Hemophilia Research Today is a free monthly online journal that collates and summarizes the latest research about Hemophilia, including details on genetics, causes, symptoms, blood transfusion.

Orthognathic surgery in a patient with hemophilia A: report of a case.

Abramowicz S, Staba SL, Dolwick MF, Chen DR

Department of Oral and Maxillofacial Surgery, University of Florida, Gainesville, Florida 32610, USA. shellya@ufl.edu

Hemophilia A (factor VIII deficiency) is a bleeding disorder that is inherited in a recessive fashion and is caused by a mutation on the X chromosome. Management of bleeding disorders during orthognathic surgery cases has only been reported 2 other times in the oral and maxillofacial surgery literature. This report describes a patient with a factor VIII deficiency who underwent bilateral sagittal split osteotomies, a genial advancement, and extraction of teeth and the management of this patient from a hematologic standpoint.

Published 10 March 2008 in Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 105(4): 437-9.
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Articles on Hemophilia published 22 February 2008:

Antithrombin affects hemostatic response to recombinant activated factor VII in factor VIII deficient plasma.   Anesth Analg, 106(3): 719-24, table of contents.

BACKGROUND: Thromboembolic complications can occur with recombinant activated factor VII (rFVIIa) treatment in trauma and surgical patients but they are infrequent in hemophiliacs. Bleeding diathesis in these conditions is often attributed to reduced thrombin generation, which may be improved with rFVIIa. Normally, thrombin that diffuses from local vascular injury sites is quickly inactivated by antithrombin (AT). Evaluating the influence of AT levels on thrombin generation in hypocoagulable ... [Abstract] [Full-text]

Articles on Hemophilia published 17 January 2008:

Conservative management resulting in complete resolution of a double intussusception in an adult haemophiliac.   Colorectal Dis, 10(2): 197-8.

Acute abdominal pain in haemophiliacs should be approached as haemorrhage until proven otherwise. With advancements in factor repletion and coagulopathic management a conservative approach should be considered. We describe a case of double colo-colonic intussusception lead by an intramural haematoma occurring spontaneously and resolving with conservative management in a young haemophiliac. This demonstrates that intussusception in these cases may be transient, and does not require surgical ... [Abstract] [Full-text]

Articles on Hemophilia published 15 January 2008:

Recombinant tissue plasminogen activator may reduce frequency of central venous access device infection in hemophilia patients undergoing immune tolerance therapy.   Pediatr Blood Cancer, 50(3): 627-9.

Many patients with hemophilia, particularly those with inhibitory antibodies, utilize central venous access devices (CVADs) to facilitate frequent infusions. Infection of these devices is a common complication of factor replacement therapy. This communication reports our center's experience with CVAD infection in three patients with severe hemophilia A undergoing immune tolerance therapy (ITT) in whom intermittent infusions of recombinant tissue plasminogen activator (rTPA, Cathflo Activase) ... [Abstract] [Full-text]

Articles on Hemophilia published 3 October 2007:

Acquired hemophilia: a case report of 2 patients with acquired factor VIII inhibitor treated with rituximab plus a short course of steroid and review of the literature.   Clin Appl Thromb Hemost, 13(4): 443-8.

Acquired hemophilia is an unusual disorder in which nonhemophiliac patients develop autoantibodies (inhibitor) against the factor VIII coagulation protein. Factor VIII inhibitor leads to life-threatening bleeding disorders classically described as new onset of diffuse bruising and prolonged partial thromboplastin time in elderly patients. Treatment is focused in the control of the acute bleeding episode and the long-term suppression of the autoantibody. Several immunosuppressive combinations ... [Abstract] [Full-text]

HindIII polymorphism in carrier detection of hemophilia A in India: a new primer design.   Clin Appl Thromb Hemost, 13(4): 432-4.

HindIII polymorphism studies were done in 67 north Indian families with hemophilia A using a new primer sequence and comparing results with the standard protocol. Amplified segment was 608 bp in size. Genetically positive hemizygous males (+) and homozygous females (+/+) showed 427, 100, and 81 size fragments, whereas heterozygous females (+/-) had 427, 181, 100, and 81-bp segments. Genotypically negative hemizygous (-) males and homozygous females (-/-) show that 427-bp and 181-bp fragments ... [Abstract] [Full-text]

Articles on Hemophilia published 13 September 2007:

Recurrent inversion with concomitant deletion and insertion events in the coagulation factor VIII gene suggests a new mechanism for X-chromosomal rearrangements causing hemophilia A.   Hum Mutat, 28(10): 1045.

Recurrent int22h-related inversions in the coagulation factor VIII gene (F8) are the most common cause of severe hemophilia A. Such inversions have repeatedly been hypothesized to be associated with concomitant deletions that are responsible for an increased risk of immune responses against therapeutic exogenous factor VIII. However, exact DNA breakpoints have not yet been reported. In a patient with persistent factor VIII-inactivating antibodies, molecular analysis of F8 including Southern ... [Abstract] [Full-text]

Articles on Hemophilia published 16 August 2007:

Increasing genetic diversity of hepatitis C virus in haemophiliacs with human immunodeficiency virus coinfection.   J Gen Virol, 88: 2513-9.

Patients with inherited bleeding disorders who received clotting factor concentrates before 1987 have high rates of hepatitis C virus (HCV) or HCV/human immunodeficiency virus (HIV) infection. To determine whether the persistent nature of HIV affects the genetic diversity of HCV by less selective pressure through the immunosuppression of HIV/HCV-coinfected patients, both the change of genetic diversity and selective pressure were examined in the HCV envelope genes (E1 and E2) of 325 genotype 1a ... [Abstract] [Full-text]

Articles on Hemophilia published 9 August 2007:

Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia.   N Engl J Med, 357(6): 535-44.

BACKGROUND: Effective ways to prevent arthropathy in severe hemophilia are unknown. METHODS: We randomly assigned young boys with severe hemophilia A to regular infusions of recombinant factor VIII (prophylaxis) or to an enhanced episodic infusion schedule of at least three doses totaling a minimum of 80 IU of factor VIII per kilogram of body weight at the time of a joint hemorrhage. The primary outcome was the incidence of bone or cartilage damage as detected in index joints (ankles, knees, ... [Abstract] [Full-text]

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