• Journal of Chest Surgery
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• v.35 no.8
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• pp.573-579
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• 2002

Background: In this study, the role and the surgical outcome of the modified Blalock-Taussig shunt in the treatment of the infants with cyanotic complex congenital heart diseases were investigated. Material and Method: Over the last 12 years, 105 modified BT shunts were performed in 100 infants. Postoperative course, shunt patency rate, complications, mortality and its risk factors were reviewed restrospectively. Result: The mean age at operation was 43.0$\pm$36.6 days. Sex ratio was 60:40(M:F). The postoperative oxygen saturations were lowest after mean duration of 11 hours after the shunt procedure. The operative mortality was 8%(8) with 3 late deaths. Causes of operative death included failure of maintenance of minimum oxygenation during the procedure(2), immediate postoperative shunt occlusion(2), respiratory failure(2), low cardiac output due to heart failure and pericardial effusion(2) and sepsis(2). Late deaths resulted from acute cardiac arrest during the follow up cardiac catheterization, hypoxic myocardial failure, and arrhythmia. Year of surgery, shunt size, age at operation, and complexity of the anomalies were not the risk factors for mortality. Six month shunt patency rate was 97% and overall patency rate was 96%. Postoperative complications comprised of shunt occlusion(6), phrenic nerve palsy(3), and wound infection(2). Conclusion: We demonstrated that modified Blalock-Taussig shunt was a useful tool to palliate the infants with complex cyanotic heart disease in whom early complete repair was not feasible with acceptable mortality and patency rate. An adequate postoperative management and a meticulous surgical technique may be key factors for the better results.

• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.75-80
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• 2005

Purpose : Internal tibial torsion is prevalent in East Asian countries such as Korea and Japan, where sitting on the floor is common behavior. Internal tibial torsion or excessive lateral tibial torsion may cause esthetical, functional, or psychological problems and also may induce degenerative arthritis in older age. The purpose of this study is to measure the tibial torsion in children of the Jeju area. Methods : Tibial torsion was measured in 1,042 lower extremities of 521 children from one to 12 years of age. The values of transmalleolar angles were analyzed for each age group divided by 6 months. Quadratic and linear regression models were used to fit patterns of changes in mean values of transmalleolar angles. The age at seven, which provides the highest coefficient of determination for quadratic regression analysis, was used as a cut-off point to fit different statistical models. Results : The mean transmalleolar angle was $0.10{\pm}5.79^{\circ}$ in all children,$ 0.90{\pm}5.49^{\circ}$ in males, and $-0.80{\pm}5.97^{\circ}$ in females. The value was $4.25{\pm}4.04$ in 1 year of age, gradually decreased to the lowest level of $-1.98^{\circ}$ in four years and seven months of age, increased again with age until it reached $0.67{\pm}1.10^{\circ}$ at seven years of age, and stayed at that level thereafter. Conclusion : Internal tibial torsion in infancy is known to correct spontaneously in the normal developing process. But in this study, the mean transmalleolar angle in children of Jeju area annually decreased after one year of age; to the lowest angle at four years and seven months of age; increased again gradually to the age of seven; and persisted in that level, about $10^{\circ}$ less than western children, not correcting further thereafter. These findings suggest tibial torsion might be caused by lifestyle, especially sitting on feet. To prevent abnormalities of joints and gaits, early diagnosis of tibial torsion in childhood and posture correction or early treatment when needed, seems to be necessary.

• Clinical and Experimental Pediatrics
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• v.51 no.3
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• pp.276-285
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• 2008

Purpose : Allergic proctocolitis is a major cause of bloody stool in early infancy. This study was aimed at ascertaining the clinical courses, sigmoidoscopic and histologic findings of allergic proctocolitis. We also analyzed the relationship between peripheral eosinophilia, the age at symptom onset, and sigmoidoscopic and histologic findings. Methods : We reviewed 25 infants retrospectively who had sigmoidoscopy and biopsy performed with a clinical diagnosis of allergic proctocolitis from April 2003 to April 2007. Results : The mean age at symptom onset was $15.2{\pm}13.2$ weeks. Fourteen infants (56.0%) were breast fed, one (4.0%) was formula fed, six (24.0%) were on combined formula, and four (16.0%) were on a weaning diet. Peripheral eosinophilia (${\geq}250/mm^3$) was seen in eighteen infants (75.0%), but total serum IgE was increased only in six (24.0%). Sigmoidoscopic findings were variable from normal (8.0%), erythema or edema (20.0%), lymphoid hyperplasia (8.0%), erosion (12.0%), hemorrhage and ulcer (4.0%) to lymphoid hyperplasia with erosion, hemorrhage, or ulcer (48.0%). Histologic findings showed focal infiltration of eosinophils in lamina propria (96.0%) and crypt epithelium (96.0%). In twenty four infants (96.0%), the number of eosinophils in mucosa was increased by a more than 60/10 high power field. There was a negative correlation between peripheral eosinophilia and the age at symptom onset. Among the twelve breast fed infants, bloody stool disappeared in ten (83.0%) with a maternal elimination diet of major food groups, but two improved spontaneously. Conclusion : Allergic proctocolitis should be considered as one of the major causes of bloody stool in healthy appearing infants. To confirm the diagnosis it is necessary to perform sigmoidoscopy and biopsy but histologic findings are more informative than sigmoidoscopic findings. Peripheral eosinophilia was prominent in the infants with an early onset of symptoms. Most infants experienced benign courses and recovered with the elimination of causative foods but did not need exclusive food restrictions.

• Clinical and Experimental Pediatrics
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• v.45 no.9
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• pp.1126-1133
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• 2002

Purpose : Prader-Willi syndrome(PWS) is a complex disorder affecting multisystems with characteristic clinical features. Its genetic basis is an expression defect in the paternally derived chromosome 15q11-q13. We analyzed the clinical features and genetic basis of PWS patients for early detection and treatment. Methods : We retrospectively studied 24 patients with PWS in Department of Pediatrics, Samsung Medical Center, from September 1997 to September 2001. We performed cytogenetic and molecular genetic techniques using high resolution GTG banding techniques, fluorescent in situ hybridization and methylation-specific PCR for CpG island of SNRPN gene region. Results : The average birth weight of PWS patients was $2.67{\pm}0.47kg$ and median age at diagnosis was 1.3 years. The average height and weight of PWS patients under one year at diagnostic time were located in a 3-10 percentile relatively, and a rapid weight gain was seen between two and six years. Feeding problems in infancy and neonatal hypotonia were the two most consistently positive major criteria in over 95% of the patients. In 18 of the 24 cases(75%), deletion of chromosome 15q11-q13 was demonstrated and one case among 18 had an unbalanced 14;15 translocation. In four cases without any cytogenetic abnormality, it may be considered as maternal uniparental disomy and the rest showed another findings. Conclusion : We suggest diagnostic testing for PWS in all infants/neonates with unexplained feeding problems and hypotonia. It is necessary for clinically suspicious patients to undergo an early genetic test. As the genetic basis of PWS was heterogenous and complex, further study is required.

• Journal of Genetic Medicine
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• v.4 no.1
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• pp.38-44
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• 2007

Purpose : Menkes disease is an X-linked recessively inherited disorder caused by the mutation of the ATP7A gene encoding copper-transporting P-type AT Pase. The phenotypic features are progressive neurological degeneration, mental retardation, loose skin, and vascular complications. Early diagnosis and treatment are very important for the prognosis of Menkes disease. Here, we describe nov el mutations of the ATP7A gene and prenatal diagnosis by mutation analysis. Methods : Five unrelated Korean Menkes patients were included in this study. They presented with depigmented wool-like hair, progressive neurologic deterioration, and hypotonia in infancy. Serum copper and ceruloplasmin levels w ere decreased. Brain magnetic resonance imaging revealed tortuous intracranial vessels. Mutation analysis has been carried out using cDNA from cultured skin fibroblasts or genomic DNA from peripheral leukocytes. Prenatal diagnosis was performed in two cases using chorionic villi samples or amniocytes. Results : Four novel mutations have been identified from four different families; c.3511+1G>A (p.E1099_N1171delinsMfsX 18), c.4005+5 G>A (p.V1268_R1335del), c.1870_2172del (p.S624_Q724del), and c.3352 G>A (p.G1118S). T he remaining one was previously reported (c.1933 C>T (p.V 1268_R1335del)). On prenatal DNA analysis, one w as diagnosed as normal, while the other turned out to be a female heterozygote with p.S624_Q724del mutation of the ATP7A gene. Conclusion : We identified 4 novel mutations of the ATP7A gene. Prenatal diagnosis in families at risk is critical in order to choose preventiv e options including an early treatment with copper-histidine therapy or therapeutic termination. Most mutations of the ATP7A gene were frame-shift mutations and prenatal diagnosis has been successfully carried out.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.60-68
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• 1998

Purpose: To determine the various prenatal factors related to the prenatal hydronephrosis diagnosed on prenatal ultrasonography. We also attempted to correlate the prenatal and neonatal renal pelvic anteroposterior diameter with the outcome in infancy Methods: Between 1985 and 1997. We retrospectively reviewed 105 renal unit (75 patient) with fetal hydronphrosis persisting postnatally. Investigation consisted of renal ultrasonography, voiding cystourethrography, diurectic renogram, and DMSA scan. Results: The 75 patient assesed had the following underlying cause: UPJ(Ureteropelvic juction) obstruction(52%). multicystic dysplastic kidney(10%). UVJ (Ureterovesicai juction) obstruction (10%) and no underlying cause (25%). Of theses cases 36 cases (40 renal unit) underwent operation, while 28 cases (50 renal unit) resoled spontaneously. 12% of mild hydornephrosis deteriorated. whereas 50% of modrate hydrophrosis and 81% of severe hydronephrosis required surgical correction. Attempting to find the renal unit that were at risk for deterioration. our study showed that urinary tract infection group and calyceal blunting group had a predictive role. Conclusion: It is necessary to follow up after birth dilatation of caylx or urinary tract infection are present. Early operation is considered when prenatal pelvic AP diameter greater is than 22 mm and postnatal diameter greater than 17 mm. This may make it possible to prevent further progression of renal damage and prompt treatment of asymptomatic hydronephrosis before complications occur.

• Journal of Yeungnam Medical Science
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• v.13 no.2
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• pp.199-210
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• 1996

Infantile hypertrophic pyloric stenosis(IHPS), which occurs three of 1,000 live births, is a major cause of nonbilious vomiting of early infancy but its etiology and pathogenesis are still obscure. The operation of pyloromyotomy as described by Ramstedt in 1912 remains the standard of care for the treatment of IHPS. From January 1993 to October 1996, 35 infants with IHPS were surgically treated and the following results were obtained. 1. Thirty-five patients comprised 32 males and 3 females, and the ratio of male to female was 10.7:1. 2. The most prevalent age group was between 2 weeks and 8 weeks. 3. Of 35 infants, first born babies were 23 cases(65.7%). 4. Breast feeding was in 23 cases(65.7%). 5. The body weight percentile at admission was lower than 50 percentile in all 35 cases. 6. Onset of symptoms was predominantly between 1week and 2 weeks in 11 cases(31.4%). 7. All had a history of nonbilious vomting, generally projectile in nature. 8. In the measured serum electrolytes, hypokalemia was noted in 9 cases(25.7%), hypochloremia was observed in 14 cases(40.0%). 9. In the preoperative ultrasonography, the average muscle thickness, diameter, and length of the pylorus were 6.2mm, 12.1mm, and 17.9mm, respectively. 10. Among 35 cases, significant sonographic criteria of IHPS, muscle thickness of 5mm or more, were noted in 26 cases(83.9%). 11. A total of seven associated anomalies were noted in six patients. 12. All 35 cases were treated with Fredet-Ramstedt pyloromyotomy. 13. There were postoperative complications of wound infection in 2 cases. Intermittent nonprojectile vomiting was presented in 8 cases(22.9%) after operation, but one of them was relieved in 13 days and the rest were relieved within one week by adjustment of oral intake.

• Clinical and Experimental Pediatrics
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• v.46 no.4
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• pp.370-375
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• 2003

Purpose : It has been suggested that the exposure to aeroallergens during early infancy after birth is important in the subsequent development of sensitization and allergic diseases. In Korea, the level of house dust mites as one of the important aeroallergens is known to be the highest in autumn. The aim of this study was to test whether the distribution of month of birth bears a relationship to the presence of mite sensitization in children with respiratory allergy. Methods : Skin prick tests and methacholine provocation tests were performed on 1,327 patients with chronic respiratory symptoms who visited Seoul National University Children's Hospital from January 1995 to May 2002. An analysis of patients' month of birth distribution according to the presence of mite sensitization was performed. Results : Atopic subjects who had at least one positive skin test numbered 864(65.1%); and non-atopic subjects numbered 463(34.9%). Among atopic subjects, 787(59.3%) had positive skin tests to mites and 77(5.8%) had positive skin test only to minor allergens. A significantly greater than expected number of mite atopic subjects were born in the months between August and November(P=0.03), however, the birth month of non-atopic subjects didn't show a consistent seasonal preference. Asthma patients numbered 543(40.9%). Among these, atopic asthmatics numbered 421(77.5%) and non-atopic asthmatics, 122(22.5%). Dust-mite atopic asthmatics numbered 387(91.9%) out of 421 atopic asthmatics. Dust-mite atopic asthmatics were born significantly higher in the season lasting from August to November in comparison to non-atopic asthmatics(P=0.002). Conclusion : Month of birth seems to be related with sensitization to allergens. Our results show that August to November is the risk period for the development of mite sensitization in Korea.

• Advances in pediatric surgery
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• v.4 no.1
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• pp.39-47
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• 1998

Infantile hypertrophic pyloric stensosis(IHPS) occurs in three of 1000 live births, and is a major cause of nonbilious vomiting of early infancy. It's etiology and pathogenesis however are still obscure. The operation of pyloromyotomy described by Ramstedt in 1912 remains the standard treatment. From January 1990 to July 1997, 64 infants with IHPS were treated at the Department of Pediatric Sursery, Yeungnam University Hospital. The ratio of male to female was 7:1, and the most prevalent age ranged from 2 weeks to 8 weeks(81.2 %) of age. Fifty-seven infants were first born (57.8 percent). The body weight of all patients at admission was below the 50 percentile. Age of onset of symptoms was between 2 and 4weeks of age in 23 cases(35.9 %). All infants had a history of nonbilious vomting, generally projectile in nature. Hypokalemia was noted in 14 cases(21.9 %) and hypochloremia in 26 cases(40.6 %). In the preoperative ultrasonography, the average muscle thickness, diameter, and length of the pylorus were 6.3 mm, 12.3 mm, and 17.8 mm. A total of 13 associated anomalies were noted in 12 patients. All cases were treated with Fredet-Ramstedt pyloromyotomy. Postoperative wound infection occured in 3 cases. Thirteen cases(20.3 %) presented intermittent nonprojectile vomiting after operation. With control of oral intake vomiting subsided within one week in 63 patients, and in thirteen days in another.

• Asia pacific journal of information systems
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• v.19 no.1
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• pp.33-62
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• 2009

Internet computing is a disruptive IT innovation. Semantic Web can be considered as an IT innovation because the Semantic Web technology possesses the potential to reduce information overload and enable semantic integration, using capabilities such as semantics and machine-processability. How should organizations adopt the Semantic Web? What factors affect the adoption and diffusion of Semantic Web innovation? Most studies on adoption and diffusion of innovation use empirical analysis as a quantitative research methodology in the post-implementation stage. There is criticism that the positivist requiring theoretical rigor can sacrifice relevance to practice. Rapid advances in technology require studies relevant to practice. In particular, it is realistically impossible to conduct quantitative approach for factors affecting adoption of the Semantic Web because the Semantic Web is in its infancy. However, in an early stage of introduction of the Semantic Web, it is necessary to give a model and some guidelines and for adoption and diffusion of the technology innovation to practitioners and researchers. Thus, the purpose of this study is to present a model of adoption and diffusion of the Semantic Web and to offer propositions as guidelines for successful adoption through a qualitative research method including multiple case studies and in-depth interviews. The researcher conducted interviews with 15 people based on face-to face and 2 interviews by telephone and e-mail to collect data to saturate the categories. Nine interviews including 2 telephone interviews were from nine user organizations adopting the technology innovation and the others were from three supply organizations. Semi-structured interviews were used to collect data. The interviews were recorded on digital voice recorder memory and subsequently transcribed verbatim. 196 pages of transcripts were obtained from about 12 hours interviews. Triangulation of evidence was achieved by examining each organization website and various documents, such as brochures and white papers. The researcher read the transcripts several times and underlined core words, phrases, or sentences. Then, data analysis used the procedure of open coding, in which the researcher forms initial categories of information about the phenomenon being studied by segmenting information. QSR NVivo version 8.0 was used to categorize sentences including similar concepts. 47 categories derived from interview data were grouped into 21 categories from which six factors were named. Five factors affecting adoption of the Semantic Web were identified. The first factor is demand pull including requirements for improving search and integration services of the existing systems and for creating new services. Second, environmental conduciveness, reference models, uncertainty, technology maturity, potential business value, government sponsorship programs, promising prospects for technology demand, complexity and trialability affect the adoption of the Semantic Web from the perspective of technology push. Third, absorptive capacity is an important role of the adoption. Fourth, suppler's competence includes communication with and training for users, and absorptive capacity of supply organization. Fifth, over-expectance which results in the gap between user's expectation level and perceived benefits has a negative impact on the adoption of the Semantic Web. Finally, the factor including critical mass of ontology, budget. visible effects is identified as a determinant affecting routinization and infusion. The researcher suggested a model of adoption and diffusion of the Semantic Web, representing relationships between six factors and adoption/diffusion as dependent variables. Six propositions are derived from the adoption/diffusion model to offer some guidelines to practitioners and a research model to further studies. Proposition 1 : Demand pull has an influence on the adoption of the Semantic Web. Proposition 1-1 : The stronger the degree of requirements for improving existing services, the more successfully the Semantic Web is adopted. Proposition 1-2 : The stronger the degree of requirements for new services, the more successfully the Semantic Web is adopted. Proposition 2 : Technology push has an influence on the adoption of the Semantic Web. Proposition 2-1 : From the perceptive of user organizations, the technology push forces such as environmental conduciveness, reference models, potential business value, and government sponsorship programs have a positive impact on the adoption of the Semantic Web while uncertainty and lower technology maturity have a negative impact on its adoption. Proposition 2-2 : From the perceptive of suppliers, the technology push forces such as environmental conduciveness, reference models, potential business value, government sponsorship programs, and promising prospects for technology demand have a positive impact on the adoption of the Semantic Web while uncertainty, lower technology maturity, complexity and lower trialability have a negative impact on its adoption. Proposition 3 : The absorptive capacities such as organizational formal support systems, officer's or manager's competency analyzing technology characteristics, their passion or willingness, and top management support are positively associated with successful adoption of the Semantic Web innovation from the perceptive of user organizations. Proposition 4 : Supplier's competence has a positive impact on the absorptive capacities of user organizations and technology push forces. Proposition 5 : The greater the gap of expectation between users and suppliers, the later the Semantic Web is adopted. Proposition 6 : The post-adoption activities such as budget allocation, reaching critical mass, and sharing ontology to offer sustainable services are positively associated with successful routinization and infusion of the Semantic Web innovation from the perceptive of user organizations.