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유전분증의 임상적 특징

Other Titles
 Clinical Characteristics of Amyloidosis 
Authors
 김형종  ;  문성진  ;  김현진  ;  최훈영  ;  류동열  ;  유태현  ;  김주성  ;  박정탁  ;  강신욱  ;  최규헌 
Citation
 Korean Journal of Nephrology (대한신장학회지), Vol.24(2) : 230-238, 2005 
Journal Title
 Korean Journal of Nephrology (대한신장학회지) 
ISSN
 1225-0015 
Issue Date
2005
Abstract
Background: Amyloidosis is a rare disease that amyloid fibril deposit in multiple organic tissue and decrease organic functions. Many case reports for amyloidosis were published but it is rare to domestic generalized report for amyloidosis. The purpose of this study is to evaluate the clinical manifestation and prognostic factors of amyloidosis. Methods: From January, 1986 to December, 2002, Total 33 patients that diagnosed of amyloidosis, pathologically were included. The patients were divided to two groups in primary amyloidosis and secondary amyloidosis according to causative diseases. Results: Thirty three patients were diagnosed to amyloidosis (men 19 patietns, women 14 patients) with mean age 54.8±13.2 years. Of the 33 patients, 21 patients had primary amyloidosis and 12 patients had secondary amyloidosis. The causes of amyloidosis were unknown 21 patients (67.7%), multiple myeloma 7 patients (16.1%), rheumatoid arthritis 3 patients (9.9%), Castleman’s disease 1 patient (3.2%), ankylosing spondylitis 1 patient (3.2%). Edema (33.3 %) and gastrointestinal symptoms (21.2%) are most common initial symptom. There are hepatomegaly (27.2%), hepatosplenomegaly (6.1%), splenomegaly (3.0%), hypotension (6.1%), hypertension (6.1%) and no abnormal finding (48.5%) on initial physical examination. Diagnostic biopsy sites were various according to amyloid deposit sites and biopsy performed at kidney (33.3%), heart+rectum (12.1%), heart (9.1%), liver (6.1%), rectum (6.1%), stomach (6.1%). Conclusion: Edema is most common initial symptom of amyloidosis but various clinical symptoms are showed according to amyloid deposit site. Biopsy should be performed at suspicious amyloidosis patients of more than 50 years and early diagnosis and proper management of amyloidosis may be helpful to treatment and prognosis of amyloidosis patients.
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1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
Yonsei Authors
강신욱(Kang, Shin Wook) ORCID logo https://orcid.org/0000-0002-5677-4756
김주성(Kim, Joo Sung)
김형종(Kim, Hyung Jong)
류동열(Ryu, Dong Ryeol)
문성진(Moon, Sung Jin)
박정탁(Park, Jung Tak) ORCID logo https://orcid.org/0000-0002-2325-8982
유태현(Yoo, Tae Hyun) ORCID logo https://orcid.org/0000-0002-9183-4507
이호영(Lee, Ho Yung)
최규헌(Choi, Kyu Hun) ORCID logo https://orcid.org/0000-0003-0095-9011
최훈영(Choi, Hoon Young) ORCID logo https://orcid.org/0000-0002-4245-0339
한대석(Han, Dae Suk)
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URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/151168
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