• Title/Summary/Keyword: 특발성 과호산구 증후군

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Dysfunction of the Prosthetic Aortic Valve in Idiopathic Hypereosinophilic Syndrome -A case report - (인공 대동맥판막 기능부전을 일으킨 특발성 과호산구 증후군 -1예 보고-)

  • Yoo, Dong-Gon;Sung, Kyu-Wan;Jung, Sang-Sig;Kang, Gil-Hyun;Kim, Chong-Wook;Park, Chong-Bin
    • Journal of Chest Surgery
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    • v.40 no.4 s.273
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    • pp.297-300
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    • 2007
  • Idiopathic hypereosinophilic syndrome is a rare systemic, leukoproliferative disorder characterized by eosinophilmediated tissue injury causing multiple organ failure, including the heart. Cardiac involvement occurs in more than 75% of patients with hypereosinophilic syndrome. Cardiac manifestations include subendocardial fibrosis, thrombus leading to peripheral emboli, restrictive cardiomyopathy, and valvular dysfunction. It is more common in men than in women (9 : 1), and trends to present between the ages of 20 and 50 years. Presentation in childhood is unusual. We report for the first time a case of a 58-year-old man with idiopathic hypereosinophilic syndrome manifested by prosthetic aortic valve dysfunction that was successfully treated by steroid and hydroxyurea therapy after surgical valvular replacement.

A Case of Idiopathic Hypereosinophilic Syndrome Associated with Pulmonary Infiltration (폐 침윤을 동반한 특발성 과호산구 증후군 1례)

  • Rhu, Hon-Mo;Kweon, Young-Soo;Chung, Jin-Hong;Lee, Kwan-Ho;Lee, Hyun-Woo;Kim, Dong-Sug;Lee, Sam-Beom
    • Journal of Yeungnam Medical Science
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    • v.11 no.2
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    • pp.375-380
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    • 1994
  • The idiopathic hypereosinophilic syndrome consists of peripheral blood eosinophilia of $1500/mm^3$ or more without a known cause, plus signs and symptoms of organ eosinophilia. The prognosis of HES without treatment is poor. However, about one third of the patients with this syndrome may respond to corticosteroid thrapy. Morever, the majority of the remainder may have a favorable response to hydroxyurea. We present here a case of hypereosinophilic syndrome without any identifiable causes, involving bone marrow, liver, lungs and cervical lymph node. We tried corticosteroid as a treatment but it showed no response. However the hydroxyurea showed good response.

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A Case of Idiopathic Hypereosinophilic Syndrome Complicated with Pulmonary Thromboembolism and Disseminated Intravascular Coagulation (폐색전증과 파종성혈관내응고가 합병된 특발성 과호산구 증후군 1례)

  • Lee, Jong Sung;Lee, Hyun-Kyung;Lee, Sung Soon;Jin, Jae Yong;Lee, Young Min;Lee, Hyuk Pyo;Kim, Joo In;Ku, Bon Il;Choi, Soo Jeon;Yum, Ho-Kee
    • Tuberculosis and Respiratory Diseases
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    • v.57 no.6
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    • pp.573-578
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    • 2004
  • The idiopathic hypereosinophilic syndrome (HES) comprises a heterogeneous group of disorders with unknown pathogenesis characterized by persistent peripheral blood and bone marrow eosinophilia. And the eosinophil infiltrates of multiple organs in HES lead to severe organ dysfunction. The disseminated intravascular coagulation (DIC) is a rare complication of HES. We have experienced a case of HES complicated with DIC and pulmonary thromboembolism. After intravenous injection of methylprednisone, blood eosinophil count was normalized but DIC was persisted. With cortico steroid and cyclosporine therapy, the disease activity was favorably remitted.

Progressive Lung Involvement during Steroid Therapy in Idiopathic Hypereosinophilic Syndrome (특발성 과호산구 증후군에서 스테로이드 투약 중 진행한 폐침윤 1례)

  • Park, Ji Young;Choi, Ju Young;Jung, Ji Min;Sim, Yun Su;Pyun, Gun Woo;Na, Youn Ju;Kang, Min Jung;Kang, In Sook;Lee, Si Nae;Kim, Yookyung;Jeong, Jee-Hyong;Lee, Jin Hwa;Cheon, Eun Mee;Chang, Jung Hyun
    • Tuberculosis and Respiratory Diseases
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    • v.59 no.1
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    • pp.97-103
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    • 2005
  • Hypereosinophilic syndrome (HES) is characterized by a sustained eosinophilia of $1,500/mm^3$ or more in the absence of any known causes or the signs and symptoms of organ involvement. We report a 64-year-old man with HES initially presenting with involvement of the liver and bone marrow. Despite controlling the eosinophilia by corticosteroid, he developed a cerebral infarction and later progressive interstitial pneumonia. Brain angiography revealed a severe stenosis of the proximal right internal carotid artery (ICA) and a complete obstruction of the intracranial ICA. An open lung biopsy revealed fibrosis and lymphoplasma cell infiltration without eosinophils, which were consistent with nonspecific interstitial pneumonia.