• 제목/요약/키워드: Multi-organ involvement

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다장기 침범을 보인 소아 Wegener 육아종증 1례 (A Case of Wegener's Granulomatosis with Multi-organ Involvement in Childhood)

  • 이현경;조희연;정해일;최용;하일수
    • Childhood Kidney Diseases
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    • 제11권1호
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    • pp.118-125
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    • 2007
  • Wegener 육아종증은 괴사성 육아종을 형성하는 작은 혈관의 혈관염으로, 주로 상기도와 하기도, 신장의 3기관을 침범한다. 주로 성인기에 발생하나 소아에서도 발생할 수 있으며, 대부분의 임상 증상은 성인과 소아에서 비슷하나, 몇몇 임상증상에서는 성인과 차이를 보인다. Wegener 육아종증은 소아에서 호흡기 감염성 질환으로 생각되기 쉬우므로 진단이 지연되어, 만성 사구체신염이 말기신부전 등까지 진행할 수 있다. 저자들은 여러 장기를 침범하여 흔하지 않은 임상 양상을 보인 Wegener 육아종증 13세 환아를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

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Severe Hypophosphatemia in a Girl with Systemic Lupus Erythematosus

  • Lim, Taek Jin;Kim, Su Young;Kim, Seong Heon
    • Childhood Kidney Diseases
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    • 제21권2호
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    • pp.156-159
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    • 2017
  • Systemic lupus erythematosus (SLE) is characterized by various symptoms and multi-organ involvement. Hypophosphatemia has been described in several diseases accompanied with systemic inflammation. However, hypophosphatemia has rarely been described in SLE patients, especially in those without nephritis. We report the case of a 13-year-old girl with SLE who developed hypophosphatemia without renal involvement. Her hypophosphatemia was caused by renal loss of phosphorus and persisted for 7 months. It improved as her complement levels increased. Therefore, hypophosphatemia may be related to disease activity in SLE patients.

골수이식 이후의 다발근육염: 만성 이식편대숙주병의 드문 증상인가? 자가면역작용인가? (Polymyositis After Bone Marrow Transplantation: As an Uncommon Manifestation of Chronic Graft-Versus-Host Disease? or Autoimmune Process?)

  • 최원철;정용한;양영일;배종석
    • Annals of Clinical Neurophysiology
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    • 제13권1호
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    • pp.58-60
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    • 2011
  • Chronic graft-versus-host disease (GVHD) is a well-known complication of allogeneic bone marrow transplantation (BMT) and has heterogeneous manifestations, with multi-organ involvement. Recently, polymyositis (PM) was reported to be a rare manifestation of chronic GVHD. Here, we report a 30-year-old woman who was diagnosed with PM after allogeneic BMT.

Myositis involving Masticatory Muscles in Behcet's disease

  • Kang, Ji-Yeon;Kim, Kyoung-Won;Lee, Eun-Young
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • 제32권1호
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    • pp.65-68
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    • 2006
  • Muscular involvement in Behcet's disease is rare manifestation in spite of basic characteristic that is vasculitis can invade multi-organ of the entire body. A few cases has been announced involving the lower extremities, the arm and generalized weakness. Like our case, myositis involving the masticatory muscles with clinically diagnosed Behcet's disease was presented with the magnetic resonance imaging (MRI) and the clinical findings, which is, not yet documented in the literature.

In Vivo Effects of Crataegus pinnatifida Extract for Healthy Longevity

  • In-sun Yu;Mina K. Kim;Min Jung Kim;Jaewon Shim
    • Journal of Microbiology and Biotechnology
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    • 제33권5호
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    • pp.680-686
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    • 2023
  • Aging is a complex series of multi-organ processes that occur in various organisms. As such, an in vivo study using an animal model of aging is necessary to define its exact mechanisms and identify anti-aging substances. Using Drosophila as an in vivo model system, we identified Crataegus pinnatifida extract (CPE) as a novel anti-aging substance. Regardless of sex, Drosophila treated with CPE showed a significantly increased lifespan compared to those without CPE. In this study, we also evaluated the involvement of CPE in aging-related biochemical pathways, including TOR, stem cell generation, and antioxidative effects, and found that the representative genes of each pathway were induced by CPE administration. CPE administration did not result in significant differences in fecundity, locomotion, feeding amount, or TAG level. These conclusions suggest that CPE is a good candidate as an anti-aging food substance capable of promoting a healthy lifespan.

소아 전두골에 발생한 랑게르한스세포 조직구증 (Unifocal Langerhans Cell Histiocytosis of Frontal Bone in a Child)

  • 홍성재;조상헌;어수락
    • 대한두개안면성형외과학회지
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    • 제14권1호
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    • pp.69-72
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    • 2013
  • Lateral eyebrow mass with primary skull lesion are rare in pediatric population. Although epidermoid cyst and dermoid cyst are the most commonly encountered skull lesions in pediatric population, Langerhans cell histiocytosis (LCH) is rarely reported. We report a case of LCH arising from the lateral eyebrow with osteolytic lesion involving the frontal bone. A 5-year-old boy was presented with a hard, fixed mass in his lateral eyebrow. Contrast magnetic resonance imaging revealed inhomogeneous enhancement of the mass with direct invasion of the frontal bone and adjacent dura mater. Under general anesthesia, linear incision at the lateral eyebrow region was made. Intraoperative evaluation revealed hard, fixed and well-defined soft tissue mass. The final extirpated mass was $2.5{\times}2.4cm$ in size, and was accompanied by a $1{\times}1cm$ sized defect on the frontal bone with intact dura mater. The surgical wound was closed primarily by a layer-by-layer fashion. Histologic examination was later performed for definite diagnosis. The histologic examination revealed abnormal proliferation of Langerhans cell with granuloma formation. Radionuclide bone scan and positron emission tomography was taken and revealed free of multi-organ involvement. At 3 months after surgery, natural looking contour at the lateral eyebrow region was observed with no tumor recurrence. Differential diagnosis of the hard and fixed mass at the lateral eyebrow region affecting the primary skull lesion from pediatric population includes epidermoid cyst, dermoid cyst and LCH. Generally, brief physical examination with plain X-ray view can be performed for clinical evaluation, but for a definite diagnosis, contrast MRI may be helpful.

옥트레오타이드를 이용한 유육종증과 동반된 유미흉의 보존적 치료 1예 (A Case of Successful Management of Sarcoidosis with Chylothorax Using Octreotide)

  • 정경수;문지애;윤설희;변민광;정우영;정재희;최상봉;김대준;표주연;김영삼;김세규;장준;김성규;박무석
    • Tuberculosis and Respiratory Diseases
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    • 제62권2호
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    • pp.119-124
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    • 2007
  • Sarcoidosis is a multi-system granulomatous disorder of an unknown etiology and affects individuals worldwide. It is characterized pathologically by the presence of non-caseating granulomas in more than one involved organ. However, pleural involvement of sarcoidosis is rare and there are no reported cases in Korea. Traditionally, sarcoidosis has often been treated with systemic corticosteroids or cytotoxic agents. In particular, chylothorax with sarcoidosis is usually treated with corticosteroid for approximately 3~6 months, followed by repeated therapeutic thoracentesis, talc pleurodesis, dietary treatment, or thoracic duct ligation where needed. We encountered a 46 years old female patient presenting with cough, dyspnea and both hilar lymphadenopathy (stage I) on chest radiograph. The patient was diagnosed with a non-caseating granuloma, sarcoidosis by a mediastinoscopic biopsy. For one month, she had suffered from dyspnea due to right side pleural effusion, which was clearly identified as a chylothorax on thoracentesis. Corticosteroid therapy with dietary adjustment was ineffective. She was treated successfully with a subcutaneous injection of octreotide for 3 weeks and oral corticosteroid. We report a case of successful and rapid treatment of chylothorax associated with sarcoidosis using octreotide and oral corticosteroid.

진성 적혈구증다증 환자에서 발현한 Erdheim-Chester Disease 1예 (A Case of Erdheim-Chester Disease Who Has Policythemia Vera)

  • 김지은;이현정;이진국;윤형규;송정섭
    • Tuberculosis and Respiratory Diseases
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    • 제64권3호
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    • pp.224-229
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    • 2008
  • ECD는 포말 대식세포의 전신 침범으로 염증 반응을 일으키는 드문 질환으로 다양한 임상양상과 불량한 예후를 보이는 질환이다. 본 증례는 골 동통과 폐막 침범이 된 자에서 흉막 생검을 통한 확진으로 ECD가 진단된 경우이며 기저질환으로 진성 적혈구 증다증이 있는 자에서 발현한 예를 경험하였기에 이를 문헌고찰과 함께 보고하는 바이다.

혈관 손상의 임상적 고찰 (Clinical Study of Vascular Injuries)

  • 정성운;김영규
    • Journal of Chest Surgery
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    • 제40권7호
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    • pp.480-484
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    • 2007
  • 서론: 주요 혈관 손상 시 적절한 진단 및 치료가 지연될 경우 환자가 생명을 잃거나 사지를 절단해야 할 위험이 있게 된다. 이러한 혈관 손상의 적절한 치료를 위한 지침을 세워보고자 본원에서 치료한 증례를 분석하였다. 대상 및 방법: 1999년 5월부터 2004년 9월까지 치료받은 외상성 혈관 손상 환자 26명을 대상으로 연령 및 성별 분포, 혈관 손상의 위치, 원인, 진단방법, 혈관 손상의 형태, 임상소견, 치료방법, 합병증 등을 살펴보았다. 결과: 환자의 연령분포는 평균 39.5세($12{\sim}86$세)였고 손상입은 혈관은 하행대동맥 6예, 대퇴 동맥 4예, 슬와 정맥 4예 등의 순으로 빈번하게 발생하였다. 손상의 원인으로는 의인성 손상이 8예로 가장 많았고 그 다음이 교통사고 7예, 자상 6예, 산업재해 5예 순이었다. 진단방법은 CT와 Arteriogram이 각각 9예씩으로 가장 많았고 수술 중에 발생한 의인성인 경우는 이학적 검사만으로도 진단이 가능하였다. 혈관손상의 형태는 동맥인 경우 가성 동맥류가 10예로 가장 많았고. 부분 절단된 경우가 5예, 완전 절단된 경우가 3예, 폐색이 3예 등이었다. 정맥손상의 경우는 부분절단이 6예, 완전 절단이 2예였고 동정맥루의 형태가 2예 등이었다. 임상 소견은 맥박손실 8예, 냉감 1예, 흉통 6예, 부종 5예, 출혈 5예 등이었다. 치료는 동맥손상인 경우 Graft interposition이 11예로 가장 많았고 primary repair가 4예, 단단 문합이 2예였고 동정맥루 2예는 모두 중재적 시술(endovascular repair)을 시행하였다. 심각한 합병증으로는 지연된 진단 및 치료로 인한 지체 소실이 3예 있었고 3예에서 동반된 신경손상으로 인한 운동장애 그리고 1예에서 다발성 장기 부전으로 사망하였다. 결론: 사지 보존율을 높이고 사망률을 줄이기 위해서는 즉각적인 진단과 치료를 시행할 수 있는 시스템 확보가 필수적이다. 또한 중재적 시술이 증가하는 요즘 경향을 볼 때 발생의 예방을 위한 의사 교육이 중요하며 발생 시 즉각적으로 혈관 외과의사가 개입할 수 있도록 원활한 의사 교환 및 협진 체제가 중요하다고 하겠다.