Avtor/Urednik		Avčin, Tadej; Cimaz, Ronaldo; Silverman, Earl D; Cervera, Ricard; Gattorno, Marco; Garay, Sstella; Berkun, Yackov; Sztajnbok, Flavio R; Silva, Clovis A; Rozman, Blaž
Naslov		Pediatric antiphospholipid syndrome: clinical and immunologic features of 121 patients in an international registry
Tip		članek
Vir		Pediatrics
Vol. in št.		Letnik 122, št. 5
Leto izdaje		2008
Obseg		str. e1100-7
Jezik		eng
Abstrakt		Objectives: The purpose of this study was to obtain data on the association of antiphospholipid antibodies with clinical manifestations in childhood and to enable future studies to determine the impact of treatment and long-term outcome of pediatric antiphospholipid syndrome. Patients and methods: A European registry extended internationally of pediatric patients with antiphospholipid syndrome was established as a collaborative project of the European Antiphospholipid Antibodies Forum and Lupus Working Group of the Pediatric Rheumatology European Society. To be eligible for enrollment the patient must meet the preliminary criteria for the classification of pediatric antiphospholipid syndrome and the onset of antiphospholipid syndrome must have occurred before the patient's 18th birthday. Results: As of December 1, 2007, there were 121 confirmed antiphospholipid syndrome cases registered from 14 countries. Fifty-six patients were male, and 65 were female, with a mean age at the onset of antiphospholipid syndrome of 10.7 years. Sixty (49.5%) patients had underlying autoimmune disease. Venous thrombosis occurred in 72 (60%), arterial thrombosis in 39 (32%), small-vessel thrombosis in 7 (6%), and mixed arterial and venous thrombosis in 3 (2%). Associated nonthrombotic clinical manifestations included hematologic manifestations (38%), skin disorders (18%), and nonthrombotic neurologic manifestations (16%). Laboratory investigations revealed positive anticardiolipin antibodies in 81% of the patients, anti-beta(2)-glycoprotein I antibodies in 67%, and lupus anticoagulant in 72%. (Abstract truncated at 2000 characters)
Deskriptorji		ANTIPHOSPHOLIPID SYNDROME ANTIBODIES, ANTIPHOSPHOLIPID ANTIBODIES, ANTICARDIOLIPIN LUPUS COAGULATION INHIBITOR THROMBOSIS CHILD ANTIBODIES, ANTINUCLEAR REGISTRIES EUROPE