• Journal of the korean academy of Pediatric Dentistry
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• v.30 no.3
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• pp.341-347
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• 2003

Smith-Magenis syndrome (SMS) is a clinically recognizable multiple congenital anomaly and mental retardation syndrome caused by an interstitial deletion of chromosome 17 p11.2. Physical features include short stature, characteristic facial appearance: flattened mid-face, down-turned mouth, prominent and often rosy cheeks; prominent jaw in older children and adults, chronic ear infections, hearing impairment, eye problems, including: strabismus (an eye which turns in or out) and myopia (nearsightedness), hoarse voice, short fingers and toes, heart defects or murmurs, problems related to the urinary system, scoliosis (curvature of the spine), an unusual gait (walking pattern), and decreased sensitivity to pain. Behavioral and developmental characteristics include speech delay and articulation problems, developmental delay, learning disability, mental retardation, hyperactivity, self-injury, including: head banging; hand biting; picking at skin, sores and nails; pulling off finger- and toenails; inserting foreign objects into ears, nose, or other body orifices, explosive outbursts, prolonged tantrums, destructive and aggressive behavior, excitability, arm hugging or hand squeezing when excited. This report is the case of a Korean 3-year-3-month old male with Smith-Magenis syndrome referred from local clinic for the treatment of dental caries. The patient was treated by physical restraint after prophylatic administration of antibiotic(Amoxacillin 50mg/kg).

• The Journal of the Korea Contents Association
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• v.17 no.7
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• pp.696-706
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• 2017

This study is to examine health care utilization and morbidity of disabled and non-disabled children in Korea to evaluate the health disparities. We used medical claim data of 2010 from the National Health Insurance Service-National Sample Cohort data. As a result of the analysis, the disabled children are not in good health condition because they have more frequency of medical service use, hospitalization rate, and more number of diseases and spent more on medical expenses than non-disabled children. Patterns of the most frequent disease differ from significantly between disable and non-disabled. Disabled children had a higher prevalence of selected birth defects and selected health conditions associated with physical disability and a lower prevalence of selected infection disease than children without disability. In conclusion, Health policy changes that would extend the access to health service for children with disabilities characterized by frequent medical care, hospitalization, excessive medical expenditure and complex diseases.

• The korean journal of orthodontics
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• v.31 no.1 s.84
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• pp.63-69
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• 2001

Cleft lip and/or palate(CLP) is the most common congenital deformity in the craniofacial region. For a practical public health system for these patients it is necessary to have an understanding on the prevalence of CLP. However, it was difficult to estimate the exact number of CLP patients due to problems in sample selection, higher miscarriage and still birth rate, difficulty in classification, and adoptions to foreign countries. Therefore this study was to estimate the prevalence of CLP and the rate of orthodontic treatment, which is usually necessary in cleft lip and/or palate patients. The samples consisted of 218,322 Korean male adults from Seoul, Kwangju, Taegu and Pusan, all born in 1979. The screening method for recognizing the CLP patients was proceeded in steps and the results are as follows. 1. The prevalence of cleft lip and/or palate in Korean male adults born in 1979 was 0.65 out of the 1000 samples. 2. In the anteroposterior aspect of the 1000 samples, the prevalence of cleft lip, cleft lip and palate, and cleft palate was 0.26, 0.36 and 0.03 respectively. 3. In the transverse aspect of the 1000 samples, the prevalence of left, right and bilateral cleft was 0.35, 0.16, 0.12 respectively. The cleft in the left showed a much higher prevalence than in the rirht, while bilateral cleft showed a lower prevalence than unilateral cleft. 4. The orthodontic treatment rate of Korean male adults among cleft lip and/or palate Patients was $35\%$, and it was in the order of cleft lip and Palate, cleft lip, and cleft Palate, being $67\%,\;29\%\;and\;29\%$ respectively. The orthodontic treatment rate in patients with the more severe cleft lip and palate was higher than in patients solely having cleft lip or cleft palate.

• Journal of Chest Surgery
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• v.41 no.3
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• pp.305-312
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• 2008

Background: Anatomic and functional abnormalities of the systemic atrioventricular (AV) valve are common in single ventricle. pathologies and continue to be associated with poor early and late outcomes in surgically palliated single. ventricle patients. We aggressively performed valvuloplasty for atrioventricular valve regurgitation (AVVR) during the course toward a Fontan operation. Material and Method: Between January 1995 and December 2004, 209 patients underwent a Fontan operation in our institution. We retrospectively evaluated the prevalence of AVVR and the influence of AV valve repair on outcome, and we analyzed the progression of AVVR after the Fontan operation for 168 patients where echocardiographic follow up results for more than 6 months after the Fontan operation were available. During the course toward a Fontan operation, 25 patients underwent 30 procedures for AVVR. These procedures. were. carried out during placement of a bidirectional cavopulmonary shunt (BCPS) for nine patients, between the time of placement of a BCPS and the Fontan operation for four patients, and during the Fontan operation for 17 patients. Five patients underwent procedures for AVVR twice. Result: The late mortality rate after the Fontan operation was 4.2% (n=7), with a median follow-up duration of 52 months (range, $6{\sim}123$ months). Seven patients (4%) had unfavorable outcomes such as significant (moderate or severe) AVVR in six patients, and significant AV valve stenosis in one patient was determined at the last follow up after the Fontan operation. Among the seven patients, four patients underwent AV valve repair after the Fontan operation, and one patient underwent subsequent AV valve replacement. Progression to AVVR of equal to or greater than grade 2 was noted in 30 patients (18%) at the last follow up after the Fontan operation, including 12 patients that underwent previous AV valve procedures. Initial grading of AVVR, a previous AV valve operation, and specific AV valve morphology such as a common AV valve or mitral atresia were significant risk factors for the progression of AVVR after the Fontan operation. Conclusion: In our surgical series, a small percentage of patients showed unfavorable outcomes. related to AVVR during the course toward a Fontan operation. However, a closer follow-up is required to evaluate the progression of the AVVR after a Fontan operation, especially for patients showing poor AV valve function at the first presentation and specific AV valve morphology.

• Clinical and Experimental Pediatrics
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• v.52 no.10
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• pp.1140-1146
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• 2009

Purpose:The pathophysiology of magnesium, the second highest common compound in humans, is still unclear, especially in preterm babies. We accessed the association between total magnesium (tMg), ionized Mg (iMg), and gestational age (GA) and that between serum magnesium (sMg) and intraventricular hemorrhage (IVH) in preterm babies. Methods:In all, 119 inborn preterm infants admitted between July 2006 and February 2008 were divided into the IVH group (19) and the control group (100) and were prospectively analyzed. TMg, iMg, pH, total calcium (tCa), and ionized Ca (iCa) levels were determined immediately after delivery or within 3 hours after birth, and their correlation with GA were investigated. Results:TMg was not correlated with GA, tCa, iCa, and pH. IMg was correlated with tMg (r=0.288, P=0.002) and iCa (r=0.212, P=0.021); however, it was not correlated with GA and pH. Mean GA and birth weight were significantly lower (P=0.002) and smaller (P=0.030) in the IVH group. Mean sMg was higher in the IVH group ($2.5{\pm}0.9mg/dL$) than in the control group ($2.1{\pm}0.6mg/dL$) (P=0.021). SMg was a risk factor even after logistic regression analysis (OR, 2.798; 95% C.I., 1.265-6.192; P=0.011). Conclusion:In less than 37-week-old preterm babies, tMg and iMg were similar, regardless of GA. High sMg may be a risk factor for IVH in premature babies, regardless of their exposure to antenatal magnesium.

• Clinical and Experimental Pediatrics
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• v.50 no.11
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• pp.1061-1066
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• 2007

Purpose : Early identification of developmental disabilities allows intervention at the earliest possible point to improve the developmental potential. The Ages and Stages Questionnaire (ASQ), a parent- completed questionnaire, can be used as a substitute for formal screening tests. The purpose of this study was to evaluate the validity of the Korean version of the ASQ (K-ASQ) as a screening tool for detecting developmental delay of young Korean children in the setting of a busy pediatric outpatient clinic. Methods : Parents completed the K-ASQ in the waiting room of the pediatric outpatient clinic of St. Mary's Hospital, Catholic University Medical College. Out of 150 completed the ASQ, 67 who were born term and had no previous diagnosis of developmental delay, congenital anomalies, or neurological abnormalities were enrolled. The cut-off values of less than 2 standard deviations (SD) below the mean for the ASQ were used to define a "fail", and children who failed in one or more domains tested were classified as "screen-positive". Diagnosis of developmental delay was made when the developmental indices fell below -1 SD of the Bayley Scales of Infant Development-II. Results : (1) The mean age of children was $16.4{\pm}7.4$ months. Ten children (14.9%) were small-for- gestational age infants. The mean birth weight and gestational age were $3.1{\pm}0.6kg$ and $38.8{\pm}1.4$ weeks. Nine children (13.4%) were twins and 33 (49.0%) were male. The mean maternal education in years was $13.6{\pm}2.4$, and 31.3% had full-time jobs. The time for completing the ASQ was $10.2{\pm}3.0$ minutes. (2) Seventeen children (25.4%) were classified as screen-positive, four of them were delayed in development. Among eight children diagnosed with developmental delay, four were screen-positive and the other four were screen-negative by the ASQ. (3) The test characteristics of the ASQ were as follows: sensitivity (50.0%); specificity (78.0%); positive predictive value (23.5%); negative predictive value (92.0%). Conclusion : The high negative predictive value of the K-ASQ supports its use as a screening tool for developmental delay in the setting of a pediatric outpatient clinic.

• Journal of Chest Surgery
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• v.42 no.5
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• pp.597-603
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• 2009

Background: Atrioventricular valve regurgitation in pediatric patients with a functional single ventricles (FSV) - has been known as one of the important risk factors for death and unfavorable long-term results after a Fontan operation. We evaluated early and mid-term results of bivalvation valvuloplasty in FSV patients. Material and Method: We retrospectively evaluated 11 patients with a functional single ventricle who underwent bivalvationvalvuloplasty between 1999 and 2007. The degree of common atrioventricular valve regurgitation (CAVVR) was determined by color Doppler echocardiography (regurgitation grade scoring, trivial; 1, mild; 2, moderate; 3, severe; 4). Mean age at valve surgery was $6.9{\pm}7.0$ months (median 4 months, 24 days$\sim$21 months)and mean body weight was $6.2{\pm}2.8\;kg$ ($3.1{\sim}11.3\;kg$). Nine patients had isomerism heart and two of them had TAPVC. The concomitant procedures were performed in all but one patient. Additional commissural closure was performed in 3 patients and commissural annuloplasty in another 3 patients. Result: There was one hospital death after. surgery. A 32-day old patient who had been preoperatively dependent on a ventilator died of air way and lung problems 4.3 months after pulmonary artery banding and bivalvation valvuloplasty. Mean follow-up duration was 40 months ($4.3{\sim}114$ months). Mean preoperative CAVVR score was $3.3{\pm}0.6$, which decreased to $1.9{\pm}0.7$ postoperatively (p<0.0001). This residual regurgitation slightly increased to $2.2{\pm}0.4$ (no statistical significance) after a mean follow-up of 1.4.3 months. Six patients (60%) required re-operations for residual regurgitation at a subsequent bidirectionalcavopulmonary shunt or Fontan operation. One patient with Ebsteinoid malformation of the right sided atrioventricular valve required valve replacement due to stenoinsufficiency. Another patient required edge-to-edge repair at the right sided AV valve (between the right mural leaflet and the bridging leaflets). The remaining 4 patients required additional suture placements between bridging leaflets with or without commissural annuloplasty. All survivor had trivial or mild CAVVR at the latest follow-up. Conclusion: Bivalvation valvuloplasty for CAVVR in FSV patients is. an effective and safe procedure. However, significant numbers of the patients have small residual regurgitation and require additional valve procedures at subsequent operations. Long-term observations to monitor progression of the CAVVR is mandatory.

• Neonatal Medicine
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• v.15 no.2
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• pp.142-151
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• 2008

Purpose : The aim of this study was to compare the clinical effects of modified porcine (Curosurf$^{(R)}$) and bovine (Newfactan$^{(R)}$) surfactants in the treatment of neonatal respiratory distress syndrome. Methods : Between April 2004 and December 2006, 65 neonates (birth weight ${\leq}$2,500 g and gestational age ${\leq}$35 weeks) with neonatal respiratory distress syndrome were treated in our neonatal intensive care unit with surfactant. Thirty-one neonates received Curosurf$^{(R)}$ and 34 neonates received Newfactan$^{(R)}$. The neonates were not enrolled if they had major congenital anomalies or meconium aspiration syndrome. We compared the changes in respiratory parameters after surfactant instillation, the incidences of acute and chronic complications, and the mortality between the two treatment groups. Results : Neonatal and maternal demographic characteristics were not different between the groups. The patterns of change in the respiratory parameters after surfactant instillation were not statistically different between the groups. The incidences of surfactant reinstillation and acute complications, such as pneumothorax, patent ductus arteriosus, pulmonary hemorrhage, and grade 3-4 intraventricular hemorrhage, were not different between the neonates who received Curosurf$^{(R)}$ and the neonates who received Newfactan$^{(R)}$. There were no statistically significant differences in the duration of mechanical ventilation, oxygen therapy, hospitalization, prevalence of bronchopulmonary dysplasia, periventricular leukomalacia, retinopathy of prematurity, necrotizing enterocolitis, and mortality between the groups. Conclusion : In the present comparative study, no significant differences in the clinical effects of Curosurf$^{(R)}$ and Newfactan$^{(R)}$ were observed.

• Journal of Chest Surgery
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• v.32 no.4
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• pp.341-346
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• 1999

Background: Thirty children ranging from 3 to 15 years of age underwent cardiac valve replacement at Dongsan Medical Center from 1982 to 1997. Material and Method: There were 16 boys and 14 girls. The mean age was 12.1. The underlying pathological cause for valve replacement was congenital heart disease in 17 children and acquired heart disease in 13. The valve replaced was mitral in 15 children, aortic in 11, tricuspid in 3, and combined aortic and mitral in 1. Twenty-one mechanical and 10 tissue valves were placed: primary mechanical valve have been utilized since 1985. Eight of ten patients with tissue valves have had successful second valve replacements 4 to 11 years after the initial operation. Result: The operative mortality was 6.7%, but mortality was higher among patients less than 5 years of age and patients who had previous cardiac operations. Of the 28 operative survivors, 4 patients were lost to follow-up: the remaining patients were observed for a total of 2091 patient/months(mean 74.7 months, maximum 187 months). There was one late death from dilated cardiomyopathy after mitral valve replacement in 7 year-old patient with atrioventricular septal defect. After the operation, all patients with mechanical valves were placed on a strict anticoagulant regimen with Coumadin. The actuarial survival rate was 96% at the end of the follow-up. No instance of thromboembolism or major bleeding were observed in the survivors. Conclusion: These results indicate that valve replacement can be performed with low mortality in children, and with satisfactory long-term survival.

• Clinical and Experimental Pediatrics
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• v.45 no.12
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• pp.1512-1518
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• 2002

Purpose : Twins have a higher mortality and morbidity than singletons. Co-twin with one fetal death is particularly at risk. We investigated the neonatal outcome of live co-twins when one fetus had died after the 20th gestational week, and associated risk factors. Methods : A retrospective study was performed in fifteen cases of twin pregnancy with single intrauterine fetal deaths after the 20th gestational week during the period from January 1996 to December 2000 at Chonnam University Hospital. Results : Gestational age was $33.7{\pm}3.2weeks$, birth weight was $1,992{\pm}592g$. Interval between one fetal death being detected and the delivery of a live co-twin was $32.4{\pm}29.5days$. There were 11 cases(73.3%) of premature babies less than 37 gestational weeks. Main causes of preterm delivery were preterm labor and premature rupture of membranes. Hematologic findings suggesting disseminated intravascular coagulopathy(DIC) were not found in all mothers before delivery, and was not associated with DIC and encephalomalacia of the live co-twin. Perinatal outcome of fifteen live co-twins was as follows : six were normal(40%), three were DIC(20.0%), three were encephalomalacia(20.0%), one suffered intrauterine growth retardation, there was one case of twin to twin transfusion syndrome, and one of congenital heart disease(atrial septal defect with pulmonary stenosis). The occurrence of DIC and encephalomalacia in live co-twins was not related to placental chorionicity, birth weight, gestational week, and the interval between the detection one fetal death and the delivery of a live co-twin. Conclusion : We could not find any maternal hematologic problems in twin pregnancies complicated by one fetal death. Twenty percent of live co-twins showed DIC and encephalomalacia. However, its associated risk factors were not found. We need to investigate more closely the cases of live co-twins with one intrauterine fetal death.