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    Localized Thymic Amyloidosis Presenting with Myasthenia Gravis: Case Report

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    https://www.riss.kr/link?id=A104783991

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    A mediastinal mass was incidentally found on chest radiography in a 46-yr-old woman who had had myasthenia gravis (MG) for 2 months. Computed tomography revealed a 4-cm in size, well-defined, and lobulating mass with nodular calcification that was located in the thymus. Microscopically, the mass consisted of diffuse amorphous eosinophilic materials. These deposits exhibited apple-green birefringence under polarized light microscopy after Congo red staining. Immunohistochemical analysis revealed that they were positive for both kappa and lambda light chains and negative for amyloid A. A diagnosis of localized primary thymic amyloidosis was finally made. After thymectomy, the symptoms of MG were controlled with reduced corticosteroid requirements. Localized thymic amyloidosis associated with MG has not been reported to date.
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    A mediastinal mass was incidentally found on chest radiography in a 46-yr-old woman who had had myasthenia gravis (MG) for 2 months. Computed tomography revealed a 4-cm in size, well-defined, and lobulating mass with nodular calcification that was loc...

    A mediastinal mass was incidentally found on chest radiography in a 46-yr-old woman who had had myasthenia gravis (MG) for 2 months. Computed tomography revealed a 4-cm in size, well-defined, and lobulating mass with nodular calcification that was located in the thymus. Microscopically, the mass consisted of diffuse amorphous eosinophilic materials. These deposits exhibited apple-green birefringence under polarized light microscopy after Congo red staining. Immunohistochemical analysis revealed that they were positive for both kappa and lambda light chains and negative for amyloid A. A diagnosis of localized primary thymic amyloidosis was finally made. After thymectomy, the symptoms of MG were controlled with reduced corticosteroid requirements. Localized thymic amyloidosis associated with MG has not been reported to date.

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    참고문헌 (Reference)

    1 Shah PL, "The importance of complete screening for amyloid fibril type and systemic disease in patients with amyloidosis in the respiratory tract" 19 : 134-142, 2002

    2 Wilson PG, "Serum amyloid A, but not C-reactive protein, stimulates vascular proteoglycan synthesis in a pro-atherogenic manner" 173 : 1902-1910, 2008

    3 Westermark GT, "Serum amyloid A and protein AA: molecular mechanisms of a transmissible amyloidosis" 583 : 2685-2690, 2009

    4 Anthony DC, "Robbins and Cotran pathologic basis of disease, professional edition" Saunders 1275-1276, 2008

    5 "Nomenclature of amyloid and amyloidosis: WHO-IUIS Nomenclature Sub-Committee" 71 : 105-112, 1993

    6 Conti-Fine BM, "Myasthenia gravis: past, present, and future" 116 : 2843-2854, 2006

    7 Merlini G, "Molecular mechanisms of amyloidosis" 349 : 583-596, 2003

    8 Ha SY, "Localized primary thymic amyloidosis presenting as a mediastinal mass: a case report" 45 : S41-S44, 2011

    9 Siddachari RC, "Laryngeal amyloidosis" 34 : 60-63, 2005

    10 Kaplan B, "Free light chains in plasma of patients with light chain amyloidosis and non-amyloid light chain deposition disease: high proportion and heterogeneity of disulfide-linked monoclonal free light chains as pathogenic features of amyloid disease" 144 : 705-715, 2009

    1 Shah PL, "The importance of complete screening for amyloid fibril type and systemic disease in patients with amyloidosis in the respiratory tract" 19 : 134-142, 2002

    2 Wilson PG, "Serum amyloid A, but not C-reactive protein, stimulates vascular proteoglycan synthesis in a pro-atherogenic manner" 173 : 1902-1910, 2008

    3 Westermark GT, "Serum amyloid A and protein AA: molecular mechanisms of a transmissible amyloidosis" 583 : 2685-2690, 2009

    4 Anthony DC, "Robbins and Cotran pathologic basis of disease, professional edition" Saunders 1275-1276, 2008

    5 "Nomenclature of amyloid and amyloidosis: WHO-IUIS Nomenclature Sub-Committee" 71 : 105-112, 1993

    6 Conti-Fine BM, "Myasthenia gravis: past, present, and future" 116 : 2843-2854, 2006

    7 Merlini G, "Molecular mechanisms of amyloidosis" 349 : 583-596, 2003

    8 Ha SY, "Localized primary thymic amyloidosis presenting as a mediastinal mass: a case report" 45 : S41-S44, 2011

    9 Siddachari RC, "Laryngeal amyloidosis" 34 : 60-63, 2005

    10 Kaplan B, "Free light chains in plasma of patients with light chain amyloidosis and non-amyloid light chain deposition disease: high proportion and heterogeneity of disulfide-linked monoclonal free light chains as pathogenic features of amyloid disease" 144 : 705-715, 2009

    11 Bhat A, "Currents concepts on the immunopathology of amyloidosis" 38 : 97-106, 2010

    12 Morgutti M, "Cellular immune response to acetylcholine receptor in myasthenia gravis: II. thymectomy and corticosteroids" 29 : 734-738, 1979

    13 Urban BA, "CT evaluation of amyloidosis : spectrum of disease" 13 : 1295-1308, 1993

    14 Hohlfeld R, "Autoimmune human T lymphocytes specific for acetylcholine receptor" 310 : 244-246, 1984

    15 Wetmore JB, "Associations of interleukin-6, C-reactive protein and serum amyloid A with mortality in haemodialysis patients" 13 : 593-600, 2008

    16 Pepys MB, "Amyloidosis" 57 : 223-241, 2006

    17 Takamori S, "Amyloid tumor in the anterior mediastinum: report of a case" 34 : 518-520, 2004

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    학술지 이력

    학술지 이력
    연월일 이력구분 이력상세 등재구분
    2023 평가 해외DB학술지평가 신청대상 (해외등재 학술지 평가)
    2020-01-01 등재 등재학술지 유지 (해외등재 학술지 평가) KCI등재
    2011-01-01 등재 등재학술지 유지 (등재유지) KCI등재
    2009-01-01 등재 등재학술지 유지 (등재유지) KCI등재
    2005-01-01 등재 SCI 등재 (등재유지) KCI등재
    2002-01-01 등재 등재학술지 선정 (등재후보2차) KCI등재
    1999-07-01 등재 등재후보학술지 선정 (신규평가) KCI등재후보
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    학술지 인용정보

    학술지 인용정보
    기준연도 WOS-KCI 통합IF(2년) KCIF(2년) KCIF(3년)
    2016 1.48 0.37 1.06
    KCIF(4년) KCIF(5년) 중심성지수(3년) 즉시성지수
    0.85 0.75 0.691 0.11
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