• Tuberculosis and Respiratory Diseases
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• v.55 no.5
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• pp.522-525
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• 2003

26세 여자환자가 우측 흉통을 주소로 응급실을 방문하였다. 흉부방사선검사에서 우측 기흉을 진단받고 흉관삽입술을 시행하였다. 환자는 6년전부터 레이노드 현상이 있었고 양손에 피부경화증이 있었으며 항 DNA 국소이성화효소 I 항체가 양성으로서 전신성 경화증을 진단받았다. 고해상력 컴퓨터단층촬영에서 양쪽 폐야에 낭종성 병변이 관찰되었고 기흉은 낭종의 파열에 의한 것으로 판단되었다. 전신성 경화증은 폐를 포함한 여러 장기를 침범하는 질환이다. 이 질환에서 기흉과 낭종성 폐병변이 발생하였던 증례들은 1954년 이래로 보고되어 왔는데 모든 증례에서 기흉은 전신성 경화증을 진단받은 뒤에야 발견된 것이었다. 본 증례는 기흉으로 발현한 전신성 경화증의 첫 증례라는 점에서 임상적 의의가 있다고 여겨진다.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.2
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• pp.256-261
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• 2005

Scleroderma is a connective tissue disease of unknown etiology, but known as a kind of auto-immune disease. It is most common in women especially in $30{\sim}50$ years, and very rare in childhood. It can be classified into two main classes : localized scleroderma & systemic sclerosis. Localized type has better prognosis, and usually involves skin only, or in some cases, the muscle below, except inner organs. Systemic type involves skin, oral mucosa and major internal organs. Involving facial skin, we can see small and sharp nose, expressionless stare and narrow oral aperture. Usually they have Raynaud's phenomenon, and in progress, show mouth opening limitation and sclerosis of tongue and gingiva. It is called CREST syndrome showing calcinosis cutis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangioectasia. Treatment of scleroderma is systemic and localized steroid therapy, use of collagen-link inhibitor (D-penicillamine), immune depressor and etc. Mouth opening limitation can be improved by mouth stretching exercise. We report a 6 years old boy, diagnosed with localized scleroderma who had mouth opening limitation. We could get additional mouth opening, and have done successful restorative treatment of mandibular and maxillary 1st and 2nd deciduous molar under deep sedation with nitrous oxide and enflurane.

• The Journal of Korean Academy of Prosthodontics
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• v.59 no.1
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• pp.116-125
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• 2021

A three-dimensional (3D) intraoral scanner, which is one of the major developments in digital dentistry, is widely used in fixed prosthodontics. The application of intraoral scanner is now increasing in removable prosthodontics. Sclerotic change induced by scleroderma causes the limitation of mouth opening and multiple loss of the teeth. Conventional prosthodontic procedures are challenging for patients with this disease. This study showed a case of digital approach to the removable prosthodontic treatment of a patient who had the scleroderma and the consequent microstomia. At the provisional stage, the optical impression of patient's oral structures was digitally obtained. Using a 3D printer, the provisional dentures were fabricated. After extraction of hopeless tooth, the definitive digital impression was taken and the metal frameworks were fabricated, based on the data acquired from the impression. The definitive removable partial dentures were completed and delivered to the patient, who was satisfied with the prostheses.

• Journal of Chest Surgery
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• v.42 no.6
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• pp.770-773
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• 2009

Rhabdomyoma has been reported to be the most common type of cardiac tumors in fetuses and children, and multiple cardiac rhabdomyomas almost certainly signify the association with tuberous sclerosis. We report here on a case of Tetralogy of Fallot (ToF) that was associated with multiple rhabdomyomas and tuberous sclerosis. A two-year-old boy, who had undergone systemic-pulmonary shunt during the neonatal period, received total correction of his ToF after the complete regression of the cardiac tumor. The postoperative course was uneventful, and he has been follow-up for 2 months. The boy is currently in an excellent condition.

• The Journal of the Korean dental association
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• v.23 no.10 s.197
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• pp.875-879
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• 1985

A 29-year-old male patient was referred to Dept. of Dentistry due to halitosis and food impaction, who had been diagnosed as progressive systemic sclerosis at Dept. of Dermatology, SNUH. Oromaxillofacially lips were slightly thin, rigid and shiny. Pale gingiva and pinched nose were found also. Radiographically, uniform extreme widening of periodontal ligament space was found.

• Journal of Veterinary Clinics
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• v.17 no.2
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• pp.490-494
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• 2000

2달령의 수컷 로트와 일러가 4일간 설사와 통증.보행 실조 등의 증상을 보여 (주)해 마루 소동물 임상 의자 연구소에 의뢰되었다 흉부 방사선 검사에서 폐야 전반에 걸친 페포성 침윤과 흉벽의 심란 비후가 관찰되어 흉막폐렴으로 진단하였으며 복강 초음파 검사에서 상 복부에서 소량의 복수가 관찰되었고 전반적인 간 echogenicity가 증가되어 있었다. 치료 후 설사 증상은 사라지고 전신 상태가 다소 호전되어 내원 후 7일째에 흉부 방사선 검사를 재 실시하였다. 흉막과 폐의 병변은 변화가 없었으나 상완골과 요골의 골간단 부분에 이상 소견 이 발견되어 골격계 방사선 검사를 실시한 결과 상완골, 요골, 대퇴골 그리고 경골의 골간단에 경화성 변화와 무정형의 골막 반응이 관찰되었고 요골, 척골과 경골의 골간단 부분에 성 장판 외의 방사선 투과성 선이 관찰되었다. 이상의 특이적인 방사선 소견을 바탕으로 비록 본 질환의 정확한 원인은 밝힐 수 없었으나, 감염성 원인에 의한 비대성 골이형성증으로 진단하였다.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.4 no.2
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• pp.77-81
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• 2008

골화석증(Osteopetrosis)은 전신의 뼈가 대리석 모양으로 골 경화를 일으키며 골수강의 폐쇄 및 골의 취약성을 보이는 드문 질환이다. 성인형(Adult form)은 우성 유전되며 경미한 증상을 나타낸다. 반면에 유아형(Infantile form)은 열성 유전되면서 심각한 증상을 나타내고, 조기에 사망에 이를 수도 있다. 골화석증은 골격계, 조혈계, 신경계에서 다양한 증상을 유발한다. 본 증례의 목적은 드물게 발생하는 유아형 골화석증을 가진 2 명의 환아를 치료한 후, 그 질환과 치과적 관리에 대해 고찰하고자 함이다. 첫 번째 증례는 골화석증으로 진단된 5세 8개월 남환이었다. 임상 및 방사선 검진 결과 악골 및 치아의 변형을 나타내었으나, 특별한 병적 소견이 없어 예방 치료 및 주기적인 검진 시행하였다. 두 번째 증례는 중증도의 치아우식증을 가진 환아로 의료진과의 협조 하에 수혈한 후 발치하였다. 유아형 골화석증은 다양한 구강 합병증을 유발하며, 질환의 심각성에 의해 치료 자체도 어렵다. 치과의사로서 골화석증 환아를 관리할 때에는 건전한 구강 상태를 유지하기 위한 지속적인 관심과 예방 치료가 필요하며, 아무리 간단한 발치나 치과 술식이더라도 의료진과 협조를 해야 한다.

• Journal of Chest Surgery
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• v.36 no.11
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• pp.858-861
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• 2003

The main cause of ischemic heart disease combined with aortic valve disease is the systemic atherosclerotic process. Coronary artery embolism by a particle from the calcified aortic valvular tissue is very rare. A 73-year-old female patient was admitted due to chest tightness of recent onset. Two dimensional echocardiogram showed severe calcific aortic valve stenosis. Preoperative coronary angiogram exhibited a stenotic lesion at the distal right coronary artery, which seemed to be embolic in origin. The coronary embolus was removed through the coronary arteriotomy and then the arteriotomy site was repaired by onlay patch angioplasty technique. Aortic valve was replaced by a bioprosthetic valve. The embolus was reported as a fibrocalcified particle of diseased valve.

• Journal of Chest Surgery
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• v.39 no.8 s.265
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• pp.619-625
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• 2006

Background: Peripheral arterial disease is frequently accompanied with systemic arteriosclerosis and more than half of the cause of deaths is due to the development of coronary arterial disease. Moreover, it is known that the most frequent cause of death after a bypass surgery of chronic arterial obstruction is heart related complications. Especially in patients with atherosclerotic arterial obstruction in the lower extremities who had no history of heart disease or had no presenting symptoms of ischemic heart disease showed a high rate of postoperative mortality and for this reason we suggest preoperative evaluation in these patients to evaluate whether or not coronary arterial disease is accompanied. Material and Method: Since Feb. 2001 to Oct. 2004, we analyzed 52 patients who were operated on for atherosclerotic arterial obstruction in the lower extremities, with the exception of patients with a past history of heart disease or symptoms of ischemic heart disease. They underwent on the same day a coronary and femoral angiography for evaluation of accompanying coronary arterial disease. Of among these patients, we compared those who received bypass surgery of the arteries of the peripheral extremities alone to those who underwent combined coronary artery bypass surgery. Result: 63% of the reported cases of atherosclerotic arterial obstruction in the lower extremities were accompanied with coronary arterial disease. Old age, hypertension, diabetes mellitus, smoking, and hypercholesterolemia are known risk factors for arteriosclerosis and of these, only old age and hypertension had statistically significance in patients with severe atherosclerotic arterial obstruction in the lower extremities accompanied with coronary arterial disease. Diabetes, smoking, and hypercholesterolemia showed no statistical significance in this group. With the increase in severity of the range and the degree of atherosclerotic arterial obstruction, coronary arterial disease is frequently accompanied and its severity also increased. Patients who received both peripheral artery and coronary artery bypass surgery showed no difference in the period of hospitalization and ICU stay period compared with patients who received bypass surgery of the arteries of the lower extremities alone. Conclusion: Patients with atherosclerotic arterial obstruction in the lower extremities without symptoms of ischem to evaluate coronary arterial disease for active treatment, especially in the patients with old age, hypertension and high AVD scores.

• Tuberculosis and Respiratory Diseases
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• v.47 no.2
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• pp.150-160
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• 1999

Background: The CREST syndrome is an indolent form of progressive systemic sclerosis. Although its clinical progress is indolent, pulmonary hypertension(PH) associated with CREST syndrome have grave prognosis with over 40 percent mortality rate at 2 year follow-up. But the pathogenesis of pulmonary hypertension in this disease is not known, and classified as either primary or secondary PH. Clonality of endothelial cell proliferation in plexiform lesion is a molecular marker which allows distinction between primary and secondary PH. We performed this study to know whether the PH associated with CREST syndrome is a variant of primary PH or is a secondary PH. Methods: We assessed the X-chromosome inactivation based on the methylation pattern of the human androgen-receptor gene by PCR(HUMARA). Endothelial cells in plexiform lesions from female patients(n=3) with PH associated with CREST syndrome were microdissected from paraffin blocks. Vascular smooth muscle cells and lung parenchyma were also microdissected for clonality studies. Results: The proliferating endothelial cells in 14 plexiform lesions were all polyclonal. Similarly proliferated smooth muscle cells from 5 vessels with medial hypertrophy were also polyclonal. Conclusion: These results suggest that the pulmonary hypertension associated with CREST syndrome has different pathogenesis from primary PH and to be classified as secondary PH.