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A Clinical Observation in 14 Children with SLE

Journal of the Korean Pediatric Society 1994;37(4):510-519.
Published online April 15, 1994.
A Clinical Observation in 14 Children with SLE
Jin Nyoung Park, Jai Kyoung Choi, Ha Baik Lee, Hahng Lee
Department of Pediatrics, Hanyang University, College of Medicine, Seoul, Korea
한국인 소아 전신성 홍반성 낭창 14례에 대한 임상적 양상의 분석연구
박진녕, 최재경, 이하백, 이항
한양대학교 의과대학 소아과학교실
Abstract
To elucidate the clinical characteristics of childhood onset systemic lupus erythematosus (SLE), we carried out a retrospective reivew on medical records of fourteen patients who were diagnosed as SLE on the base of 뱓he 1982 revised criteria for the SLE?(by american Rheumatism Association) at the Department of pediatrics, Hanyang University Hospital from January 1980 through August 1992. The results were summerized as follows : 1) Eleven girls and three boys were recruited in our study. The eight of them were early teens at the time of SLE diagnosis. 2) The frequencies of their clinical manifestations on admission were persistent facial rashes, edema, hepatosplenomegaly, renal abnormalities were proteinuria and hematuria. 3) Immunologic abnormalities were noted as follows : Positive ANA 100%, positive LE cell 64%, elevated anti DS-DNA antibody 64%, and decreased only C3 level 28%, decreased both C3 & C4 64% of the patients. 4) Serum anti-DS DNA (>10 units) titer was increased in clinical exacerbated three patients and decreased in four resolved. 5) The high ANA titers (> 1 : 80) obtained in eleve and/or homogenous pattern shown in seven strongly suggested SLE. 6) Renal biopsies of the four patients revealed lupus nephritis and three of the four patients had lupus band in their biopsied skin. 7) There of 14 patients were recoverd spontaneously with supportive care. Other 11 patients were treated : 7 with prednisolone only, 4 combined prednisolone and other agents. After treatment five were clinically improved. But 4 patients were frequently exacerbated 2 years, and 2 patients were died of their complications. In conclusion, serum anti-DS DNA level and ANA may be useful for screening of SLE and for predicting clinical course and prognosis of SLE.
Key Words: Systemic Lupus Erythematosus (SLE), Antinuclear Antibody (ANA), Anti-Double Stranded DNA antibody


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