Cerebral sinovenous thrombosis in children

Cerebral sinovenous thrombosis in childhood is a serious disease. There are many predisposing and influencing factors which should be unraveled to identify patients at risk and to establish treatment regimens in children. We report two cases, in which the predisposing risk factors for the cerebral sinovenous thrombosis were antithrombin III deficiency in one case, and severe dehydration in the other. In both cases diagnosis was confirmed by standard imaging methods, computerized tomography [CT] followed by magnetic resonance imaging/venography [MRI and MRV]

Venous thromboembolism: the intricacies.

Venous thromboembolism (VTE) has been a subject of great interest of late. Since Rudolph Virchow described the famous Virchow's triad in 1856, there have been rapid strides in the understanding of the pathogenesis and factors responsible for it. Discovery of various thrombophilic factors, both primary and acquired, in the last 40 years has revolutionized prognostication and management of this potentially life-threatening condition due to its associated complication of pulmonary thromboembolism. Detailed genetic mapping and linkage analyses have been underlining the fact that VTE is a multifactorial disorder and a complex one. There are many gene-gene and gene-environment interactions that alter and magnify the clinical picture in this disorder. Point in case is pregnancy, where the risk of VTE is 100-150 times increased in the presence of Factor V Leiden, prothrombin mutation (Prothrombin 20210A) and antithrombin deficiency. Risk of VTE associated with long-haul air flight has now been well recognized. Thrombotic events associated with antiphospholipid syndrome (APS) are 70% venous and 30% arterial. Deep venous thrombosis and pulmonary embolism are the most common venous events, though unusual cases of catastrophes due to central vein thrombosis like renal vein thrombosis and Budd-Chiari syndrome (catastrophic APS) may occur.

Budd-Chiari syndrome due to antithrombin III deficiency.

Budd-Chiari syndrome is a disease complex with varied etiology and is one of the causes of post-hepatic portal hypertension. We report a 2 year-old boy who presented with Budd-Chiari syndrome due to congenital antithrombin III deficiency, who was managed with an expandable metal stent placed in the inferior vena cava and oral anticoagulation.

Venous thrombosis: prevalence of prothrombotic defects in north Indian population.

431 patients with thrombosis of different venous system were evaluated for underlying acquired and inherited prothrombotic states. Associated acquired risk factors were observed to be present in 28.7% patients and possible inherited in 32.3%, in the rest, no cause could be identified. Major acquired risk factors included coexistence of liver disease (12.2%), oral contraceptives (4.1%), puerperium (2.5%), malignancy (2.3%) and lupus anticoagulant (2%). Low levels of protein C were detected in 21.1% and of which 11.3% were attributed to acquired factors. Protein S deficiency was found in 19.0% and of these 10.4% cases were associated with acquired risk factors. Antithrombin III (AT III) deficiency was detected in 6.4% of patients, of which 4.8% were secondary to acquired factors. In the rest, deficiency of protein C, protein S and AT III were attributed to inherited factors as no associated acquired risk factor was present. Activated protein C resistance (APC-R) was present in 12.5% cases.

The prevalence of hereditary thrombophilia in the Trakya region of Turkey

The prevalences of deficiencies in antithrombin III (AT III), protein C (PC), protein S (PS) and in the activated protein C (APC) resistance in the thrombotic population of the Trakya region, Turkey were investigated. 37 patients with venous thrombosis (VT) and 17 patients with arterial thrombosis (ArT) were included in this study. The mean ages of the patients with VT and ArT were 46 years (range 20-70) and 38 years (range 32-40), respectively. The activity of AT III was measured by commercially available immuno-turbidimetric assay. The activities of PC and PS were determined by coagulometric assay. The APC resistance was measured using a modified APTT-based clotting assay. Among the VT patients, there were 2 cases (5.4%) with AT III, 5 (13.51%) with PC deficiency, 5 (13.51%) with PS deficiency and 2 (5.4%) with APC resistance. In the ArT patient group, there was 1 patient (5.88%) with AT III, 3 (17.64%) with PC deficiency, 1 (5.88%) with PS deficiency and no APC resistant patients, while there was one (2.08%) with PC deficiency and one (2.08%) with APC resistance in the control group (49 persons, mean age 41 years). The relative risk of thrombosis (odds ratio) was 1.7 in the deficiency of PC and 5.6 in the deficiency of PS. The data presented suggests that the prevalences of AT III, PC and PS deficiencies causing thrombophilia in the Trakya region of Turkey are higher than in other reported studies while the APC resistance is lower than in others. Further studies including more patients would be required to clarify these discrepancies.

Déficit congénito de antitrombina III: reporte de un caso clínico de una paciente sometida a cirugía oral / Congenital deficiency of antithrombine III: report of a patient submitted to oral surgery

El déficit congénico de antitrombina III (AT III) es una patología de escasa prevalencia que se manifiesta clínicamente por fenómenos tromboembólicos que ocurren principalmente en territorio venoso, especialmente de las extremidades inferiores. En estos pacientes la incidencia de trombosis venosa aumenta con la edad, al igual que al someter a los pacientes a factores disponibles como son cirugía, parto, trauma, reposo prolongado, etc. Por esta razón la terapia profiláctica-anticoagulante en estos enfermos es necesaria cuando debe someterse a una cirugía. La administración de AT III exógenal, sola o junto a heparina, aparece como el tratamiento profiláctico más efectivo en prevenir los eventos trombólicos. En el presente trabajo se describe la evolución y manejo terapéutico de una paciente portadora de un déficit congénito de AT III, en relación a su segundo parto y cirugía de terceros molares

Venous sinus thrombosis due to inherited anti-thrombin lll deficiency in a neonate and review of the literature

Venous sinus thrombosis in the neonate is possibly an under recognized condition which presents most commonly with seizures The condition is idiopathic in the majority of reported cases however hypercoagulable states have to be looked for in the absence of obvious precipitating causes We report a newborn baby who had venous sinus thrombosis due to inherited antithrombin III deficiency and the relevant literature is reviewed. To our knowledge, this is the first reported case of venous sinus thrombosis due to antithrombin III deficiency in the United Arab Emirates