Platelet function defects comprise a large and heterogeneous group of bleeding disorders that range in severity from mild to severe. Patients may be asymptomatic; however, the majority who are diagnosed present with easy bruising and mucocutaneous bleeding, or with excessive haemorrhage following injury or surgery. As the complex internal biochemical and signal transduction pathways are further understood, and as structural analysis of platelets advances, more of the mechanisms leading to platelet function defects will be uncovered. Despite advances in the understanding of the etiology of these defects in function, treatment remains fairly rudimentary. For platelet function disorders associated with a defect in a plasma coagulation factor, such as vWD and afibrinogenemia, treatment consists of replacement of the deficient coagulation factor. Adjunctive therapies (such as antifibrinolytics, microfibular collagen, fibrin glue, etc.), DDAVP/Stimate, and platelet transfusions remain the mainstay of therapy available at this time. Corticosteroids may play a role in the management of some of these disorders and may lead to a shortening of bleeding time, especially in storage pool defects or release defects failing to respond to DDAVP/Stimate.
Clinical History.\p=m-\This1-year-old girl had a fever for 24 hours prior to admission to hospital. There was a history of a urinary tract infection at 6 months of age. The child was other-wise healthy, and there was no history of other illnesses. She was the product of a full-term delivery without incident.Physical Examination.\p=m-\Temperature on admission was 38.9 C. The child was generally ill and irritable. There was no icterus. There was a fullness in the right flank, and the bladder was somewhat distended. The liver was not palpable, and there was no edema of the extremities. The remainder of the examination was within normal limits.Results of urinalysis revealed numerous WBCs per high power field. The hemogram, blood urea, and serum creatinine determinations were normal. An intravenous urogram (Fig 1) and a cystogram (Fig 2) were obtained after antibiotic treatment of the urinary infection.
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