HEREDITARY DEFICIENCY OF THE THIRD COMPONENT OF COMPLEMENT IN TWO SISTERS WITH SYSTEMIC LUPUS ERYTHEMATOSUS-LIKE SYMPTOMS
β Scribed by Yuji Sano; Hiroaki Nishimukai; Hajime Kitamura; Kazuyoshi Nagaki; Shinya Inai; Yasuteru Hamasaki; Ikuro Maruyama; Akihiro Igata
- Publisher
- John Wiley and Sons
- Year
- 1981
- Tongue
- English
- Weight
- 514 KB
- Volume
- 24
- Category
- Article
- ISSN
- 0004-3591
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β¦ Synopsis
Abstract
We observed two sisters with lupusβlike syndrome with homozygous C3 deficiencies. A 19βyearβold woman and her 15βyearβold sister developed malar rash, arthralgia, and photosensitivity, but antinuclear antibodies and LE cell preparations were negative. The older sister experienced recurrent bronchitis in her childhood, but the younger sister had no recurrent infections. Serum C3 was not detected immunochemically in either sister, and total complement activity and C3 hemolytic activity were extremely low.
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## Abstract The in vivo metabolism of purified C3, the third component of complement, labeled with ^125^βiodine (^125^IβC3) was studied in 17 normal volunteers and in 24 patients with systemic lupus erythematosus (SLE). The latter had varying degrees of disease severity and differing clinical and s
## Abstract We studied 31 children with systemic lupus erythematosus (SLE) and 108 firstβdegree relatives of the children to determine if HLA type, familial relationship to patient, or gender influenced the familial aggregation of SLE and the serologic and serum complement abnormalities associated