Effect of Multifaceted Interventions for Ward Nurses on the Storage, Conditions, and Transportation of Specimens for Microbial Culture

Purpose@#The purpose of this study was to provide multifaceted interventions for nurses and to confirm changes in their knowledge, perception, and actual practice on the adequate storage, conditions, and transportation of culture specimens. @*Methods@#A onegroup pretest-posttest experimental design was conducted with 41 nurses in two general wards of a tertiary acute care hospital in Seoul. Multifaceted interventions including education, feedback, posting guidelines and reminders, and improvement in specimen management accessibility were provided from May 2019 to January 2020. Outcomes were measured before and after the interventions. Knowledge and perception of the nurses were evaluated using self-reported questionnaires and actual practice by observation. @*Results@#After the interventions, the average knowledge score on transportation time was significantly increased (Z = -4.89, p < .001). However, the knowledge score on storage methods was not significantly increased. The perception score was significantly increased (t = -3.19, p = .003). The proportion of specimen storage times, places, and conditions managed properly was significantly increased from 43.0% (46/107) to 77.1% (84/109) (p < .001). The average transportation time of blood samples to the laboratory significantly decreased from 3 hours 36 minutes ( ± 1 hour 52 minutes) to 3 hours 1 minute ( ± 1 hour 41 minutes) (t = 2.51, p = .013). The percentage of blood culture specimens arriving within 2 hours was increased significantly from 22.9% to 39.2% ( χ 2 = 6.90, p = .009). @*Conclusion@#The interventions were effective. However, some specimens remained in the ward longer than expected after the interventions. This requires further interventions.

Acute Myocarditis in Children: a 10-year Nationwide Study (2007–2016) based on the Health Insurance Review and Assessment Service Database in Korea

Background and Objectives@#We performed a nationwide study to assess the incidence, treatment patterns, and outcomes of acute myocarditis in Korean children. @*Methods@#The nationwide incidence, treatment patterns, and outcomes of acute myocarditis in Korean children were assessed using data between 2007 and 2016 from the Health Insurance Review and Assessment database. @*Results@#We investigated 1,627 children during the study period. The overall incidence of acute myocarditis was 1.4 per 100,000 children in 2007 and 2.1 per 100,000 children in 2016, which indicates a significant increase in the trend over time. A bimodal age distribution was observed with a larger peak in infancy and a smaller peak in the mid-teenage years. No sex difference was observed in the incidence rate of acute myocarditis in children aged ≤5 years (373 boys vs. 366 girls); however, the incidence rate of acute myocarditis in adolescents aged ≥13 years showed significant male preponderance (482 boys vs. 152 girls). Acute fulminant myocarditis occurred in 371 children (22.8%) who needed extracorporeal membrane oxygenation and/or mechanical ventilator support. Of the 371 children with acute fulminant myocarditis, 258 (69.5%) survived. The survival rate of children with acute fulminant myocarditis remained nearly identical over the 10-year study period. @*Conclusions@#This was the first nationwide epidemiological study to investigate acute myocarditis in Korean children. In our view, this study would help clinicians in decisionmaking and planning for optimal management of acute myocarditis in children.

Lethal Cardiac Complications in a Long-Term Survivor of Spinal Muscular Atrophy Type 1 / 고신대학교의과대학학술지

Spinal muscular atrophy (SMA) is a rare neuromuscular disease characterized by degeneration of the anterior horn cells of the spinal cord and motor nuclei in the lower brainstem, resulting in hypotonia, progressive proximal muscle weakness, paralysis, and progressive respiratory insufficiency. We report the case of a 6-year-old girl diagnosed with spinal muscular atrophy type 1 (Werdnig-Hoffman disease) who has been treated at home with non-invasive ventilation (assist-control mode with a back-up respiratory rate of 26 per minute). She presented with an atrioventricular block and atrial fibrillation, as well as paroxysmal fluctuation of blood pressure and heart rate indicating autonomic dysfunction. Although it is known that patients with spinal muscular atrophy type 1 do not generally demonstrate cardiac problems, it can be concluded based on findings in our case that long-term survivors with spinal muscular atrophy type 1 may develop cardiac rhythm disturbances. We therefore recommend that the possibility of cardiac complications and autonomic dysfunction should be borne in mind in the management of such patients.

Increased arterial stiffness causing resistant hypertension in an adolescent with Neurofibromatosis type 1 / 고신대학교의과대학학술지

Neurofibromatosis type 1 is an autosomal dominant genetic disorder characterized by the presence of café au lait spots, axillary and inguinal freckling, Lisch nodules, and neurofibromas. Hypertension is a relatively frequent complication, usually caused by renal artery stenosis or pheochromocytomas. We describe the case of a 15-year-old boy with neurofibromatosis type 1 who was also diagnosed with resistant hypertension. Despite an extensive evaluation, the etiology of his hypertension remained indeterminate. Estimation of the brachial-ankle pulse wave velocity and ambulatory arterial stiffness index could validate the existence of arterial stiffness. Further, a combination of carvedilol and angiotension receptor blockers was administered, which successfully controlled his resistant hypertension. We propose that the estimation of the brachial-ankle pulse wave velocity measure and ambulatory arterial stiffness index is a noninvasive method, and these two parameters are relatively simple tools that can be used for the detection of arterial stiffness due to neurofibromatosis type 1-related vasculopathy.

Meta-analysis on risk stratification of malignant ventricular tachyarrhythmic events in arrhythmogenic right ventricular cardiomyopathy / 영남의대학술지

BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a cardiomyopathy characterized by predominant right ventricular fibro-fatty replacement, right ventricular dysfunction and ventricular arrhythmias. It is a rare but important cause of sudden cardiac death in children and young adults. A meta-analysis on risk stratification of major ventricular tachyarrhythmic events indicating the need for implantable cardioverter defibrillator therapy in ARVC was performed. METHODS: The pubmed database was searched from its inception to May 2015. Of the 433 citations identified, 12 were included in this meta-analysis. Data regarding major ventricular tachyarrhythmic events were retrieved in 817 subjects from the studies. For the variables, a combined odds ratio (OR) was calculated using a fixed-effects meta-analysis. RESULTS: Extensive right ventricular dysfunction (OR, 2.44), ventricular late potential (OR, 1.66), inducible ventricular tachyarrhythmia during electrophysiology study (OR, 3.67), non-sustained ventricular tachycardia (OR, 3.78), and history of fatal event/sustained VT (OR, 5.66) identified as significant risk factors (p < 0.0001). CONCLUSION: This meta-analysis shows that extensive right ventricular dysfunction, ventricular late potential, inducible ventricular tachyarrhythmia during electrophysiological study, non-sustained ventricular tachycardia, and history of sustained ventricular tachycardia/fibrillation are consistently reported risk factors of major ventricular tachyarrhythmic events indicating implantable cardioverter defibrillator therapy in patients with ARVC.

SCN1A Variants in Patients with Dravet Syndrome / 대한소아신경학회지

PURPOSE: The aim of this study is to examine the SCN1A variants in Korean patients with Dravet syndrome. METHODS: We conducted a retrospective study of clinically confirmed thirty-nine patients with Dravet syndrome who visit our hospital from January 2007 to May 2015. We analyzed the SCN1A variants by direct sequencing. We analyzed and classified SCN1A variants according to ACMG/AMP (American College of Medical Genetics and Genomics and the Association for Molecular Pathology) guideline. RESULTS: A total thirty-nine patients (female 22, male 17) were included. Among them, twenty patients (51.2%) with Dravet syndrome had pathogenic or likely pathogenic SCN1A mutations including fifteen truncating mutations (12 nonsense and 3 splice region mutations), 5 missense mutations. The remained variants in nineteen patients with Dravet syndrome classified into ten variants of unknown significances, and 9 benign variants. In our study, truncation mutations are located whole span of SCN1A protein, while half of missense mutations are located at higher density on pore loop (S5-S6) regions. CONCLUSION: Unlike previous known study, lower positive rate of SCN1A mutation of Dravet syndrome was revealed in our study. The importance of parental test (trio test) and other additional tests have been emphasized.

SCN1A Variants in Patients with Dravet Syndrome / 대한소아신경학회지

PURPOSE: The aim of this study is to examine the SCN1A variants in Korean patients with Dravet syndrome. METHODS: We conducted a retrospective study of clinically confirmed thirty-nine patients with Dravet syndrome who visit our hospital from January 2007 to May 2015. We analyzed the SCN1A variants by direct sequencing. We analyzed and classified SCN1A variants according to ACMG/AMP (American College of Medical Genetics and Genomics and the Association for Molecular Pathology) guideline. RESULTS: A total thirty-nine patients (female 22, male 17) were included. Among them, twenty patients (51.2%) with Dravet syndrome had pathogenic or likely pathogenic SCN1A mutations including fifteen truncating mutations (12 nonsense and 3 splice region mutations), 5 missense mutations. The remained variants in nineteen patients with Dravet syndrome classified into ten variants of unknown significances, and 9 benign variants. In our study, truncation mutations are located whole span of SCN1A protein, while half of missense mutations are located at higher density on pore loop (S5-S6) regions. CONCLUSION: Unlike previous known study, lower positive rate of SCN1A mutation of Dravet syndrome was revealed in our study. The importance of parental test (trio test) and other additional tests have been emphasized.

The evolution of electrocardiographic changes in patients with Duchenne muscular dystrophies / 소아과

PURPOSE: Myocardial dysfunction and dysrhythmias are inevitable consequences of Duchenne muscular dystrophy. We aimed to evaluate specific trends of electrocardiographic changes that reflect the progress of cardiomyopathy in patients with Duchenne muscular dystrophy. METHODS: Fifty electrocardiograms (ECGs) of 30 patients (ages 1 to 27 years) who had not been prescribed medications for heart failure treatment at the time of examination were retrospectively analyzed and compared with 116 ECGs of age-matched healthy 116 controls. Heart rate, leads with fragmented QRS (fQRS), corrected QT, Tpeak-to-Tend, and Tpeak-to-Tend/QT were analyzed. RESULTS: The patients with Duchenne muscular dystrophy failed to show a normal age-related decline in heart rate but showed an increasing trend in the prevalence of fQRS, corrected QT, corrected Tpeak-to-Tend, and Tpeak-to-Tend/QT over time. In the ≤10-year-old patient group, a significant difference was found only in the prevalence of fQRS between the patients and the controls. The prevalence of fQRS, heart rate, Tpeak-to-Tend/QT, and corrected Tpeak-to-Tend demonstrated significant differences between the patients and the controls in the middle age group (11 to 15 years old). All the indexes were statistically significantly different in the ≥16-year-old patient group. CONCLUSION: The prevalence of lead with fQRS representing regional wall motion abnormalities was higher in the young patients than in the young healthy controls, and this might be one of the first signs of myocardial change in the patients. Markers of depolarization and repolarization abnormalities were gradually prominent in the patients aged >10 years. Further studies are needed to confirm these findings.

Meta-analysis on risk stratification of malignant ventricular tachyarrhythmic events in arrhythmogenic right ventricular cardiomyopathy / 영남의대학술지

BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a cardiomyopathy characterized by predominant right ventricular fibro-fatty replacement, right ventricular dysfunction and ventricular arrhythmias. It is a rare but important cause of sudden cardiac death in children and young adults. A meta-analysis on risk stratification of major ventricular tachyarrhythmic events indicating the need for implantable cardioverter defibrillator therapy in ARVC was performed.METHODS: The pubmed database was searched from its inception to May 2015. Of the 433 citations identified, 12 were included in this meta-analysis. Data regarding major ventricular tachyarrhythmic events were retrieved in 817 subjects from the studies. For the variables, a combined odds ratio (OR) was calculated using a fixed-effects meta-analysis.RESULTS: Extensive right ventricular dysfunction (OR, 2.44), ventricular late potential (OR, 1.66), inducible ventricular tachyarrhythmia during electrophysiology study (OR, 3.67), non-sustained ventricular tachycardia (OR, 3.78), and history of fatal event/sustained VT (OR, 5.66) identified as significant risk factors (p < 0.0001).CONCLUSION: This meta-analysis shows that extensive right ventricular dysfunction, ventricular late potential, inducible ventricular tachyarrhythmia during electrophysiological study, non-sustained ventricular tachycardia, and history of sustained ventricular tachycardia/fibrillation are consistently reported risk factors of major ventricular tachyarrhythmic events indicating implantable cardioverter defibrillator therapy in patients with ARVC.

Vitamin D deficiency in children aged 6 to 12 years: single center's experience in Busan

PURPOSE: This study investigated the prevalence and risk factors associated with vitamin D deficiency in children. METHODS: We analyzed the medical records of 330 patients from the age of 6 to 12, who visited the endocrinology clinic of the Department of Pediatrics at Pusan National University Hospital, from September, 2013 to May, 2014. According to their serum 25-hydroxyvitamin D (25(OH)D) levels, the patients were grouped into either the deficiency group (25(OH)D<20 ng/mL), or the sufficiency group (25(OH)D≥20 ng/mL). The differences between the 2 groups were compared. RESULTS: There were 195 patients (59.1%) who had vitamin D deficiency. Their mean serum 25(OH)D level was 14.86±3.20 ng/mL. The differences in sex, age, and pubertal status between the 2 groups were not statistically significant. Weight standard deviation score (SDS), and body mass index SDS, were significantly higher in the vitamin D deficiency group (P=0.002 for each), compared to the sufficiency group. Compared with Autumn, both Spring (odds ratio [OR], 9.7; 95% confidence interval [CI], 4.3–22.0), and Winter (OR, 5.9; 95% CI, 3.5–10.0), were risk factors for vitamin D deficiency. In multiple logistic regression analysis, only seasonal differences have been confirmed to have an effect on vitamin D deficiency. CONCLUSION: Vitamin D deficiency in children aged 6 to 12 years is very common. Spring and Winter are the most important risk factors for vitamin D deficiency. We suggest that it is necessary to supplement the guideline for the vitamin D intake according to our situation.

Vitamin D deficiency in children aged 6 to 12 years: single center's experience in Busan

PURPOSE: This study investigated the prevalence and risk factors associated with vitamin D deficiency in children. METHODS: We analyzed the medical records of 330 patients from the age of 6 to 12, who visited the endocrinology clinic of the Department of Pediatrics at Pusan National University Hospital, from September, 2013 to May, 2014. According to their serum 25-hydroxyvitamin D (25(OH)D) levels, the patients were grouped into either the deficiency group (25(OH)D<20 ng/mL), or the sufficiency group (25(OH)D≥20 ng/mL). The differences between the 2 groups were compared. RESULTS: There were 195 patients (59.1%) who had vitamin D deficiency. Their mean serum 25(OH)D level was 14.86±3.20 ng/mL. The differences in sex, age, and pubertal status between the 2 groups were not statistically significant. Weight standard deviation score (SDS), and body mass index SDS, were significantly higher in the vitamin D deficiency group (P=0.002 for each), compared to the sufficiency group. Compared with Autumn, both Spring (odds ratio [OR], 9.7; 95% confidence interval [CI], 4.3–22.0), and Winter (OR, 5.9; 95% CI, 3.5–10.0), were risk factors for vitamin D deficiency. In multiple logistic regression analysis, only seasonal differences have been confirmed to have an effect on vitamin D deficiency. CONCLUSION: Vitamin D deficiency in children aged 6 to 12 years is very common. Spring and Winter are the most important risk factors for vitamin D deficiency. We suggest that it is necessary to supplement the guideline for the vitamin D intake according to our situation.

Giant coronary aneurysm caused by Kawasaki disease: consistency between catheter angiography and electrocardiogram gated dual-source computed tomography angiography / 소아과

We present the case of a 5-year-old child with coronary complications due to Kawasaki disease; this patient unintentionally underwent both dual-source computed tomography (DSCT) coronary angiography and invasive coronary angiographic examination in 2 months. This case highlights the strong consistency of the results between DSCT coronary angiography and invasive coronary angiography. Compared to conventional invasive coronary angiography, DSCT coronary angiography offered additional advantages such as minimal invasiveness and less radiation exposure.

Development of Smartphone Educational Application for Patients with Coronary Artery Disease / 대한의료정보학회지

OBJECTIVES: This study was conducted to develop a smartphone application (app) as an educational learning instrument for coronary artery disease (CAD) patients and to assess the users' level of satisfaction. METHODS: This methodological research involves elicited learning content for CAD patients to develop a learning instrument using the smartphone app. The app was developed according to the steps of Assessment, Design, Development, Implementation, and Evaluation, which is a systematic instructional design model. The levels of satisfaction with the developed smartphone app among 30 outpatients with CAD were assessed via a questionnaire during their visits to a cardiology outpatient department. RESULTS: A smartphone app 'Strong Heart' was developed through reviewing the literature associated with education for CAD patients under professional supervision and searching for medical smartphone apps that are already available. The learning contents include six main sections containing essential learning issues in managing CAD and additional information to attract the user's attention, such as patient cases and quizzes. After modification with feedback from experts, the app was finally developed and evaluated by patients who reported that they were satisfied with the usefulness of the app. CONCLUSIONS: The developed smartphone app is available on both the iPhone App Store and the Android Play Store. Patients with CAD may utilize the app for supporting educational material without limitations of time and space.

QT dispersion in children with Kawasaki disease / 영남의대학술지

BACKGROUND: We analyzed the changes in QT dispersion (QTd) in children with Kawasaki disease (KD), and determined the presence of repolarization abnormality in these children even in the absence of coronary artery abnormalities. METHODS: Ninety-one children with KD and 20 healthy controls were enrolled in this retrospective study. Serial echocardiographic and electrocardiographic (ECG) measurements in the beginning of treatment, 2nd month and 6th month after the diagnosis were compared. Fifty-one of 91 children had at least 2 serial ECG data. The number of patients who had 3 consecutive ECG data was 23. RESULTS: Among the 67 KD patients with no coronary artery changes, the consecutive mean QTd values were 41.86 ms, 37.84 ms, and 25.47 ms, respectively (26 ms for controls). In the analysis of changes among KD patients without coronary artery abnormalities, QTd showed a significant decrease with time (p=0.01). Especially, the 1st month and the 6th month QTd values were significantly different (p=0.028). The mean QTd values in KD patients with coronary artery changes were significantly higher than those in KD patients with no coronary artery changes at each time (1st, 2nd, and 6th month exam). CONCLUSION: QTd is significantly increased in children during the early stage of KD. Repolarization abnormality may exist during the acute stage of KD, regardless of the echocardiographic changes.

Stomach and Colon Metastasis from Breast Cancer / 이화의대지

Gastric metastasis from breast cancer is rare and only six cases have been reported in Korea. Colon metastasis is more rare than gastric metastasis. We report a 63-year-old woman with gastric and colon metastases of invasive lobular carcinoma of breast. She was diagnosed as right breast cancer, received right modified radical mastectomy 10 years ago and has been treated with chemotherapy and hormone therapy. Investigating for melena and a small caliber of stool, we found gastric and colon metastases. The diagnosis of metastatic breast cancer was made through gross pathologic and immunohistochemistry staining. We report a case with gastric and colon metastases from breast cancer and a review of the associated six case reports in Korea.

Cytomegalovirus appendicitis with concurrent bacteremia after chemotherapy for acute leukemia

No abstract available.

Cause and incidence of eosinophilia in children: a single center study in one year

PURPOSE: Eosinophilia may be associated with various primary and reactive conditions. However, studies on the cause and incidence of eosinophilia in Korean children are rare. This study aimed to evaluate the cause and incidence of eosinophilia in patients at a single university hospital. METHODS: We studied 8,285 pediatric patients under the age of 18 years who had eosinophilia at Pusan National University Hospital. Premature and newborn infants were excluded. Eosinophilia was defined as an absolute eosinophil count greater than 450/microL. Eosinophilia was categorized as mild (450-1,500/microL), moderate (1,500-5,000/microL), and severe (>5,000/microL). The underlying conditions of eosinophilia were retrospectively investigated. RESULTS: Of 8,285 patients who had a hematology profile, 497 (5.9%) were found to have eosinophilia. Of patients with eosinophilia, 333 patients (67.0%) had identifiable and possible causes. The major causes of eosinophilia were allergic diseases (61.3%), infectious diseases (19.8%), immunologic diseases (9.0%) and hemato-oncologic disease (5.1%). Immunological disease such as Idiopathic hypereosinophilic syndrome, drug induced hypersensitivity syndrome and Graft-versus-host disease was the common condition with moderate to severe eosinophilia in which eosinophil count in peripheral blood was more than 1,500/microL. CONCLUSION: The most common cause of eosinophilia was allergic disease. Immunological disease was the common condition with moderate to severe eosinophilia.

Fasting serum C-peptide is useful for initial classification of diabetes mellitus in children and adolescents

PURPOSE: With rising obesity rates in children, it is increasingly difficult to differentiate between type 1 and type 2 diabetes mellitus (T1DM, T2DM) on clinical grounds alone. Using C-peptide as a method of classifying diabetes mellitus (DM) has been suggested. This study aimed to find a correlation between fasting C-peptide level and DM types in children and adolescents. METHODS: A total of 223 diabetic children, newly diagnosed at 5 hospitals between January 2001 and December 2012, were enrolled in this study. Initial DM classification was based on clinical and laboratory data including fasting C-peptide at diagnosis; final classification was based on additional data (pancreatic autoantibodies, human leukocyte antigen type, and clinical course). RESULTS: Of 223 diabetic children, 140 were diagnosed with T1DM (62.8%) and the remaining 83 with T2DM (37.2%). The mean serum C-peptide level was significantly lower in children with T1DM (0.80 ng/mL) than in children with T2DM (3.91 ng/mL). Among 223 children, 54 had a serum C-peptide level 3.0 ng/mL; 48 of them (97.9%) were diagnosed with T2DM. CONCLUSION: In this study, we found that if the C-peptide level was 3.0 ng/mL, a T1DM diagnosis is unlikely. This finding suggests that serum fasting C-peptide level is useful for classifying DM type at the time of diagnosis in youth.

Percutaneous ultrasound-guided thrombin injection is effective even in infants with external iliac artery pseudoaneurysms / 소아과

Iatrogenic pseudoaneurysms are extremely rare in children. Conventional management of pseudoaneurysms in adults has included surgical repair, ultrasound-guided compression, and more recently, endovascular embolization. However, in infants and children, there is little information regarding the applicability of such treatment modalities, which have been effective in adults, because of its rarity. Here, we present the case of a 6-month-old infant who developed a postprocedural pseudoaneurysm of the external iliac artery, which was successfully treated with ultrasound-guided percutaneous thrombin injection.

A Case of Reversible Very Low Voltage Electrocardiogram in Fulminant Myocarditis

Clinical features of acute myocarditis range from a subclinical state to a fulminant state. Fulminant myocarditis with ventricular arrhythmia or atrioventricular block is associated with a high mortality rate. In cases in which aggressive medical therapy for fulminant myocarditis is not likely to be successful, intensive and emergency mechanical circulatory support, such as extracorporeal membrane oxygenation (ECMO) or intra-aortic balloon pump, should be considered. We report life salvage of acute fulminant myocarditis in a 53-year-old woman presented with malignant arrhythmia and cardiogenic shock supported by ECMO.