• 대한수의학회지
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• 제46권1호
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• pp.67-70
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• 2006

An 8-month-old 3.15 kg female Cocker-spaniel with history of ataxia referred to the Veterianary Medical Teaching Hospital, Chungnam National University. There were no abnormalities in CBC and serum chemical analysis. Agenesis of dens was found on dorsoventral view in cervical radiography. Compressed cervical spinal cord and enlarged cerebral ventricle were observed in magnetic resonance imaging. It was diagnosed as atlantoaxial instability with hydrocephalus. For conservative therapy, neck brace was applied and diuretics and prednisolone were administered. The dog's ataxia became better gradually.

• 대한소아치과학회지
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• 제28권2호
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• pp.316-322
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• 2001

감각신경아세포종으로 생후 1년 6개월부터 약 1년간 6주기의 화학요법과 생후 1년 6개월에 29회에 걸친 방사선 조사(AP 4540 R+LAT 1080 R : total 5620 R)를 받은 후, 후유증으로 상악치아 및 상악골의 발육장애를 보이는 10세 남아의 임상적, 방사선학적 관찰 및 치료 후 다음과 같은 지견을 얻었기에 보고하는 바이다. 1. 성장중인 어린이에서 악성종양의 치료를 위한 방사선 조사는 연조직 및 경조직(골, 연골, 치아)등의 성장장애를 유발할 수 있으므로 시술 전 충분한 고려가 필요하다. 2. 치배의 손상은 치관 및 치근의 형성장애를 유발하고 이에 따라 치조골의 성장장애가 나타나므로 치근이 없는 치아일지라도 치조골의 흡수를 억제하기 위하여 hawley type의 부분의치 등을 이용하여 잔존시켜야 한다. 3. 향후 성장이 완료된 이후(만 18세 이상)에 무치근 치아 및 무치악 부위에 틀니(denture)나 임플란트 등의 보철수복이 필요할 것으로 여겨진다.

• Molecules and Cells
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• 제39권6호
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• pp.501-507
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• 2016

The corpus callosum is a bundle of nerve fibers that connects the two cerebral hemispheres and is essential for coordinated transmission of information between them. Disruption of early stages of callosal development can cause agenesis of the corpus callosum (AgCC), including both complete and partial callosal absence, causing mild to severe cognitive impairment. Despite extensive studies, the etiology of AgCC remains to be clarified due to the complicated mechanism involved in generating AgCC. The biological function of PI3K signaling including phosphatidylinositol-3,4,5-trisphosphate is well established in diverse biochemical processes including axon and dendrite morphogenesis, but the function of the closely related phosphatidylinositol-3,4,-bisphosphate (PI(3,4)P2) signaling, particularly in the nervous system, is largely unknown. Here, we provide the first report on the role of inositol polyphosphate 4-phosphatase II (INPP4B), a PI(3,4)P2 metabolizing 4-phosphatase in the regulation of callosal axon formation. Depleting INPP4B by in utero electroporation suppressed medially directed callosal axon formation. Moreover, depletion of INPP4B significantly attenuated formation of Satb2-positive pyramidal neurons and axon polarization in cortical neurons during cortical development. Taken together, these data suggest that INPP4B plays a role in the regulating callosal axon formation by controlling axon polarization and the Satb2-positive pyramidal neuron population. Dysregulation of INPP4B during cortical development may be implicated in the generation of partial AgCC.

• Journal of Chest Surgery
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• 제40권1호
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• pp.60-62
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• 2007

폐 형성 저하증은 폐 무발생의 한 분류이다. 폐 무발생은 대부분 출생시 진단되는 매우 드문 질환으로, 심혈관계 기형과 같은 다른 기형이 흔히 동반된다. 소아기에 사망할 수도 있지만, 정상적인 성장을 하는 경우도 있다. 저자들은 수술 전 흉부 전산화 단층촬영에서 종격동의 심한 우측 편위와 우측 폐 실질의 허탈이 관찰되었던 폐 형성 저하증이 있는 젊은 여자에서 우측 측방 개흉술을 통한 승모판막 성형술을 시행하였다.

• Journal of Yeungnam Medical Science
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• 제22권2호
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• pp.241-246
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• 2005

체질성 8세염색체는 비교적 드문 염색체 이상으로 신생아 25,000-50,000명당 한 명 정도의 출생빈도를 보이며 여아보다 남아에서 발생 빈도가 높다. 순수한 8세염색체는 생존이 어려운 것으로 보이며 보고된 대부분의 증례는 섞임증이다. 저자들은 뇌량 무발생, 선천성 심장질환과 소악증을 가진 신생아에서 말초혈액 T림프구 핵형분석 및 형광동소교잡법을 통해 체질성 선천성 8세염색체 섞임증을 진단하였다. 체질성 8세염색체 섞임증의 표현형은 거의 정상에 가까운 형태에서 심한 신체적 혹은 지능 장애까지 매우 다양하지만 그 표현형의 정도가 전체 핵형에서 8세염색체 핵형이 차지하는 비율, 즉 섞임증의 정도와 비례하지는 않는다. 또한 조직마다 섞임증의 정도가 매우 다양하여 그 예후를 예측하기는 매우 어렵다. 8세염색체 세포에서 골수성 혈액질환의 발생빈도가 높은 것으로 알려져 있으므로 향후 주의깊은 추적관찰이 필요하다.

• 대한심미치과학회지
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• 제13권2호
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• pp.7-11
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• 2004

The Schwachman-Diamond syndrome is an autosomal recessive syndrome(1/20,000 births), consisting of pancreatic insufficiency, neutopenia, which may be intermittent, neutrophil chemotaxis defects, metaphyseal dysostosis, failure to thrive and short stature. Patients present in infancy with poor growth and grease, foul-smelling stools that are characteristic of malabsorption. These children can be readily differentiated from those with cystic fibrosis by their normal sweat chloride levels, lack of the cystic fibrosis gene, and characteristic metaphyseal lesions. Pathologically, the pancreatic acini are replaced by fat with little fibrosis. The neutropenia may be cyclic. Recurrent pyogenic infections otitis media, pneumonia, dermatitis(fig 1), sepsis are common and a frequent cause of death. In dental examination, these patients had a poor oral hygine and moderate generalized marginal gingivitis, also show delayed primary tooth exfoliation and oral development. This report illustrates a case that pancreatic agenesis 6 yeas-old boy with various esthetic dental problems has been served the esthetic dental restoration of 6 years.

• 대한치과교정학회지
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• 제47권5호
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• pp.323-333
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• 2017

Orthodontic treatment of a complex case that involves retained deciduous mandibular second molars with missing permanent successors is challenging. Usually, congenitally missing teeth are manifested with other dental anomalies that further complicate orthodontic treatment, such as retained deciduous teeth, impactions, transpositions and peg-shaped lateral incisors. Even though the long term prognosis of the retained deciduous tooth is not fully predictable, if the teeth are in good condition, the patient and clinician may incline towards a decision to preserve the deciduous teeth as long as possible. This case report demonstrates that deciduous teeth, in this case the mandibular second molars and maxillary canine, can be incorporated into final occlusion with clinically stable long-term results.

• Advances in pediatric surgery
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• 제1권2호
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• pp.181-185
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• 1995

Segmental intestinal dilatation is rare, which causes symptom of bowel obstruction and requires resection. The resection is not only diagnostic but also curative procedure. Recently, author experienced 2 cases of segmental dilatation of the ileum due to focal agenesis of the intestinal muscularis in 7 day & 4 day-old female neonates. The post operative recovery was excellant in the first case after resection of dilated ileum(15cm in length) and end to end anastomosis, and discharged at 20th day. But in the second case, the passage disturbance was not relieved after resection of dilated ileum (30cm in length), and author re-resected 80cm more of dilated proximal ileum at 2 weeks after the first operation. This baby discharged after diarrhea control with Loperin on 1 month after the second operation. Final histologic examination showed 1) normal population of ganglion cells in both narrowed & dilated ileum in both cases. 2) focal abscence of muscularis propria in both cases. 3) relative hypertrophy of inner circular muscle layer and thinned, multiple fragmented outer longitudinal muscle layer in case 2.

• Journal of Chest Surgery
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• 제15권1호
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• pp.21-26
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• 1982

Fourteen cases of funnel deformity, 11 were male and the others female, treated over a eleven-year period, are presented. The overall results with the methods described by the authors, Wada, Shannon, Adkins and Ravitch appear to be excellent. The symmetric depression was more common In children under the age of 12 years and asymmetric one Increased after the age of 12. Six cases of abnormal cardiac auscultatory findings revealed no consistent hemodynamic abnormalities. An 21-year-old female was associated with left lung agenesis and dextroversion of the heart, and a 6-year-old boy with congenital bronchogenic cyst who underwent right upper lobectomy. EKG changes associated with the anomaly were observed in all. Pulmonary function test showed the range from normal to moderate restriction which did not Improve in only one patient after operation, but the patient did not complain any restriction In activity. Some transient complications were developed which resulted in improvement.

• Journal of Chest Surgery
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• 제7권2호
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• pp.153-162
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• 1974

Four patients with funnel chest deformity corrected in the Department of Thoracic Surgery, Seoul National University Hospital are presented. The first case was a 21-year old female with cyanosis, clubbed fingers and systolic murmur on the left infrascapular region on physical examination associated with agenesis of the right lung. The deformity was of asymmetrical funnel chest, in which the left hemithorax was more sunken. She was corrected by the method of Funnel Costoplasty of Wada. The second case was a three years old boy whose anterior chest wall was symmetrically deformed, and he was corrected by the method of Ravitch using Adkins strut under the sternum. The third was a 22-year old man with symmetrical deformity, and was corrected by the method described by Shannon in 1973. The last patient was a 22-year old man and he had dyspnea on exertion, palpitation and apical systolic murmur with symmetrical funnel chest deformity. He was also corrected by Ravitch operation, All of them has excellent result.