Starke I D, Corrin B, Selby P J, Webster A D, Turner-Warwick M
Thorax. 1981 Jul;36(7):502-7. doi: 10.1136/thx.36.7.502.
Four girls born to second cousin parents developed chronic chest infection and bronchiectasis in infancy. Three were studied in detail: they all had the same HLA haplotype, all showed random orientation of cilia or compound cilia in the respiratory tract, and all had low levels of the C1 and C2 components of the complement system. Although the cause of the respiratory disease in this family remains unclear, it is suggested that the low C1 levels may have contributed to the disease in two of the children while the low C2 levels were artefacts and the ciliary abnormalities were secondary to chronic chest infection.
四位由近亲父母所生的女孩在婴儿期出现了慢性胸部感染和支气管扩张。对其中三位进行了详细研究：她们都具有相同的人类白细胞抗原单倍型，呼吸道中均显示纤毛随机定向或复合纤毛，并且补体系统的C1和C2成分水平均较低。尽管该家族中呼吸系统疾病的病因尚不清楚，但有人认为低C1水平可能导致了其中两名儿童患病，而低C2水平是人为因素造成的，纤毛异常是慢性胸部感染的继发症状。
