Fibrous Dysplasia Flashcards
1
Q
What is fibrous dysplasia?
A
Disorganized mixture of fibrous and osseous elements in the medullary region of affected bones
2
Q
What gene has been assoicated with evolution of fibrous dysplasia?
A
Activating mutations in the GNAS1 gene
3
Q
What is the most common form of fibrous dysplasia?
A
Monostotic fibrous dysplasia (only affects one bone)
4
Q
What are characteristics of McCune-Albright Syndrome?
A
- Endocrine Dysfunction
- Acromegaly
- Cushing Syndrome
- Hyperthyroidism
- Vitamin D-resistant rickets
5
Q
What is the most common endocrine abnormality seen in girls with McCune-Albright Syndrome?
A
Precocious Puberty
6
Q
What skeletal growth characteristics are observed in patients with McCune-Albright Syndrome?
A
Decreased stature due to premature closure of growth plates
7
Q
What skin lesions are characteristic of McCune Albright Syndrome?
A
- Pigmented Macules (“Cafe-au-lait” spots)
- Irregular borders (“Coast of Maine mark”) That does not cross the midline
