• Clinical and Experimental Pediatrics
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• 제53권6호
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• pp.669-679
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• 2010

The risk of mortality and morbidity of patients with congenital heart defects (CHDs) is highest during neonatal period and increases when diagnosis and proper management are delayed. Neonates with critical CHDs may present with severe cyanosis, respiratory distress, shock, or collapse, all of which are also frequent clinical presentations of various respiratory problems or sepsis in the newborn. Early diagnosis and stabilization and timely referral to a tertiary cardiac center are crucial to improve the outcomes in neonates with CHDs. In this review, the clinical presentation of critical and potentially life-threatening CHDs is discussed along with brief case reviews to help understand the hemodynamics of these defects and ensure proper decision-making in critically ill patients.

• Journal of Chest Surgery
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• 제48권6호
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• pp.429-431
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• 2015

Congenital pericardial defects (CPDs) are infrequent anomalies that are usually asymptomatic and are discovered incidentally during unrelated interventions. Here we report the case of a CPD with herniation of an enlarged left atrial appendage identified during total thoracoscopic ablation (TTA) for persistent atrial fibrillation (AF). The persistent AF was successfully treated with a hybrid procedure, in which TTA was followed by an electrophysiological study.

• Advances in pediatric surgery
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• 제2권2호
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• pp.129-132
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• 1996

This is a case report of a sliding hiatal hernia with severe gastroesophageal reflux(GER) after repair of congenital diaphragmatic hernia(CDH). It was not possible to determine whether the hiatal hernia is a de novo lesion which was missed at the original operation or a consequence of overzealous repair of the Bochdalek defect at the expense of weakening of the diaphragmatic crura. This case demonstrates that a sliding hiatal hernia can be a cause of severe gastroesophageal reflux that should be managed surgically.

• Journal of Genetic Medicine
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• 제5권1호
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• pp.1-6
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• 2008

Knowledge of developmental biology is essential for clinicians who seek to develop a rational approach to the diagnostic evaluation of patients with birth defects. After an accurate diagnosis, a clinician can make predictions about prognosis, recommend management options, and provide an indication of recurrence risk for the parents and relatives. In this paper, we first review the basic mechanisms of embryological development and clinical dysmorphology. We then review cellular and molecular mechanisms in development and related congenital anomalies. Developmental anomalies have a major impact on public health. Genetic counseling and prenatal diagnosis, with the option to continue or to terminate a pregnancy, are important for helping families faced with the risk of a serious congenital anomaly in their offspring. Moreover, primary prevention of birth defects, for example, supplementation of prenatal folic acid and prevention of consumption of alcohol which has teratogenic effects, can be accomplished using developmental biology knowledge.

• 대한두개안면성형외과학회지
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• 제20권6호
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• pp.408-411
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• 2019

Hairless scalp areas can occur due to trauma, tumors, or congenital disease. This aesthetically unpleasing condition can lead to psychosocial distress, and thin skin flaps may be prone to scarring. Treating the hairless scalp by simple excision is challenging because of skin tension. Tissue expanders are a good option for hairless scalp resurfacing. However, a single expansion may be inadequate to cover the entire defect. This report describes good results obtained using a serial resurfacing method involving re-expansion of the flap with a tissue expander to treat two patients with large lesions: one due to aplasia cutis congenital and another who underwent dermatofibrosarcoma protuberance resection. The results suggest that scalp resurfacing by serial tissue expansion using a tissue expander can be used for extensive lesions.

• Journal of Chest Surgery
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• 제22권6호
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• pp.1088-1091
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• 1989

Foramen of Morgagni hernia is the least common type of all congenital diaphragmatic hernias. These foraminal hernias result from a congenital defect in the development or attachment of the diaphragm to the sternum and costal arch. They occur most commonly on the right side, possibly because of pericardial reinforcement of the left. In our case, occurred on the right side and the contents of the hernial sac were omental fat and a part of transverse colon. We performed transthoracic approach for reduction and repair of foraminal hernia with ligation and interrupted mattress sutures of the margin of the defected diaphragm to the posterior part of the sternum and costal cartilage. The postoperative course was uneventful except posttraumatic delirium and discharged at 21st postoperative day.

• 생명과학회지
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• 제7권1호
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• pp.66-72
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• 1997

The term of apoptosis, programmed cell death, was firstly coined to distinguish with necrosis, pathologic cell death, by Kerr in 1972. Although various pathogenic factors are able to occur apoptosis, ti is essential process for normal development and physiology in the animals. Recently in the field of medicine, apoptosis researeh is especially focused in serveral Kind of pathopoiesis problems including cancer, immunodeficiency associated HIV and other virus, autommunity, alzheimer and congenital anormality. The information obtained from the animal model system for apoptosis should be directly applicable to both life science for understanding of development and medicine for practical the rapy. To know the common mechanism of apoptosis, it is prerequisite that the genes and factors responsible for apoptosis should be defined and characterized on the molecular level. The study of apoptosis should contribute largely to biology inculuding cell physiology and development, and both basic and clinical medicine to understand cause of diseases for therapy as well as congenital defect.

• Journal of Chest Surgery
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• 제21권4호
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• pp.757-760
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• 1988

Double mitral valve orifice is an unusual congenital abnormality, and is rarely of clinical importance. That abnormality not produce hemodynamic deficits, but it has been associated with other congenital heart diseases, especially atrioventricular canal defects, and so it is surgically important condition. After the first report by M.S. Greenfield in 1876, there are only a few reported cases all over the world. We experienced two cases of double mitral valve orifice with atrioventricular canal defect. The small accessory mitral orifice was placed in anterior mitral leaflet as a hole type in both cases. The A-V canal defects are corrected with a one patch technique, but the accessory orifices are left unclosed because there were no mitral regurgitations through the orifice. On the 4th postoperative day, the patients have been performed echocardiography; both had no mitral regurgitation. Both of patients are now being follow-up and we find no specific problems. A small accessory mitral orifice may be left unclosed and rarely produce significant regurgitation, conclusively.

• 대한치과보철학회지
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• 제55권2호
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• pp.198-204
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• 2017

악안면부의 결손은 선천적으로 결손을 가지고 있거나 외상이나 수술적인 절제 등에 의한 후천적 원인에 의해 발생할 수 있다. 특히 구강 내 결손을 가진 환자 중 상악의 결손과 연관되어 있는 경우가 높은 비중을 차지하고 있으며 보철적 치료의 필요성이 높다. 하악의 부분적 결손을 가질 경우 기능적 회복에 상당한 한계점을 보이나 양측 턱관절이 정상적으로 남아 있을 경우 국소의치를 이용한 악안면 보철물을 제작함으로써 양호한 결과를 기대할 수 있다. 본 증례의 환자는 58세 남자 환자로서 안면부 총상으로 인한 구개부 및 좌측 하악 구치부 결손을 가진 분으로 obturator와 하악 RPD 재제작을 위해 내원하였다. 환자의 상악 결손 범위는 Aramany 분류법 Class IV에 해당되는 상태이며, 하악은 Cantor와 Curtis 분류법 Type V resection 상태이다. 상, 하악의 우측 구치부가 잔존하였으나, 수술 후 악골 변형으로 인해 서로 교합되지 않았으며 치주상태 불량으로 인한 동요도도 존재하는 상태였다. Obturator의 유지를 위해 잔존 지대치를 최대한 활용하였고 안정적인 교합이 형성되도록 치아를 배열하였다. 하악 RPD는 하악 절제술로 변형된 연조직에 적합하도록 제한된 범위를 피개하는 RPD를 제작하였다. 이에 환자분의 저작, 연하, 발음 기능이 많이 개선되었으며 만족할 만한 결과를 보여주어 본 증례를 발표하는 바이다.

• Clinical and Experimental Pediatrics
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• 제45권2호
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• pp.208-213
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• 2002

목 적 : 수술 중, 후의 심근 손상은 선천성 심질환에 대한 개심술 후 심장 기능 부전의 주원인이다. 이 연구에서는 심근 세포에서만 발견되는 cardiac troponinI를 측정함으로서 수술 중, 후 심근 손상 정도와 회복 여부를 반영할 생화학적 표지자로서의 가치를 확인하고자 하였다. 방 법: 2001년 4월부터 7월까지 선천성 심질환으로 진단받고 개심술을 위해 본원에 입원한 환아 34명을 대상으로 하였다. Cardiac troponin I 치의 측정을 위해 술전 24시간 이내, 술후 1, 2, 3, 7일째에 각각 채혈을 시행했고, CPB 시간, ACC 시간, 기도 삽관시간, 수술 후 입원 기간을 측정하였다. 결 과 : Cardiac troponin I 치는 수술 후 1일째가 수술 전에 비해 유의한 증가가 있었고, 2, 3, 7일째에는 점차적으로 유의한 감소를 보였다. 심질환 별로는 수술 후 1일째 대혈관 전위에서 cardiac troponin I 치가 가장 높았고, 활로씨 4징, 방실중격결손, 심실중격결손, 심방중격결손 순으로 높은 치를 나타내었다. 심장폐 우회술 시간이 길수록, ACC 시간이 길수록 수술 후 1일째 cardiac troponin I 농도가 높았고, cardiac troponin I 농도가 높을수록 기도삽관 시간이 길었으나 입원 기간과는 상관관계가 없었다. 결 론: 개심술 후 시간에 따라, 개심술을 시행한 심질환에 따라 cardiac troponin I 농도의 의미 있는 증가와 점차적인 감소를 보임으로써 cardiac troponinI는 개심술 후 심근 손상의 정도 및 심근 손상에서의 회복 여부를 반영하는 생화학적 표지자로 가치가 있을 것으로 생각된다.