ANTIPHOSPHOLIPID SYNDROME WITH ACQUIRED COAGULATION FACTOR DEFICIENCY AND GENETICALLY DETERMINED PREDISPOSITION TO THE THROMBOTIC EVENTS IN CHILDHOOD
Mukvich E.N., Omelchenko L.I., Diachenko N.M., Hridina T.A., Yakovenko A.O.
Summary. Antiphospholipid syndrome (APS) in children is a potentially life-threatening autoimmune multisystem disease that mainly manifests as vascular thrombosis with a high risk of recurrent thrombosis (20–30%) and a high mortality rate (7%). Although thromboses in children are much less common than in adults, the proportion of thrombosis associated with antiphospholipid antibodies (APA) is higher in pediatric practice. The aim of the study is to acquaint doctors with the features of clinical manifestations and diagnosis of primary antiphospholipid syndrome in children with hematological complications, the importance of conducting an extensive examination, including the study of all links of hemostasis. Clinical case. A clinical case of personal observation of primary antiphospholipid syndrome in a child with a debut in adolescence, post-thrombotic disease, pronounced laboratory signs of deep disturbance of various links of hemostasis is presented. Conclusions. APS is a rare disorder with a very limited number of reported cases in children, but with diverse clinical manifestations and disease outcomes. Carrying out an extended examination of a child with APS, including the study of all links of hemostasis, allows to correctly choose the tactics of treatment and observation, to determine measures for the prevention of severe complications.
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