• Journal of Chest Surgery
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• v.42 no.1
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• pp.87-91
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• 2009

The surgical management of complete transposition of the great arteries, ventricular septal defect, and pulmonary stenosis still remain a significant challenge. The Rastelli (REV procedure) remains the most widely applied procedure for surgical repair of these lesions. Although the Rastelli procedure can be performed with good early results, the intermediate- and long-term results have been less than satisfactory because of deterioration of the hemodynamic performance of the LVOT or RVOT. We performed a modified Nikaidoh procedure as an alternative surgical procedure in a 19-month-old boy weighing 10.4 kg with this anomaly. Aortic translocation with biventricular outflow tract reconstruction resulted in a more "normal" anatomic repair and postoperative echocardiography showed straight, direct, and unobstructed ventricular outflow.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.14 no.12
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• pp.6413-6419
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• 2013

This study examined the irradiation effect of high energy x-rays on the hatching process of fertilized eggs, particularly with regard to malformation and blood cells change. The experimental groups were five day old fertilized eggs irradiated with x rays at doses of 5, 7.5, 10 Gyusing alinear accelerator. The control group was not irradiated. After three weeks, hatched chicks were sacrificed and examined for blood sampling. The survival rate of the x-ray irradiated groups were significantly lower than that of the control group (46.7vs 80%). The malformation rate of the experimental groups was60%, whereas no congenital malformations were observed in the control group. The experimental groups had a significantly higher malformation rate. The types of malformation were left wing defect, proptosis, microcephaly, cervical spine, and feet anomaly. The incidence of malformation increased with increasing radiation dose. The white blood cell count of control group and eachexperimental groups (5 and 7.5 Gy) were 87.64, 100.76 and 81.42 ($10^3/{\mu}L$), respectively. X-ray irradiation of 5 day old chick embryos increased the rate of death and malformation significantly.The malformations were estimated to have occurred by chromosomal abnormalities. Further genetic studies will be needed to confirm the correlation between high energy X-rays and the cause of malformations.

• Tuberculosis and Respiratory Diseases
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• v.71 no.2
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• pp.126-133
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• 2011

Unilateral pulmonary artery hypoplasia (UPAH) is a rare disease in adults and is frequently accompanied by a congenital cardiac anomaly at a young age. The diagnosis is usually based on computed tomography (CT), angiography, and magnetic resonance imaging (MRI). However, no reports are available on retrograde flow in patients with UPAH. We describe a 68-year-old man with isolated UPAH and retrograde blood flow. He was admitted for dyspnea on exertion for the past 23 years. His diagnosis was delayed, as his symptoms and signs mimicked his underlying pulmonary diseases, such as emphysema and previous tuberculous pleurisy sequelae. A discrepancy was detected between the results of a ventilation-perfusion scan and the CT image. This was resolved by MRI, which showed retrograde blood flow from the right to the left pulmonary artery. Using MRI, we diagnosed this patient with isolated pulmonary artery hypoplasia and retrograde flow.

• Tuberculosis and Respiratory Diseases
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• v.65 no.2
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• pp.131-136
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• 2008

Cholethorax (bilious pleural effusion) is an extravasation of bile into the thoracic cavity via a pleurobiliary fistula (and also a bronchobiliary fistula). It is an extremely rare complication of thoraco-abdominal injuries. It can be caused by congenital anomaly and also by hepatobiliary trauma, severe infection or iatrogenic procedures. The definitive diagnosis is made with aspiration of bilious fluid from the pleural space during thoracentesis, by finding a fistulous tract during endoscopic retrograde cholangiopancreatography (ERCP) or cholagioscopy, or with finding an abnormal pleural accumulation of radioisotope during hepatobiliary nuclear imaging. Its symptoms include coughing, fever, dyspnea and pleuritc chest pain. Herein we report on a case of cholethorax following performance of percutaneous transhepatic cholangioscopy (PTCS) to remove incidentally discovered common bile duct (CBD) stones.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.23 no.3
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• pp.263-269
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• 2001

Median cleft of the lower lip and/or mandible is a rare congenital anomaly, first mentioned by Couronne in 1819. Monroe(1966), Fujino(1970), Ranta(1984) and Oostrom(1996) conducted comprehensive reviews and list cases in literature. Median cleft varies greatly, from a simple vermilion notch to a complete cleft of the lip involving the tongue, the chin, the mandible, the supporting structures of the median of the neck, and the manubrium sterni. The associated anomalies include ankyloglossia, cleft tongue, neck contraction, heart lesion, absence of hyoid bone, and so on. The etiology of median cleft is unknown. Various possibilities, such as failure of mesodermal penetration into the midline, failure of fusion of mandibular processes, external factors apart from the embryogenic pattern such as pressure, position in utero, circulatory failure caused placental adhesion, diseases in pregnancy, and so on, have been discussed. A 8-year-old girl was referred to the Dept. of Oral & Maxillofacial Surgery, Kyungpook National University Hospital and had been aware of the fact that at birth "she had something wrong with her mouth." Shortly after birth she had been examined by a plastic surgeon and at that time surgical procedure had been performed to release the tongue from the lower jaw and lip at local hospital. On admission, she had a slight notching of lower lip and two fibrous frenum ran from the lip along the ventral surface of the tongue, diastema between her mandibular central incisors, and slightly constricted bifid mandible associated independent movement of the two halves of mandible. The patient had autogenous iliac bone graft to reconstruct the mandibular midline defect. The postoperative result was uneventful. In future, the correction of the soft tissue deformities such as notching of the lower lip and partial ankyloglossia will be required for the esthetic and functional improvement.

• Journal of Genetic Medicine
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• v.5 no.2
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• pp.125-130
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• 2008

Propose: To analyze the indications and cytogenetic results of midtrimester amniocentesis. Material and Methods: This study reviewed 2,523 cases of midtrimester prenatal genetic amniocentesis performed at MizMedi Hospital between January 2000 and December 2007. Results: The most frequent indication for midtrimester amniocentesis was advanced maternal age (45.9%), followed by positive serum markers (29.9%). Chromosomal aberrations were diagnosed in 110 cases (4.4%), for which numerical aberration accounted for 38 cases (34.5%), structural aberration accounted for 65 cases (59.1%), and mosaicism accounted for 7 cases (6.4%). Among the autosomal aberrations, there were 20 cases of trisomy 21 and 8 cases of trisomy 18. With respect to structural aberrations, there were 14 cases of reciprocal translocation and 8 cases of robertsonian translocation. The frequencies of chromosomal aberrations according to the indication were highest in individuals with a family history of chromosome abnormality 14.0% (8/57) followed by previous congenital anomaly 5.9% (2/34). Conclusion: Midtrimester amniocentesis is an effective tool for prenatal diagnosis. Indications such as advanced maternal age, maternal serum markers, and ultrasound are important for predicting abnormal fetal karyotypes.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.8 no.2
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• pp.137-142
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• 2005

Purpose: To evaluate the safety, efficacy and technical problems of the endoscopic balloon dilatation of esophageal anomalies in children. Methods: The medical records of 8 children treated by endoscopic balloon dilatation for esophageal anomalies over a 10-year period at Pusan National University Hospital were reviewed retrospectively. The balloon catheter (Maxforce TTS or CRE, Boston Scientific Co., USA) was positioned across the area of narrowing by direct visualization. The balloon was slowly inflated with normal saline to specified pressures for each balloon and maintained for 60 seconds and then deflated. After 60 seconds pause, the procedure was repeated with a larger sized balloon (increments of 1 mm for each subsequent dilation) till effective dilatation was confirmed by direct visualization without complications. Results: Three male and five female were included and their mean age was 4.2 years. A total of 27 (average of 3.2 per patient) dilatation were performed. Underlying diseases of patients are postoperative stricture of esophageal atresia in 3 cases, esophageal ring in 2 cases, achalasia, corrosive esophagitis and hypertensive LES in one case respectively. The size of initial dilating balloon was chosen on the basis of the diameter of the narrowing determined by endoscopy. The first dilation in patients with severe esophageal stricture was made with a 6 mm sized balloon. Complications observed were esophageal perforation and respiratory holding during the procedure in one case respectively. Successful outcome was seen in 6 patients (75%). Conclusion: Endoscopic balloon dilatation can provide a safe and effective mean of treating esophageal anomalies in children and should be considered the treatment of choice in the initial management of those cases.

• Journal of Chest Surgery
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• v.34 no.9
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• pp.680-685
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• 2001

Background: Tricuspid valve replacement is very rarely performed procedure and its long-term result is not yet satisfactory. Moreover, it is not well known whether bioprosthesis or mechanical prosthesis is the best selection for artificial valve. We reviewed 72 cases of tricuspid valve replacements in 71 patients between January 1989 and December 1998, trying to analyze the overall results and risk factors for mortality and morbidity. Material and Method: Average age of the patients at the time of operation was 42$\pm$13 years(range 16 to 65 years) and the sex ratio of male versus female was 32/39. Primary diagnosis consisted of 50 cases of aquired valvular heart disease and 18 cases of congenital heart disease, such as Ebstein’s anomaly. 4 cases had isolated tricuspid valve regurgitation. Implanted valves were 69 mechanical prosthesis and 3 bioprosthesis. Concomitant mitral or aortic valve replacements were performed in 50 cases. One patient received concomittant pulmonary valve replacement. Result: There were 7(9.72%) operative deaths and 7(13.0%) late deaths. Actuarial survival at 10 years was 59.2$\pm$7.2%. Prosthetic tricuspid valve thrombosis occurred 11 times in 5 patients. Reoperation for prosthetic tricuspid valve failure was performed in 1 patient. In this case, examination of the explanted prostheses showed that the tricuspid stenosis was the result of valve thrombosis. Among the 47 survivors, 46 patients(98%) were in functional class I or II. Conclusion: In our ten-year experience of tricuspid valve replacement, mortality and morbidity were satisfactory. Mechanical prosthesis in tricuspid position showed comparable clinical results as bioprosthesis.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.4
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• pp.568-574
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• 2009

Tongue-tie is a congenital anomaly characterized by an abnormally short lingual frenum, which may restrict tongue tip mobility, In the neonate and infant, tongue-tie was said to cause difficult breast-feeding, In the older infant, toddler and young child, poor speech has been frequently listed because movement of tongue tip is limit ed. Also as a abnormal tongue position, various orthodontic problems, later mechanical and social manifestations could be developed. Treatment options such as observation, speech therapy, frenotomy and frenectomy have been suggested. The optimal timing for the surgery has not been determined, but early intervention may be appropriate for the children with significant tongue-tie who has the significant potential to speech difficulties and later social and mechanical problems. Sedation is an effective method for incapable of cooperative and the handicapped children, necessary to early intervention. We report three cases, using oral sedation for the frenectomy in young children with severe tongue-tie. After treatments, we could find out sufficient tongue movement and improved speech ability.

• Journal of Chest Surgery
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• v.19 no.4
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• pp.569-577
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• 1986

Between October 1979 and June 1986, modified Fontan procedures have been performed on 22 patients by the Department of Thoracic and Cardiovascular Surgery, Yonsei University College of Medicine. Twelve patients had tricuspid atresia; one, congenital tricuspid stenosis; five, single ventricle; two, I-transposition of the great vessels; one, double outlet right ventricle, and one, pulmonary atresia with an intact ventricular septum. There were 9 operative deaths [mortality; 40.9%]. The causes of death were right heart failure in six patients and pulmonary venous hypertension in one who misdiagnosed preoperatively. Another two were deceased due to sepsis and cerebrovascular accident at postoperative 35 and 34 days in each. There were 7 patients below 4 years of age at the time of operation and among them 4 patients were deceased. The operative death was not related with patients` age above and below 4[p=0.211]. The relation between operative death of tricuspid anomaly and another cardiac malformations was statistically significant [p=0.048]. The operative procedures with or without valved conduit [woven dacron] was related significantly [p=0.043] in the case of the 21 of the patients, but the modified Fontan operation with a valved conduit was performed early stage in this series. Since 1982, we operated on 4 patients, doing a right atrium-right ventricle anastomosis without a conduit. All survived and remained in functional class I [NYHA]. The right atrial pressure [RAP] was elevated significantly after operation [mean 9.9$\pm$4.8 ~16.9$\pm$3.6 mmHg, p<0.001]. The relation between the postoperative RAP of the survival group [16.5$\pm$4.3 mmHg] and the group who died [17.4$\pm$2.2 mmHg] was statistically significant [p=0.047]. There was no relation between any operative death and any previous palliation. All patients were followed for 4 months to 80 months, except one who was lost to follow up at 2 months following surgery [mean 11.4 months, 238 patient. months]. All were in functional class I with 5 on medications and 7 not. One was reoperated at 70 months following the first operation, due to conduit stenosis. She was moderately impaired in activity, with hepatomegaly after the second operation.