%0 Journal Article
%T Adrenal failure followed by status epilepticus and hemolytic anemia in primary antiphospholipid syndrome
%A Patrick Gerner
%A Michael Heldmann
%A Peter Borusiak
%A Vladimir Bures
%A Stefan Wirth
%J Thrombosis Journal
%D 2005
%I BioMed Central
%R 10.1186/1477-9560-3-6
%X Adrenal failure is a rare complication of APS in children with only five cases reported to date. As shown in our patient, this syndrome can manifest in a diverse set of simultaneously occurring symptoms.The antiphospholipid syndrome is characterized by clinical evidence of arterial or venous thrombosis and repeated presence of antiphospholipid autoantibodies. The disease, first described by Hughes in 1983 [1], may occur in a variety of conditions including neoplasms, infections, other autoimmune disease such as lupus erythematodes, and after administration of certain drugs. If found without any other demonstrable disease, it is termed primary antiphospholipid syndrome.The autoantibodies, of which the most important are lupus anticoagulant and cardiolipin-antibodies, comprise a heterogeneous group which are mainly directed against complexes of anionic phospholipid with some phospholipid-binding proteins such as beta-2-glycoprotein I and human prothrombin. In a proportion of individuals the circulation of the antibodies may induce thrombosis of virtually any vein or artery. The most common are deep vein thrombosis, pulmonary, or cerebrovascular embolisms. In addition, other complications such as cardiomyopathy, hepatitis, hemolytic anemia, bleeding, vasculitis and renal failure have been reported [2-5].The complexity of manifestations and the risk of severe complications, led us to conclude that this syndrome is an important differential diagnosis in patients with the described symptoms.In September 2001, a previously healthy, Caucasian, 14-year-old boy was admitted to our hospital. Two weeks prior to his arrival, he had developed abdominal pain, a recurring fever of up to 39ˇăC, and an intermittent cough. These symptoms were worsening and his general condition was deteriorating.On examination he had diffuse abdominal pain located primarily in the upper abdomen, and his temperature was 38.5ˇăC. His skin and the further physical examination was normal. Pertinent laborato
%U http://www.thrombosisjournal.com/content/3/1/6