OBJECTIVE: @entity2883 ( @entity2883 ) is a predominantly @entity10763 associated with antineutrophil cytoplasmic antibodies (ANCAs). There are few reports describing its clinical features and outcome in @entity1 . We report on the experience at a single tertiary referral center over 21 years. METHODS: We conducted a retrospective chart review of all @entity1 diagnosed with @entity2883 at The Hospital for Sick @entity1 between 1984 and 2005. RESULTS: Twenty-five @entity1 were identified. Median age at diagnosis and median followup were 14.5 years and 32.7 months, respectively. Male-to-female ratio was 1:4. Median duration of symptoms before diagnosis was 2 months. Of 22 @entity1 , 21 were ANCA positive during their disease course (classic ANCA 78.9%). Constitutional symptoms were the most common clinical feature at presentation (24 of 25). @entity631 was present in 22 @entity1 at presentation. Only 1 of 11 @entity1 who presented with or developed @entity8 had normalization of serum @entity1011 . Upper airway involvement occurred in 21 @entity1 at presentation and 24 over followup; only 1 had @entity5142 . Twenty @entity1 had initial @entity358 , most commonly nodules (44%) and @entity548 (44%). Five @entity1 required ventilation for @entity548 . Four @entity1 (16%) had @entity49 ( @entity49 ). Treatment included @entity229 (100%), @entity786 (76%), @entity500 (40%), and @entity1499 (32%). CONCLUSION: Pediatric @entity2883 typically presents in adolescence and has a female predominance. @entity631 and @entity358 are common at diagnosis and frequently present as a @entity11278 . Loss of renal function is common and rarely completely reversible. As in adults, @entity1 with @entity2883 are at risk of @entity49 .

Clinical features and outcome of pediatric XXXX .

@entity1 :: ('9606', 'Species') :: ['children', 'patients', 'Children'] @entity8 :: ('MESH:D007674', 'Disease') :: ['renal impairment'] @entity548 :: ('MESH:D006470', 'Disease') :: ['pulmonary hemorrhage'] @entity500 :: ('MESH:D001379', 'Chemical') :: ['azathioprine'] @entity229 :: ('MESH:D011241', 'Chemical') :: ['prednisone'] @entity786 :: ('MESH:D003520', 'Chemical') :: ['cyclophosphamide'] @entity49 :: ('MESH:D019851', 'Disease') :: ['VTEs', 'venous thrombotic events'] @entity10763 :: ('MESH:C565222', 'Disease') :: ['small-vessel vasculitis'] @entity11278 :: ('MESH:C538458', 'Disease') :: ['pulmonary-renal syndrome'] @entity358 :: ('MESH:D008171', 'Disease') :: ['pulmonary involvement', 'pulmonary disease'] @entity2883 :: ('MESH:D014890', 'Disease') :: ['WG', "Wegener's granulomatosis"] @entity1011 :: ('MESH:D003404', 'Chemical') :: ['creatinine'] @entity1499 :: ('MESH:D008727', 'Chemical') :: ['methotrexate'] @entity631 :: ('MESH:D005921', 'Disease') :: ['Glomerulonephritis'] @entity5142 :: ('MESH:D007829', 'Disease') :: ['subglottic stenosis']
@entity2883 :: (MESH:D014890,Disease) :: ['Wegener's granulomatosis']
