Evans D I, Holzel A, MacFarlane H
Arch Dis Child. 1977 Sep;52(9):691-5. doi: 10.1136/adc.52.9.691.
The defect in Leiner's disease, which presents in early infancy with extensive dermatitis, diarrhoea, and failure to thrive, has been attributed to a defect of the fifth component of complement (C5). We report 2 brothers with extensive dermatitis and dysgammaglobulinaemia. Both died. The older showed symptoms of Leiner's disease: C5 tests were not performed. The younger had extensive dermatitis and was found to have the C5 defect. He developed normally, but died suddenly with pertussis. We postulate that the C5 defect is not the sole cause of Leiner's disease as has been suggested, but that hypogammaglobulinaemia or other lymphoid deficiency is also required for its expression.
莱纳氏病的缺陷表现为婴儿早期出现广泛的皮炎、腹泻和发育不良，这一缺陷被归因于补体第五成分（C5）的缺陷。我们报告了2例患有广泛皮炎和低丙种球蛋白血症的兄弟。两人均已死亡。哥哥表现出莱纳氏病的症状：未进行C5检测。弟弟有广泛的皮炎，发现存在C5缺陷。他发育正常，但因百日咳突然死亡。我们推测，C5缺陷并非如之前所认为的是莱纳氏病的唯一病因，而是低丙种球蛋白血症或其他淋巴样缺陷对于其表现也是必需的。
