• 제목/요약/키워드: Mixed connective tissue disease

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Pathological interpretation of connective tissue disease-associated lung diseases

  • Kwon, Kun Young
    • Journal of Yeungnam Medical Science
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    • 제36권1호
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    • pp.8-15
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    • 2019
  • Connective tissue diseases (CTDs) can affect all compartments of the lungs, including airways, alveoli, interstitium, vessels, and pleura. CTD-associated lung diseases (CTD-LDs) may present as diffuse lung disease or as focal lesions, and there is significant heterogeneity between the individual CTDs in their clinical and pathological manifestations. CTD-LDs may presage the clinical diagnosis a primary CTD, or it may develop in the context of an established CTD diagnosis. CTD-LDs reveal acute, chronic or mixed pattern of lung and pleural manifestations. Histopathological findings of diverse morphological changes can be present in CTD-LDs airway lesions (chronic bronchitis/bronchiolitis, follicular bronchiolitis, etc.), interstitial lung diseases (nonspecific interstitial pneumonia/fibrosis, usual interstitial pneumonia, lymphocytic interstitial pneumonia, diffuse alveolar damage, and organizing pneumonia), pleural changes (acute fibrinous or chronic fibrous pleuritis), and vascular changes (vasculitis, capillaritis, pulmonary hemorrhage, etc.). CTD patients can be exposed to various infectious diseases when taking immunosuppressive drugs. Histopathological patterns of CTD-LDs are generally nonspecific, and other diseases that can cause similar lesions in the lungs must be considered before the diagnosis of CTD-LDs. A multidisciplinary team involving pathologists, clinicians, and radiologists can adequately make a proper diagnosis of CTD-LDs.

골연화증과 저인산혈증을 유발한 인산뇨성 간엽성 종양 -증례 보고- (Osteomalacia and Hypophosphatemia Caused by Phosphaturic Mesenchymal Tumor Mixed Connective Tissue Variant (PMTMCT) -A Case Report-)

  • 정재윤;김준혁;이상훈;김한수
    • 대한골관절종양학회지
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    • 제10권2호
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    • pp.124-129
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    • 2004
  • 인산뇨성 간엽성 종양 복합성 결합조직 변이형(PMTMCT)은 매우 드문 질환으로, 인산뇨, 저인산혈증, 정상혈청 칼슘농도, 그리고 감소된 1,25-dihydroxyvitamin D3등의 부종양성 증후군을 보이는 종양성 골연화증을 흔히 유발하는 것으로 알려져 있다. 45세 여자 환자에서 종양성 골연화증의 증상을 동반하는 우측 둔부내 인산뇨성 간엽성 종양을 진단하여, 수술적인 치료로 좋은 결과를 얻었기에 문헌고찰과 함께 보고하는 바이다.

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레이노드 증후군으로 초기 발현된 복합 교원성 질환 1예 (A case of mixed connective tissue disease presenting initially with Raynaud's phenomenon)

  • 김수영;최영석;김영옥;우영종
    • Clinical and Experimental Pediatrics
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    • 제51권8호
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    • pp.886-891
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    • 2008
  • 복합 교원성 질환은 류마티스 관절염, 경피증, 전신성 홍반성 낭창, 다발성 근염의 다양한 임상양상을 나타내면서 혈청학적 검사에서 항 ENA 항체를 갖는 질환이다. 성인에서 1972년 25례의 복합 교원성 질환의 첫 보고 이래 국내에서 성인에서 발생한 복합 교원성 질환에 대한 몇몇 보고가 있었지만, 소아에서는 복합 교원성 질환의 보고가 드물다. 7세 여아가 내원 3개월 전부터 시작된 양 손가락에, 글쓰기 및 한랭노출에 의해 유발되는, 가역적인 색깔변화와 차가움을 주소로 내원하였다. 신체 검사에서 양측 손가락들의 부종 및 오른쪽 집게손가락 끝에 궤양이 관찰되었다. 혈청학적 검사에서 다른 교원성 질환의 증거 없이 높은 역가의 ANA와 항 ENA 항체 중 항 RNP 항체가 고역가로 검출되었다. Raynaud 현상과 궤양에 대해 slow releasing nifedipin을 사용하였으나 호전 보이지 않아 스테로이드를 투약 하였고, 이후 궤양은 치유 되었으며, Raynaud 현상의 빈도도 현저히 감소하였다. 그러나 steroid를 점차 감량하는 동안 탈모 및 관절염 증상이 새로 발현되었으며, 현재 겨울에 악화되는 Raynaud 증상과 관절염으로 추적 관찰 중이다. 이에 본 저자들은 Raynaud 현상으로 발현되고 추적 동안에 탈모와 관절염이 나타난 복합 교원성 질환의 드문 소아 증례를 보고하는 바이다.

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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    • 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.

가족성 거대 백악종 (Familial gigantiform cementoma)

  • 한원정;김은경
    • Imaging Science in Dentistry
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    • 제36권3호
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    • pp.157-162
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    • 2006
  • Familial gigantiform cementoma is a rare fibre-cemento-osseous disease of the jaws which appears to be transmitted as an autosomal dominant trait with variable expressivity of the phenotype. A 7-year-old girl visited DKUDH complaining of the painless facial deformity. Clinically, significant facio-lingual expansion was observed at the left maxilla, left mandibular body and symphysis portion. Malposition of lower anterior teeth was found. Panoramic radiograph and CT scan showed the extensive expansile mixed lesion at maxilla and mandible. Bone scan revealed hot spot at the maxilla and left side of mandible. Histologic examination revealed moderately dense fibrous connective tissue with scattered masses resembling cementum. The patient's mother had a history of the mandibular resection due to benign tumor. Her younger brother had buccal expansion of right mandible. We report our finding of a family that has exhibited clinical, radiographic and histologic findings consistent with the familial gigantiform cementoma.

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痔瘡과 痔瘻에 對한 文獻的 考察 (A Literatural study on the hemorrhoids and hemorrhoids complicated by anal fistula)

  • 노현찬;노석선
    • 한방안이비인후피부과학회지
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    • 제10권1호
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    • pp.284-305
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    • 1997
  • A Literatural study on the etiological factors, classification, prescription of hemorrhoids and hemorrhoids complicated by anal fistula following results were obtained. 1. The cause of hemorrhoids are long time sit, long time gate, overfatigue, overeating, imbalance of stool( constipation or diarrhea), pregnant fertility(overfatigue after childbirth, insufficiency of middle warmer energy), uncontrol sexual excess, pathgenic factors of wetness, heat, wind, dry, genetic cause, excess of anxiety, pile up of heat poison, weakness of entrails and viscera. The cause of hemorrhoid complicated by anal fistula are attack of external wind, heatness, dry, fire, wetness(pathgenic factors), inapporiate treatment and chronic disease, greasy diet, excess of anxiety, constipation, uncontrol sexual excess, obstacle of circulation of vital energy and blood on anal site. 2. Classification of hemorrhoids are female hemorrhoids, male hemorrhoids, pulse hemorrhoids, intestines hemorrhoids, vital energy hemorrhoids, wine hemorrhoids, blood hemonhoids, flowing hemorrhoids. Classification with other method are external hemorrhoids, internal hemorrhoids, mixed hemorrhoids, excrescence hemorrhoids, nipple homorrhoids. External hemorrhoids is classified of varicosis of hemorrhoidal vein, connective tissue form, thrombus form. Classification of hemorrhoid complicated by anal fistula are simple lower hemorrhoid, lower mixed hemorrhoid, deep hemorrhoid, outer of one hole hemorrhoid, a horseshoe hemorrhoids. Once more classificated of four are space of sphincter muscle form, penetration sphincter muscle form, upper of sphincter muscle form, outer of sphincter muscle form. 3. Therapy method of hermorrhoid and hemorrhoid complicated by anal fistula are internal method, fumigation method method, ointment, method of close with medicine, necrotizing method, hot medicated compress( gxternal method), injection, insertion, bind, (operation) and acupuncture therapy (the others method) 4. Herb medicine for many used of internal method are Scutellaria baikalensis George(黃芩), Coptis japonia Makino(黃連), Rehmania giutinosa Liboschitz ex Fischer & Meyer(生地黃), Poncirus trifoliata Refinesque(枳殼), Sanguisorba officinalis Linne(地楡), Sophora japonica L.(槐花), Cnidium officinale Makino (川芎), Astragalus membranaceus Bunge(황기), Angelica gigas Nakai (當歸). 5. Herb medicine for many used of fumigation are Schlechtendalia Chinesis J. Bell (五倍子), Artemisia Vulgaris L. var indica Maxim(艾葉), Poncirus trifoliata Refinesque (枳殼), Nepeta japonica Maximowicy(荊芥), And herb medicine for many used of ointment are Calomelas(輕粉), Alum(白礬), Boswellia carterii Birdwood(乳香), Os Draconis Fossilia Ossis Mastodi(龍骨).

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면역저하 환자에서 발생한 단순포진바이러스 폐렴 (Herpes Simplex Virus Pneumonia in Immunocopmromised Host)

  • 김진구;이충현;강경우;서지영;정만표;김호중;권오정;이종헌;한정호
    • Tuberculosis and Respiratory Diseases
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    • 제46권1호
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    • pp.82-88
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    • 1999
  • Herpes simplex virus pneumonia in immunocompromised host is difficult to diagnose with non-invasive method, and has high mortality rate. Because early diagnosis and early treatment can significantly decrease the mortality rate, the enthusiastic efforts for the early diagnosis should be done. A 41-year-old woman who took prednisolone due to mixed connective tissue disease developed gradually increasing dyspnea with radiological features of interstitial lung disease. Initially, we treated her with empirical antibiotics, but failed to improve her dyspnea. So we performed bronchoalveolar lavage and open lung biopsy. Open lung biopsy specimen showed herpes simplex virus pneumonia. Herpes simplex virus was also isolated from bronchoalveolar lavage fluid. There was both clinical and radiological improvement after treatment with acyclovir for 14 days.

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사례로 본 한방임상에서 양약으로 인한 약인성간손상에 대한 인식 필요성 (Awareness of the Causes of Drug-Induced Liver Injury: A Case of Hepatotoxicity Resulting from Antipsychotics)

  • 손창규
    • 대한한방내과학회지
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    • 제44권4호
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    • pp.751-756
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    • 2023
  • 목적: 본 연구는 임상에서 많은 빈도로 사용되고 있는 향정신성 약물이 간독성을 발생시킬 수 있음을 임상 예를 통해 보여줌으로써, 한의진료현장에서 이에 대한 경각심과 더불어 약인성간손상에 대한 최신 정보를 제공하고자 한다. 방법: 본 임상 예의 논문은 향정신성 약물을 사용한 후 약인성간독성의 의심과 진단 및 약물의 중단 후 증상과 간손상 효소의 개선과정을 자세히 제시하였다. 결과: 평소 혼합결합조직병으로 한방병원에서 수년 동안 침과 뜸 치료 등으로 잘 유지되고 있던 56세의 여성 환자가, 어느날부터 갑자기 심한 피로감과 전신적 불편편함을 호소하였다. 혈액검사를 시행한 결과, 혈청 AST, ALT가 정상 경계의 2.5-배 이상 증가하였고, 촤근 복용한 향정신성약물에 대한 RUCAM score가 9점으로 약인성간손상 진단에 부합하였다. 이 의심 약물들을 중지한 뒤에 주관적 불편함이 빠르게 개선되었으며, 혈청 간손상 효소 수치 또한 2주 안에 정상화되었다. 결론: 본 증례는 향정신성약물로 생길 수 있는 간손상의 전형적인 임상 예로서, 향정신성약물의 일반화된 상황에 비추어 한의원에서 환자의 진료 과정에서 하나의 중요한 고려사항을 암시한다.