Metabolical and morphological study for endothelial cell injry and platelet aggregation in childhood disorders.

已有报道在实验性5-羟色胺肌病和Duchenne肌营养不良症(Duchenne型肌营养不良症)中观察到血管内皮细胞的改变，包括水疱样肿胀。Wistar大鼠腹腔注射5-羟色胺(75 mg/kg)。光镜下未见结构损伤，但肌肉毛细血管超微结构改变明显。更令人印象深刻的异常之一是大的水疱状肿胀，它似乎起源于内皮细胞的管腔表面，并延伸到管腔，有时几乎完全闭塞。这些现象与在DMD肌肉组织中看到的非常相似。(2)用抗DMD基因产物(Dystrophin)的抗体进行免疫组织化学研究。在大、小鼠的各种组织中，dystrophin明显定位于各种大小血管的平滑肌层(中膜)的表面膜，尤其是肌性动脉。注射5-羟色胺10小时后，大鼠肺内、甚至肌肉动脉内的水泡失去了抗肌营养不良反应。这些结果表明，由于dystrophin缺陷和5-羟色胺注射引起的继发性损伤等导致的中膜功能障碍，导致内皮细胞损伤和血小板聚集。再想一想，我们认为，任何引起血小板聚集，导致血管内释放大量5-羟色胺的条件，也会导致内皮细胞损伤。

It has been reported that endothelial alterations including blister-like swelling, were observed in experimental serotonin myopathy as well as Duchenne muscular dystrophy (DMD).In the first study, we observed blood vessels in rat muscle tissues after serotonin injection.(1).Serotonin experiment. serotonin (75mg/Kg) was injected to Wister rats intraperitoneally. Althouge no structural damage was observed with the light microscope, ultrastructural changes were prominent in-muscle capillaries. One of the more impressive abnormalities consisted of large blister-like swelling, which appeared to originate from the luminal surface of the endothelial cell and extend into the lumen, at times almost completely occluding it. These phnomena were quite similar to those seen in DMD muscle tissue.(2).Immunohistochemical study using antibodies against DMD gene product (dystrophin). Dystrophin was clearly localized in the surface membrane of smooth muscle layers (tunica media) of blood vessels of any kinds and sizes, especially muscular arteries, in various tissues from rats and mice. Ten hours after serotonin injection, bolld vesse in lung , even muscular arteries, lost dystrophin reaction. These results suggested that, dysfunction of tunica media, due to defective dystrophin and secondary damage by serotonin injection for instance, induce endothelial cell damage and platelet aggregation. On second thought, it wa suggested that, any conditions which induce platelet aggregation resulting abundant serotonin release in blood vessels, also produce endothelial cell injury.