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THE HEMOSTATIC DEFECT IN GLYCOGEN STORAGE DISEASE, TYPE I
Author(s) -
Gilchrist Gerald S.,
Fine Richard N.,
Donnell George N.
Publication year - 1968
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1968.tb04679.x
Subject(s) - medicine , platelet , hemostasis , coagulation , glycogen storage disease , glycogen , disease , glycogen storage disease type i , lesion , platelet aggregation , gastroenterology , surgery
Summary 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.