• Journal of Chest Surgery
• /
• v.14 no.1
• /
• pp.9-16
• /
• 1981

Twenty eight patients with conpnital heart disuse underwent lung biopsy to assets pulmonary obstructive vascular disease at cardiac surpry. Thirteen patients had patent ductus arteriosus, 10, ventricular septal defects and S, atrial septal defects. The aaes were between 2 and 30 years. In patients with patent ductus arteriosus lung biopsy was performed from the IIngular Hlment. The anterior seament of the right upper lobe was blopsled in cases with ventricular septal defect and atrial septal defect. Grading of pulmonary obstructive vascular disease could not be assessed In 9 cases. In 2 cases poor quality of the slides made us impossible to evaluate and In 7 cases there were no suitable small muscular arteries to evaluate in the slides of lung tissue especially taken from the IIngular seament. Nineteen cases were evaluated pulmonary obstructive vascular disease. Among them 17 cases had Heath-Edwards changes of grade 1 and 2 patients had that of grade 3. The thickness of media was measured. It was expressed as percentage of medial thickness to outer diameter of artery. The medial thickness was correlated proportionally with elevation of pulmonary arterial pressure and pulmonary vascular resistance to systemic vascular resistance ratio. There were no complications related to the procedure of lung biopsy.

• Archives of Plastic Surgery
• /
• v.47 no.5
• /
• pp.392-403
• /
• 2020

Severe cartilage defects and congenital anomalies affect millions of people and involve considerable medical expenses. Tissue engineering offers many advantages over conventional treatments, as therapy can be tailored to specific defects using abundant bioengineered resources. This article introduces the basic concepts of cartilage tissue engineering and reviews recent progress in the field, with a focus on craniofacial reconstruction and facial aesthetics. The basic concepts of tissue engineering consist of cells, scaffolds, and stimuli. Generally, the cartilage tissue engineering process includes the following steps: harvesting autologous chondrogenic cells, cell expansion, redifferentiation, in vitro incubation with a scaffold, and transfer to patients. Despite the promising prospects of cartilage tissue engineering, problems and challenges still exist due to certain limitations. The limited proliferation of chondrocytes and their tendency to dedifferentiate necessitate further developments in stem cell technology and chondrocyte molecular biology. Progress should be made in designing fully biocompatible scaffolds with a minimal immune response to regenerate tissue effectively

• Clinical and Experimental Pediatrics
• /
• v.45 no.2
• /
• pp.273-277
• /
• 2002

Syndrome of 4q deletion is characterized by an abnormal shape of the skull, craniofacial dysmorphism, cardiovascular malformations, genitourinary defects, limb and digital anomalies, and developmental delay. We experienced a case of 4q interstitial deletion in a 2 day-old female neonate who showed short extremities, partial agenesis of corpus callosum and congenital heart defects. We report the case with a brief review of the literature.

• Journal of Chest Surgery
• /
• v.28 no.4
• /
• pp.335-339
• /
• 1995

Secundum atrial septal defect occupies about 10 to 15% of congenital heart diseases, and the surgical accomplishment is outstanding, so that the operative mortality is getting near to zero percent. But, the methods of correction, direct closure versus patch closure are still controversial and there is no absolute method about it. Some surgeons prefer direct closure technique for its simplicity and lesser thrombogenicity but others, afraid of arrhythmia and suture detachment after closure of large defect, prefer patch closure. Usually most surgeons use direct suture technique in small and moderate sized defects and patch closure in large defects. In our hospital, 156 cases of isolated secundum atrial septal defect were closed directly by double continuous over and over suture using 5-0 polypropylene[prolene , regardless their sizes and the amounts of shunt flow. There were no operative mortality and no serious complications such as heart block, suture detachment and embolism.

• Journal of Chest Surgery
• /
• v.47 no.3
• /
• pp.280-282
• /
• 2014

Atrial septal defect (ASD) is one of the most common congenital heart defects in adults. Surgical repair is the most common treatment approach, but device closure has recently become widely performed in accordance with the trend toward less invasive surgical approaches. Although surgery is recommended when ASD is accompanied by atrial fibrillation, this study reports a case in which a complete cure was achieved by closure of a device and totally thoracoscopic ablation.

• Journal of Interdisciplinary Genomics
• /
• v.5 no.1
• /
• pp.1-4
• /
• 2023

Kleefstra syndrome is caused by chromosome 9q34.3 deletion or heterozygous mutations in the Euchromatin Histone Methyl Transferase 1 (EHMT1) gene. The prevalence is estimated 1:25,000 to 1:35,000. Intellectual disability, distinctive facial features, hypotonia in childhood can be accompanied. The spectrum of Kleefstra syndrome includes behavioral/psychiatric problems, hearing and visual impairments, seizures, congenital heart defects, genitourinary defects, and obesity. Therefore, it is necessary to understand the pathophysiology and various manifestation of Kleefstra syndrome and discussing with a multidisciplinary team will help diagnose and treat Kleefstra syndrome patients.

• Journal of Chest Surgery
• /
• v.17 no.2
• /
• pp.184-188
• /
• 1984

As OHS is prevalent on whole world, LV-RA shunts once thought as quite rare congenital heart disease are reported frequently. Two cases of LV-RA shunts were operated at N.M.C. in 1983: One of them combined VSD, the other membranous ventricular septal aneurysm protruding into right atrial chamber. In case 1, which was diagnosed correctly, right atriotomy was enough to close the defect under ECC, but in case 2, which was misdiagnosed as ASD preoperatively, atriotomy was added to ventriculotomy. The LV-RA defects were closed by U-shaped direct suture with Teflon felt pledget. Postoperative course was uneventful in both of them.

• Journal of Chest Surgery
• /
• v.15 no.3
• /
• pp.325-330
• /
• 1982

A case of a 7 year old girl with rare congenital anomalies is reported. The anomalies as called Cantrell`s pentalogy is consisted of defect in supraumbilical abdominal wall, ventral diaphragm, adjacent pericardium, and lower sternum associated with cardiac malformation. Her cardiac lesion was muscular diverticulum of left ventricle. The diverticulum was resected and the other defects were repaired successfully.

• Clinical and Experimental Pediatrics
• /
• v.49 no.8
• /
• pp.833-838
• /
• 2006

Giant platelet syndrome is a group of unique disorders characterized by the presence of abnormally large platelets, and usually accompanied by thrombocytopenia. Most cases of giant platelets are encountered in idiopathic thrombocytopenic purpura(ITP). In contrast, inherited giant platelet disorders, a group of heterogeneous diseases, are rare. Bernard-Soulier syndrome and its variants, and MYH9 related diseases have been defined at the molecular level. Abnormalities in transcription factors are implicated in a couple of macrothrombocytopenia syndromes. However, the molecular defects are unknown in gray platelet syndrome. It is important to make a proper diagnosis of congenital macrothrombocytopenia to avoid unnecessary medications and potentially dangerous treatment for presumed ITP.

• Journal of Chest Surgery
• /
• v.27 no.3
• /
• pp.196-201
• /
• 1994

Endocardial cushion defect is a rare congenital heart disease. From September 1985, we experienced 20 cases of endocardial cushion defects and postoperative follow up was taken.We reviewed preoperative clinical data, echocardiography, cardiac cath data, operative method & time. After operation, we performed echocardiography and examined mitral valve function & integrity of patch closure sites. Postoperative follow up was taken regularly & follow up period was from 2 month to 91 months. Several postoperative complications were overcome with adequate treatment. There were 3 cases of early death and mortality rate was 15% [3/20].