Prothrombotic disorders in children with moyamoya syndrome.
OBJECTIVE
Moyamoya syndrome is an uncommon chronic occlusive cerebrovascular disease in children. The origin of moyamoya syndrome remains undetermined. The role of the prothrombotic disorders contributing to its pathogenesis has not been completely elucidated. The purpose of this study was to determine the frequency of prothrombotic disorders in a pediatric population with moyamoya syndrome.


METHODS
From May 1992 to April 2000, a prospective study of 10 consecutive children with moyamoya syndrome was carried out at a single center. Evaluation included the following assays: protein C, protein S, antithrombin, plasminogen, activated protein C resistance, factor V Leiden, and prothrombin gene mutations. Lupus anticoagulant, anticardiolipin antibodies, and anti-beta(2)-glycoprotein I antibodies assays were also performed. The clinical characteristics, underlying diseases, family history of thrombosis, radiological findings, treatment, and outcome were also recorded.


RESULTS
In our series, prothrombotic disorders were detected in 4 patients (40%). Inherited protein S deficiency was found in 1 patient; lupus anticoagulant and anticardiolipin antibodies were detected in the remaining 3 patients. One presented persistent lupus anticoagulant for 2.7 years until his death. In the case of the other 2 patients, 1 has maintained lupus anticoagulant for 9 months, whereas the other has kept anticardiolipin/anti-beta(2)-glycoprotein I antibodies for 10 months.


CONCLUSIONS
We report the hemostatic data of the largest prospective pediatric study carried out at a single center in the western hemisphere. In 4 patients (40%), a prothrombotic disorder was detected. It is tempting to speculate that these hemostatic abnormalities may contribute to the pathogenesis of moyamoya syndrome in some of our patients.
Bonduel
M
M
Servicio de Hematología-Oncología, Hospital de Pediatría, Buenos Aires, Argentina.
Hepner
M
M
Sciuccati
G
G
Torres
A F
AF
Tenembaum
S
S
D000293:Adolescent; D000328:Adult; D017153:Antibodies, Anticardiolipin / Q000097:blood; D017152:Antibodies, Antiphospholipid / Q000097:blood; D001241:Aspirin / Q000627:therapeutic use; D001778:Blood Coagulation Disorders / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D001780:Blood Coagulation Tests; D002548:Cerebral Revascularization / Q000009:adverse effects; D002648:Child; D002675:Child, Preschool; D005260:Female; D005343:Fibrinolytic Agents / Q000627:therapeutic use; D006023:Glycoproteins / Q000276:immunology; D006801:Humans; D007223:Infant; D016682:Lupus Coagulation Inhibitor / Q000097:blood; D008297:Male; D008875:Middle Aged; D009072:Moyamoya Disease / Q000150:complications* / Q000276:immunology / Q000628:therapy; D011446:Prospective Studies; D018455:Protein S Deficiency / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D012307:Risk Factors; D015996:Survival Rate; D017211:Treatment Failure; D016896:Treatment Outcome; D053482:beta 2-Glycoprotein I
D017153:Antibodies, Anticardiolipin; D017152:Antibodies, Antiphospholipid; D005343:Fibrinolytic Agents; D006023:Glycoproteins; D016682:Lupus Coagulation Inhibitor; D053482:beta 2-Glycoprotein I; D001241:Aspirin