• Journal of Chest Surgery
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• v.41 no.3
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• pp.363-365
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• 2008

Accessory mitral valve tissue is a very rare congenital cardiac malformation and it is an uncommon cause of left Ventricular outflow tract obstruction(LVOTO). The pathogenetic mechanism of subaortic obstruction is thought to be systolic ballooning of the abnormal valve tissue into LVOT. We are reporting a case of an accessory mitral valve tissue that was associated with LVOTO, and this was completely relieved after trans-aortic surgical excision of the accessory tissue.

• Journal of Chest Surgery
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• v.14 no.1
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• pp.40-48
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• 1981

Total anomalous venous return defines a group of congenital heart disease which have in common the entire pulmonary venous drainage returning directly or indirectly to the right atrium instead of to the left atrium. Despite of recent advance in treatment, this severe malformation in its various anatomical forms has a high surgical mortality during early infancy. Because of the high mortality in the untreated infant and the surgical risk in the first year of life, the timing of the operation remains important for optimal result. Three cases of T APV R, two supracardiac types and one mixed type, were treated with extracorporeal circulation during last three years in the Dept. of Thoracic and Cardiovascular Surgery, Seoul National University Hospital. The first one was 10 months old male with supracardiac type which drained through left innominate vein, and he was operated with profound hypothermia and total circulatory arrest but failed. The second case was 7 years old male with supracardiac type drained through left innominate vein, and he was well post operatively, and followed periodically for 12 months. The third case was 24 years old female with mixed type drainage (left upper pulmonary vein drained through left innominate vein, and the others through coronary sinus) was successfully corrected, and she was followed for 4 month without problem. All cases were diagnosed with cardiac catheterization and angiocardiogram, and also with echocardiogram in last two cases. In first two cases of supracardiac type, total circulatory arrest was used in brief period during anastomosis between common pulmonary venous trunk and left atrium. In the last case of mixed type, usual cardiopulmonary bypass with moderate hypothermia was used and total circulatory arrest was not needed.

• Journal of the korean academy of Pediatric Dentistry
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• v.30 no.3
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• pp.510-514
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• 2003

The Axenfeld-Rieger syndrome is a rare autosomal dominant disorder characterized by dental and ocular abnormalities. The essential ocular features include partial or complete bilateral hypoplasia of the iris stroma, abnormalities of the angle structures with congenital iris adhesions, and anterior displacement of Schwalbe's corpuscles. Common oral findings are hypodontia(especially in anterior maxillary segment), microdontia, misshaped teeth, delayed eruption of the teeth. Additionally, other systemic symptoms can be seen and early detection by the pedodontist through dental diagnosis should prevent visual impairment.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.3 no.1
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• pp.17-21
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• 2007

Alagille syndrome is an autosomal dominant genetic disorder and occurs in approximately 1 in 100,000 live births. Diagnostic criteria was established by Alagille. It is mainly caused by a mutation in the Jagged1 gene. Major clinical features of this syndrome are paucity of intrahepatic bile duct with cholestasis, characteristic facies, cardiac murmur, defects of vertebrae, and embryotoxon. And minor clinical features are mental retardation, renal involvement, growth retardation, other skeletal abnormalities, a high-pitched voice. The surviving prognosis of Alagille syndrome patients depends on the severity of cardiovescular malformation in the early ages of infant. However, with the increasing years, it depends on the severity of the liver disease. Cholestasis causes congenital jaundice, malnutrition and growth retardation. Also, the increase of serum cholesterol level cause xanthoma and pruritus. Even though the severity of these problems are reduce with age, there is cases where there is no way but liver transplantation. For oral features of Alagille syndrome patients, green discoloration of entire dentition, induced by bilirubin infiltration into dentinal tubules, is especially. Also, xanthoma on gingiva and partial hypodontia have been reported. This report is on the oral features of an Alagille syndrome patient who visited to Kyung-Pook University Hospital.

• Clinical and Experimental Pediatrics
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• v.52 no.1
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• pp.124-128
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• 2009

Pulmonary arteriovenous malformations (PAVMs) are direct communications between pulmonary arteries and pulmonary veins, resulting in right-to-left shunts that may cause cyanosis, dyspnea, and digital clubbing. Neurological complications such as intracerebral hemorrhage or brain abscess may result from cerebral thrombosis or emboli. In most cases, they remain unrecognized until the late teenage years. Here, we report a case of a 6-year-old boy who presented with perioral cyanosis, digital clubbing, and dyspnea on exertion. A plain chest X-ray showed a focal nodular opacity in the right lower lobe (RLL), and a diagnosis of a large PAVM in the RLL was confirmed by chest computed tomography. A right lower lobectomy was successfully performed without any complications. Although their incidence in children is low, PAVMs should be suspected as a possible cause of cyanosis and dyspnea of non-cardiac origin, and should be treated promptly to prevent further neurological complications.

• Journal of the Korean Society for Precision Engineering
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• v.24 no.10
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• pp.123-130
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• 2007

Hypoplastic left heart syndrome is currently the most lethal cardiac malformation of the newborn infant. Survival following a Norwood operation depends on the balance between systemic and pulmonary blood flow, which is highly dependent on the fluid dynamics through the interposition shunt between the two circulations. The purpose of this study is an optimization of the systemic-to-pulmonary artery shunt. In this study, We used computational fluid dynamic(CFD) models to determine the velocity profile in a systemic-to-pulmonary artery shunt and suggested a simplified method of calculating the blood flow in the shunt based on Ultrasound systems. We analyzed the flow characteristic variations and oscillatory shear index(OSI) due to the anastomosis angle and shunt diameter changing. Four different CFD models were constructed with the shunt sizes ranging from 3 to 3.5mm. The angle between the brachiocephalic trunk(BCT) and the shunt were $30^{\circ}$ and $45^{\circ}$, respectively. When the diameter is 3.0 mm, the oscillatory shear index decreased by 1.2% at $30^{\circ}$ as opposed to at $45^{\circ}$. When the diameter is 3.5 mm, it increased by 18% more at $30^{\circ}$ as opposed to at $45^{\circ}$. When the joint angle is $30^{\circ}$ and the diameter is 3.0 mm, the oscillatory shear index decreased by 4.1% in comparison with the 3.5 mm diameter. When the angle is $45^{\circ}$ and the diameter is 3.0 mm, the index increased by 14.6% in comparison with the 3.5 mm diameter.

• Clinical and Experimental Pediatrics
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• v.63 no.8
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• pp.329-334
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• 2020

Background: Birth asphyxia is a leading cause of neonatal mortality. Ischemia-modified albumin (IMA) levels may have a predictive role in the identification and prevention of hypoxic disorders, as they increase in cases of ischemia of the liver, heart, brain, bowel, and kidney. Purpose: This study aimed to assess the value of IMA levels as a diagnostic marker for neonatal hypoxic-ischemic encephalopathy (HIE). Methods: Sixty newborns who fulfilled 3 or more of the clinical and biochemical criteria and developed HIE as defined by Levene staging were included in our study as the asphyxia group. Neonates with congenital malformation, systemic infection, intrauterine growth retardation, low-birth weight, cardiac or hemolytic disease, family history of neurological diseases, congenital or perinatal infections, preeclampsia, diabetes, and renal diseases were excluded from the study. Sixty healthy neonates matched for gestational age and with no maternal history of illness, established respiration at birth, and an Apgar score ≥7 at 1 and 5 minutes were included as the control group. IMA was determined by double-antibody enzyme-linked immunosorbent assay of a cord blood sample collected within 30 minutes after birth. Results: Cord blood IMA levels were higher in asphyxiated newborns than in controls (250.83±36.07 pmol/mL vs. 120.24±38.9 pmol/mL). Comparison of IMA levels by HIE stage revealed a highly significant difference among them (207.3±26.65, 259.28±11.68, 294.99±4.41 pmol/mL for mild, moderate, and severe, respectively). At a cutoff of 197.6 pmol/mL, the sensitivity was 84.5%, specificity was 86%, positive predictive value was 82.8%, negative predictive value was 88.3%, and area under the curve was 0.963 (P<0.001). Conclusion: IMA levels can be a reliable marker for the early diagnosis of neonatal HIE and can be a predictor of injury severity.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.1
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• pp.60-65
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• 2014

Smith-Lemli-Opitz syndrome (SLOS) is an autosomal recessive disease caused by a defect in cholesterol biosynthesis. This mutation encodes 7-dehydrocholesterol reductase (DHCR7), which is located on chromosome 11q13. It is characterized by typical facial appearances, microcephaly, small up-turned nose, cleft palate, syndactyly, and is correlated with cardiac, gastrointestinal and genital malformations. There may also be mental retardation, behavioral problems and growth retardation. It causes a broad spectrum of effects, ranging from a mild disorder of learning and behavior to a lethal malformation. There are four reports of Smith-Lemli-Opitz syndrome in Korean children. Here, we describe a two months old female with microcephaly, toe syndactyly and a cleft soft palate who was diagnosed as SLOS with c. 1054 C>T (p.R352W) and c.907G>A (p. G303R) mutations.

• Journal of Chest Surgery
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• v.16 no.2
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• pp.157-163
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• 1983

Ebstein's anomaly is a rare congenital cardiac malformation oand the ideal surgical correction seems controversial at present, and some problems are left unsolved in the surgical correction of this anomaly. Between June 1978 and June 1982, 12 patients with Ebsteins' anomaly underwent corrective open heart surgery at Seoul National University Hospital. Except for one patient, who had no ASD, all had a huge right atrium, secundum type ASD, and definite atrialized right ventricle. Typically, displaced tricuspid valve leaflets were found in all cases, but the degree of displacement and deformity were variable. In the point of NYHA functional classification, five were in class II, six were in class III, and one was in class IV. Ten patients were operated on by tricuspid valve replacement and pication. Two patients were operated on only by plication and annuloplasty techniques, and in all cases, ASD was closed. Postoperatively, four patients suffered from complete A-V block, and two of them died immediately. The remaining two patients took pacemaker generator implantation with good results. The other eight patients were in good condition. Tricuspid valve replacement using tissue valve and plication of the atrialized rght ventricle seems to be a good method of surgical correction for Ebstein's anomaly.

• Journal of Veterinary Science
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• v.22 no.1
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• pp.2.1-2.10
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• 2021

Background: Diseases related to cerebrospinal fluid flow, such as hydrocephalus, syringomyelia, and Chiari malformation, are often found in small dogs. Although studies in human medicine have revealed a correlation with cerebrospinal fluid flow in these diseases by magnetic resonance imaging, there is little information and no standard data for normal dogs. Objectives: The purpose of this study was to obtain cerebrospinal fluid flow velocity data from the cerebral aqueduct and subarachnoid space at the foramen magnum in healthy beagle dogs. Methods: Six healthy beagle dogs were used in this experimental study. The dogs underwent phase-contrast and time-spatial labeling inversion pulse magnetic resonance imaging. Flow rate variations in the cerebrospinal fluid were observed using sagittal time-spatial labeling inversion pulse images. The pattern and velocity of cerebrospinal fluid flow were assessed using phase-contrast magnetic resonance imaging within the subarachnoid space at the foramen magnum level and the cerebral aqueduct. Results: In the ventral aspect of the subarachnoid space and cerebral aqueduct, the cerebrospinal fluid was characterized by a bidirectional flow throughout the cardiac cycle. The mean ± SD peak velocities through the ventral and dorsal aspects of the subarachnoid space and the cerebral aqueduct were 1.39 ± 0.13, 0.32 ± 0.12, and 0.76 ± 0.43 cm/s, respectively. Conclusions: Noninvasive visualization of cerebrospinal fluid flow movement with magnetic resonance imaging was feasible, and a reference dataset of cerebrospinal fluid flow peak velocities was obtained through the cervical subarachnoid space and cerebral aqueduct in healthy dogs.