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Circulating CCR7(lo)PD-1(hi) Follicular Helper T Cells Indicate Disease Activity and Glandular Inflammation in Patients with Primary Sjögren's Syndrome

Kim JW, Lee J, Hong SM, Lee J, Cho ML, Park SH

Since primary Sjögren's syndrome (pSS) is an autoummune disease of B cell hyperactivity and pathologic autoantibody response, follicular helper T (Tfh) cells and follicular regulatory T (Tfr) cells are suggested...
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Cutaneous and Systemic Plasmacytosis Associated with Renal Amyloidosis

Lee TG, Jeong WS, Moon SH, Hwangbo H, Lee SK, Lee DR, Kwon TG

Cutaneous and systemic plasmacytosis (CSP) is a rare disorder of unknown etiology characterized by cutaneous polyclonal plasma cell infiltrates associated with various extracutaneous involvement and polyclonal hypergammaglobulinemia. Here, we report...
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Identification of a Novel Mutation in the CYBB Gene, p.Asp378Gly, in a Patient With X-linked Chronic Granulomatous Disease

Song SM, Park MR, Kim DS, Kim J, Kim YJ, Ki CS, Ahn K

Chronic granulomatous disease (CGD) is a rare immunodeficiency disease, which is characterized by the lack of a functional nicotinamide adenine dinucleotide phosphate (NADPH) oxidase in phagocytes. The disease presents leukocytosis,...
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Azidothymidine and recombinant human interferon-alpha therapy in a cat with feline immunodeficiency virus

Jang HJ, Ho YK, Kang MH, Kim SG, Park WJ, Choi IS, Kim DY, Park HM

  • KMID: 1709212
  • J Biomed Res.
  • 2014 Jun;15(2):96-99.
A 7-year-old, spayed female, domestic short hair cat showed signs of a 2-week history of chronic anorexia, depression, and severe weight loss. Upon physical examination, pyrexia, mild gingivitis, and pale...
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A Case of Cutaneous Rosai-Dofman Disease Treated with Isotretinoin and Pulsed Dye Laser

Han J, Kim JE, Park GH, Won CH, Chang SE, Lee MW, Choi JH, Moon KC

  • KMID: 2388401
  • Korean J Dermatol.
  • 2013 Jun;51(6):465-469.
Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy (SMHL) is a benign histiocytic proliferative disorder of unknown etiology. The disease is usually accompanied by massive bilateral lymphadenopathy, fever, elevated...
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A Case of Autoimmune Hepatitis Combined with Ulcerative Colitis

Lee KJ, Lee JS, Bae WK, Kim NH, Kim KA, Moon YS, Joo M

  • KMID: 2258870
  • Korean J Med.
  • 2011 Jun;80(Suppl 2):S106-S110.
Autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and primary biliary cirrhosis (PBC) are the major immune-mediated chronic liver diseases. They are characterized by circulating autoantibodies, hypergammaglobulinemia, unique clinical features, and...
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A Case of Autoimmune Hepatitis Following Acute Hepatitis A

Kim YD, Kim KA, Rou WS, Lee JS, Song TJ, Bae WK, Kim NH

The pathogenesis of autoimmune hepatitis (AIH) is unclear, but viral infections have been proposed as a potential trigger in patients with genetic predisposition. We report a case of AIH following...
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Two Cases of Eosinophilic Fasciitis

Chun JH, Lee KH, Sung MS, Park CJ

Eosinophic fasciitis (EF) is an uncommon connective tissue disease characterized by scleroderma-like cutaneous changes, peripheral eosinophilia, hypergammaglobulinemia, and an elevated erythrocyte sedimentation rate (ESR). Typical histopathologic findings include chronic inflammatory...
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Systemic Plasmacytosis: A Case Report with a Review of the Literature

Lee SH, Yoo CY, Jung JH, Yoo JY, Kang SJ, Kang CS

  • KMID: 1979067
  • Korean J Pathol.
  • 2011 Dec;45(6):632-638.
Systemic plasmacytosis is an uncommon disorder characterized by widely disseminated macular skin eruptions composed of polyclonal lymphoplasmacytic infiltrates associated with variable extracutaneous involvement. An aggressive clinical course has been observed...
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Castleman's Disease with Cutaneous Involvement Manifestating as Multiple Violaceous Plaques on Entire Body

Park HY, Lee JJ, Lee JB, Kim SJ, Lee SC, Won YH, Yun SJ

Castleman's disease (CD) is an uncommon B-cell lymphoproliferative disorder characterized by lymph node hyperplasia with vascular proliferation. Cutaneous involvement in CD is rare. A 65-year-old man presented with a 7-year...
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Suspicious Eosinophilic Fasciitis Preceding Peripheral T Cell Lymphoma

  • KMID: 2299541
  • Korean J Asthma Allergy Clin Immunol.
  • 2010 Sep;30(3):233-236.
Eosinophilic fasciitis (EF) is characterized by scleroderma-like skin lesions, peripheral eosinophilia and hypergammaglobulinemia. While EF is generally benign course, it may precede hematologic malignancy or Hodgkin's disease, association with peripheral...
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Castleman's Disease Presenting with Uveal Effusion Syndrome

Park SH, Song SJ

We report a rare case of multicentric Castleman's disease that presented with ophthalmic involvement, along with a review of the literature. A 63-year-old male presented with decreased visual acuity in...
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A Case of Sjogren's Syndrome That Presented with Alcohol-induced Purpura

Cheong SH, Han YJ, Choi YW, Myung KB, Lee JS, Choi HY

Sjogren syndrome (SS) is a systemic autoimmune disease that mainly affects the salivary and lacrimal glands. It may exist as a primary condition or in association with other systemic autoimmune...
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A Case of Non Secretory Type Multiple Myeloma Associated with Systemic Lupus Erythematosus

Jeong YJ, Kim HS, Kim EJ, Kim YS, Park CY, Shin BS, Park G

The development of malignant tumors is a more frequent finding in lupus patients than the general population. However, the incidence of multiple myeloma associated with systemic lupus erythematosus (SLE) is...
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A Case of Cutaneous and Systemic Plasmacytosis with an Unusual Clinical Presentation

Lee DW, Yang JH, Won CH, Chang SE, Lee MW, Choi JH, Moon KC

  • KMID: 2247894
  • Korean J Dermatol.
  • 2009 May;47(5):558-561.
Cutaneous and systemic plasmacytosis is a rare disorder that's characterized by a cutaneous plasma cell infiltrate accompanied by polyclonal hypergammaglobulinemia. Clinically, it appears as multiple red-brown plaques that mainly occur...
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A case of concomitant epstein-barr virus-induced hepatitis on chronic autoimmune hepatitis

Jung JG, Moon DG, Yang HJ, Kwon JH, Kang JH, Jung ES, Yoon SK

  • KMID: 2256198
  • Korean J Med.
  • 2008 Jun;74(6):658-662.
Autoimmune hepatitis (AIH), a rare cause of chronic liver disease in Korea, is a chronic, predominantly periportal hepatitis characterized by hypergammaglobulinemia, tissue autoantibodies, and responsiveness to immunosuppressive therapy. Here we...
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Eosinophilic Fasciitis Localized on the Left Lower Extremity: A case report

Jeong HJ

  • KMID: 2324606
  • J Korean Acad Rehabil Med.
  • 2007 Aug;31(4):478-481.
Eosinophilic fasciitis is a rare disorder which can markedly affect the quality of life in individual patients by disabling joint mobility. Eosinophilic fasciitis causes symmetrical pain, swelling and contracture of...
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A Case of Rosai-Dorfman Disease Treated with Hydroxychloroquine

Baek JO, Lee HY, Lee JR, Kim M, Roh JY, Kim YC

  • KMID: 2302140
  • Korean J Dermatol.
  • 2007 Oct;45(10):1055-1059.
Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy (SMHL) is a benign histiocytic proliferative disorder of unknown etiology. It is accompanied by fever, leukocytosis, elevated erythrocyte sedimentation rate and polyclonal...
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Autoimmune Hepatitis: Recent Korean Trend

Lee YS

Autoimmune hepatitis is defined as an unresolving, predominantly periportal hepatitis of unknown etiology, usually with hypergammaglobulinemia and tissue autoantibodies, which is responsive to immunosuppressive therapy. Clinical manifestations, diagnostic criteria, differential...
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Clinical Characteristics, Histology and Prognosis of Autoimmune Hepatitis in Korean Children

Chung DL, Seo JK, Yang HR, Ko JS, Park SH

  • KMID: 2275201
  • Korean J Pediatr Gastroenterol Nutr.
  • 2004 Sep;7(2):186-196.
PURPOSE: Autoimmune hepatitis is a chronic inflammatory liver disease with unknown cause that is characterized by liver histology, circulating autoantibodies and increased levels of immunoglobulin G. Only sporadic reports are...
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