partial-thromboplastin time and fibrinogen yielded normal results. The postoperative course was uncomplicated; the factor VIIa levels increased between 5 to 12-times (average of 693 IU mL )1 in 16 samples obtained at different times during the whole period of rFVIIa administration (range 420–1894 UI mL) from baseline values, and the levels of FVIII activity and antigen increased 24 h after the OLT (Fig. 1). On the other hand, no thrombotic complications were observed and hepatocellular carcinoma and cirrhosis were confirmed in the excised liver. As a result of the unavailability of FEIBA , we used rVIIa because there is no standard solution for the recurrence of the inhibitor after OLT and an antibody response against the new FVIII synthesized by the transplanted liver could be expected postoperatively as reported by Khakhar et al. [6]. This is the first report of OLT in a patient with HA and low titre of inhibitors treated with rVIIa from Argentina. FVIII production by the liver transplant began 12 h after surgery and reached normal levels within 24 h. (Fig. 1). Similar results have been reported by other authors in different cohorts of patients with HA undergoing OLT. On the other hand, large transfusion requirements during OLT are related with increased morbidity and mortality, considering that a median transfusion requirement includes 10 units of blood-derived products and the complications of OLT such as coagulopathy, thrombocytopenia and venous congestion of the mesenteric venous system make these the surgeries more technically demanding. In this study, the use of rVIIa significantly reduced the need of transfusion during the surgery and the patient showed complete restoration of the haemostatic function at discharge and also a negative inhibitor 60 days postsurgery.
[1]
A. Cama.
Spinal epidural hematoma in hemophilic children: controversies in management
,
2009,
Child's Nervous System.
[2]
F. Rocco,et al.
Spinal epidural hematoma in hemophilic children: controversies in management
,
2009,
Child's Nervous System.
[3]
J. H. Davis,et al.
Torticollis as a sign of cervico‐thoracic epidural haematoma in an infant with severe haemophilia A
,
2006,
Haemophilia : the official journal of the World Federation of Hemophilia.
[4]
N. Simanovsky,et al.
The use of ultrasound for the diagnosis of spinal hemorrhage in a newborn.
,
2004,
Pediatric neurology.
[5]
C. Owens,et al.
Spinal ultrasound in infants.
,
2002,
The British journal of radiology.
[6]
M. Freund,et al.
US of the spinal cord in newborns: spectrum of normal findings, variants, congenital anomalies, and acquired diseases.
,
2000,
Radiographics : a review publication of the Radiological Society of North America, Inc.