• Journal of Chest Surgery
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• v.18 no.4
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• pp.589-597
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• 1985

Congenitally corrected transposition of great arteries is a rare congenital heart anomaly. We experienced two case of corrected transposition of great arteries, one [S,L,L] and one [I, D,D] associated with patent foramen ovale, ventricular septal defect and pulmonary stenosis. The patent foramen ovales were closed directly under right atriotomy, the ventricular septal defects were closed with Dacron patch under morphological left ventriculotomy and the pulmonary valvular and subvalvular stenosis were corrected under pulmonary arteriotomy. The postoperatively course was uneventful in case I, the permanent pacemaker was implanted in case ll.

• Journal of Microbiology and Biotechnology
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• v.27 no.5
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• pp.1005-1009
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• 2017

Primary cytomegalovirus (CMV) infection during pregnancy can cause congenital defects. Available data for CMV infection during pregnancy in north China are inadequate. The aim of this study was to evaluate the epidemiology of maternal CMV infection and explore the incidence of congenital infection. In this prospective study, serum CMV IgG and IgM antibodies were measured in 2,887 pregnant women using ELISA, and the IgG avidity test was performed on all IgM-positive subjects. The seroprevalence of anti-CMV IgG was 94.70%, and of anti-CMV IgM was 1.28%. CMV IgG prevalence increased significantly with age (p < 0.01). Women living in downtown areas showed higher IgG prevalence than those residing in urban areas (p = 0.023). CMV-IgM seroprevalence was highest in autumn (p = 0.021). There was no difference in IgM seroprevalence by age, socioeconomic status, geographical area, or gravida. The rate of primary CMV infection was 0.45% (13/2,887) at the first trimester. The seroconversion rate during pregnancy was 0.76% (22/2,887). One woman underwent seroconversion during pregnancy and gave birth to an infant with asymptomatic CMV infection. Congenital CMV infection was diagnosed in five of the 14 infants from 14 mothers with active infection, for a vertical transmission rate of 35.71% (5/14). Three infants were asymptomatic, whereas two infants presented symptomatic infection with hearing deficits. Although CMV IgG prevalence is relatively high in north China, significant attention to primary CMV infection during pregnancy is still needed.

• Journal of Chest Surgery
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• v.53 no.2
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• pp.41-48
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• 2020

Background: Chylothorax after congenital heart surgery is not an uncommon complication, and it is associated with significant morbidity. However, consensus treatment guidelines are lacking. To improve the treatment outcomes of patients with postoperative chylothorax, we implemented a standardized management protocol at Severance Hospital in September 2014. Methods: A retrospective review of patients treated at a single center was done. All corrective and palliative operations for congenital heart disease performed at our institution between January 2008 and April 2018 were reviewed. The incidence and treatment outcomes of postoperative chylothorax were analyzed. Results: The incidence of chylothorax was 1.9%. Sixty-one percent of the patients could be managed with a low-fat diet, while 28% of the patients required complete restriction of enteral feeding. Thoracic duct embolization was performed in 2 patients and chest tube drainage decreased immediately after the procedure. No patient required thoracic duct ligation or pleurodesis. After implementation of the institutional management protocol, the number of chest tube drainage days decreased (median, 24 vs. 14 days; p=0.45). Conclusion: Implementing a strategy to reduce postoperative chylothorax resulted in an acceptable incidence of postoperative chylothorax. Instituting a clinical practice protocol helped to curtail the treatment duration and to decrease the requirement for surgical treatment. Image-guided embolization of the thoracic duct is an effective treatment for postoperative chylothorax.

• Journal of the korean academy of Pediatric Dentistry
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• v.30 no.4
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• pp.673-677
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• 2003

Congenital aglossia is a very rare condition. The oral manifestations of an aglossia include micrognathia, high arched or cleft palate, defects of the lower lip, an absence of lateral incisors and a mandibular growth deficiency. Although the etiology of congenital aglossia is unclear, both genetic and teratogenic mechanisms have been proposed. Treatment of aglossia patients depends on the nature and severity of the condition which includes surgical rehabilitation of the tongue tip to some extent, orthopedic expansion of the mandible to guide mandibular growth, and mandibular expansion by a distraction osteogenesis. In the present case, a 6 year old female aglossia patient with situs inversus was treated. A bonded hyrax screw was used to increase her mandibular primary intercanine width and intermolar width. A second phase orthodontic and surgical treatment will be possible after some retention phase.

• Tuberculosis and Respiratory Diseases
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• v.47 no.3
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• pp.394-399
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• 1999

We report a case of congenital and familial antithrombin III deficiency developing massive pulmonary thromboembolism. A 44-year-old man was admitted to our hospital because of sudden chest pain and severe dyspnea. Five years ago, he was operated due to a mesenteric vein thrombosis of unknown cause. On admission, radioisotopic venogram showed deep vein thrombosis and lung scintigram showed multiple segmental perfusion defects. His plasma antithrombin III level was 10.5 mg/dL which was less than 50% of normal and those of a son and two daughters were also decreased. After treatment with tissue plasminogen activator, heparin and coumadin, his symptom and lung scintigram were significantly improved. As far as we reviewed, there were very rare reports with congenital antithrombin III deficiency presenting as pulmonary thromboembolism in Korea.

• Korean Journal of Cleft Lip And Palate
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• v.11 no.2
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• pp.49-58
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• 2008

The development of fetal surgery has led to promising options for many congenital malformations, such as congenital diaphragmatic hernia (CDH), obstructive uropathy, twin-to-twin transfusion syndrome (TTTS), and sacrococcygeal teratoma. However, preterm labor (PTL) and premature rupture of membranes continue to be uniquitous risks for both mother and fetus. To reduce maternal morbidity and the risk of prematurity, minimal access techniques were developed and are increasingly employed recently. Lift-threatening diseases as well as severely disabling but not life-threatening conditions are potentially amenable to treatment. Recently, improvement of video-endoscopic technology has boosted the development of operative techniques for feto-endoscopic surgery, which has been demonstrated to be less invasive than the open approach. Fetal surgery for repair of cleft lip and palate, a congenital anomaly which is not life threatening, is inappropriate until such time that the benefits are shown to outweigh the risks of both the procedure itself and preterm delivery. Further animal studies will be needed before intrauterine surgery for humans should be considered. For the better understanding of recent techniques and complications associated with fetal intervention of congenital facial defect patients, we reviewed recent related articles about the current knowledge and new perspectives of experimental fetal fetal surgery in the cleft lip and palate defects.

• Neonatal Medicine
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• v.16 no.1
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• pp.85-88
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• 2009

The amniotic band syndrome is an uncommon congenital fetal abnormality, presumably due to fetal entanglement in strands of ruptured amniotic sac. The defects caused by this syndrome vary from simple limb defects to major visceral and craniofacial defects. We cared for a newborn infant with this syndrome, who showed constriction rings of the right leg and right axilla, right club foot, thoracic scoliosis, polydactyly, absence of the right thumb and aplasia cutis of the scalp. We report this case with a brief review of the literature.

• Archives of Plastic Surgery
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• v.32 no.2
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• pp.189-193
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• 2005

Reconstruction of calvarial bone defects from congenital anomaly or from bone loss due to traumatic or neoplastic processes remains a significant problem in craniofacial surgery and neurosurgery. To facilitate bone regeneration, there have been many trials such as autologous bone graft or allograft, and the addition of demineralized bone matrix and matrix-derived growth factor. Guided bone regeneration is one of the methods to accelerate bone healing for calvarial bone defects especially in children. Pericranium is one of the most usable structure in bone regeneration. It protects the dura and sinus, and provides mechanical connection between bone fragments. It supplies blood to bone cortex and osteoprogenitor cells and enhances bone regeneration. For maximal effect of pericranium in bone regeneration, authors used pericranium as a flap for covering calvarial defects in surgeries of 11 craniosynostosis patients and achieved satisfactory results: The bone regeneration of original cranial defect in one year after operation was 74.6%(${\pm}8.5%$). This pericranial flap would be made more effectively by individual dissection after subgaleal dissection rather than subperiosteal dissection. In this article, we reviewed the role of pericranium and reported its usefulness as a flap in surgery of craniosynostosis to maximize bone regeneration.

• BMB Reports
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• v.42 no.1
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• pp.6-15
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• 2009

The most common genetic disorder Down syndrome (DS) displays various developmental defects including mental retardation, learning and memory deficit, the early onset of Alzheimer's disease (AD), congenital heart disease, and craniofacial abnormalities. Those characteristics result from the extra-genes located in the specific region called 'Down syndrome critical region (DSCR)' in human chromosome 21. In this review, we summarized the recent findings of the DYRK1A and RCAN1 genes, which are located on DSCR and thought to be closely associated with the typical features of DS patients, and their implication to the pathogenesis of neural defects in DS. DYRK1A phosphorylates several transcriptional factors, such as CREB and NFAT, endocytic complex proteins, and AD-linked gene products. Meanwhile, RCAN1 is an endogenous inhibitor of calcineurin A, and its unbalanced activity is thought to cause major neuronal and/or non-neuronal malfunction in DS and AD. Interestingly, they both contribute to the learning and memory deficit, altered synaptic plasticity, impaired cell cycle regulation, and AD-like neuropathology in DS. By understanding their biochemical, functional and physiological roles, we hope to get important molecular basis of DS pathology, which would consequently lead to the basis to develop the possible therapeutic tools for the neural defects in DS.

• Journal of Preventive Medicine and Public Health
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• v.27 no.2 s.46
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• pp.337-365
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• 1994

Rubella is a viral disease with mild constitutional symptoms and generalized rashes. In childhood, it is an inconsequential illness, but when it occurs during early pregnant period, there are significant risks of heart defects, cataract, mental retardation to the fetus. The series of congenital defects induced by rubella is called 'congenital rubella syndrome'. Many research have been performed to find out more effective prevention program on rubella. The objectives of this study are, first, to calculate the incidence rate of acute rubella infection and congenital rubella syndrome in Korea, second, to evaluate economic efficiency of several rubella vaccination policies and to offer data for the most reasonable decision on vaccination policy. Study populations are 663,312 children of one year-old in 1992. The author has performed cost-benefit analyses according to the three vaccination policies-U.S.A.'s. U.K.'s and Sweden's. In this Study, the author got the incidence rate of acute rubella infection using the catalytic model. In the meantime, the author used 50 per 100,000 live births as the incidence rate of congenital rubella syndrome. The discount rate used in this study was 5 percent per annum. The sensitivity analyses were done with different discount rates (4%, 7%) and different incidence rate of congenital rubella syndrome (10,100 per 100,000 live births) : The study results are as follows: 1. Without vaccination, lifetime expenditures per patient for acute rubella infeciton amount to 14,822 won and the total expenditures to about 3.1 billion won. Meanwhile, lifetime expenditures per patient for congenital rubella syndrome amount to about 91 million won and the total expenditures to about 16.3 billion won without vaccination. 2. The cost of vaccination for a child of one year old was 2,322 won and the total cost for the one year old children was about 1.5 billion won (American style). The cost for vaccination of female children at fifteen was about 339 million won (Birtish style). And the cost of vaccination at one for both sex and female children at fifteen was about 1.9 billion won (Swedish style). 3. The benefit to cost ratios of vaccination of female children at fifteen that is the british mode of rubella vaccination, was 60.0 at the level of 80% population coverage and 48.6 at 100% coverage. It shows much higher benefit to cost ratio than those of the other two vaccination policies. 4. Both net benefits of vaccination at one (American style) and that of vaccinations at one and fifteen (Swedish style) range from about 17.0 billion to 17.8 billion won, those were larger than that of vaccinations of female children at fifteen (Birtish style, about 16.0 billion). 5. In marginal cost-benefit analysis of only additional program of revaccination, the benefit to cost ratios were 3.6 (80% coverage rate) or 0.6 (100% coverage rate). It implies that additional program was less efficient or inefficient. 6. In sensitivity analysis with different discount rates(4% or 7%) and different incidence rates of congenital rubella syndrome (10 or 100 per 100,000 live births), the benefit to cost ratios has fluctuated in wide range. However, all the ratios of vaccination of female children at fifteen were higher than those of the others. Even under the most conservative assumption, the benefit to cost ratios of all the rubella vaccination policies were higher than 3.3. In conclusion, all the rubella vaccination policies found to be cost-effective and particularly the vaccination of female children at fifteen was strongly recommended.