• 대한소아치과학회지
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• 제32권2호
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• pp.256-261
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• 2005

피부 경화증(scleroderma)은 만성 결체조직 질환으로 정확한 원인은 밝혀지지 않았으나 자가 면역 질환으로 알려져 있다. 대개 $30{\sim}50$세의 여성에서 호발하며 소아에서는 매우 드물다. 장기의 이환여부에 따라 국소성과 전신성 피부 경화증으로 분류할 수 있다. 국소적 형태는 예후가 양호하고 피부에만, 또는 피하 근육까지 이환되며, 내부 장기에는 이환되지 않는다. 전신적인 형태는 피부와 구강점막 및 위장계, 호흡계, 심혈관계 등을 포함하늘 전신 다발적 발생 (multisystemic involvement)이 특징적이다. 안면피부 이환시 작고 예리한 코, 무표정한 응시 (expressionless stare), 좁아진 입모양(narrow oral aperture) 등이 특징적이다. 대개 레이노 현상(Raynaud's phenomenon)이 존재하며 질환이 진행되면서 개구장애와 혀, 치은의 경화가 나타나게 된다. 피부 석회증(calcinosis cutis), 레이노현상(Raynaud's phenomenon). 식도 기능부전(esophageal dysfunction), 공지증(sclerodactyly), 모세혈관 확장증(telangioectasia)이 복합된 질환을 크레스트 증후군(CREST syndrome)이라 부른다. 피부 경화증의 치료는 국소적 및 전신적 스테로이드 치료, collagen cross-link inhibitor (D-penicillamine), 면역억제제 등을 사용한다. 개구제한의 치료는 설압자를 이용한 mouth stretching exercise를 통해 개선될 수 있다. 본 증례에서는 국소성 피부 경화증으로 진단되고 개구제한이 있는 6세 남아에게 아산화질소와 enflurane을 이용한 깊은 진정요법을 이용하여 상하악 제 1, 2 유구치의 우식치료 치험례를 보고한다.

• Journal of Oral Medicine and Pain
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• 제32권1호
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• pp.69-79
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• 2007

하악과두 흡수(condylar resorption)는 하악과두 용해라고도 하며 과두형태변화와 크기 감소가 점진적으로 또는 갑작스럽게 일어나는 것으로 정의할 수 있다. 원인은 잘 알려져 있지 않지만 류머티스 관절염, 전신성 홍반성 낭창, 스테로이드 복용(usage), 교정치료, 악교정 수술후 일어나는 과두흡수(secondary condylar resorption)와 특별한 원인 없이 일어나는 특발성 과두흡수(primary condylar resortion)로 나눌 수 있다. 이러한 하악과두 흡수가 발생하는 경우에는 골소실로 인해 이환측 과두에서 후방 지지의 갑작스런 상실이 초래된다. 이로인해 하악이 변위될수 있으며 교합변화, 개방교합과 같은 교합변화와 하악의 후퇴가 나타날 수 있다. 이에 저자는 하악과두 흡수가 일어난 증례를 보고하며 과두흡수와 관련된 인자, 병리기전, 환자관리에 대해 고찰하고자 한다.

• Tuberculosis and Respiratory Diseases
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• 제44권6호
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• pp.1426-1432
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• 1997

Sj$\ddot{o}$gren's syndrome(SS) is a chronic inflammatory disorder characterized by lymphocytic infiltration of lacrimal and sailvary glands, which results in dry eyes and dry mouth. SS may exist as a primary condition or as a secondary condition in association with connective tissue disease such as rheumatoid arthritis, systemic lupus erythematosus, or progressive systemic sclerosis. We experienced a patient with primary SS who developed multiple bullae, nodular type of pulmonary amyloidosis and lymphocytic interstitial peumonitis. We believe this to be the first reported case of SS acompanied by these three types pulmonary manifestations at the same time.

• Journal of Yeungnam Medical Science
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• 제35권1호
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• pp.89-93
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• 2018

Idiopathic non-cirrhotic portal hypertension (INCPH) is a disease with an uncertain etiology consisting of non-cirrhotic portal hypertension and portal pressure increase in the absence of liver cirrhosis. In INCPH, patients exhibit normal liver functions and structures. The factors associated with INCPH include the following: Umbilical/portal pyremia, bacterial diseases, prothrombic states, chronic exposure to arsenic, vinyl chloride monomers, genetic disorders, and autoimmune diseases. Approximately 70% of patients present a history of major variceal bleeding, and treatment relies on the prevention of complications related to portal hypertension. Autoimmune disorders associated with INCPH are mainly systemic sclerosis, systemic lupus erythematosus and rheumatoid arthritis. To the best of our knowledge, a case of ankylosing spondylitis (AS) associated with INCPH has not been reported thus far. Therfore, we report our experience of a patient with AS accompanied by INCPH, who showed perisplenic varices with patent spleno-portal axis and hepatic veins along with no evidence of cirrhosis on liver biopsy, and provide a brief literature review.

• Biomolecules & Therapeutics
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• 제23권1호
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• pp.1-11
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• 2015

Lysophosphatidic acid (LPA) is a signaling lipid that binds to six known lysophosphatidic acid receptors (LPARs), named $LPA_1-LPA_6$. These receptors initiate signaling cascades relevant to development, maintenance, and healing processes throughout the body. The diversity and specificity of LPA signaling, especially in relation to cancer and autoimmune disorders, makes LPA receptor modulation an attractive target for drug development. Several LPAR-specific analogues and small molecules have been synthesized and are efficacious in attenuating pathology in disease models. To date, at least three compounds have passed phase I and phase II clinical trials for idiopathic pulmonary fibrosis and systemic sclerosis. This review focuses on the promising therapeutic directions emerging in LPA signaling toward ameliorating several diseases, including cancer, fibrosis, arthritis, hydrocephalus, and traumatic injury.

• Korean Circulation Journal
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• 제53권1호
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• pp.52-54
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• 2023

• Toxicological Research
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• 제33권1호
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• pp.63-69
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• 2017

Transmembrane protein 39A (TMEM39A) belongs to the TMEM39 family. TMEM39A gene is a susceptibility locus for multiple sclerosis. In addition, TMEM39A seems to be implicated in systemic lupus erythematosus. However, any possible involvement of TMEM39A in cancer remains largely unknown. In the present report, we provide evidence that TMEM39A may play a role in brain tumors. Western blotting using an anti-TMEM39A antibody indicated that TMEM39A was overexpressed in glioblastoma cell lines, including U87-MG and U251-MG. Deep-sequencing transcriptomic profiling of U87-MG and U251-MG cells revealed that TMEM39A transcripts were upregulated in such cells compared with those of the cerebral cortex. Confocal microscopic analysis of U251-MG cells stained with anti-TMEM39A antibody showed that TMEM39A was located in dot-like structures lying close to the nucleus. TMEM39A probably located to mitochondria or to endosomes. Immunohistochemical analysis of glioma tissue specimens indicated that TMEM39A was markedly upregulated in such samples. Bioinformatic analysis of the Rembrandt knowledge base also supported upregulation of TMEM39A mRNA levels in glioma patients. Together, the results afford strong evidence that TMEM39A is upregulated in glioma cell lines and glioma tissue specimens. Therefore, TMEM39A may serve as a novel diagnostic marker of, and a therapeutic target for, gliomas and other cancers.

• 대한두경부종양학회지
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• 제29권2호
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• pp.65-67
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• 2013

Osteomyelitis is an infection of bone or bone marrow, caused by pyogenic bacteria or mycobacterium. Osteomyelitis can be acute or chronic, inflammatory process of the bone and its structures. Chronic osteomyelitis will result in variable sclerosis and deformity of the affected bone. With an infection of the bone, the subsequent inflammatory response will elevate this overlying periosteum, leading to a loss of the nourishing vasculature, vascular thrombosis, and bone necrosis, resulting occasionally in formation of sequestra. These become areas that are more resistant to systemic antibiotic therapy due to lack of the normal Havesian canals that are blocked by scar tissue. At this aspect, not only systemic antibiotic therapy, but also surgical debridement maybe required to remove the affected bone and prevent disease propagation to adjacent areas. We experienced a patient who diagnosed tongue cancer and underwent wide partial glossectomy few years before, with an ulcerative lesion around right retromolar trigon. We diagnosed cancer recurrence because PET indicated hot uptake on mandible which was nearby previous tongue tumor site. The patient received hemiglossectomy via paramedian mandibulotomy, partial mandibulectomy and fibula osteocutaneous free flap reconstruction. But final diagnosis was mandible osteomyelitis on pathology report. Here, we present the case with a review of the related literatures.

• Tuberculosis and Respiratory Diseases
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• 제82권4호
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• pp.285-297
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• 2019

Connective tissue disease (CTD) is a collection of disorders characterized by various signs and symptoms such as circulation of autoantibodies in the entire system causing damage to internal organs. Interstitial lung disease (ILD) which is associated with CTD is referred to as CTD-ILD. Patients diagnosed with ILD should be thoroughly examined for the cooccurrence of CTD, since the treatment procedures and prognosis of CTD-ILD are vary from those of idiopathic interstitial pneumonia. The representative types of CTD which may accompany ILD include rheumatoid arthritis, systemic sclerosis (SSc), Sjogren's syndrome, mixed CTD, idiopathic inflammatory myopathies, and systemic lupus erythematous. Of these, ILD most frequently co-exists with SSc. If an ILD is observed in the chest, high resolution computed tomography and specific diagnostic criteria for any type of CTD are met, then a diagnosis of CTD-ILD is made. It is challenging to conduct a properly designed randomized study on CTD-ILD, due to low incidence. Therefore, CTD-ILD treatment approach is yet to been established in absence of randomized controlled clinical trials, with the exception of SSc-ILD. When a patient is presented with acute CTD-ILD or if symptoms occur due to progression of the disease, steroid and immunosuppressive therapy are generally considered.

• 대한한방내과학회지
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• 제25권2호
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• pp.352-360
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• 2004

Tuberculosis, one of the oldest diseases known to affect humans, is caused by bacteria belonging to the Mycobacterium luberculosis complex. The diseases usually affects the lungs, although in up to one-third of cases other organs are affected. If properly treated, tuberculosis caused by drug-susceptible strains is curable in virtually all cases. If untreated, the disease may be fatal within 5 years in more than half of cases. Transmission usually takes place through the airborne spread of droplets of nuclei produced by patients with infectious pulmonary tuberculosis. Two patients with Pulmonary Tuberculosis and one with Tuberculous Meningitis were recently examined. The changes in these patient's symptoms through both western medical treatment and oriental medical treatment are reported.