• Clinical and Experimental Pediatrics
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• v.53 no.6
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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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• v.48 no.6
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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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• v.2 no.2
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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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• v.5 no.1
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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.

• Archives of Craniofacial Surgery
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• v.20 no.6
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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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• v.22 no.6
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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.

• Journal of Life Science
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• v.7 no.1
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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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• v.21 no.4
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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.

• The Journal of Korean Academy of Prosthodontics
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• v.55 no.2
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• pp.198-204
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• 2017

Maxillofacial defect comes from congenital defect, trauma and surgical resection. Patients with intraoral defect are commonly related to maxillary defect and they need prosthetic rehabilitation. Functional reconstruction of partially edentulous mandible has many limitations. However, if both condyles are intact, maxillofacial prosthesis using partial denture give competent results. In this case, a patient of 58 year-old male has a defect on palate and left mandibular posterior teeth from gunshot. The maxillary defect of this patient is Class IV according to Aramany classification and the mandibular one is Type V according to Cantor and Curtis classification. For retention of the obturator, remaining teeth are fully utilized and artificial teeth are arranged harmoniously to provide stable occlusion. Mandibular RPD covered limited range of deformed soft tissue derived from mandibular resection surgery. With these treatments, the patient in this case showed improvements in mastication, swallowing and speech.

• Clinical and Experimental Pediatrics
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• v.45 no.2
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• pp.208-213
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• 2002

Purpose : The major cause of cardiac dysfunction, after open heart surgery for congenital heart disease, is perioperative myocardial injury. Cardiac troponin I is found only within the myocardial cell, so it can be used as a biochemical marker of the myocardial injury. We performed this study to evaluate the worth of cardiac troponin I as a biochemical marker reflecting the extent of perioperative myocardial injury and recovery. Methods : Thirty-four patients who had undergone elective open heart surgery of congenital heart disease(CHD) from April to July 2001 were enrolled in this study. We measured types of CHD, serial cardiac troponin I(baseline 1 day before operation, postoperative day 1, 2, 3, 7), duration of cardiopulmonary bypass(CPB), aortic cross clamping(ACC), intubation and postoperative hospital stay. Results : Compared with the baseline before operation, there was a significant, increase of cardiac troponin I on the postoperative day 1 and a significant gradual decrease on the day 2, 3, 7. The levels of cardiac troponin I were the highest in the transposition of great artery(TGA) repair on the postoperative day 1 and high in the tetralogy of Fallot(TOF), atioventricular septal defect (AVSD), ventricular septal defect(VSD) and atrial septal defect(ASD) repair with decreasing sequence. The longer duration of CPB, ACC and intubation, the higher of cardiac troponin I, but there were no significant correlations between cardiac troponin I levels and duration of hospital stay. Conclusion : Because there was significant increases or decreases of cardiac troponin I according to the perioperative time and types of the congenital heart disease, it is a worthy biochemical marker which reflects the extent of perioperative myocardial injury and recovery after open heart surgery.