• Journal of Chest Surgery
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• 제28권7호
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• pp.726-730
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• 1995

Congenital cystic adenomatoid malformation[CCAM of the lung is extremely rare. We have experienced an unusual case of congenital cystic adenomatoid malformation. The patient was 20-year-old male and had chest pain for 10 days. On simple chest x-ray and Thoracic CT scan, there was a large cystic mass surrounded with multiloculated round cysts with air fluid level on the right lower lobe of a lung. Right lower lobectomy was performed and the pathologic result was congenital cystic adenomatoid malformation.

• Journal of Chest Surgery
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• 제28권2호
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• pp.193-195
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• 1995

Congenital lobar emphysema is an uncommon disease affecting newborns and infants with respiratory distress. This report describes a 1.9kg premature baby of 31weeks gestation who developed respiratory distress symptoms of congenital lobar emphysema involved left upper lobe.Left upper lobe lobectomy was carried out and good result was obtained. So, we present one case report with literature review.

• Clinical and Experimental Pediatrics
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• 제50권11호
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• pp.1055-1060
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• 2007

Right-sided heart failure is a major problem among patients with congenital heart diseases, due to the prevalence of congenital heart defects and the association of pulmonary hypertension. More attention is focused on the structure of the right heart particularly in association with congenital heart defects and chronic lung disease. The right ventricle (RV) may support the pulmonary circulation, and sometimes the systemic circulation (systemic RV) in congenital heart defects. Despite major progress being made, assessing the RV remains challenging, often requiring a multi-imaging approach and expertise (echocardiography, magnetic resonance imaging, nuclear and cineangiography). Evidence is accumulating that RV dysfunction develops in many of these patients and leads to considerable morbidity and mortality. While there is extensive literature on the pathophysiology and treatment of left heart failure, the data for right-sided heart failure is scarce. Therefore RV function in certain groups of congenital heart disease patients needs close surveillance and timely and appropriate intervention to optimise outcomes. An understanding of RV physiology and hemodynamics will lead to a better understanding of current and future treatment strategies for right heart failure. This will review right-sided heart failure with the implications of volume and pressure loading of the RV in congenital heart diseases.

• Clinical and Experimental Pediatrics
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• 제53권1호
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• pp.14-20
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• 2010

Cytomegalovirus (CMV) is currently the most common agent of congenital infection and the leading infectious cause of brain damage and hearing loss in children. Symptomatic congenital CMV infections usually result from maternal primary infection during early pregnancy. One half of symptomatic infants have cytomegalic inclusion disease (CID), which is characterized by involvement of multiple organs, in particular, the reticuloendothelial and central nervous system (CNS). Moreover, such involvement may or may not include ocular and auditory damage. Approximately 90% of infants with congenital infection are asymptomatic at birth. Preterm infants with perinatal CMV infection can have symptomatic diseases such as pneumonia, hepatitis, and thrombocytopenia. Microcephaly and abnormal neuroradiologic imaging are associated with a poor prognosis. Hearing loss may occur in both symptomatic and asymptomatic infants with congenital infection and may progress through childhood. Congenital infection is defined by the isolation of CMV from infants within the first 3 weeks of life. Ganciclovir therapy can be considered for infants with symptomatic congenital CMV infection involving the CNS. Pregnant women of seronegative state should be counseled on the importance of good hand washing and other control measures to prevent CMV infection. Heat treatment of infected breast milk at $72{^{\circ}C}$ for 5 seconds can eliminate CMV completely.

• Advances in pediatric surgery
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• 제16권2호
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• pp.143-153
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• 2010

Congenital diaphragmatic disease is one of the common major congenital anomalies, and its mortality remained still high despite recent medical advances. The aim of this study is to examine the clinical characteristics of congenital diaphragmatic diseases. A total of 39 patients with congenital diaphragmatic disease that underwent surgery from January, 1997 to December, 2009 at Pusan National University Hospital were included in this study. Medical records were retrospectively reviewed. The male to female ratio was 30:9. Six out of 39 cases died (NS) before surgery, 17 patients had Bochdalek's hernia (BH), 11 patients hiatus hernia (HH), 4 diaphragmatic eventration (DE), and 1 Morgagni hernia (MH). There were no differences in mean birth weight and mean gestational age. NS (83.3 %). BH (35.3 %) was diagnosed more frequently than other diseases in the prenatal period. Three patients (17.6 %) of BH expired due to pulmonary hypoplasia and 1 patient had co-existing congenital heart disease. BH was diagnosed more frequently in the prenatal stage and had a higher motality rate than other conditions. Therefore, BH needs to be concentrated more than other anomalies.

• Clinical and Experimental Otorhinolaryngology
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• 제11권4호
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• pp.233-241
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• 2018

Objectives. As endoscopic instrumentation, techniques and knowledges have significantly improved recently, endoscopic ear surgery has become increasingly popular. Transcanal endoscopic ear surgery (TEES) can provide better visualization of hidden areas in the middle ear cavity during congenital cholesteatoma removal. We aimed to describe outcomes for TEES for congenital cholesteatoma in a pediatric population. Methods. Twenty-five children (age, 17 months to 9 years) with congenital cholesteatoma confined to the middle ear underwent TEES by an experienced surgeon; 13 children had been classified as Potsic stage I, seven as stage II, and five as stage III. The mean follow-up period was 24 months. Recurrence of congenital cholesteatoma and surgical complication was observed. Results. Congenital cholesteatoma can be removed successfully via transcanal endoscopic approach in all patients, and no surgical complications occurred; only one patient with a stage II cholesteatoma showed recurrence during the follow-up visit, and the patient underwent revision surgery. The other patients underwent one-stage operations and showed no cholesteatoma recurrence at their last visits. Two patients underwent second-stage ossicular reconstruction. Conclusion. Although the follow-up period and number of patients were limited, pediatric congenital cholesteatoma limited to the middle ear cavity could be safely and effectively removed using TEES.

• Journal of Chest Surgery
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• 제47권6호
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• pp.545-547
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• 2014

A coronary artery fistula was surgically ligated in a 38-year-old woman via a left anterior mini-thoracotomy without the use of cardiopulmonary bypass. In selected cases, this surgical approach can provide an excellent surgical exposure for coronary artery fistula ligation. It also offers an excellent cosmetic result and shorter hospital stay.

• 보건의료산업학회지
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• 제7권3호
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• pp.111-123
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• 2013

The purpose of this study was to identify factors affecting stress and adaptation in newborn congenital heart disease patients of parents. Data were obtained through questionnaires from 181 newborn congenital heart disease inpatients of parents from P university in Y city. The data were analyzed SPSS 18.0, using descriptive statistics, Pearson correlation coefficients and stepwise multiple regression. Stress was negative correlated with level of patents adaptation. Social support, marital communication and family cohesion were positive correlated with level of patents adaptation. The explained variances for patents adaptation were 45.2% and factors affecting patients adaptation were congenital heart disease, family cohesion, stress, social support and marital communication. Educational intervention program for parents of newborn with congenital heart disease, reduce stress, and to increase the adaptation will be developed.

• Clinical and Experimental Pediatrics
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• 제59권sup1호
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• pp.1-4
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• 2016

Congenital aniridia is a rare ocular malformation that presents with severe hypoplasia of the iris and various ocular manifestations. Most cases of congenital aniridia are known to be related to mutations in the paired box gene-6 (PAX6 ), which is an essential gene in eye development. Herein, we report a familial case of autosomal dominant congenital aniridia with four affected members in 3 consecutive generations and describe the detailed ophthalmologic findings for one of these members. As expected, mutational analysis revealed a nonsense mutation (p.Ser122*) in the PAX6 gene. Thus, our findings reiterate the importance of PAX6 mutations in congenital aniridia.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제38권3호
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• pp.171-176
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• 2012

Congenital syngnathia refers to the fusion of bony tissues, a rare disorder with only 41 cases reported in the international literature from 1936 to 2009. The occurrence of syngnathia without any other associated systemic disease or congenital anomaly is extremely rare. This report presents a case of congenital syngnathia with unilateral maxillomandibular bony adhesion without any other oral or maxillofacial anomaly. No recommended protocol for surgery exists due to the rarity of the disorder. There is a very low survival rate for the few patients who have forgone surgical management. This case describes a 74-year-old female patient who was suffering from limitation of mouth opening and was subsequently diagnosed with congenital syngnathia. The surgical staff performed separation surgery and reconstructed the malformed oral vestibule and cheek using the radial forearm free flap operation.