• Journal of the korean academy of Pediatric Dentistry
• /
• v.32 no.2
• /
• pp.256-261
• /
• 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 Korea Institute of Information, Electronics, and Communication Technology
• /
• v.8 no.5
• /
• pp.367-372
• /
• 2015

In this study, locality of scleroderma was detected. Diagnostic method is difficult for scleroderma (skin curing; Scleroderma), and it is done by comparing the images of the normal subjects to the scleroderma patients, after performing monochrome processing. The saturation, brightness, and contrast are adjusted, and they were converted by using the process of Well Filter. As a result, the images were able to be used to clearly distinguish the symptoms of scleroderma. In addition, in a video of a healthy person, the line of sight of the observation given the image of scleroderma patients above sea level of height as $0^{\circ}$ is to implement the closing process to the rear Well Filter even only in so that the horizontal plane, and out at intervals of graph the amplitude difference of the video have I asked. The diagnostic criteria were determined for the healthy subjects and the scleroderma patients.

• Clinical and Experimental Pediatrics
• /
• v.51 no.7
• /
• pp.766-770
• /
• 2008

Primary tumors of the heart are uncommon among pediatric patients. Rhabdomyoma is the most frequent cardiac tumor in infants and children, which is commonly associated with tuberous sclerosis. Tuberous sclerosis is a neurocutaneous syndrome affecting the brain, heart, skin, and other organs. Cardiac rhadomyomas are reported in 50-64% of infants with tuberous sclerosis. Tuberous sclerosis involves multiple locations in the atrium, ventricle and septum, and may induce mechanical obstruction of the outflow tract and heart failure depending on the location, number, size, and degree of invasion of tumors. Arrhythmias may also develop in infants with cardiac rhadomyomas, but only a few of these patients require prolonged anti-arrhythmic therapy because arrhythmia often disappears with spontaneous regression of the tumors, and the ultimate prognosis may be decided by the cerebral manifestations.

• Korean Journal of Head & Neck Oncology
• /
• v.25 no.2
• /
• pp.150-152
• /
• 2009

혈관근육지방종은 지방, 평활근, 두꺼운 벽의 혈관이 다양한 비율로 구성된 흔하지 않은 과오종이며, 이는 결정 경화증을 동반하거나 산발적으로 발생한다. 저자들은 혈관근육지방종이 드물게 발생하는 두경부에서 발생한 귓바퀴 1예, 구개점막 2예의 혈관근육지방종을 보고하고자 한다. 병리소견상, 3예 모두에서 성숙지방조직, 불규칙한 혈관, 그리고 HMB-45와 Melan-A에 음성을 보이는 평활근육세포로 이루어진 조직소견을 보였으며, 여러군데에서 림프구 침윤이 3예 모두에서 관찰되었다. 점막피부 혈관근육지방종으로 진단하였다. 세증례 모두 결절경화증은 동반되지 않았다. 점막피부 혈관근육지방종에서는 혈관주변세포가 HMB-45와 Melan-A에서 음성을 보였으며, 이는 간이나 신장의 혈관근육지방종에서의 특징적인 양성반응과는 다른 점이었다. 간이나 신장에서 생긴 혈관근육지방종과 다른 임상병리적 특징을 비교 기술하고자 두경부에서 발생한 점막피부 혈관근육지방종 3예를 보고한다.

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

• Childhood Kidney Diseases
• /
• v.18 no.2
• /
• pp.132-136
• /
• 2014

Tuberous sclerosis complex (TSC) is a genetic disorder that affects multiple organ systems and causes tumors. It is important that physicians are aware of the manifestations of TSC, and that they follow the recommendations for screening and evaluation. Several types of renal abnormalities may develop in individuals with TSC. Individuals with TSC may require ongoing treatment that can be adapted for each arising manifestation of renal disease. Herein, we report 4 patients with TSC who presented with a range of different renal manifestations, including angiomyolipoma, renal cell carcinoma, renal infarction, renal cyst, and nephrolithiasis.

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

• Childhood Kidney Diseases
• /
• v.11 no.1
• /
• pp.100-105
• /
• 2007

Cockayne syndrome is a rare autosomal recessive disorder characterized by cachectic dwafism, mental retardation, loss of facial subcutaneous adipose tissue, microcephaly and photosensitive dermatitis. It is associated with renal abnormalities characterized by hyalinization of glomeruli, atrophy of tubules and interstitial fibrosis. To our knowledge, this is the first report of a case of Cockayne syndrome with FSGS in Korea. A 7-year old boy was admitted for evaluation of hypertension and proteinuria, which were detected 2 month ago. He was followed for short stature(<3 percentile), mental retardation(IQ 55), strabismus and dental caries since 3 years ago. He also showed microcephaly, a bird-like face and relatively large hands and feet. Laboratory findings showed decreased creatinine clearance($C_{Cr}$ 76.1 mL/min/$1.73m^2$) and proteinuria(1,548 mg/day). Renal biopsy demonstrated focal segmental glomerulosclerosis of the hilar type with large hyaline deposits, moderate tubular atrophy and interstitial fibrosis. His cardinal features, mental retardation, and renal biopsy findings were consistent with Cockayne syndrome. We report here a very rare case of Cockayne syndrome with FSGS presenting with proteinuria and hypertension.

• Clinical and Experimental Pediatrics
• /
• v.50 no.2
• /
• pp.178-181
• /
• 2007

Purpose : The renal manifestations of tuberous sclerosis complex (TSC) are remarkably diverse, including polycystic kidney disease, simple renal cysts, renal cell carcinomas, and angiomyolipomas. All of these occur in children as well as adults in TSC. Angiomyolipomas, which can cause spontaneous life-threatening hemorrhages, are by far the most prevalent and the greatest source of morbidity. Here, we will address our experience, adding to the literature on pediatric patients with TSC requiring evaluation and treatment for renal manifestations. Methods : A retrospective analysis was made on 19 patients in whom TSC was diagnosed between May 2001 and Oct. 2005 at Severance Hospital. All patients had clinical diagnoses of TSC as defined by the 1998 tuberous sclerosis complex consensus conference. Results : The patients consisted of 13 boys and 6 girls with a mean age of 7.3 years (range 1 to 22). The renal disease associated with TSC included angiomyolipoma in nine patients (47.4 percent), renal simple cyst in one (5.3 percent), hydronephrosis in one (5.3 percent) patient. Eight patients (42.1 percent) presented with normal kidney contours at abdominal ultrasonography. One patient underwent renal replacement therapy due to chronic renal insufficiency after nephrectomy. Hemorrhage from angiomyolipoma was not detected. Conclusion : In our review of 19 cases of TSC, renal manifestations are reported in 57.9 percent of patients. Asymptomatic angiomyolipoma associated with TSC grow gradually, although severe hemorrhages are rare. So patients with TSC should be followed up with serial computerized tomography or abdominal ultrasonography. And also, renal function should be monitored conservatively.

• Journal of TMJ Balancing Medicine
• /
• v.13 no.1
• /
• pp.32-38
• /
• 2023

Objectives: The purpose of this study is to report the effect of Korean medicine on trigeminal neuralgia in a patient with systemic sclerosis. Methods: We treated a 49-year-old male patient who has systemic sclerosis and trigeminal neuralgia by Korean medicine. He was treated from October 29th, 2022 to November 12th, 2022. The treatment applied was herbal medicine, acupuncture, pharmacopuncture. The evaluation was conducted by Visual Analogue Scale (VAS). Results: After 14 days of treatment, the symptoms were alleviated. Facial pain and dullness of taste improved. VAS of discomfort was decreased from 10 to 2. Conclusions: These results showed that Korean medicine may have an effect on reducing symptoms of systemic sclerosis and trigeminal neuralgia.