HEMOSTATIC DEFECT IN GLYCOGEN STORAGE DISEASE TYPE I

被引：22

作者：

GILCHRIST, GS

FINE, RN

DONNELL, GN

机构：

[1] Divisions of Hematology and Metabolic-Renal Disease, Childrens Hospital of Los Angeles, Department of Pediatrics, University of Southern California School of Medicine, Los Angeles, California

来源：

ACTA PAEDIATRICA SCANDINAVICA | 1968年 / 57卷 / 03期

关键词：

bleeding time; Glycogen storage disease; hemostatic defect; platelet adhesiveness; platelet factor 3;

D O I：

10.1111/j.1651-2227.1968.tb04679.x

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Because of the ill‐defined bleeding tendency which has been reported in patients with Glycogen Storage Disease, Type 1, various aspects of hemostasis and coagulation were studied in 8 patients. Definite abnormalities in the bleeding time and platelet factor 3 availability were found in the older symptomatic individuals but all the patients, irrespective of age, exhibited significant reduction in platelet adhesiveness in spite of normal platelet aggregation after addition of ADP. Although an intracellular defect may account for the abnormal platelet function, environmental plasma factors appear to play a role in the development of the platelet lesion. Copyright © 1968, Wiley Blackwell. All rights reserved

引用

页码：205 / +

页数：1

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