• 제목/요약/키워드: Multiple sclerosis

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다발성 심장 횡문근종과 동반된 활로씨 사징증 - 1예 보고 - (Tetralogy of Fallot Associated with Multiple Cardiac Rhabdomyomas - A case report -)

  • 정희석;장원경;윤태진
    • Journal of Chest Surgery
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    • 제42권6호
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    • pp.770-773
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    • 2009
  • 심장횡문근종은 영, 유아나 소아기에서 가장 발생 빈도가 높은 원발성 심장종양으로서, 다발성인 경우 빈번히 결절성 경화증을 동반한다. 저자들은 심장 횡문근종 및 결절성 경화증과 동반된 활로씨 사징증을 가진 환아에서 신생아기에 전신-폐 단락술을 시행하고 횡문근종이 자연적 소멸된 후 2세에 완전 교정술을 시행하여 양호한 결과를 얻었기에 보고하는 바이다.

근위축성측삭경화증 가족 돌봄제공자의 심리사회적 안녕감에 영향을 미치는 요인 (Factors Influencing Psychosocial Well-Being in Family Caregivers of People with Amyotrophic Lateral Sclerosis)

  • 추현식;탁영란;김승현
    • 대한간호학회지
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    • 제48권4호
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    • pp.454-464
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    • 2018
  • Purpose: The purpose of this study was to identify factors influencing psychosocial well-being in family caregivers of patients with amyotrophic lateral sclerosis (ALS). Methods: A descriptive correlational design was used. The transactional model of stress and coping was used to investigate the psychosocial well-being of 137 family caregivers of patients with ALS. Data were collected through self-reported questionnaires from January to November 2016. Data were analyzed using an independent t-test, one-way ANOVA, Pearson's correlation, and hierarchical multiple regression analysis with the SPSS WIN 21.0 program. Results: The regression model had an adjusted $R^2$ of .49, which indicated that meaning-focused coping, social support, ALS patient-family caregiver relationship (especially a spousal relationship), and tracheostomy were significant predictors of caregivers' psychosocial well-being. Conclusion: Meaning-focused coping and social support significantly influenced caregivers' psychosocial well-being. Therefore, interventions to improve caregivers' psychosocial well-being must focus on increasing meaning-focused coping and social support resources.

흉부질환을 병발한 Von-Recklinghausen`s Diseas (Von Recklinghausen`s Disease Involving the Chest)

  • 이선희
    • Journal of Chest Surgery
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    • 제21권4호
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    • pp.766-771
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    • 1988
  • Von Recklinghausen`s neurofibromatosis, tuberous sclerosis and encephalotrigeminal angiomatosis[Sturge-Kalischer-Weber syndrome] are frequently classified under the heading of organic neurocutaneous syndromes. Both neurofibromatosis and tuberous sclerosis are believed to represent instances of simple autosomal dominant heredity. Multiple neurofibroma and cafe*-au-lait spots are the hallmarks of the van-Recklinghausen`s disease. The characteristic features of the fully developed syndrome are [1] pigmentation of the skin, including cafe*-au-lait spots, pigmented freckles and males, and occasionally a generalized darkening of the skin; [2] subcutaneous nodules and deep neurofibromatous tumors and diffuse plexiform growths of neural tissue; [3] skeletal anomalies, especially scoliosis; and [4] predilection to malignancy. In recent years cystic lung disease, usually of the so-called honeycomb lung variety, has been reported on several occasions in patients with tuberous sclerosis. This association has been shown to our sporadically as well as in members of a single family. Little attention has been paid to the presence of cystic lung disease in association with neurofibromatosis. Currently, most think of thoracic involvement in neurofibromatosis in terms of posterior mediastinal neuroma, pheochromocytoma, meningocele or, less commonly, parenchymal pulmonary neurofibromatosis. Author have experienced a case of von Recklinghausen`s disease. This case developed a huge neurofibroma in the both side thorax and invaded to the Lt. 7th rib.

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Structure of a Human Insulin Peptide-HLA-DQ8 Complex and Susceptibility to Type 1 Diabetes

  • Lee, Kon-Ho
    • 한국생물물리학회:학술대회논문집
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    • 한국생물물리학회 2002년도 제9회 학술 발표회 프로그램과 논문초록
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    • pp.16-17
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    • 2002
  • The major histocompatibility complex (MHC) is an important susceptibility locus for many human autoimmune diseases. The structural and functional properties of HLA-DR molecules that are associated with susceptibility to several autoimmune diseases, such as rheumatoid arthritis and multiple sclerosis, have been defined.(omitted)

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Multiple Cancers in a Patient with Systemic Sclerosis and Aggravated Interstitial Lung Disease by Chemotherapy

  • Park, Chan Kwon;Lee, Seok Jong;Cho, Hyung Jun;Lee, Kyeong Soo;Kim, Sung Jun;Cho, Gu Min;Lee, Ha Ni
    • Tuberculosis and Respiratory Diseases
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    • 제75권3호
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    • pp.111-115
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    • 2013
  • Although the relationship between malignancy risk with systemic sclerosis (SSc) has been inconclusive, there are some previous studies for a positive correlation. Most patients with SSc have some degree of lung parenchymal involvement in the form of interstitial thickening and fibrosis. Interstitial lung disease is the most common pulmonary manifestation of SSc. Interstitial lung disease following chemotherapy (5-fluorouracil, leucovorin, and oxaliplatin [FOLFOX]) is an uncommon life-threatening complication and it is induced by oxaliplatin. We report a case of multiple cancers in a patient with SSc and aggravated interstitial lung disease by chemotherapy.

Sphingosine 1-Phosphate Receptor Modulators and Drug Discovery

  • Park, Soo-Jin;Im, Dong-Soon
    • Biomolecules & Therapeutics
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    • 제25권1호
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    • pp.80-90
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    • 2017
  • Initial discovery on sphingosine 1-phosphate (S1P) as an intracellular second messenger was faced unexpectedly with roles of S1P as a first messenger, which subsequently resulted in cloning of its G protein-coupled receptors, $S1P_{1-5}$. The molecular identification of S1P receptors opened up a new avenue for pathophysiological research on this lipid mediator. Cellular and molecular in vitro studies and in vivo studies on gene deficient mice have elucidated cellular signaling pathways and the pathophysiological meanings of S1P receptors. Another unexpected finding that fingolimod (FTY720) modulates S1P receptors accelerated drug discovery in this field. Fingolimod was approved as a first-in-class, orally active drug for relapsing multiple sclerosis in 2010, and its applications in other disease conditions are currently under clinical trials. In addition, more selective S1P receptor modulators with better pharmacokinetic profiles and fewer side effects are under development. Some of them are being clinically tested in the contexts of multiple sclerosis and other autoimmune and inflammatory disorders, such as, psoriasis, Crohn's disease, ulcerative colitis, polymyositis, dermatomyositis, liver failure, renal failure, acute stroke, and transplant rejection. In this review, the authors discuss the state of the art regarding the status of drug discovery efforts targeting S1P receptors and place emphasis on potential clinical applications.

Short-Term Lung Function Changes and Predictors of Progressive Systemic Sclerosis-Related Interstitial Lung Disease

  • Kaenmuang, Punchalee;Navasakulpong, Asma
    • Tuberculosis and Respiratory Diseases
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    • 제83권4호
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    • pp.312-320
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    • 2020
  • Background: Systemic sclerosis (SSc) involves multiple organ systems and has the highest mortality among connective tissue diseases. Interstitial lung disease is the most common cause of death among SSc patients and requires closer studies and follow-ups. This study aimed to identify lung function changes and predictors of progressive disease in systemic sclerosis-related interstitial lung disease (SSc-ILD). Methods: A retrospective study extracted SSc patients from an electronic database January 2002-July 2019. Eligible cases were SSc patients >age 15 diagnosed with SSc-ILD. Factors associated with progressive disease were analyzed by univariate and multivariate logistic regression analyses. Results: Seventy-eight SSc-ILD cases were enrolled. Sixty-five patients (83.3%) were female, with mean age of 44.7±14.4, and 50 (64.1%) were diffuse type SSc-ILD. Most SSc-ILD patients had crackles (75.6%) and dyspnea on exertion (71.8%), and 19.2% of the SSc-ILD patients had no abnormal respiratory symptoms but had abnormal chest radiographic findings. The most common diagnosis of SSc-ILD patients was non-specific interstitial pneumonia (43.6%). The lung function values of diffusing capacity of the lung for carbon monoxide (DLCO) and DLCO per unit alveolar volume declined in progressive SSc-ILD during a 12-month follow-up. Male and no previous aspirin treatment were the two significant predictive factors of progressive SSc-ILD with adjusted odds ratios of 5.72 and 4.99, respectively. Conclusion: This present study showed that short-term lung function had declined during the 12-month follow-up in progressive SSc-ILD. The predictive factors in progressive SSc-ILD were male sex and no previous aspirin treatment. Close follow-up of the pulmonary function tests is necessary for early detection of progressive disease.

Human-yeast genetic interaction for disease network: systematic discovery of multiple drug targets

  • Suk, Kyoungho
    • BMB Reports
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    • 제50권11호
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    • pp.535-536
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    • 2017
  • A novel approach has been used to identify functional interactions relevant to human disease. Using high-throughput human-yeast genetic interaction screens, a first draft of disease interactome was obtained. This was achieved by first searching for candidate human disease genes that confer toxicity in yeast, and second, identifying modulators of toxicity. This study found potentially disease-relevant interactions by analyzing the network of functional interactions and focusing on genes implicated in amyotrophic lateral sclerosis (ALS), for example. In the subsequent proof-of-concept study focused on ALS, similar functional relationships between a specific kinase and ALS-associated genes were observed in mammalian cells and zebrafish, supporting findings in human-yeast genetic interaction screens. Results of combined analyses highlighted MAP2K5 kinase as a potential therapeutic target in ALS.

Effects of Panax ginseng in Neurodegenerative Diseases

  • Cho, Ik-Hyun
    • Journal of Ginseng Research
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    • 제36권4호
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    • pp.342-353
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    • 2012
  • Ginseng, the root of the Panax ginseng, has been a popular and widely-used traditional herbal medicine in Korea, China, and Japan for thousands of years. Now it has become popular as a functional health food and is used globally as a natural medicine. Evidence is accumulating in the literature on the physiological and pharmacological effects of P. ginseng on neurodegenerative diseases. Possible ginseng- or ginsenosides-mediated neuroprotective mechanisms mainly involve maintaining homeostasis, and anti-inflammatory, anti-oxidant, anti-apoptotic, and immune-stimulatory activities. This review considers publications dealing with the various actions of P. ginseng that are indicative of possible neurotherapeutic efficacies in neurodegenerative diseases and neurological disorders such as Parkinson's disease, Alzheimer's disease, Huntington's disease, and amyotrophic lateral sclerosis and multiple sclerosis.