A Comparative Analysis of Clinical Screening Test and Language Specific Test in Language Delay Children / 전남의대학술지

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.

Infliximab Treatment for Intravenous Immunoglobulin-resistant Kawasaki Disease: a Multicenter Study in Korea

BACKGROUND AND OBJECTIVES@#We investigated the status of infliximab use in intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) patients and the incidence of coronary artery aneurysms (CAAs) according to treatment regimens.@*METHODS@#Between March 2010 and February 2017, 16 hospitals participated in this study. A total of 102 (32.3±19.9 months, 72 males) who received infliximab at any time after first IVIG treatment failure were enrolled. Data were retrospectively collected using a questionnaire.@*RESULTS@#Subjects were divided into two groups according to the timing of infliximab administration. Early treatment (group 1) had shorter fever duration (10.5±4.4 days) until infliximab infusion than that in late treatment (group 2) (16.4±4.5 days; p 5). Overall response rate to infliximab was 89/102 (87.3%) and the incidence of significant CAA was lower in group 1 than in group 2 (1/42 [2.4%] vs. 17/60 [28.3%], p < 0.001).@*CONCLUSIONS@#This study suggests that the early administration of infliximab may reduce the incidence of significant CAA in patients with IVIG-resistant KD. However, further prospective randomized studies with larger sample sizes are required.

Infliximab Treatment for Intravenous Immunoglobulin-resistant Kawasaki Disease: a Multicenter Study in Korea

BACKGROUND AND OBJECTIVES: We investigated the status of infliximab use in intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) patients and the incidence of coronary artery aneurysms (CAAs) according to treatment regimens. METHODS: Between March 2010 and February 2017, 16 hospitals participated in this study. A total of 102 (32.3±19.9 months, 72 males) who received infliximab at any time after first IVIG treatment failure were enrolled. Data were retrospectively collected using a questionnaire. RESULTS: Subjects were divided into two groups according to the timing of infliximab administration. Early treatment (group 1) had shorter fever duration (10.5±4.4 days) until infliximab infusion than that in late treatment (group 2) (16.4±4.5 days; p 5). Overall response rate to infliximab was 89/102 (87.3%) and the incidence of significant CAA was lower in group 1 than in group 2 (1/42 [2.4%] vs. 17/60 [28.3%], p < 0.001). CONCLUSIONS: This study suggests that the early administration of infliximab may reduce the incidence of significant CAA in patients with IVIG-resistant KD. However, further prospective randomized studies with larger sample sizes are required.

Effects of low-dose topiramate on language function in children with migraine / 소아과

PURPOSE: This study aimed to verify the safety of low-dose topiramate on language development in pediatric patients with migraine. METHODS: Thirty newly diagnosed pediatric patients with migraine who needed topiramate were enrolled and assessed twice with standard language tests, including the Test of Language Problem Solving Abilities (TOPs), Receptive and Expressive Vocabulary Test, Urimal Test of Articulation and Phonology, and computerized speech laboratory analysis. Data were collected before treatment, and topiramate as monotherapy was sustained for at least 3 months. The mean follow-up period was 4.3±2.7 months. The mean topiramate dosage was 0.9 mg/kg/day. RESULTS: The patient's mean age was 144.1±42.3 months (male-to-female ratio, 9:21). The values of all the language parameters of the TOPs were not changed significantly after the topiramate treatment as follows: Determine cause, from 15.0±4.4 to 15.4±4.8 (P>0.05); making inference, from 17.6±5.6 to 17.5±6.6 (P>0.05); predicting, from 11.5±4.5 to 12.3±4.0 (P>0.05); and total TOPs score, from 44.1± 13.4 to 45.3±13.6 (P>0.05). The total mean length of utterance in words during the test decreased from 44.1±13.4 to 45.3±13.6 (P0.05). In the articulation and phonology validation in both groups, speech pitch and energy were not significant, and all the vowel test results showed no other significant values. CONCLUSION: No significant difference was found in the language-speaking ability between the patients; however, the number of vocabularies used decreased. Therefore, topiramate should be used cautiously for children with migraine.

Comparison of Percutaneous Endoscopic Gastrostomy and Surgical Gastrostomy in Severely Handicapped Children / 대한소아소화기영양학회지

PURPOSE: Gastrostomy is commonly used procedures to provide enteral nutrition support for severely handicapped patients. This study aimed to identify and compare outcomes and complications associated with percutaneous endoscopic gastrostomy (PEG) and surgical gastrostomy (SG). METHODS: A retrospective chart review of 51 patients who received gastrostomy in a single tertiary hospital from January 2000 to May 2016 was performed. We analyzed the patients and the complications caused by the procedures. RESULTS: Among the 51 patients, 26 had PEG and 25 had SG. Four cases in the SG group had fundoplication for gastroesophageal reflux disease. PEG and SG groups were followed up for an average of 29 months and 44 months. Major complications occurred in 19.2% of patients in the PEG group and 20.0% in the SG group, but significant differences between the groups were not observed. Minor complications occurred in 15.4% of patients in the PEG group and 52.0% in the SG group. Minor complications were significantly lower in the PEG group than in the SG group (p=0.006). The average use of antibiotics in the PEG and SG groups was 6.2 days and 15.7 days (p=0.002). Thirteen patients died of underlying disease but not related to gastrostomy, and only one patient died due to complications associated with general anesthesia. CONCLUSION: The duration of antibiotics use and incidence of minor complications were significantly lower in the PEG group than those in the SG group. Early PEG could be recommended for nutritional supports.

A Case of Unusual Visceral Heterotaxy Syndrome with Isolated Levocardia

Situs inversus of the abdominal organs in the presence of normally placed heart on the left side of the thorax is known as situs inversus with isolated levocardia. This rare condition is commonly associated with severe congenital defects of the heart. We report a case of situs inversus with levocardia in a 19-year-old asymptomatic male patient with completely normal heart on the left chest. Spiral computed tomography of the thorax and abdomen and echocardiographic studies revealed situs inversus of abdominal organs, normal heart (levocardia), mirrored left lungs, a midline liver, a left-sided inferior vena cava connecting to the right atrium, multiple splenic masses in the abdominal right upper quadrant, and aneurysmal dilatation of a splenic artery.

The Development Study of a Medical Ethics Education Program by Using Simple ISD Model / 한국의학교육

PURPOSE: Simple instructional systems design (ISD) model is based on a fast development, usability test, and continuos feedback, which are necessary for educational program development in medical school. This study aims to figure out the usability of Simple ISD model for a medical ethics education program by describing a developmental details of each phase and its evaluation results. METHODS: Research has been conducted in two steps. First, while researchers participated in the program development by using Simple ISD model, we collected empirical data of each development activities. Second, the developed program was evaluated by students' web-based usability test, a 8-students' focus group interview and 5 faculty members' individual interviews in 4 domains; learning contents, instructional methods and strategies, achievement evaluation, and self-evaluation. RESULTS: Following the circular process of analysis, design, development, and usability test of Simple ISD model, a 10-week medical ethics program covering 9 instructional topics was developed. The average points of response on the developed medical ethics program in 2008 and 2009 are increased from 3.96 to 4.59 and 4.41, respectively. The prospects and limitations of the program are discussed. CONCLUSION: From a development study of the medical ethics program by using Simple ISD model, we could implement a more usable medical ethics program, and found 4 different usability of the Simple ISD model; the rapid development of educational program, program improvement by continuous feedback, faculty members' engagement in instructional design, and professional development of the faculty members.

Acoustic variations associated with congenital heart disease / 소아과

PURPOSE: To investigate the nature of deviant voice physiology in preoperative children with congenital heart disease. METHODS: Ninety-four children with congenital heart disease were enrolled. Their cries and related acoustic variables (fundamental frequency, duration, noise to harmonic ratio, jitter, and shimmer) were analyzed using a multi-dimensional voice program. RESULTS: The average fundamental frequency showed a significant decrease in patent ductus arteriosus, ventricular septal defect, and tetralogy of Fallot, except in atrial septal defect and pulmonary stenosis. The length of the analyzed sample (duration) did not show a significant difference when compared with the control group. There was a significant increase in jitter percent in ventricular septal defect, patent ductus arteriosus, and atrial septal defect. There was an increase in shimmer in ventricular septal defect, patent ductus arteriosus, and atrial septal defect. The noise-to-harmonic ratio increased in ventricular septal defect, patent ductus arteriosus, and atrial septal defect but there was no significant difference in pulmonary stenosis and tetralogy of Fallot. While analyzing acoustic variables, the voice change was significantly higher, especially in patent ductus arteriosus followed by ventricular septal defect and atrial septal defect. Most of these acoustic variables were deviant in left-to-right shunt lesions in congenital heart disease, especially in patent ductus artriosus. CONCLUSION: The results of the voice change analysis of preoperative children with congenital heart disease revealed that the acoustic variables differed by each congenital heart disease. Moreover, the acoustic variables were prominently deviant in congenital heart disease with left-to-right shunts.

A Survey of Pediatricians in Private Practices Who Participated in Community-Based Clerkships: An Intellectual, Inspirational and Professional Growth Experience

PURPOSE: To examine how pediatricians in private practices are affected by the process of training medical students in their clinics as part of a community-based clerkship program. MATERIALS AND METHODS: In 2007, a questionnaire was sent to 35 pediatricians who had provided private clinical settings for clerkship training for the previous 3 years. The questionnaire covered a number of points, including the pediatricians' motivation to join and/or reasons to quit the program; if there were changes seen in their stress levels while supervising students; changes in their treatment procedures or attitudes because of the students' presence; responses of patients and/or their guardians in regard to have medical students treating them, and whether the doctors were inspired to grow professionally by participating in the program. RESULTS: Of the 35 pediatricians, 31 (88.5%) responded. Eighteen respondents (58%) selected 'responsibility to cooperate with medical school' as a reason to participate. Fifteen physicians (48.3%) answered that the clerkship program had a positive impact on their treatment procedures and their attitude towards patients. CONCLUSION: Based on the pediatricians' responses, the community-based clerkship program may instill intellectual inspiration and promote professional growth among the pediatricians in private practices, resulting in potentially better treatment for patients.

Effectiveness of head-up tilt test for the diagnosis of syncope in children and adolescents / 소아과

PURPOSE: We aimed to examine the effectiveness of the head-up tilt test (HUT) for the diagnosis of syncope or presyncope in children and adolescents. METHODS: HUT results and clinical features of 160 children and adolescents with syncope or presyncope were studied from May 2003 through March 2008 at the Chonbuk National University Hospital. The children and adolescents were subjected to 70degrees HUT for 45 minutes. The testees were divided into 2 groups: group I (children) comprising 39 children in the age range 7-12 years (mean, 10.59+/-1.60 years) and group II (adolescents) comprising 121 adolescents in the age range 13-20 years (mean, 15.93+/-2.28 years). Positive result rates of the HUT and types of hemodynamic response to the test in the 2 groups were compared. RESULTS: Of the 160 testees, 92 (57.5%) showed positive HUT results; they showed 3 patterns of response to tilting. Twelve patients showed a predominantly vasodepressor response; 10 patients showed a cardioinhibitory response; and 70 patients showed a mixed response. The positive result rates were 43.6% (17/39) and 62.0% (75/121) in groups I and II, respectively. Mixed response was the predominant positive hemodynamic response in both the groups. CONCLUSION: The HUT is a useful diagnostic tool for evaluating the condition of pediatric patients, including adolescents, with syncope. Further, it may be considered as the first step for evaluating the condition of such patients.

Results of radiofrequency catheter ablation in children and adolescent with tachyarrhythmia / 소아과

PURPOSE: Radiofrequency catheter ablation (RFCA) has become an effective therapeutic modality for treating pediatric tachyarrhythmias. Using conventional RFCA catheters, ablation of parahisian accessory pathways may be difficult and have high risk for heart block. We reviewed the efficacy and complications of the RFCA in children and adolescent with arrhythmias including parahisian accessory pathways. METHODS: We studied 48 patients (aged 2 years to 20 years) who had undergone RFCA from August 2003 to March 2007. We reviewed clinical findings, electrophysiologic studies, RFCA data, complications, and follow-up results of the patients. RESULTS: Mean age of the patients was 13.1 years. Numbers and types of arrhythmias (age, acute success rate) were as follows: 19 WPW syndrome including 5 parahisian accessory pathways (13.74.6 yr, 18/19), 11 atrioventricular reentrant tachycardia with concealed bypass tract (12.35.0 yr, 10/11), 13 atrioventricular nodal reentrant tachycardia (12.64.4 yr, 13/13), 4 atrial flutter (13.07.4 yr, 3/4), and 1 ventricular tachycardia (20 yr, 1/1). Associated cardiac structural lesion was not detected in 48 patients. The recurrence rate was 6.5%, and the final success rate was 93.8%. CONCLUSION: These results suggest that RFCA is a highly effective treatment method in children and adolescent with tachyarrhythmia.

Results of radiofrequency catheter ablation in children and adolescent with tachyarrhythmia / 소아과

PURPOSE: Radiofrequency catheter ablation (RFCA) has become an effective therapeutic modality for treating pediatric tachyarrhythmias. Using conventional RFCA catheters, ablation of parahisian accessory pathways may be difficult and have high risk for heart block. We reviewed the efficacy and complications of the RFCA in children and adolescent with arrhythmias including parahisian accessory pathways. METHODS: We studied 48 patients (aged 2 years to 20 years) who had undergone RFCA from August 2003 to March 2007. We reviewed clinical findings, electrophysiologic studies, RFCA data, complications, and follow-up results of the patients. RESULTS: Mean age of the patients was 13.1 years. Numbers and types of arrhythmias (age, acute success rate) were as follows: 19 WPW syndrome including 5 parahisian accessory pathways (13.74.6 yr, 18/19), 11 atrioventricular reentrant tachycardia with concealed bypass tract (12.35.0 yr, 10/11), 13 atrioventricular nodal reentrant tachycardia (12.64.4 yr, 13/13), 4 atrial flutter (13.07.4 yr, 3/4), and 1 ventricular tachycardia (20 yr, 1/1). Associated cardiac structural lesion was not detected in 48 patients. The recurrence rate was 6.5%, and the final success rate was 93.8%. CONCLUSION: These results suggest that RFCA is a highly effective treatment method in children and adolescent with tachyarrhythmia.

A Case of Balloon Valvuloplasty using Three Balloon Catheters in a Child with Pulmonic Stenosis

Balloon dilatation of congenital stenotic lesion of the pulmonic valve has been used. Repeated balloon dilatation of restenosed lesion after previous balloon dilatation for the pulmonic stenosis is needed in some case. We treated a case of pulmonic restenosis with using three balloon catheters in a boy. The most critical problems related the valvuloplasty are severe systemic hypotension and bradycardia due to stasis of blood flow. The use of three balloon catheters instead of single or double balloons for the pulmonic stenosis could be a alternative interventional method to preserve the preexistent forward blood flow during inflation and to minimize vascular injury in children with large valve annulus.

Joubert syndrome with peripheral dysostosis: A case report of long term follow-up / 소아과

This report describes the long-term follow-up of a 10-year-old female patient with Joubert syndrome with short stature and brachydactyly. She presented with hyperpnea alternated with hypopnea, uncontrolled jerking eye movements, and hypotonia during early infancy. She was diagnosed with Joubert syndrome based on clinical symptoms and typical MRI findings at 5 months of age. Abnormal ventilation and eye movements disappeared at around 4 years of age. Head circumference kept within normal range for her age, but her height and weight growth were markedly retarded. Simple X-ray showed an enlarged skull with increased digital markings, hypoplasia of facial bones, and abnormal enchondral bone formations in hands and feet. This article is the first report of Joubert syndrome with peripheral dysostosis.

Clinical Manifestations and Prognosis of Acute Myocarditis in Children

PURPOSE: We made investigation into the etiology, clinical manifestations, laboratory findings, treatments, outcomes, prognosis and prognostic factors of acute myocarditis in children. METHODS: We have reviewed the medical records of the patients who were admitted to the Chonbuk National University Hospital for recent 10 years. Patients were divided in two groups with base on the outcomes. One is recovery group and the other is mortality group. And we analyzed the clinical findings and compared between two groups. RESULTS: Ten of 27 patients were expired with fulminant myocarditis or complication of acute myocarditis. Seventeen patients were recovered. Left ventricular fractional shortening on the echocardiogram was a valuable prognostic factor. Seventeen patients were treated with IVIG. Seven patients were evaluated the viral etiology. Coxsackie B5, coxsackie B3, coxsackie A10 and adenovirus were detected. CONCLUSION: Left ventricular fractional shortening was a valuable prognostic factor. It is necessary to research antegrade multicenter study about acute myocarditis.

Left Ventricular Anomalous Muscle Band and Electrocardiographic Repolarization Changes in a 5 Year Old Girl

We report here on a previously healthy 5-year-old girl with significant electrocardiographic repolarization abnormalities and a left ventricular mass. After the clinical evaluation, a large muscular false tendon was found within the left ventricular cavity.

Comparision of PCR-RFLP, PCR-SSCP, Amplication Refractory Mutation System (ARMS) in Leu72Met Polymorphism of Ghrelin Gene / 소아과

PURPOSE: The role of ghrelin, which promotes the secretion of growth hormone, was not well known until now. Recently it was found that the mutation of ghrelin gene is related to obesity and diabetes. This study is to find the screening method that can easily and effectively detect the polymorphism of Leu72Met in ghrelin gene of obesity patients and apply it to clinical usage. METHODS: We compared PCR-RFLP, PCR-SSCP and ARMS methodologies for analyzing of the polymorphism of Leu72Met in ghrelin gene of obesity children, and also studied the merits and demerits of these methodologies. RESULTS: In this study, we were able to find out the band of peculiar allele of Leu72Met in ghrelin gene using PCR-RFLP, PCR-SSCP and ARMS analyses. The polymorphism of Leu72Met in ghrelin gene determined by all above methodologies was in complete agreement. Compared to the PCR- RFLP and PCR-SSCP, ARMS analysis is simple, inexpensive and also consume less time. It is very sensitive to analyze the polymorphism and easy to understand the results of test. CONCLUSION: Though PCR-RFLP, PCR-SSCP and ARMS analyses were sensitive to analyze the polymorphism of Leu72Met in ghrelin gene, ARMS analysis appears to be more efficient than PCR-RFLP and PCR-SSCP. Therefore, we conclude that ARMS analysis is suitable to analyze the polymorphism of Leu72Met in ghrelin gene for large quantity of specimens.

Rehabilitation of Children with Congenital Heart Disease: From Children to Adolescents and Adults / 소아과

No abstract available.

Electrocardiographic and Echocardiographic Characterisitics of Wolff-Parkinson-White Syndrome in Preschool Children

PURPOSE: This study was conducted to estabilish the prevalence, clinical features and relationship between ECG findings and echocardiographic findings of Wolff-Parkinsion-White(WPW) syndrome in asymptomatic preschool children. METHODS: An electrocardiographic screening study was performed on 77,824 preschool children in Jeonbuk province from April, 1999 to August, 2001. Patients with WPW syndrome underwent echocardiographic study. RESULTS: Twenty three patients with WPW syndrome were discovered by electrocardiographic screening of preschool children. The prevalence rate was 2.9 per 10,000 preschool children and there was no significant sexual difference. Two patients had a history of symptoms related to tachyarrythmia. According to the ECG classification of Rosenbaum et al., five patients were type A and 18 were type B. Utilizing the criteria of Gallagher et al, right anterior, 12 patients; right anteiror paraseptal, four patients; left anteiror, three patients. Nineteen of 23 patients underwent echocardiographic study. Four of five patients with type A WPW syndrome had abnormal early systolic anterior motion of left ventricular posterior wall. Twelve of 14 patients with type B had abnormal interventricular septal motion characterized by early sytolic posterior motion immediately after inscription of the delta wave. CONCLUSION: The prevalence rate of preschool children in Jeonbuk province was 2.9/10,000. By the classification according to the electrocardiographic findings, the accessory pathway location was dominant right side than left side. In the echocardiographic study, type A WPW syndrome showed abnormal left ventricular posterior wall motion and type B WPW showed abnormal interventricular septal motion.

Role of the Nuclear Transcription Factor NF-kappa B Caused by Acute Hypoxia in the Heart

PURPOSE: Nuclear factor-kappa B(NF-kappa B) is now recognized as playing a potential role in programmed cell death and the adaptive response to various stress. Cellular hypoxia is a primary manifestation of many cardiovascular diseases. It seems that vascular endothelial growth factor (VEGF) and insulin like growth factor-I(IGF-I) have a function as a protective molecule in the heart against several stress including hypoxia. In this study, the role of NF-kappa B to the cellular response and regulation of protective molecules against the acute hypoxia in the heart was studied. METHODS: To cause acute hypoxic stress to the heart, Sprague Dawley rats were exposed to hypoxic chamer(N2 92% and O2 8%). After the hypoxic exposure, nuclear proteins, total proteins and mRNA were isolated from heart. Translocation of the transcription factors NF-kappa B, NF-ATc, AP-1 and NKX-2.5 were evaluated by electrophoretic mobility shift assay(EMSA). The expression of IGF-I and VEGF were studied before and after the hypoxic stress by competitive-PCR, Northern hybridization and Western hybridization. To confirm the role of the NF-kappa B in the heart, the rats also were pretreated with diethyl-dithiocarbamic acid(DDTC) into peritoneal cavity to block NF-kappa B translocation into nucleus. RESULTS: The expression of NF-kappa B, AP-1 and NF-ATc were increased by the hypoxic stress. Increased expression of the VEGF and IGF-I were also observed by the hypoxic stress. However, the blocking of the NF-kappa B translocation reduced those expressions of VEGF and IGF-I. CONCLUSION: These results suggest that NF-kappa B has a protective role against the acute hypoxia through several gene expression, especially VEGF and IGF-I in heart muscle.