Pagets Disease Flashcards
What is Paget’s disease?
A disorder of bone remodeling characterized by increased osteoclast-mediated bone resorption followed by increased bone formation.
What process is primarily increased in Paget’s disease?
Osteoclast-mediated bone resorption.
What follows the increased bone resorption in Paget’s disease?
Increased bone formation.
What is the etiology of Paget’s disease?
The etiology is largely unknown, with hereditary factors suggested.
How does family history relate to Paget’s disease?
There is a higher occurrence in patients’ relatives than in controls, with 15-30% of patients having a positive family history.
What genetic markers are associated with Paget’s disease?
HLA DQw 1 and DR antigens, as well as the sequestrosome 1 gene on chromosome 5.
What potential trigger is associated with the onset of Paget’s disease in genetically predisposed individuals?
Viral infection may be necessary to trigger the disease in those who have inherited the associated gene.
At what age is Paget’s disease typically diagnosed?
It is typically diagnosed in mid or older age.
What is the male-to-female ratio of Paget’s disease in the US?
The ratio is approximately 3:2, with men being more affected than women.
How does the prevalence of Paget’s disease vary by age?
The disease is more common in mid to older age groups.
What part of the skeleton is more commonly affected by Paget’s disease?
The axial skeleton
Which specific bones are commonly affected by Paget’s disease?
The skull, spine, pelvis, and proximal long bones like the femur.
What percentage of Paget’s disease cases are monostotic, and which bones are typically involved?
Approximately 20% are monostotic, commonly affecting the tibia and iliac bones.
What percentage of Paget’s disease cases are polystotic?
About 80%, affecting multiple bones.
What characterizes the hot phase of Paget’s disease
An initial phase of increased osteoclastic activity affecting both the cortex and cancellous bone.
What happens during the intermediate or mixed phase of Paget’s disease?
Bone destruction occurs alongside new bone formation.
What is the cold phase of Paget’s disease?
It is characterized by disorganized new bone formation.
What deformity is commonly seen in long bones affected by Paget’s disease?
Bowing of long bones.
What percentage of Paget’s disease patients experience fractures, and which bones are commonly affected?
Approximately 7%, commonly in the femur, tibia, humerus, and forearm.
How does Paget’s disease affect adjacent joints?
can lead to osteoarthritis (OA) of nearby joints due to stress from enlarged bones.
What is acetabular protrusion in the context of Paget’s disease?
A complication where the acetabulum (hip socket) protrudes due to bone changes, potentially affecting hip function.
What is a common result of Paget’s disease in the skull?
Head enlargement.
What deformity is associated with Paget’s disease of the skull?
Frontal bossing.
What percentage of patients may experience deafness due to Paget’s disease?
Approximately 13%, along with headaches and dizziness from auditory nerve entrapment.