Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT 14-06: Congenital Disorders of Platelet Function + Key Features Download Section PDF Listen +++ ++ Results from a qualitative or quantitative abnormality in the platelet glycoprotein IIb/IIIa receptor on the platelet membrane and the fibrinogen receptor critical for linking platelets during initial platelet aggregation/platelet plug formation Inheritance is autosomal recessive + Clinical Findings Download Section PDF Listen +++ ++ Onset of bleeding is usually in infancy or childhood, but some forms are milder and present later in life Degree of deficiency in IIb/IIIa may not correlate well with bleeding symptoms Bleeding due to defective platelets is classically mucocutaneous, but is not limited to mucocutaneous surfaces Bleeding Mucosal (epistaxis, gingival) Menorrhagia Postoperative Variable severity but may be severe + Diagnosis Download Section PDF Listen +++ ++ Platelet aggregation studies show marked impairment of aggregation in response to stimulation with various agonists + Treatment Download Section PDF Listen +++ ++ Platelet transfusions when necessary Desmopressin acetate (DDAVP), antifibrinolytic agents, and recombinant human activated factor VII also have been used successfully