• Title/Summary/Keyword: ANCA

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A Case of Microscopic Polyangiitis with Diffuse Alveolar Hemorrhage (폐출혈을 동반한 현미경적 다발혈관염 1예)

  • Lee, Sang-Jin;Lee, Jae-Woung;Kim, Hye-Jin;Shin, Kyeong-Cheol;Chung, Jin-Hong;Lee, Kwan-Ho;Park, Hye-Jung
    • Journal of Yeungnam Medical Science
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    • v.21 no.1
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    • pp.101-107
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    • 2004
  • Diffuse alveolar hemorrhage is a rare but serious and frequently life-threatening complication of a variety of conditions. The first goal in the management of patients with diffuse alveolar hemorrhage is to achieve or preserve stability of the respiratory status. Subsequently, the differential diagnosis is aimed at the identification of a remediable cause of the alveolar hemorrhage. The most common causes of diffuse alveolar hemorrhage with glomerulonephritis are microscopic polyangiitis and Wegener's granulomatosis, followed by Goodpasture syndrome and systemic lupus erythematosus. Microscopic polyangiitis (MPA) is a distinct systemic small vessle vasculitis affecting small sized vessels with few or no immune deposits and with no granulomatosus inflammation. The disease may involve multiple organs such as kidney, lung, skin, joint, muscle, gastrointestinal tract, eye, and nervous system. MPA is strongly associated with antineutrophil cytoplasmic autoantibody (ANCA) that is a useful serological diagnostic marker for the most common form of necrotizing vasculitis. Our report concerns a case of microscopic polyangiitis with diffuse alveolar hemorrhage in a 54-year-old man. He was admitted to our hospital due to dyspnea upon exertion and recurrent hemoptysis. Laboratory findings showed hematuria, proteinuria and deterioration of renal function. In the chest CT scan, diffuse ground glass appearance was seen in both lower lungs. A lung biopsy revealed small vessel vasculitis with intraalveolar hemorrhage and showed a positive reaction to against perinuclear ANCA. The patient was treated with prednisolone and cyclophosphamide. Chest infiltration decreased and hemoptysis and hypoxia improved. He is still being followed up in our hospital with a low dose of prednisolone.

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A Case of Microscopic Polyangiitis Initially Suspected with Henoch-Sch$\ddot{o}$nlein Purpura Nephritis (Henoch-Sch$\ddot{o}$nlein 자반병 신염으로 의심했던 현미경적 다발혈관염 1례)

  • Im, Jong Geun;Moon, Kyung Chul;Koo, Ja Wook
    • Childhood Kidney Diseases
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    • v.16 no.2
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    • pp.132-137
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    • 2012
  • Microscopic polyangiitis (MPA) is systemic small vessel vasculitis that is very rare in childhood. MPA is characterized by pauci-immune necrotizing small vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation. Approximately 90% of patients have glomerulonephritis that is accompanied by a variety of other organ involvement. A 10-year-old girl visited our clinic with clinical manifestations suggestive Henoch-Sch$\ddot{o}$nlein purpura nephritis such as purpuric skin rash, abdominal pain, arthralgia on both knees, massive proteinuria and microscopic hematuria. So initially we suspected Henoch-Sch$\ddot{o}$nlein purpura nephritis. However, later her perinuclear-antineutrophil cytoplasmic antibodies(p-ANCA) test was positive, and her renal biopsy was consistent with microscopic polyangiitis. We began steroid therapy, combined with cyclophosphamide, ACE inhibitor. Currently she is a 12-year old, and until now she has been regularly examined in the outpatient. We report a case of microscopic polyangiitis initially suspected with Henoch-Sch$\ddot{o}$nlein purpura nephritis.

Pulmonary Cavitary Lesions Associated with High Fever (고열을 동반한 폐의 공동성 병변)

  • Lee, So-Hyeon;Pyun, Eun-Kyung;Lee, Hye-Jin;Lee, Jin-Hwa;Cho, Young-Joo;Han, Woon-Sup;Kim, Hyae-Young;Park, Sung-Sook;Chang, Jung-Hyun
    • Tuberculosis and Respiratory Diseases
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    • v.49 no.5
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    • pp.644-648
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    • 2000
  • A 35-year-old male presented high fever and cough. The pateint showed three, discrete, "punched-out", shallow ulcers appearing as pyodermic gangrenosum on the trunk and the back, and a painless subcutaneous nodule on the medial side of the left thigh. The chest X-ray showed multiple cavities on the both lungs. The diagnosis of Wegener's granulomatosis was established by pathology of the skin and the lung, radiologic findings of the chest and positive result of c-ANCA test.

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C-ANCA-positive glomerulonephritis associated with subacute infective endocarditis caused by Bartonella infection

  • Kim, Min Jeong;Jang, Ha Nee;Lee, Tae Won;Cho, Hyun Seop;Chang, Se-Ho;Kim, Hyun-Jung
    • Journal of Yeungnam Medical Science
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    • v.34 no.1
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    • pp.140-145
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    • 2017
  • Glomerulonephritis (GN) is sometimes associated with infective endocarditis (IE). Bartonella endocarditis is difficult to diagnose because it is rare and cannot be detected by blood culture. This is the first report of cytoplasmic anti-neutrophil cytoplasmic antibody-positive subacute endocarditis-associated GN caused by Bartonella infection in South Korea. A 67-year-old man was hospitalized due to azotemia. He complained of weight loss and anorexia for 6 months. A diagnosis of IE was made based upon echocardiographic detection of vegetations on the mitral and aortic valves and a Bartonella antibody titer of 1:2,048. Renal histology identified focal crescentic GN. Azotemia and proteinuria improved after doxycycline and rifampin treatment combining with steroid therapy.

Pathology of Thrombotic Microangiopathy (혈전성 미세혈관병증의 병리)

  • Sol, Mee Young
    • Childhood Kidney Diseases
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    • v.17 no.1
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    • pp.6-12
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    • 2013
  • Thrombotic microangiopathy (TMA) is a microvascular thrombotic lesion caused by endothelial injury and subsequent formation of platelet rich thrombus. TMA is first described as a classical pathologic feature of HUS/TTP. Renal biopsy finding of TMA represents kidney involvement of HUS/TTP as well as other diseases such as malignant hypertension, drug toxicity, eclampsia, pre-eclampsia, and several systemic infections. Autoimmune diseases and transplant kidney sometime also have TMA. It is needed to consider a complete autoimmune work-up of patients presenting with TMA including tests for ANA, ANCA, and ADAMTS13 inhibitory antibodies, because there are several reports of association with TMA in patients of SLE, anti-phospholipid syndrome, and ANCA-associated vasculitis.

A Case of Churg-Strauss Syndrome in Infancy (영아기 Churg-Strauss Syndrome 1예)

  • Shon, Su-Min;Kim, Kyo-Il;Park, Moon-Ho;Kang, Yu-Na;Lee, Hee-Jung;Hwang, Jin-Bok
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.10 no.1
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    • pp.66-70
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    • 2007
  • Churg-Strauss syndrome (CSS) is a disorder characterized by pulmonary and systemic smallvessel vasculitis, extravascular eosinophilic infiltration and hypereosinophilia; it is rarely diagnosed in the pediatric age group. We experienced a case of CSS in a male infant who had repeated symptoms of asthma with hypereosinophilia and transitory non-fixed pulmonary infiltrates on chest radiographs. He also had rectal bleeding in early infancy and multiple erosions with extravascular eosinophilic infiltrations in the sigmoid colon. We report a rare case of CSS in a 14-month-old infant and review the medical literature.

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A Case of Microscopic Polyangiitis with Pulmonary Hemorrhage and Rapidly Progressive Glomerulonephritis (폐출혈과 급속 진행성 사구체신염을 동반한 현미경적 다발성 동맥염 (Microscopic Polyangiitis) 1례)

  • Kwon Hae Sik;Lee Young-Mock;Kim Ji Hong;Kim Pyung-Kil;Kang Hae Youn;Hong Soon Won;Jeong Hyeon Joo
    • Childhood Kidney Diseases
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    • v.5 no.2
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    • pp.213-218
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    • 2001
  • Microscopic Polyangiitis(MPA) belongs to a spectrum of systemic vasculits, and particularly antineutrophil cytoplasmic autoantibodies(ANCA)-associated small-vessel vasculitis which is characterized by involvement of the lung and kidney. The diagnosis of MPA is often difficult to make, and delayed because of the variability of the clinical presentation. Renal biopsies have a very important diagnostic and prognostic value in MPA. We experienced a case of microscopic polyangiits which was confirmed by renal biopsy and positive serum perinuclear ANCA, associated with alveolar hemorrhage and gastrointestinal bleeding. We began methylprednisolone pulse therapy, combined with a low dose of cyclophosphamide and plasmapheresis therapy. ACE inhibitor and Ca channel blocker were used when proteinuria and hypertention developed. On admission, the patient's lab findings showed BUN l17 mg/dL, Cr 2.3 mg/dL, while on the 60th hospital day BUN/Cr values fell to 20.8 mg/dL / 1.6 mg/dL and though proteinuria persisted, the patient's condition was tolerable and is currently under observation on an out-patient basis. The last lab values were BUN 26 mg/dL / Cr 1.6 mg/dL. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 213-18)

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Seven-Year Follow Up of Microscopic Polyangiitis Presenting with Rapidly Progressive Glomerulonephritis (급속 진행성 사구체신염으로 시작된 현미경적 다발성 동맥염(Microscopic Polyangiitis) 환자의 7년간의 장기 추적관찰 1례)

  • Oh, Jin-Won;Kim, Pyung-Kil;Lee, Jae-Seung;Jeong, Hyeon-Joo
    • Childhood Kidney Diseases
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    • v.12 no.1
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    • pp.99-104
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    • 2008
  • Microscopic polyangiitis(MPA) is a systemic necrotizing vasculitis that involves many organ systems including the skin, joint, kidneys, and lungs. In spite of early diagnosis and intensive care, the five-year actuarial patient and kidney survival rates are 65% and 55%. We experienced a case in 7-year-old girl of microscopic polyangiitis presenting with rapidly progressive glomerulonephritis which was confirmed by renal biopsy and positive serum perinuclear antineutrophil cytoplasmic autoantibodies(p-ANCA). The diagnosis of patients first renal biopsy was MPA, p-ANCA-associated crescentic glomerulonephritis. The patients second renal biopsy was done 5 years 6 months later since first renal biopsy, and pathologic diagnosis was chronic sclerosing glomerulonephritis, advanced, due to MPA. We began methylprednisolone pulse therapy, combined with a low dose of cyclophosphamide and plasmapheresis therapy. ACE inhibitor, angiotensin II receptor blocker, and cyclophosphamide were used until now and the patients current age is 14 years old. On admission, the patients laboratory findings showed BUN 117 mg/dL and Cr 2.3 mg/dL, while on the hospital day BUN and Cr values fell to 20.8 mg/dL and 1.6 mg/dL. But renal function was progressed to chronic failure with latest laboratory data BUN 51.7 mg/dL and Cr 3.2 mg/dL. ACE inhibitor, angiotensin II receptor blocker and small dose of immunosuppressant with close observation is the key to maintain the patient survival.

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An Image Retrieval System with Adjustment for Human Subjectivity

  • Fukushima, Shigenobu;Ralescu, Anca
    • Proceedings of the Korean Institute of Intelligent Systems Conference
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    • 1993.06a
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    • pp.1309-1312
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    • 1993
  • We present a flexible retrieval system of face photographs based on their linguistic descriptions in terms of fuzzy perdicates. While natural for describing a face, linguistic expressions are also subjective, which affects the retrieval result. Thus, the capability of a retrieval system to adjust to different users becomes very important. In this research we use fuzzy logic techniques, for describing image data, inference for retrieval and adjustment to a new user. Experimental results of the adjustment are also included.

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Representation of Spatial Relations between Regions in a 2D Segmented Image

  • Ralescu, Anca;Miyajima, Koji
    • Proceedings of the Korean Institute of Intelligent Systems Conference
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    • 1993.06a
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    • pp.1317-1320
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    • 1993
  • We are concerned with developing a robust method for comprehensive scene analysis. In particular, we address the problem of representing spatial relations between regions in a segmented 2D image. Spatial relations are modeled as fuzzy sets. The method has two aspects, symbolic and quantitative, consisting of assigning labels for spatial relations and numeric degrees to which a relation holds respectively. The procedure of deriving a spatial relation is hierarchical taking into account geometric/physical characteristics of the regions in question.

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