Temple A S, Hinton P, Narcisi P, Pope F M
MRC Clinical Research Centre, Harrow, Middlesex, U.K.
Br J Dermatol. 1988 Jan;118(1):17-26. doi: 10.1111/j.1365-2133.1988.tb01745.x.
Immunofluorescence showed that cultured skin fibroblasts from 15 out of 17 patients with Ehlers-Danlos syndrome type IV retained abnormal amounts of type III collagen within the cytoplasm. This was not shown by fibroblasts from normal subjects or from patients with other inherited connective tissue diseases. The diagnosis of Ehlers-Danlos syndrome type IV may be facilitated by this finding.
免疫荧光显示,17例IV型埃勒斯-当洛综合征患者中有15例的培养皮肤成纤维细胞在细胞质内保留了异常数量的III型胶原蛋白。正常受试者或其他遗传性结缔组织疾病患者的成纤维细胞未出现这种情况。这一发现可能有助于IV型埃勒斯-当洛综合征的诊断。