• The Journal of Korea Assosiation for Disability and Oral Health
• /
• v.7 no.2
• /
• pp.103-106
• /
• 2011

다운 증후군(Down syndrome, Trisomy 21)은 가장 흔하게 나타나는 증후군으로 대략 1/1000의 확률로 태어난다. 세 개의 유전적 유형이 있지만, 95% 정도가 3개의 21번 염색체를 갖는 비분리 염색체 유형이다. 주된 구강 증상으로는 거대설, 균열설, 과잉치, 결손치, 왜소치, 유치와 영구치의 맹출지연 그리고 이에 따른 부정교합 등이 있다. 75%의 환자에서는 어린 나이에 치주질환에 이환되기 쉬우며, 치아우식증에는 낮은 이환율을 보인다는 보고가 있다. 급성 림프구성 백혈병(Acute Lymphoblastic Leukemia)은 주로 어린이에서 나타나며, 특히 다운 증후군 환자에서의 발병율은 정상인에 비해 20배 높다. 치은비대와 출혈의 양상이 주로 나타나며, 간혹 상악골과 구개골에 종괴가 관찰 되기도 한다. 본 증례에서는 서울대학교치과병원 소아치과에 내원한 환자 중 급성림프구성 백혈병을 앓고 있는 다운 증후군 환아가 있어 이를 보고하고자 한다.

• The Journal of Korean Academy of Prosthodontics
• /
• v.51 no.4
• /
• pp.339-346
• /
• 2013

In case of missing of permanent teeth by trauma or innate defect, the decision of treatment modalities and application timing have an important effect on the prognosis of oral rehabilitation. In this case report, interdisciplinary approach between the orthodontic and prosthodontic treatment, the way to re-establish the collapsed occlusal vertical dimension, and implant prosthetic considerations will be discussed. Proper diagnosis on teeth and craniofacial skeleton was made prior to treatment and provisional restorations were used in regard of growth patterns of the patient. Finally, the edentulous areas were restored with fixed implant prostheses. Diagnosis, treatment rationale and prognosis will be discussed thoroughly.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.19 no.1
• /
• pp.1-11
• /
• 2019

Purpose: Congenital hypothyroidism (CH) is the most common congenital endocrine disorder. The purpose of the present study was to determine the incidence of CH in South Korea during the period from January 1991 to March 2004. Methods: Central data from each city branch of SCL (Seoul Clinical Reference Laboratories) in Yongin, South Korea, was gathered and collectively analyzed. Newborn screening (NBS) for CH was based on measuring the levels of neonatal thyroid stimulating hormone (TSH) and free T4 (a cut-off of 20 mIU/L and less than 0.8 ng/dL, respectively). Results: During the study period, 671,805 live births were screened for CH based on TSH and free T4 ELISA assays. A total of 159 newborns were deemed positive for CH out of 671,805, with a corresponding incidence of 1 in 4,225. When a cut-off of 20 mIU/L was used in TSH assays, the associated sensitivity, specificity, and positive predictive values (PPV) were 100.0%, 99.7%, and 10.8%, respectively. When a cut-off of 0.8 ng/dL in free T4 assays was used, the associated sensitivity, specificity, and PPV were 100.0%, 98.5%, and 3.9%, respectively. Conclusion: CH incidence in South Korea as evidenced by the results of NBS was compared with its incidence and comparable to the other countries prior to 2004.

• The korean journal of orthodontics
• /
• v.10 no.1
• /
• pp.55-64
• /
• 1980

The purpose of this study was to investigate the interrelationship of the experimental group and control group by analyzing case histories, intraoral radiographs, orthopantomographs, intraoral slide films and dental casts. The data for this study were complied from 654 outpatients of the Department of Orthodontics, Seoul National University Hospital. The following conclusions were obtained. 1. When one or more thins molar teeth were congenitally missing, the incidence of the other congenitally missing teeth was high. 2. The frequency of congenitally missing teeth was comparatively higher in male, maxilla, class II and class III. 3. The congenitally missing srea of the third molar by Angle's classification was not significant. 4. The order of frequency of congenitally missing teeth was the third molar, the second premolar, the lateral incisor, the first premolar, the central incisor, the canine, the first molar, the second molar.

• Journal of Chest Surgery
• /
• v.30 no.10
• /
• pp.1024-1027
• /
• 1997

Anomalous origin of left coronary artery from pulmonary artery(ALCAPA) is a rare fatal congenital anormaly that needs early surgical intervention. Many reports say that the choice of operative procedure is reimplantation of the left coronary artery into the ascending aorta. We experienced the surgical management of a case of the ALCAPA. The patient was 44 days old and 3.45 kg weighed female baby who had a symptom of congest ve heart failure. She underwent implantation of coronary artery on the aorta with cardiopulmonary bypass and recovered without any complications.

• Maxillofacial Plastic and Reconstructive Surgery
• /
• v.11 no.2
• /
• pp.67-73
• /
• 1989

Sturge - Weber Syndrome is a congenital disorder and characterized by facial hemangioma following one or more divisions of the trigeminal nerve, epilepsy, mental retardation, contralateral hemiplegia, occlular involvement, gingival involvement. A 34 year old Korean man, a 25 year old Korean woman and a 48 year old Korean woman were found to have red - purple colored pigmentation on the hemifacial area and upper oral mucosal area.

• Journal of Chest Surgery
• /
• v.37 no.1
• /
• pp.76-79
• /
• 2004

The remote location of VSD relative to the aortic valve imposes considerable surgical difficulties in the repair of DORV with noncommitted VSD. We report a successful biventricular repair of the anomaly with VSD rerouting to pulmonary artery followed by arterial switching operation.

• Journal of Chest Surgery
• /
• v.32 no.9
• /
• pp.847-850
• /
• 1999

Tracheobronchomegaly or Mounier-Kuhn syndrome a is rare disease characterized by marked dilatation of the trachea and main bronchi. It is thought to be due to a congenital anomaly, but is uncertain. It has variable clinical manifestations from causing chronic respiratory infections and bronchiectasis to being asymptomatic for the lifetime. Recently, we experienced a case of Mounier-Kuhn syndrome patient with tracheal diverticulum, who had lung cancer. Our case is reported with literature reviews.

• Journal of Chest Surgery
• /
• v.41 no.5
• /
• pp.671-674
• /
• 2008

Jejunum and fasciocutaneous and myocutaneous flaps are theoretically recommended as esophageal substitutes in reconstruction of the esophagus after several occurrences of failed reconstruction. However, other esophageal substitutes should also be considered. Secondary esophageal reconstruction was successfully performed on a 24-year-old woman who had a history of cervical, thoracic, and abdominal operations and esophageal stricture secondary to several failed reconstructions for esophageal atresia and tracheo-esophageal fistula 21 years prior. The esophageal reconstruction was done subcutaneously by end-to-side anastomosis at the left cervical area using a deformed stomach graft. The patient was discharged with the ability to consume a regular diet after the operation.

• Journal of Chest Surgery
• /
• v.41 no.4
• /
• pp.512-515
• /
• 2008

An anomalous systemic arterial supply to the left basal segments without sequestration is a rare congenital abnormality within the spectrum of pulmonary sequestration. But this is rather different from the definition of pulmonary sequestration in that it has normal bronchial connections. We describe here our experience with surgical treatments for an anomalous systemic arterial supply to the left basal segments without sequestration, and this condition was confirmed preoperatively.