• Title/Summary/Keyword: 관절염질환

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A Boy With Blau Syndrome Misdiagnosed as Refractory Kawasaki Disease

  • Kyungwon Cho;Yoonsun Yoon;Joon-sik Choi;Sang Jin Kim;Hirokazu Kanegane;Yae-Jean Kim
    • Pediatric Infection and Vaccine
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    • v.29 no.3
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    • pp.166-172
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    • 2022
  • Blau syndrome is a systemic autoinflammatory disease presenting with non-caseating granulomatous dermatitis, chronic uveitis, and arthritis. It is caused by a gain-of-function variant of the nucleotide-binding oligomerization domain protein 2 gene, which leads to the overactivation of inflammatory cytokines and eventually causes autoinflammation. Since the symptoms of Blau syndrome are nonspecific and usually do not appear simultaneously, it is challenging to differentiate Blau syndrome from other inflammatory disorders. This is a case report of a 13-month-old boy who had suffered from recurrent skin rash and fever. The patient was previously misdiagnosed as refractory Kawasaki disease twice and was treated with intravenous immunoglobulin and systemic glucocorticoid, which only resulted in transient improvement of the symptoms. He was eventually diagnosed with Blau syndrome.

N'-[(2-Hydroxy-1-naphthyl)methylene]arylhydrazides as Potent HIF-2α Inhibitors (N'-[(2-Hydroxy-1-naphthyl)methylene]arylhydrazide 화합물의 HIF-2α 저해 활성)

  • Lee, Hyosung
    • Journal of the Korea Convergence Society
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    • v.13 no.1
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    • pp.161-166
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    • 2022
  • HIF-2α is a transcription factor activated mainly in hypoxic condition known to play crucial roles in a wide variety of pathophysiological events including cancer, metabolic syndrome, arthritis etc. In this context, a number of N'-aryl isonicotinolyhydrazides, in which known pharmacophores are included, have been selected from commercial chemical library and tested for the inhibitory activities targeting HIF-2α in cultured HTB94 cell. HRE-luciferase and HIF-2α were introduced into the cell by transfection and adenoviri infection, respectively and the reporter gene assay discovered the potency of 2-hydroxy-1-naphthyl structure. Accordingly, the scaffold has been adjusted based on this structure and subjected to anti-HIF-2α activity test, identifying 2 compounds as HIF-2α inhibitors. The activities were confirmed by false positive test. This study has been performed via the convergence of biology and chemistry and the results may be useful for discovering novel inhibitors and HIF-2α biology studies, and contribute to the development of therapeutic agents.

Clinical Features and Radiological Differential Diagnoses of Symptomatic Sesamoid Bones and Accessory Ossicles: A Pictorial Essay (증상이 있는 종자골과 부골의 임상적 소견과 영상적 감별진단: 임상화보)

  • Hyun Gun Kim;Hee Young Choi;Ji Seon Park;Kyung Nam Ryu;So Young Park;Wook Jin
    • Journal of the Korean Society of Radiology
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    • v.82 no.1
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    • pp.82-98
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    • 2021
  • Sesamoid bones and accessory ossicles are normal anatomic variants with varying morphological appearances and incidences. They are usually small osseous fragments with well-corticated margins located adjacent to the joint space and bone. Patients with sesamoid bones and accessory ossicles are usually asymptomatic and commonly encountered in clinical practice. These sesamoids and accessory bones are occasionally painful because of fractures, dislocations, degenerative changes, avascular necrosis, accessory bone infections, or abnormalities of the adjacent tissue, such as nerve entrapment, tenosynovitis, or soft tissue impingement. This article aimed to illustrate the imaging features of symptomatic sesamoids bones and accessory ossicles at various anatomic locations and describe their clinical features and radiological differential diagnosis.

Comparison of the health behavior and nutrition status of young-old women according to the vitality in their quality of life: based on the 2019, 2021 Korea National Health and Nutrition Examination Survey (전기노인 여성의 삶의 질 중 기운에 따른 건강행태와 영양상태 비교: 2019년, 2021년 국민건강영양조사 자료를 이용하여)

  • Jiyoung Jeong;Yoon Jung Yang
    • Journal of Nutrition and Health
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    • v.56 no.5
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    • pp.496-509
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    • 2023
  • Purpose: This study aimed to identify the general characteristics, chronic diseases, health behavior, mental health, and nutritional status of young-old women based on their vitality. Methods: This study used data from the 2019 and 2021 Korea National Health and Nutrition Examination Survey (KNHANES). The subjects were 1,113 young-old women aged 65 to 74 years old. The health-related quality of life concept with an 8-item questionnaire was used to measure the quality of life. Subjects were categorized into 4 groups (always, often, sometimes, never) based on their vitality. General characteristics, chronic diseases, health behavior, dietary behavior, food intake, and nutrient intake were compared among the groups. Results: Age, education level, household income, employment, fruit intake, dietary supplements, abundance of food, and nutrition labeling recognition were associated with the vitality of the subjects. Young-old women with arthritis, diabetes, and osteoporosis displayed lower vitality. Moreover, subjective health status, exercise, activity restrictions, and average daily sitting hours were related to vitality, while no significant difference was found in vitality between smoking and drinking. In terms of mental health factors, higher vitality was associated with 6-8 hours of sleep, lower stress levels, and reduced depression. The high-vitality group exhibited a higher intake of potatoes, starch, mushrooms, fruits, meat, milk, animal oils, and beverages than the low-vitality group. Additionally, the group with higher levels of vitality showed a higher intake of protein, fat, saturated fatty acids, monounsaturated fatty acids, polyunsaturated fatty acids, n-6 fatty acids, dietary fiber, sugars, phosphorous, potassium, magnesium, iron, zinc, and riboflavin. Conclusion: This study suggests that the vitality of young-old women is related to socioeconomic factors, health behavior, mental health, and food intake. To maintain a vibrant lifestyle in elderly women, it is necessary to have social and economic stability, prevent arthritis, diabetes, and osteoporosis, exercise regularly, get sufficient sleep, maintain mental health, and have a balanced diet.

Advance Understanding and New Treatment of Alopecia Areata (원형탈모증(alopecia areata)의 최신 이해와 치료)

  • Kang, Kyung-Hwa
    • Journal of Life Science
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    • v.26 no.11
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    • pp.1345-1354
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    • 2016
  • Alopecia areata (AA) is a common and tissue-specific autoimmune disease of hair follicle resulting in the loss of hair on the scalp and elsewhere on the body. Hair follicles is a unique organ because it has its own immune system and hormonal milieu and has a different immune state at each hair cycle stage. The collapses of anagen-dependent hair follicle immune privilege arise autoimmune attack, inducing ectopic MHC class I expression in the hair follicle epithelium and autoantigen presentation to autoreactive CD8+T cells, which results in AA. Clinical and experimental studies have pointed that psychological stress may also influence the hair follicle immune/hormone systems and contribute to the induction of AA. The key pathogenesis of AA is associated with immune privilege guardians (including ACTH, ${\alpha}-MSH$, and $TGF-{\beta}$), natural killer group 2D-positive (NKG2D+) cells (including NK and CD8+T cells), and stress hormones (including CRH and substance P). Effective treatments for AA are still demanded. One of the future targets of treatment will be the modification of hair follicle immune privilege including stress. Recent studies have reported that JAK inhibitors and immunomodulators used in other autoimmune disease, such as psoriasis, atopic dermatitis, and rheumatoid arthritis, Tregs, platelet-rich plasma therapy, statins, and prostaglandin anaolgues are effective for AA. Here the article reviews the recent understanding in the pathogenesis associated with perifollicular endocrine/immunology and new treatments of AA.

Effects of health promotion education experience on present health status of elderly (건강증진 보건교육 경험이 노인의 현재 건강상태에 미치는 영향)

  • Lee, Heung Hun;Hwang, Eun Jeong
    • Journal of the Korea Academia-Industrial cooperation Society
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    • v.20 no.11
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    • pp.106-114
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    • 2019
  • This study aims to identify the effects of experiencing health promotion education on the present health status of elderly people. The subjects of this study were a total 10,532 elderly people (aged 65 years or older), and who were selected from the data of the '2017 Community Health Survey'. The health promotion education experience consisted of handwashing education/campaign, a non-smoking campaign and non-smoking education. The chronic disease control education experience consisted of education on hypertension, diabetes mellitus and arthritis. Psychiatric counseling consisted of consultations on stress, depression and suicide. The present health status consisted of the patients' ability to exercise, their self-care ability, their activities of daily living, their pain/discomfort and their anxiety/depression. The data was analyzed utilizing the chi-square test, the Mann-Whitney test and multivariate logistic regression analysis. The general characteristics of the subjects were 4,075 males (38.69%) and 6,457 females (61.31%). The average age was 73.71(±6.18) years old. The significant factors influencing the elderly peoples' present health status were age (OR=0.909, 95% CI=0.886-0.933), handwashing education/campaign (OR=2.463, 95% CI=1.703-3.563), a stop smoking campaign (OR=1.624, 95% CI=1.146-2.301) and consultation for mental problems (OR=0.533, 95% CI=0.359-0.791). In conclusion, to modify the personal habits that the elderly have had for a long time and to show the effects of education, long-term continuous education that focuses on the characteristics of the people will be effective.

Three Dimensional Curvature Analysis of Femoral Shaft Bowing based on CT Images (CT 영상을 이용한 대퇴체부 휨의 3차원적 곡률 분석)

  • Lim, Ki Seon;Oh, Wang Kyun;Lee, Tae Soo
    • Journal of the Korean Society of Radiology
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    • v.7 no.5
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    • pp.313-320
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    • 2013
  • For some patients with joint illnesses such as rheumarthritis or varus deformity, the total knee arthroplasty (TKA) procedures are performed. However, when inserting metal cutting guide for the procedures, due to the femoral shaft bowing, complications such as the cortex of the femoral shaft damages or secondary fractures can be caused. If the central coordinate value of the femoral shaft is known, the metal cutting guide could be inserted into the anatomical center, so such complications can be prevented. In this study, CT images of femoral shafts of 10 individuals in the experiment group who are in need of receiving the total knee arthroplasty procedures and those of 10 individuals in the control group without illness in the femoral shaft have been utilized to locate the 3-dimensional coordinate values. Then, Matlab was utilized to identify the central coordinate value in order to obtain a graph reflecting the anatomical shapes as well as to acquire the 3-dimensional radial curvature values by section. As a result, the average curvature range and standard deviation of femoral shafts of the experiment group was determined to be $758.15{\pm}206.3mm$ whereas the that of the control group was determined to be $1672.97{\pm}395.6mm$. The statistical significance of the measured results was verified through f-distribution analysis. Based on these results, it was verified that the level of curvature of the femoral shaft of the experiment group was higher. If the anatomical central points are located and analyzed using this methodology, it would be helpful in performing orthopedic operations such as the total knee arthroplasty.

Effects of Nutrient Intake on Oral Health and Chewing Difficulty by Age Group (연령층별 구강건강과 저작불편이 영양소 섭취에 미치는 영향)

  • Kim, Seol-Hee
    • Journal of the Korea Academia-Industrial cooperation Society
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    • v.19 no.2
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    • pp.202-209
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    • 2018
  • This study analyzed the effects of the nutrient intake on oral health and chewing difficulty according to the age group. The subjects were 5,855 participants of the third Korea National Health and Nutrition Examination Survey(KNHANES VI), 2015, Korea Centers for Disease Control and prevention and aged 20 years and over. The data were analyzed using SPSS Ver 21.0, classified as the difficulty in chewing group (DC) and no difficulty in chewing group (NDC). As a result, the DC rates were 5 times higher in the 60+ year age group (39.5%) than in the 20-39 year age group (8.1%). The DC group were experience periodontal disease (33.4%), dental caries (30.1%), diabetes (41.8%), myocardial infarction (57.3%), arthritis (44.0%), asthma (48.0%), and depression (41.9%). In addition, 86% of the DC group were experiencing speech problems. The DC group had significantly lower intakes (1446.59g), than the NDC group (1666.62g), and the protein, carbohydrate, dietary fiber and other dietary intake were significantly lower. These findings suggest that the chewing difficulty is related to the nutrient intake, and psychological status in the elderly DC group. Therefore, the care of chewing difficulties is essential for the elderly to maintain a healthy lifestyle. Accordingly, oral care and myofunctional therapy are needed to maintain oral health.

A Novel COMP Gene Mutation in a Korean Kindred with Multiple Epiphyseal Dysplasia

  • Ko, Jung-Min;Kwack, Kyu-Sung;Baek, Kum-Nyeo;Cho, Dae-Yeon;Kim, Hyon-Ju
    • Journal of Genetic Medicine
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    • v.6 no.1
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    • pp.81-86
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    • 2009
  • Multiple epiphyseal dysplasia (MED) is a clinically and genetically heterogeneous chondroplasia, characterized by delayed development of the ossification centers and, deformities of the extremities that involve only the epiphysis and result in mild short stature. Mutations in the cartilage oligomeric matrix protein (COMP) gene are most commonly found, and most of the mutations are located in the calmodulin-like repeats and the C-terminal domain. We report a Korean kindred of 12 family members with MED in four generations who were found to have a novel mutation in the COMP gene. A pedigree showed early onset osteoarthritis requiring arthroplasty that was an autosomal dominant inherited trait. Radiological examinations demonstrated the presence of osteochondral defects in the medial femoral condyles, and the knee and hip joints showed variable degrees of precocious degenerative changes. Mutation analysis of the COMP gene in the proband and five other affected family members identified a novel missense mutation, c.1280G>C (p.Gly427Ala) in exon 12, which was not found in three unaffected family members. Direct sequencing of the COMP gene may yield pathogenic mutations in dominantly inherited MED cases, and may provide opportunities of carrier detection among high-risk family members, leading to genetic counseling for early diagnosis and intervention before the onset of complications.

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A Case of Hypersensitivity Pneumonitis Caused by Methotrexate (Methotrexate에 의한 약제 유발 과민성 폐렴 1례)

  • Suh, Hyun Joo;Chung, Man Pyo;Park, Eun Ha;Shin, Sung Chul;Jeon, Kyeong Man;Yu, Chang Min;Pyun, Yu Jang;Lee, Kyung Soo;Han, Joungho
    • Tuberculosis and Respiratory Diseases
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    • v.56 no.2
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    • pp.203-209
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    • 2004
  • Background : Methotrexate (MTX) has been used to treat a wide range of malignant and benign diseases including osteosarcoma, advanced stage non-Hodgkin's lymphoma, psoriasis, severe rheumatoid arthritis, sarcoidosis, and Wegener's granulomatosis. MTX-induced lung injury occurs in up to 10% of treated patients. Although both acute and chronic presentations have been described, typical manifestation of MTX-induced lung injury is subacute with symptoms usually developing within several months after starting therapy. Nonspecific interstitial pneumonia (NSIP) is the most common histopathologic manifestation of MTX-induced lung disease, while bronchiolitis obliterans organizing pneumonia (BOOP) and diffuse alveolar damage (DAD) are less common. Granuloma formation is reported in 34.7%. In Korea, Two reports of MTX pneumonitis have been published. The one presented with NSIP and the other with DAD. We recently experienced a case of MTX pneumonitis with presentation of hypersensitivity pneumonitis.