Haemophilia (Subscribe)  

Definition - Inherited disorder of intrinsic clotting
Background
Haemophilia A
Haemophilia B
Von Willebrands disease
Combined incidence of ~ 1/10,000
Manifestations of factor VIII and IX deficiency indistinguishable

PURPOSE: To evaluate the clinical usefulness of phosphorus-32 chromic phosphate synoviorthesis in patients with hemophilia, recurrent hemarthrosis, and synovitis.
CONCLUSION: P-32 colloid synoviorthesis is a clinically useful out-patient procedure in patients with hemophilia, recurrent hemarthrosis, and synovitis in whom hemostatic therapy has failed.
Hemarthrosis and synovitis associated with hemophilia: clinical use of...

Powerpoint presentation on the orthopaedic aspects of haemophilia.
Orthopedic Surgery Presentations May 2001

The purpose of this essay is to illustrate features of the musculoskeletal complications of hemophilia as shown by CT, sonography, and MR imaging. MR can be used to detect early synovial and cartilaginous changes that may not be evident on conventional radiography and to differentiate between acute and chronic bleeding...

* incidence: knees> elbows> ankles> hips> shoulders> wrists
* results in secondary OA (DDx: acromegaly, ochronosis, spondyloepiphyseal dysplasia, chronic trauma)
xray manifestations:
* premature closure of epiphysis leading to short bone with enlarged end
* "squared" patella (flattened inferiorly)
* widened intercondylar notch
* subchondral cysts
* severe osteopenia

Hemophilia is the oldest known bleeding disorder. This condition first came to public attention when the disease appeared in the offspring of Queen Victoria of England.
The deficiency or absence of either of 2 clotting elements — factor VIII or factor IX — leads to the clinical condition described as hemophilia...

Hemophilia Objectives
1. List the two clotting factors in which deficits result in hemophilia
2. Describe laboratory workup for clotting deficiencies
3. Discuss the importance of inhibitors
4. Describe the natural history of hemophiliac arthropathy
5. Discuss methods currently available for...

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...

As a result of repeated episodes of articular bleeding from early childhood adult patients with haemophilia commonly have severe damage to the knee. They may present to the orthopaedic surgeon with pain and severe functional disability arising from advanced haemophilic arthropathy. There are many reports of successful operations on the...

A clotting screen should always be performed in a child with a swollen joint, even if there is a history of minor trauma, to exclude underlying bleeding disorders. A prompt diagnosis of haemophilia will allow early arrest of bleeding by appropriate treatment, which will in turn minimise complications and reduce...