencing were performed for the analysis of melanoma samples (n=22) for the detection of mutations in exon 15 of the BRAF gene, and exons 2 and 3 of the NRAS gene in genomic DNA. Mutations of the BRAF gene were correlated with the clinicopathologic features of patients and the BRAF mutation status was compared in 18 paired primary and metastatic tumors. RESULTS: Incidence of somatic mutations within the BRAF and NRAS genes was 27.3% (6/22) and 0% (0/22), respectively. Age, gender, Breslow thickness, and ulceration did not show correlation with BRAF mutations. Among 18 patients with metastasis, BRAF mutation was detected in 22.2% of cases (4/18), and all four cases with BRAF mutations were identified in metastatic lymph node tissues. BRAF mutations were only found in lymph node metastases, which was statistically significant (28.6% vs 0%, P<0.01). CONCLUSION: The incidence of BRAF mutation is as low as in other Asian reports and the NRAS mutation was not found in patients of our institute.

Efficacy of Inhaled Iloprost in Cor Pulmonale and Severe Pulmonary Hypertension Associated with Tuberculous Destroyed Lung

Chronic obstructive pulmonary disease (COPD) is one of the causes of cor pulmonale. Cor pulmonale patients with pulmonary hypertension have a significant lower survival rate than patients without. However, there is no conclusive treatment options in cor pulmonale and pulmonary hypertension associated with COPD until now. We report a patient with cor pulmonale and pulmonary hypertension associated with severe form of COPD and tuberculous destroyed lung who achieved marked clinical, functional and echocardiographic hemodynamic improvements with inhaled iloprost for six months.

Functional Class and Targeted Therapy Are Related to the Survival in Patients with Pulmonary Arterial Hypertension

PURPOSE: Pulmonary arterial hypertension (PAH) is an orphan disease showing poor prognosis. The purpose of study was to evaluate clinical factors influencing outcomes in PAH. MATERIALS AND METHODS: Patients who were diagnosed with PAH at a single center were reviewed retrospectively. Forty patients (34.9+/-14.5 years, 80% of female) were enrolled. RESULTS: Causes were congenital heart disease in 24 (60%), connective tissue disease in 8 (20%) and idiopathic PAH in 6 (15%). Sixteen patients (40%) were WHO functional class III or IV at the time of diagnosis. Twenty seven patients (67.5%) received molecular targeted therapy. During follow-up (53.6+/-45.5 months), 10 patients (25%) died and 1-, 2-, and 8 year survival rates were 91.3%, 78.7%, and 66.8%, respectively. As expected, median survival of patients with functional class I or II were significantly longer than patients with III or IV (p=0.041). Interestingly, patients with molecular targeted therapy showed longer survival than conventional therapy (p=0.021). CONCLUSION: WHO functional class at the time of diagnosis was the strong predictor of survival, and molecular targeted therapy could significantly improve the survival. Therefore, early screening and intensive management would be crucial to improve the prognosis in the patient with PAH.

Inhaled iloprost for the treatment of patient with Fontan circulation / 소아과

Decreased exercise capacity after Fontan surgery is relatively common and the failure of the Fontan state gradually increases with age. However, there is no further treatment for patients with Fontan circulation. Pulmonary vasodilation therapy is an effective method to solve this problem because pulmonary vascular resistance is a major factor of the Fontan problem. Inhaled iloprost is a chemically stable prostacyclin analogue and a potent pulmonary vasodilator. We experienced two cases of Fontan patients treated with inhaled iloprost for 12 weeks. The first patient was an 18-year-old female with pulmonary atresia with an intact ventricular septum, and the second patient was a 22-year-old male with a double outlet right ventricle. Fifteen years have passed since both patients received Fontan surgery. While the pulmonary pressure was not decreased significantly, improved exercise capacity and cardiac output were observed without any major side effects in both patients. The iloprost inhalation therapy was well tolerated and effective for the symptomatic treatment of Fontan patients.

Correction: Clinical Features of Symptomatic Meckel's Diverticulum in Children: Comparison of Scintigraphic and Non-scintigraphic Diagnosis / 대한소아소화기영양학회지

The name "Sung Min Kim" should be "Seong Min Kim" and "Yoon Mi Kim" should be "Yun Mi Kim".

Clinical Features of Symptomatic Meckel's Diverticulum in Children: Comparison of Scintigraphic and Non-scintigraphic Diagnosis / 대한소아소화기영양학회지

PURPOSE: Meckel's diverticulum (MD) has various clinical manifestations, and diagnosis or selectection of proper diagnostic tools is not easy. This study was conducted in order to assess the clinical differences of MD diagnosed by scintigraphic and non-scintigraphic methods and to find the proper diagnostic tools. METHODS: We conducted a retrospective review ofthe clinical, surgical, radiologic, and pathologic findings of 34 children with symptomatic MD, who were admitted to Gachon University Gil Medical Center, Inha University Hospital, and The Catholic University of Korea, Incheon St. Mary's Hospital between January 2000 and December 2012. The patients were evaluated according to scintigraphic (12 cases; group 1) and non-scintigraphic (22 cases; group 2) diagnosis. RESULTS: The male to female ratio was 7.5 : 1. The most frequent chief complaint was lower gastrointestinal (GI) bleeding in group 1 and nonspecific abdominal pain in group 2, respectively. The most frequent pre-operative diagnosis was MD in both groups. Red blood cell (RBC) index was significantly lower in group 1. MD was located at 7 cm to 85 cm from the ileocecal valve. Four patients in group 1 had ectopic gastric tissues causing lower GI bleeding. The most frequent treatment modality was diverticulectomy in group 1 and ileal resection in group 2, respectively. CONCLUSION: To diagnose MD might be delayed unless proper diagnostic tools are considered. It is important to understand indications of scintigraphic and non-scintigraphic methods according to clinical and hematologic features of MD. Scintigraphy would be weighed in patients with anemia as well as GI symptoms.

Pattern of Hospital-Associated Infections in Children Admitted in the Intensive Care Unit of a University Hospital / 소아감염

PURPOSE: Hospital associated infection (HAI) caused by multidrug-resistant (MDR) microorganisms has been recognized as an important issue in the world, especially in critically ill patients such as the patients admitted in the intensive care unit. There are fewer papers about MDR-HAI in pediatric patients compared to adult patients. In this study, we investigated the incidence and associated factors of MDR-HAI in children admitted to the intensive care unit (ICU) of a university hospital. METHODS: We retrospectively evaluated 135 children who were admitted in ICU for at least 3 days between January 2009 and December 2010. HAI cases were divided into MDR-HAI group and non-MDR-HAI group. Clinical characteristics and various associated factors were compared between those groups. RESULTS: In 39 patients, 45 cases of ICU-related HAI were developed. ICU-related HAI incidence was 47.7 per 1000 patient-days. Thirty-six cases (80.0%) were MDR-HAI. Acinetobacter baumannii was isolated more commonly in MDR-HAI group. And the followings were found more frequently in MDR-HAI group than non-MDR-HAI group: medical condition as an indication for ICU admission, mechanical ventilation, urinary catheterization and previous use of broad-spectrum antibiotics. Among the risk factors, previous use of broad-spectrum antibiotics was the independent risk factor for MDR-HAI. CONCLUSION: ICU-related HAI incidence was higher than previously reported. Previous use of broad-spectrum antibiotics was the independent risk factor for MDR-HAI. To investigate the characteristics of MDR-HAI in children admitted in ICU, further studies with a larger sample size over a longer period of time are warranted.

The effect of perioperative inhaled iloprost on congenital heart disease with severe pulmonary arterial hypertension / 소아과

A 47-year-old male patient in whom atrial septal defect (ASD) had been diagnosed 15 years previously was admitted for cardiac catheterization. He had definite cyanotic lips and nail beds and severe pulmonary arterial hypertension (PAH). He had received medical treatment only for the last few years after being diagnosed with Eisenmenger syndrome. After cardiac catheterization, he received iloprost inhalation therapy pre and postoperation and was discharged after successful surgical closure of the ASD.

The Results of Transcatheter Occlusion of Patent Ductus Arteriosus: Success Rate and Complications Over 12 Years in a Single Center

BACKGROUND AND OBJECTIVES: Percutaneous occlusion of patent ductus arteriosus (PDA) has become increasingly attractive with the evolution of devices and techniques. We reviewed results for percutaneous occlusion of PDA using various devices in a single center. SUBJECTS AND METHODS: A retrospective review was done for 118 consecutive procedures performed in 111 patients with PDA between January 1996 and December 2007. RESULTS: The median age of the patients was 4.5 years (0.9 to 60.3 years); body weight was 16.9 kg (6.8 to 74.7 kg). The median PDA diameter at the pulmonic end was 3.8 mm (0.7 to 10 mm); mean pulmonary artery pressure was 21.0 mmHg (7 to 60 mmHg). Complete occlusion occurred in 76/111 (68.4%) immediately after implantation and in 100/111 (90.0%) at one year of follow-up. Second procedures for residual shunts were done in 7 patients. After the year 2001, the complete closure rate was 95.2% compared to 71.4% before 2001. Complications associated with the procedure were left pulmonary artery narrowing (all <20 mmHg) in 14, arrhythmia in 2, and death in 1. CONCLUSION: Evolution of devices, cumulative experience, and health insurance covering the cost of devices have contributed to good outcomes in our center for percutaneous occlusion of PDA. Our results have improved over the years, particularly with the use of the Amplatzer duct occluder.

Clinical Characteristics of Chlamydia trachomatis Respiratory Tract Infection in Young Infants / 소아알레르기및호흡기학회지

PURPOSE: Chlamydia trachomatis (C. trachomatis) is the most prevalent etiology of sexually transmitted diseases and also a cause of respiratory infections in infants. The purposes of this study were to determine the epidemiology, presentation, and laboratory findings of C. trachomatis respiratory infection, and to investigate its severity and hospital courses. METHODS: Between February 2002 and January 2010, we enrolled infants younger than 6 months admitted to Gil Hospital with a diagnosis of acute bronchiolits and pneumonia. Clinical findings and demographic data were reviewed in all patients. Basic laboratory examinations included white blood cell and eosinophil count. C. trachomatis was detected by enzyme immunoassay for C. trachomatis specific immunoglobulin M. RESULTS: Of 1,708 patients, C. trachomatis was detected in 142 (8.3%). The incidence increased from 2002 to 2009 and was higher in spring. C. trachomatis infection was distinguished by less fever (p<0.01) and more crackle (p<0.05) on auscultation, and strongly correlated with eosinophilia (P=0.01). The severity and clinical course of C. trachomatis infection was similar to those of infections by the other etiologies. CONCLUSION: C. trachomatis is prevalent among infants younger than 6 months with a diagnosis of acute bronchiolits and pneumonia. We must consider C. trachomatis as the etiologic agent in infantile respiratory infections and must make more effort to detect C. trachomatis.

Acute Lupus Pneumonitis as Initial Manifestation of Systemic Lupus Erythematosus / 소아알레르기및호흡기학회지

We report case of a 14-year-old girl with systemic lupus erythematosus who initially presented with acute lupus pneumonitis. She had a 4-week history of exertion for the past 4 weeks, and was transferred from a regional hospital due to abnormality on chest radiographs. Chest radiographs revealed bilateral infiltration and pleural effusion on both lower lung fields. We assumed her to be infected and prescribed antibiotics. The response to antibiotics was ineffective, and viral, bacterial, and mycobacterial cultures were negative. Antinuclear and anti-dsDNA antibodies in serum were positive. The open lung biopsy revealed diffuse alveolar damage. She was diagnosed as having acute lupus pneumonitis in systemic lupus erythematosus and recovered gradually after receiving corticosteroids. Acute lupus pneumonitis may be considered even though interstitial lung involvement in systemic lupus erythematosus is relatively rare in pediatric practice and its diagnosis is difficult.

Predictive indicators of coronary artery complications in Kawasaki disease / 소아과

PURPOSE: Kawasaki disease-the most common cause of acquired heart disease in children-incidence is increasing yearly. Therefore, we evaluated the predictive indicators of coronary complications of Kawasaki disease based on clinical and laboratory data. METHODS: Between January 2005 and March 2008, of the 201 children with Kawasaki disease treated at the Gil Hospital of Gachon University of Medicine and Science, 51 had coronary artery lesions (Group II) and 150 had no lesions (Group I). The reasons for coronary artery lesions were deduced from the clinical and laboratory data. RESULTS: Analysis of the 2 groups revealed that fever duration and days of fever after and before initial intravenous gammaglobulin (IVIG) treatment were significantly longer in Group 2 than in Group I. IVIG infusions were statistically higher in Group II than in Group I. As per the laboratory data, C-reactive protein (CRP) value was significantly higher in Group II. Collectively, >10 days of fever duration, >48 h of fever duration after, and >10 days of fever before IVIG treatment increased the risk of coronary artery lesions 6-, 5-, and 3.5-fold, respectively. Furthermore, additional IVIG courses and higher CRP level increased the risk of coronary artery lesions 4-fold and 2.3-fold, respectively. CONCLUSION: The following 3 factors were responsible for increased risk of coronary artery lesions in children with Kawasaki disease: fever duration and days of fever after and before IVIG treatment. To identifythe predictive indicators of coronary complications, it is necessary to further elucidate the relationship between well-known forecasting factors.

Correlation between Clinical Findings of Epileptic Seizures and Electroencephalography in Children / 대한소아신경학회지

PURPOSE: Electroencephalography(EEG) is an essential method carried out for classifying seizures and taking appropriate treatment. The aim of this study is to investigate the concordance between clinical findings of epileptic seizures and EEG in children. METHODS: We enrolled 461 patients from those who visited Gil hospital from January 1, 2000 to September 30, 2008 with the chief complaint of epileptic seizure more than once and checked their first EEG at the same hospital. The clinical findings of seizure were based on the charts and interictal waking and sleep EEGs were done. RESULTS: The mean age of the patients was 6.7 years old. 497 epileptic seizures occurred and its clinical finding included 310 of partial seizures and 187 of generalized seizures. In 315 waking EEG 158 were abnormal including 118 of partial seizures, 59 of generalized seizures and 19 of both seizures, in 431 sleep EEG 239 were abnormal including 196 of partial seizures, 77 of generalized seizures and 34 of both seizures, and in waking and/or sleep EEG 273 were abnormal including 216 of partial seizures, 97 of generalized seizures and 40 of both seizures. Epileptic syndromes were observed in 90 patients. 146(41.5%) of partial seizures, 44(23.4%) of generalized seizures, and 79(87.8%) of epileptic syndromes accorded with EEG. CONCLUSION: When diagnosing seizures in children, we must consider not only clinical findings but also accurate EEG findings.

Drug Compliance of Pediatric Patients with Epilepsy / 대한소아신경학회지

PURPOSE: The sole available treatment for the majority of epilepsy patients is antiepileptic drug therapy. Drug compliance is an important factor for adequate treatment. This study is for investigating pediatric epilepsy patients' compliance to antiepileptic drug treatment at Gil hospital, in South Korea. METHODS: From February 1997 to July 2005, retrospective studies were made on 327 patients with epilepsy in Gil hospital of Gachon medical school. The patients who were taking medication prescribed at other hospitals, were admitted to the hospital for over 30 days, had seizures due to meningitis, encephalitis or trauma were excluded. The participants included patients followed for two years or until the seizures were no longer present. RESULTS: Of the total 327 patients, the mean drug compliance was 80.8%; over 80% for 203 patients(62.1%), 80-50% for 97, under 50% for 28(8.6%). Gender, the age of patients, dose frequency, results of the EEG, MRI abnormalities, etiology of epilepsy, and the duration of the first seizure did not significantly influence drug compliance. However, the patients taking tablets or capsules showed higher compliance than those treated with powder medication. In addition, the patients who started to take drugs after the year 2000 had higher compliance than those before 2000. CONCLUSION: The patients taking tablets or capsules and those who started after 2000 had higher compliance. Although most patients had satisfactory drug compliance, 8.6% had poor compliance(under 50% of compliance); this group requires further attention to improve their compliance.

The Clinical and Histopathologic Findings of Lymphonodular Hyperplasia of the Colon in Infancy and Childhood / 대한소아소화기영양학회지

PURPOSE: Lymphonodular hyperplasia of the colon (LNHC) is a rare finding in children and its significance as a pathologic finding is unclear. The aim of this study was to investigate the clinical significance of LNHC by analyzing clinical and histopathologic findings in children with LNHC. METHODS: We analyzed data from 38 patients who were confirmed to have LNHC by colonoscopy. We checked age, birth history, past history, family history, and clinical symptoms. A hematologic exam, stool exam, and image studies were performed and biopsy specimens were examined by a pathologist. All patients were asked to have short- and long-term follow-up. RESULTS: The mean age of the patients was 12.5+/-14.4 months. All patients presented with complaints of bloody stool. They appeared healthy and the hematologic findings were within a normal range, with the exception of one case. There was no other identified source of bleeding. On histologic exam, 36 patients (94.7%) had lymphoid follicles and 34 patients (84.5%) fulfilled the criteria of allergic colitis. Regardless of diet modification and presence of residual symptom, there was no recurrence of bloody stool through long-term follow-up in all patients. CONCLUSION: LNHC is more common in infants who are affected by allergic colitis, but it can appear even after infancy. LNHC should be regarded as the etiology when there are any other causes of rectal bleeding, especially in healthy children. We suggest that LNHC has a benign course regardless of diet modification and it might not require excessive concerns.

The efficacy and safety of transcatheter closure of atrial septal defect with Amplatzer septal occluder in young children less than 3 years of age / 소아과

PURPOSE: Applicability of transcatheter closure of atrial septal defect (ASD) has been expanded by accumulation of clinical experiences and evolutions of the device. This study was performed to evaluate the safety and efficacy of transcatheter closure of ASD with Amplatzer septal occluder (ASO) in young children less than 3 years of age. METHODS: From May 2003 to December 2005, 295 patients underwent transcatheter closure of ASD with ASO in the Severance Cardiovascular Hospital, Yonsei University Health System. Among them, 51 patients less than 3 years of age were enrolled in this study. We investigated procedural success rate, rate of residual shunt, frequency of complications, procedure/fluoroscopy time, and need of modified techniques for device implantation. RESULTS: The median age was 2.1 years and median body weight was 12 kg. Implantation of device was successful in 50 patients (98%). Seven patients (15%) showed a small residual shunt 1 day after the procedure, but complete occlusion had been documented at 6 month follow-up in all patients (100%). The pulmonary to systemic flow ratio (Qp/Qs), peak systolic pulmonary artery pressure, and peak systolic right ventricular pressure had decreased significantly after closure of ASD. There were 2 complications including device embolization (1, 2%) and temporary groin hematoma (1, 2%). CONCLUSION: Transcatheter closure of ASD with ASO can be performed with satisfactory results and acceptable risk even in young children less than 3 years of age. We could suggest that even in very young children with ASD, there is no need to wait until they grow to a sufficient size for the transcatheter closure.

Clinical Characteristics of Childhood Henoch-Schonlein Purpura with Duodenal Involvement by Upper Gastrointestinal Endoscopy / 대한소아소화기영양학회지

PURPOSE: The aim of this study was to investigate the clinical usefulness of upper gastrointestinal (GI) endoscopy in children with Henoch-Schonlein purpura (HSP). METHODS: We retrospectively analyzed the clinical, endoscopic, and histopathologic records of children with HSP who had been admitted to the Department of Pediatrics of Gil Hospital and underwent upper GI endoscopy between January 2002 and June 2009. Patients were classified into the following two groups for statistical analysis: duodenal involvement (+) and duodenal involvement (-). RESULTS: Fifty-one children with HSP underwent upper GI endoscopy; the mean age was 7.2+/-2.9 years. The upper GI endoscopy showed abnormalities of the duodenum in 38 cases (74.5%), 22 of which had duodenal ulcers. Among the biopsy specimens obtained from the duodenum of 37 cases, 13 cases (35.1%) had leukocytoclastic vasculitis, neutrophil debri, and/or extravasation of RBCs. Steroid use was more frequent in the duodenal involvement (+) group (86.8%) than the duodenal involvement (-) group (53.8%; p=0.02). The mean length of hospitalization was 13.9+/-8.43 days in the duodenal involvement (+) group and 8.1+/-4.62 days in the duodenal involvement (-) group (p=0.003). The recurrence rate was significantly higher in the duodenal involvement (-) group than the duodenal involvement (+) group (p=0.027), whereas none of the other study parameters, such as the age of onset, renal involvement, and steroid use, led to significantly higher or lower recurrence rates. CONCLUSION: These results suggest that duodenal involvement can influence the clinical course and prognosis of HSP in children.

Multifocal Atrial Tachycardia in a Newborn

Multifocal atrial tachycardia (MAT) is a rare arrhythmia in the newborn. MAT can be difficult to diagnose; it is frequently confused with atrial fibrillation. MAT is difficult to treat but often resolves spontaneously within the first year of life. A newborn with a rapid and irregular pulse rate was diagnosed with multifocal atrial tachycardia by eletrocardiography (ECG) using a hand-made transesophageal electrode. Treatment with propranolol was attempted but ineffective. Treatment with digoxin and sotalol was attempted. The heart rhythm gradually reverted to a sinus rhythm with this treatment. We report our experience managing a neonate with MAT diagnosed by ECG using a hand-made transesophageal electrode.