• Title/Summary/Keyword: Systemic Sclerosis

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A Case of Sj$\ddot{o}$gren's Syndrome with Multiple Bullae Secondary to Pulmonary Amyloidosis and Lymphocytic Infiltration of Interstitium and Bronchioles (폐 유전분증(Amyloidosis)과 다발성 수 (Bullae), 폐 간질내 임파구 침윤이 동반된 Sj$\ddot{o}$gren's 증후군 1예)

  • Kim, Dong-Il;Lim, Yun-Jeong;Oh, Yung-Ha;Kim, Hyung-Soo;Lee, Jin-Sung;Kim, Dong-Soon
    • Tuberculosis and Respiratory Diseases
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    • v.44 no.6
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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.

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Non-cirrhotic portal hypertension in an ankylosing spondylitis patient

  • Park, Sukki;Lee, Ji Hyun;Choi, Joon Sul;Kim, Hyun Woo;Shim, Beom Jin;Choi, Won Kyu;Kim, Sang Hyun
    • Journal of Yeungnam Medical Science
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    • v.35 no.1
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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.

Promising Pharmacological Directions in the World of Lysophosphatidic Acid Signaling

  • Stoddard, Nicole C.;Chun, Jerold
    • Biomolecules & Therapeutics
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    • v.23 no.1
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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.

Recognition of Transmembrane Protein 39A as a Tumor-Specific Marker in Brain Tumor

  • Park, Jisoo;Lee, Hyunji;Tran, Quangdon;Mun, Kisun;Kim, Dohoon;Hong, Youngeun;Kwon, So Hee;Brazil, Derek;Park, Jongsun;Kim, Seon-Hwan
    • Toxicological Research
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    • v.33 no.1
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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.

A Case of Mandible Osteomyelitis Mimicking Recurrent Tongue Cancer (재발성 설암으로 오인된 하악골 골수염 1예)

  • Park, Sangheon;Jung, Kwangjin;Park, Min Woo;Jung, Kwang-Yoon
    • Korean Journal of Head & Neck Oncology
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    • v.29 no.2
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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.

Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 5. Connective Tissue Disease Associated Interstitial Lung Disease

  • Koo, So-My;Kim, Song Yee;Choi, Sun Mi;Lee, Hyun-Kyung;Korean Interstitial Lung Diseases Study Group
    • Tuberculosis and Respiratory Diseases
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    • v.82 no.4
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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.

The Clinical Report on the Pulmonary Tuberculosis, Tuberculosis Meningitis (Pulmonary Tuberculosis, Tuberculous Meningitis의 임상치험 3례)

  • Son, Yun-Jung;Lim, Joong-Hwa;Lee, Do-Hyoung
    • The Journal of Internal Korean Medicine
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    • v.25 no.2
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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.

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Micro-channel Embedded Soft Contact Lens for Tear Biomolecule Sampling (눈물의 생체 분자 추출을 위한 미세유관이 탑재된 유연한 콘택트 렌즈)

  • Kim, Ockchul;Park, HyungDal;Song, Young-won;Kim, Jinseok
    • Journal of Biomedical Engineering Research
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    • v.37 no.5
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    • pp.178-185
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    • 2016
  • Tear is a promising biological fluid for non-invasive health monitoring. It has been studied in the past to be a possible candidate for the diagnosis of certain systemic diseases, such as breast cancer, multiple sclerosis, prostate cancer, and diabetes. However, currently existing methods for collecting and extracting tear from the human eye causes inconsistencies in the biomolecule concentrations of the tear sample due to the irritating nature of the process. In response, we designed and fabricated a microfluidic system embedded soft contact-lens for the purpose of tear sampling. The lens was then tested with artificial tear for its tear sampling capability, and found to be able to find concentration equilibrium within 50 minutes. Additionally, simulation was carried out to further optimize the design so that tear sampling rate matched the natural tear turn-over rate of 1 microliter per minute.

Tc-99m-MIBI Uptake in Active Pulmonary Tuberculosis : A Case Report (활동성 폐 결핵에서의 Tc-99m-MIBI 섭취 : 1예 보고)

  • Lee, Chang-Ho;Park, Chan-Hee;Hwang, Hee-Sung;Bae, Moon-Sun
    • The Korean Journal of Nuclear Medicine
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    • v.30 no.3
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    • pp.379-381
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    • 1996
  • Technetium-99m MIBI was developed as a myocardiac perfusion imagine agent and has been used effectively in the detection and post-therapeutic evaluation of various neoplasm such as thyroid, lung, bone and breast tumors. As an infrequent findings, Tc-99m MIBI agent has shown in non-neoplastic pulmonary conditions Including fibrosing alveolitis, pulmonary actinomycosis, active pulmonary sarcoidosis, pulmonary interstitial fibrosis in progressive systemic sclerosis and active osteomyelitis. In a recent report conducted by Cetin Oncel, Tc-99m MIBI imaging is an effective method in the detection and follow-up of pulmonary tuberculosis We have also experienced Tc-99m MIBI uptake in active pulmonary tuberculosis incidentally found in a patient with suspected proliferative villonodular synovitis of the left ankle.

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A Case Report on Scars from Autoimmune Disorder Treated with Incisional Needle Therapy (절개침법을 적용한 자가면역질환 흉터 치료 1례)

  • Joo-Yeon Cho;Tae-Kyung Lim;Min-Hee Kim
    • The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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    • v.37 no.1
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    • pp.107-112
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    • 2024
  • Objectives : This case report aims to demonstrate the effectiveness of incisional needle therapy in treating facial scars resulting from autoimmune disorder. Methods : A single case of a 29-year-old female patient with facial scars due to systemic sclerosis was treated with incisional needle therapy. The treatment was applied over 14 sessions from February to September 2023. The efficacy of the treatment was evaluated using clinical photographs and the Physician's Global Assessment(PGA). Results : The treatment significantly improved scar appearance and reduced blood vessel visibility. The PGA scores, with an average of 2.66, indicating moderate improvement. Conclusions : Incisional needle therapy demonstrated potential as an effective treatment for scars caused by autoimmune disorders, due to minimal side effects and non-interference with ongoing treatments. It offers a promising alternative to conventional treatments, suggesting a need for further research for wider applications.