Pediatric Antiphospholipid Syndrome: Clinical and Immunologic Features of 121 Patients in an International Registry

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%. Comparisons between different subgroups revealed that patients with primary antiphospholipid syndrome were younger and had a higher frequency of arterial thrombotic events, whereas patients with antiphospholipid syndrome associated with underlying autoimmune disease were older and had a higher frequency of venous thrombotic events associated with hematologic and skin manifestations. CONCLUSIONS. Clinical and laboratory characterization of patients with pediatric antiphospholipid syndrome implies some important differences between antiphospholipid syndrome in pediatric and adult populations. Comparisons between children with primary antiphospholipid syndrome and antiphospholipid syndrome associated with autoimmune disease have revealed certain differences that suggest 2 distinct subgroups. Pediatrics 2008; 122: e1100-e1107

The role of videopericardioscopy in evaluating indeterminate pericardial effusions

Background. The pericardial biopsy has opened a new perspective for the etiologic diagnosis of pericardial effusions, because adequate pericardial visualization via the use of a video camera can provide more accurate results. We assessed the usefulness of videopericardioscopy for the diagnosis and treatment of pericardial effusion of indeterminate origin. Methods. We conducted a retrospective study of clinical data from patients who underwent videopericardioscopy examination for pericardial effusion without an established diagnosis. The video-assisted pericardioscopy procedure was performed through a small incision in the xiphoid area. Results. From January 1998 to January 2007, 101 consecutive patients underwent videopericardioscopy evaluation for pericardial effusion. Ten patients were excluded because of lack of data. Fifty men and 41 women were included ( mean age, 50 years; range, 14-76 years). All of the patients had moderate or significant pericardial effusion as demonstrated by echocardiography or computed tomography. The following diagnoses for the pericardial effusions were established: nonspecific inflammation, 50 cases ( 54.94%); neoplastic disorders, 22 cases ( 24.17%); tuberculous, 11 cases ( 12.08%); bacterial inflammatory process, 3 cases ( 3.29%); chylopericardial, 2 cases ( 2.19%); fungal infection, 2 cases ( 2.19%); and viral infection, 1 case ( 1.09%). Pericardioscopy evaluation provided the definitive diagnosis via the pericardial biopsy in 36.26% of the cases and via the results of fluid analyses in 13.18% of the cases; the use of both methods established the definitive diagnosis in 45.05% of the cases in this group of patients. The overall morbidity rate was 4.3%, and the most common complication was arrhythmia due to intraoperative manipulation, which ceased with the removal of the instruments from the pericardial cavity. We had 1 death, by cardiac tamponade, in the perioperative period. Conclusion. Videopericardioscopy is a safe and efficient method for obtaining a better diagnosis of and satisfactory therapeutic results for pericardial effusions of indeterminate cause, and such results are obtained via an improved exploration of the pericardial cavity.