Clinical, Serological and Genetic Study in Patients with CREST Syndrome.
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- AKIYAMA Yuji
- the Department of Rheumatology, Saitama Medical School
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- TANAKA Masahiko
- the Department of Rheumatology, Saitama Medical School
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- TAKEISHI Michio
- the Department of Rheumatology, Saitama Medical School
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- ADACHI Daisuke
- the Department of Rheumatology, Saitama Medical School
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- MIMORI Akio
- the Department of Rheumatology, Saitama Medical School
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- SUZUKI Teruhiko
- the Department of Rheumatology, Saitama Medical School
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Objective To assess the clinical, serological and genetic features of Japanese patients with CREST syndrome.<br> Patients and Methods Clinical features, autoantibodies and human histocompatibility leukocyte antigen (HLA) typing were studied in thirty patients with CREST syndrome, including 29 females and one male, with a mean age of 59.0 years (ranging from 40 to 76 years).<br> Results Interstitial pneumonia on chest X-ray and renal involvement were rare. Mitral regurgitation and tricuspid regurgitation were present in 56.7% and 76.7%, respectively. Sjören's syndrome (SS) and primary biliary cirrhosis (PBC) were highly associated, however the positivity of the marker antibodies to those syndromes, such as anti-SSA, anti-SSB, anti-mitochondrial (AMA) and antismooth muscle autoantibodies were less frequent than that of primary SS and PBC without the other autoimmune diseases. The histological findings of PBC were all early stages in Scheuer's classification. HLA-Cw6 were associated with CREST-PBC overlap syndrome (p<0.05). However the HLA antigen was not correlated with CREST syndrome, and the frequency of HLA-DR2 between CREST syndrome with or without PBC was significantly different (p<0.01).<br> Conclusion It was suggested that there was a genetic difference between CREST syndrome alone and CREST-PBC overlap syndrome and there were differences (the positivity of AMA and the severity of bile duct lesion) between PBC and CREST-PBC overlap syndrome.<br>(Internal Medicine 39: 451-456, 2000)
収録刊行物
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- Internal Medicine
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Internal Medicine 39 (6), 451-456, 2000
一般社団法人 日本内科学会
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詳細情報 詳細情報について
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- CRID
- 1390282679843703680
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- NII論文ID
- 10006994977
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- NII書誌ID
- AA10827774
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- COI
- 1:STN:280:DC%2BD3cvpt1OksA%3D%3D
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- ISSN
- 13497235
- 09182918
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- NDL書誌ID
- 5384789
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- PubMed
- 10852162
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- 本文言語コード
- en
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- データソース種別
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- JaLC
- NDL
- Crossref
- PubMed
- CiNii Articles
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- 抄録ライセンスフラグ
- 使用不可