23325Rhode Island Hospital, Providence, RI, USA.
Brown University, Providence, RI, USA.
Vet Pathol. 2021 Nov;58(6):1100-1106. doi: 10.1177/03009858211018660. Epub 2021 May 31.
The objectives of this study were to describe the anatomy, histology, and ultrastructure of the equine filum terminale (FT) and to describe the FT in hereditary equine regional dermal asthenia (HERDA), a model of human Ehlers-Danlos syndromes (EDS). Those humans suffer from tethered cord syndrome (TCS) caused by an abnormally structured FT wherein its attachment at the base of the vertebral column leads to long-term stretch-induced injury to the spinal cord. The pathophysiology of TCS in EDS is poorly understood, and there is a need for an animal model of the condition. Histopathologic and ultrastructural examinations were performed on FT from HERDA ( = 4) and control horses ( = 5) and were compared to FT from human TCS patients with and without EDS. Adipose, fibrous tissue, and neuronal elements were assessed. CD3 and CD20 immunohistochemistry was performed to clarify cell types (HERDA = 2; control = 5). Collagen fibrils were assessed in cross-section for fibril diameter and shape, and in longitudinal section for fibril disorganization, swelling, and fragmentation. The equine and human FT were similar, with both containing fibrous tissue, ependyma, neuropil, and nerve twigs. Hypervascularity was observed in both HERDA horses and human EDS-TCS patients and was not observed in equine or human controls. Moderate to severe abnormalities in collagen fibril orientation and architecture were observed in all HERDA horses and were similar to those observed in human EDS-TCS patients.
本研究的目的是描述马的终丝（FT）的解剖结构、组织学和超微结构，并描述遗传性马的区域性皮肤虚弱症（HERDA）中的 FT，HERDA 是人类埃勒斯-当洛斯综合征（EDS）的模型。这些人患有因异常结构的 FT 引起的脊髓栓系综合征（TCS），其在脊柱底部的附着导致脊髓长期拉伸引起的损伤。EDS 中 TCS 的病理生理学尚未完全了解，因此需要该疾病的动物模型。对来自 HERDA（n = 4）和对照马（n = 5）的 FT 以及来自患有和不患有 EDS 的人类 TCS 患者的 FT 进行了组织病理学和超微结构检查，并进行了比较。评估了脂肪、纤维组织和神经元成分。进行了 CD3 和 CD20 免疫组织化学染色，以阐明细胞类型（HERDA：n = 2；对照：n = 5）。评估了胶原纤维在横切面上的纤维直径和形状，以及在纵切面上的纤维紊乱、肿胀和碎片化。马和人类的 FT 相似，均含有纤维组织、室管膜、神经胶质和神经分支。在 HERDA 马和人类 EDS-TCS 患者中均观察到血管增生，而在马和人类对照中未观察到。在所有 HERDA 马中均观察到胶原纤维取向和结构的中度至重度异常，与在人类 EDS-TCS 患者中观察到的异常相似。
