BACKGROUND:    Inherited factor (F)VII deficiency is the commonest of the @entity3459 , with a wide set of hemorrhagic features. Other than for the severe clinical forms (for which treatment guidelines are well defined), consistent recommendations regarding perioperative replacement management do not exist for mild and asymptomatic @entity942 @entity1 . OBJECTIVES:    The present study aimed to evaluate the influence of @entity548 history, FVII procoagulant activity levels (FVII:C) and the type of surgical procedure on the management of @entity74 @entity1 before surgery. @entity1 :    One hundred and fifty-seven surgical procedures, performed without replacement therapy, in 83 unrelated @entity942 @entity1 (median FVII:C=5%, range 0.6%-35%) were analyzed. RESULTS:    The overall @entity548 rate was 15.3%. We found a significant relationship between previous deep traumatic @entity1410 and @entity548 at surgery, although relationships with previous common @entity1415 , easy @entity2981 and @entity931 were not significant. The receiver-operating characteristic (ROC) curve analysis performed on the first 83 procedures allowed us to define a cut-off value of 7% with a sensitivity of 87% (negative predictive value: 94%). To enhance the sensitivity, and to take into account the potential variation resulting from non-standardized FVII:C measurements, we would suggest applying a threshold of 10%. CONCLUSION:    We have proposed recommendations for the perioperative management of @entity942 @entity1 based on FVII:C levels, a thorough @entity548 history and the type of surgery involved. By applying these recommendations, minor procedures that risk only external or controlled @entity548 can be performed in asymptomatic or mildly affected adults, even those with FVII:C levels below 10%.

A retrospective analysis of 157 surgical procedures performed without replacement therapy in 83 unrelated factor VII-deficient XXXX .

@entity1 :: ('9606', 'Species') :: ['patients', 'PATIENTS'] @entity548 :: ('MESH:D006470', 'Disease') :: ['hemorrhage', 'bleeding'] @entity1415 :: ('MESH:D004844', 'Disease') :: ['epistaxis'] @entity3459 :: ('MESH:D025861', 'Disease') :: ['rare bleeding disorders'] @entity1410 :: ('MESH:D006406', 'Disease') :: ['hematomas'] @entity74 :: ('MESH:D030342', 'Disease') :: ['inherited FVII-deficient'] @entity931 :: ('MESH:D008595', 'Disease') :: ['menorrhagia'] @entity2981 :: ('MESH:D003288', 'Disease') :: ['bruising'] @entity942 :: ('MESH:D003677', 'Disease') :: ['FVII-deficient']
@entity1 :: (9606,Species) :: ['patients']
