• Journal of the korean academy of Pediatric Dentistry
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• v.25 no.3
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• pp.598-603
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• 1998

Amelogenesis imperfecta is a group of hereditary defects of enamel unassociated with any other generalized defects. The prevalence of this condition has been estimated to range from 1 in 14,000 to 1 in 16,000, depending on the population studied. It may be differentiated into three general types : hypoplastic, hypocalcified, and hypomaturation, depending on the clinical presentation of the defects and the likely stage of enamel formation that is primarily affected. The dentin and root form are usually normal, but the enamel may lack the normal prismatic structure, being laminated throughout its thickness or at the periphery, with the result that these teeth are more resistant to decay. This case is that of an six-year-old girl brought to the pediatric dentistry department by her parents for esthetic reasons and also because of slight dental sensitivity. Clinical and radiographic examinations confirmed amelogenesis imperfecta. The author has treated with the crowning of the primary molars, using prefomed NiCr crowns and periodic fluoride application on whole dentition.

• Journal of Chest Surgery
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• v.35 no.6
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• pp.475-478
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• 2002

A 45 year old man was admitted for aggravated dyspnea, abdominal distension and poor oral intake. On Echocardiogram, mitral stenosis(severe), tricuspid regurgitaion(IV), and LA thrombus were diagnosed. We used heparin with continuous infusion for prevention of systemic thrombo embolism. On the 11th day of admission, the patient showed thrombocytopenia and we suspected Heparin-induced thrombocytopenia. Hirudin was used in this case as alternative anticoagulant during cardiopulmonary bypass to prevent serious complication of heparin. The patient was recovered without any complication as postoperative bleeding or systemic thromboembolism.

• The korean journal of orthodontics
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• v.32 no.3 s.92
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• pp.175-183
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• 2002

Crouzon syndrome Is one of the craniofacial syndromes characterized by craniosynostosis, midfacial hypolpasia and ocular proptosis. Distraction osteogenesis is becoming Important technique to treat craniofacial dyplaisa. It has nuy advantages compared with standard orthognathic surgery. Maxillary distraction osteogenesis after Le Fort III osteotomy with the RED system presents successful maxillary protraction to Crouzon syndrome patient with severe maxillary deficiency. It also allows remarkable improvement of facial esthetics and respiratory functions.

• Journal of Chest Surgery
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• v.34 no.4
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• pp.329-337
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• 2001

배경: 승포판 전엽이나 교련 또는 여러 판엽에 걸쳐 광범위한 병변으로 인한 승모판 역류의 경우 수술수기에 대한 뚜렷한 정립이 되어 있지 않은 실정이다. 최근 이러한 병변의 승모판 역류의 경우 PTEE(Polytetrafluro-ethylene)을 이용한 신건삭 형성술의 시행이 점차 늘고 있다. 이에 저자들은 PTEE를 이용한 신건삭 형성술이 승모판 성형술에 어떠한 영향을 미치는지 알아보고자 하였다. 대상 및 방법: 1994년 1월부터 1999년 12월까지 승모판막 성형술을 시행 받은 322명의 환자 중에서 신건삭 형성술을 이용하여 승모판 성형술을 시행한 144례(I군)과 신건삭 형성술 이외의 방법으로 승모판 성형술을 시행한 178례(II군)를 대상으로 하였다. 두 군에서 수술 직후ㅘ 수술 후 6개월, 1년 및 1년 단위로 주기적인 심초음파를 시행하였고 이를 통계적 검정하였다. 결과: 같은 기간 승모판 성형술은 승모판막 폐쇄 부전증으로 내원한 환자의 95%(306)에서 추적 관찰이 가능하였다. 두 구난에 수술 전 혈류 역학적인 차이를 보이지 않았고, 수술 전 평균 승모판막 폐쇄 부전의 정도는 I군이 3.8$\pm$0.4, II군이 3.6$\pm$0.3였으나 수술 후 추적 관찰에서 각각 1.3$\pm$0.9와 1.1$\pm$0.7 정도의 양호한 판막 성형술의 결과를 보였고 승모판막 면적의변화나 승모판막에서의 평균 압력차이 등 혈류 역학적인 결과에도 차이를 보이고 있지 않았다. 술 후 3명(0.9%)의 조기 사망과 4명 (1.2%)의 후기 사망이 있었으나 두 군간의 차이는 보이지 않았다. 만기 생존율( 93.7$\pm$3.3 vs 88.0$\pm$1.95) 및 판막살패가 발생하기 않을 확률( 93.7$\pm$3.6 vs 93.3$\pm$3.4%) 과 재수술이 필요치 않을 활률(99.3$\pm$0.7 vs 96.0$\pm$1.9%), 색전증, 감염성 심내막염, 판막과 관련된 합병증 발생률 등에서 두 군간에 의미 있는 차이는 없었다. 전엽과 후엽에서 신건삭 형성술을 시행한 환자에서는 통계학적인 차이가 없었다(p=0.29) 결론: 신건삭 형성술을 이용한 승모판 성형술은 승모판 성형술의 증가(r=0.98, p<0.01)를 가져 왔을 뿐만 아니라 내구성과 안정성에 있어 기존의 안전화된 수술 수기와 견줄만하다. 특히 이러한 신건삭 형성술은 승모판 전엽에 병변이 있는 경우와 교련부 및 여러 엽에 광범위한 병변으로 인하여 재건술이 어려운 경우에도 승모판 재건을 가능하게 하는 우수한 수술 수리가 사료된다.

• Journal of Chest Surgery
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• v.40 no.8
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• pp.536-545
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• 2007

Background: Compared to adult patients, mitral regurgitation in pediatric patients is uncommon and it shows a wide spectrum of morphologic abnormalities. We retrospectively evaluated the midterm results of mitral valve repair in pediatric patients. Material and Method: Between December 1993 and August 2006, mitral valve repair was performed in 35 patients who were aged less than 18 years, The mean age was $5.3{\pm}5.3$ years and the mean body weight was $20,0{\pm}16.3\;kg$. 18 patients had associated cardiac anomalies. The most common pathologic finding was leaflet prolapse (n=17). The most common method of repair was the double orifice technique (n=15). Result: There was no early mortality. Eight patients underwent reoperation (24.2%), and five of them required mitral valve replacement. Among the four ring annuloplasty cases, two have developed mitral stenosis. Four out of the 14 double orifice cases required reoperation. One case of early mortality and one case of late mortality occurred in the reoperation cases. The 5-year survival rate and the freedom from reoperation rate were $93.3{\pm}4.6%$ and $76.1{\pm}8.2%$, respectively. The 5-year freedom from mitral valve replacement rate was $83.6{\pm}6.7%$. There was no significant risk factor for reoperation. Conclusion: The midterm results of mitral valve repair are very acceptable in pediatric patients compared to the adult cases, although the reoperation rate is slightly higher.

• Tuberculosis and Respiratory Diseases
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• v.46 no.1
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• pp.116-121
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• 1999

Unilateral hypoplasia of the pulmonary artery is an uncommon anomaly, which commonly develops in combination with congenital cardiovascular defects such as tetralogy of Fallot, patent ductus arteriosus and septal defect of atrium or ventricle, but may also present as an isolated lesion. We have recently experienced a case of the left pulmonary artery hypoplasia in adult by chance of during the general health screen, which diagnosed by chest X-ray, chest spiral CT, lung perfusion and ventilation scan, digital substraction angiogram and bronchoscopy, then presented hereby with the review of relevant literature.

• Tuberculosis and Respiratory Diseases
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• v.39 no.6
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• pp.548-553
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• 1992

The Unilateral absence of a pulmonary artery (UAPA) is an uncommon congenital anomaly. Approximately 160 cases have been reported in the literature since Frantzel's first report in 1968. Most of the patients with UAPA are asymptomatic but some patients may suffer from recurrent respiratory infections, hemoptysis, or pulmonary hypertension. The diagnosis could be suspected from the chest roentgenogram and lung scan, and definitely confirmed by pulmonary angiography. We experienced a case of UAPA in a 39-year-old male with the recurrent hemoptysis. Chest X-ray revealed that the left lung volume was moderately decreased and the heart and mediastinum were displaced to the left side. Lung perfusion scan showed that the left lung was not perfused. Pulmonary angiography revealed the absence of the left main pulmonary artery. Aortic arch and descending aorta on aortogram were right sided. Blood supply to the left lung was originated from numerous systemic collaterals from intercostal and brachiocephalic origin. No other intrinsic or internal abnormalities of the cardiac chambers were noted.

• Journal of Chest Surgery
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• v.32 no.6
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• pp.584-587
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• 1999

The DiGeorge syndrome is a rare congenital abnormality consisting of aplasia or hypoplasia of the thymus and parathyroid glands resulting from malformation of the third and fourth pharyngeal pouches. This syndrome usually includes congenital cardiac anomalies and abnormal facial features. We experienced a case of congenital cardiac anomaly associated with DiGeorge syndrome. The patient was 1 month old boy weighing 3.5 kg. The congenital cardiac anomalies included ventricular septal defect, atrial septal defect, coactation of aorta, and patent ductus arteriosus. We performed one-stage operation with two separate incisions for these cardiac anomalies. Postoperative course was uneventful and the patient at 6 months of follow up is doing well.

• Journal of Chest Surgery
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• v.29 no.8
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• pp.828-835
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• 1996

From January 1993 to April 1995, 27 neonates (under age of 30 days underwent open heart surgery in the Department of Thoracic and Cardiovascular Surgery, Dong-A Medical Center. Mean age and weight were 12.1 days(2days∼306ays) and 3.29 kg(2.6kg∼4.1 kg) respectively. Cardiac anomalies were simple complete transposition of great arteries(TGA) in 11 neonates, TGA with coarctation of aorta(COA) in 1 , total anomalous pulmonary venous connection(TAPVC) in 5, double inlet right ventricle with TAPVC in 1, interrupted aortic arch(IAA) with ventricular septal defect(VSD) in 3, pulmonary atresia(PA) with intact ventricular septum(IVS) in 3, pulmonary stenosis with IVS in 1, Taussig-Bing anomaly with IAA in 1, and hypoplastic left heart syndrome(HLHS) in 1 . Postoperative complications were myocardial and/or pulmonary edema which caused open sternum in 13 patients(54.2%), acute renal failure( RF) in 10(37.0%), Intractable low cardiac output syndrome (LCOS) including weaning failure from cardiopulmonary bypass in 7(25.9%), bronchopulmonary dysplasia in 1, wound infection in 1, and paroxysmal supraventricular tachycardia in 1. Nine of 13 patients with postoperative open sternum were recovered with delayed sternal closure, and seven of 10 patients survived postoperative ARF with peritoneal dialysis. There were 8 operative deaths(29.6%): 3 in the patients with simple complete TGA, 1 In TCA with COA, 1 in PA with IVS, 1 in Taussig-Bing anomaly with IAA, 1 in DIRV with TAPVC, and 1 in HLHS. One late death occurred after arterial switch operation in simple TGA. The mosts common cause of death was low cardiac output syndrome. Our initial experience of open heart surgery in neonates showed high operative mortality and morbidity, especially in complex anomalies.

• Clinical and Experimental Pediatrics
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• v.45 no.5
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• pp.596-602
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• 2002

Purpose : The purpose of this study was to analyze the association of peripheral neutrophil count with the development of respiratory failure in preterm infants. Methods : A retrospective study was conducted from January 1993 to December 1999 on 44 preterm infants, who were admitted to the neonatal intensive care unit of St. Francisco hospital. Preterm infants(birth weight 500 to 1,350 gm) who had a complete blood count obtained within 2 hours after delivery. Patients in the lowest of neutrophil count(early neutropenia, < $1.0{\times}10^9/L$) were compared with patients in the remaining group. Results : Low neutrophil count were transient in early neutropenia group. The concentration the circulating neutrophil count rose from $0.85{\pm}0.11{\times}10^9/L$ at average of 2 hours after delivery to $5.3{\pm}2.7{\times}10^9/L$ at 24 hours after delivery in the early neutropenia group and from $3.6{\pm}1.6{\times}10^9/L$ to $5.8{\pm}3.2{\times}10^9/L$ in the non-neutropenia group during the same time period. Compare to the non-neutropenia group, the neutropenia group had a lower birth weight($1,046.50{\pm}180.76gm$ Vs $1,156.70{\pm}124.99gm$), a lower Apgar score(1 min : $3.41{\pm}1.18$ Vs $4.30{\pm}1.46$, 5 min : $5.41{\pm}0.87$ Vs $6.15{\pm}0.95$), and a higher incidence of bronchopulmonary dysplasia(27.27% Vs 7.0%). Patients who had early neutropenia were more likely to require mechanical ventilation, supplemental oxygen and hospital stay. Also, main effect factors for the two groups were birth weight(Odds ratio=5.457, 95% CI=1.551-27.525), initial peripheral blood white cells(odds ratio=8.308, 95% CI=2.054-52.699), and bronchopulmonary dysplasia(odds ratio=0.099, 95% CI=0.017-0.397). Conclusion : A low count of neutrophil in the systemic circulation of premature infants within 2 hours of birth is associated with more severe respiratory distress.