Sjaastad O V, Blom A K, Stormorken H, Nes N
Department of Physiology and Nutrition, Norwegian College of Veterinary Medicine, Oslo.
Am J Med Genet. 1990 Mar;35(3):373-8. doi: 10.1002/ajmg.1320350312.
Bleeding times, concentrations of serotonin in whole blood, and concentrations of adenine nucleotides as well as aggregation properties of platelets were examined in 18 blue foxes with Chediak-Higashi-like syndrome (CHS) and 16 controls. A claw of each ketamine-sedated fox was cut until bleeding started and the bleeding time was recorded as the time from the first to the last drop. The bleeding time was greatly increased in CHS foxes. Platelet counts of CHS foxes were normal, but aggregation induced by adenosine diphosphate (ADP), serotonin, collagen, and arachidonate was impaired. Adrenaline and serotonin was impaired. Adrenaline and serotonin potentiated the aggregatory effect of ADP on control as well as on CHS platelets. The mean concentration of ADP in CHS platelets was about one-third that in controls, whereas adenosine triphosphate (ATP) was approximately one-half that in controls. Serotonin could not, in most cases, be detected in blood of CHS foxes. These findings suggest that the prolonged bleeding time in the CHS foxes is, at least partly, due to a storage pool deficiency. The drastically reduced, and in some cases absent, aggregation of CHS platelets in response to arachidonate suggests that defective arachidonate metabolism contributes to the impaired hemostasis.
对18只患有类切-东综合征(CHS)的蓝狐和16只对照蓝狐进行了出血时间、全血中血清素浓度、腺嘌呤核苷酸浓度以及血小板聚集特性的检测。对每只经氯胺酮镇静的狐狸的一只爪子进行切割,直至开始出血,并将出血时间记录为从第一滴血到最后一滴血的时间。CHS狐狸的出血时间大幅延长。CHS狐狸的血小板计数正常,但二磷酸腺苷(ADP)、血清素、胶原蛋白和花生四烯酸盐诱导的聚集受损。肾上腺素和血清素的作用也受损。肾上腺素和血清素增强了ADP对对照血小板以及CHS血小板的聚集作用。CHS血小板中ADP的平均浓度约为对照血小板的三分之一,而三磷酸腺苷(ATP)约为对照血小板的二分之一。在大多数情况下,CHS狐狸的血液中检测不到血清素。这些发现表明,CHS狐狸出血时间延长至少部分是由于储存池缺陷。CHS血小板对花生四烯酸盐的聚集大幅减少,在某些情况下甚至不存在,这表明花生四烯酸盐代谢缺陷导致止血功能受损。