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Printable Handouts
Navigable Slide Index
- Introduction
- Definition and prevalence
- Etiopathogenesis
- Endocrine system
- Skin
- Muscle
- Bone
- FD/MAS etiopathogenesis and histopathology
- Periosteal bone is not primarily affected
- Osteogenic cells form thin, irregular bone trabeculae
- Dysplastic bone irregularly replaces normal bone
- Dysplastic bone and fibrous tissue are highly vascularized
- Fibroblast growth factor 23 (FGF23)
- Skeletal involvement in FD/MAS
- Progression of skeletal lesions in FD/MAS
- FD/MAS malignant transformation
- FD/MAS clinical manifestations
- Imaging in FD/MAS: radiology and CT scan
- Imaging in FD/MAS: MRI and Tc bone scan
- Pain in FD/MAS
- Typical lower limb deformities: 6 typical deformities
- Typical lower limb deformities: other
- Worsening of skeletal deformities in FD/MAS
- Fractures in FD/MAS
- Scoliosis in FD/MAS
- FD/MAS diagnosis (1)
- FD/MAS diagnosis (2)
- Medical treatment: bisphosphonates
- Medical treatment: denosumab
- Denosumab: clinical trial
- Denosumab: mouse models
- Medical treatment: denosumab and zoledronic acid
- Medical treatment: burosumab
- Surgical treatment of FD/MAS: basic principles
- Two stage surgery in severe coxa vara ≤90o
- Intramedullary nailing (IN) for lower limb FD
- Titanium elastic nails (TENs)
- Complementary surgery: performed during growth in 37% of cases
- Evaluation scale at follow-up
- Results (IN in child with FD)
- Good results: 16 years follow-up
- Poor results
- Complications
- IN in patients with previous unsuccessful treatment
- Results (IN in adults with previous unsuccessful treatment)
- Results: good and poor
- Complications
- Conclusions: etiopathogenesis
- Conclusions: diagnosis and treatment
- Conclusions: surgery
- Conclusions: the future
- Acknowledgements
Topics Covered
- McCune-Albright Syndrome (MAS)
- Fibrous Dysplasia (FD)
- Etiopathogenesis of MAS
- Skeletal involvement in FD/MAS
- Imaging FD/MAS
- FD/MAS diagnosis
- FD/MAS medical treatment
- Surgical treatment of FD/MAS
- Intramedullary Nailing (IN)
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Talk Citation
Ippolito, E. and Lardo, D. (2024, July 31). Fibrous dysplasia of bone in McCune-Albright syndrome: pathophysiology, clinical aspects and treatment [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved October 13, 2024, from https://doi.org/10.69645/BUOJ6752.Export Citation (RIS)
Publication History
Financial Disclosures
- There are no financial interests to disclose.
- There are no financial interests to disclose.
Fibrous dysplasia of bone in McCune-Albright syndrome: pathophysiology, clinical aspects and treatment
Published on July 31, 2024
22 min
A selection of talks on Metabolism & Nutrition
Transcript
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0:00
Fibrous Dysplasia of Bone in
McCune-Albright Syndrome.
Pathophysiology, Clinical
Aspects, and Treatment
by Ernesto Ippolito,
Professor Emeritus of
Orthopedic Surgery,
University of Rome,
Tor Vergata, Italy;
and Davide Lardo,
senior resident,
Department of
Orthopedic Surgery,
University of Rome, Tor Vergata,
and Senior Medical Officer of
the Military Hospital of Rome,
Attilio Frigeri, Italy.
0:37
Definition and prevalence.
McCune-Albright syndrome is
a rare genetic
pathologic condition
affecting endocrine
glands, skin, and bone.
0:51
Etiopathogenesis.
The mutated GNAS
gene overstimulates
the production of
intracellular cAMP in
endocrine glands,
skin melanocytes,
and osteogenic cells.
The mutation is post-zygotic,
producing a mosaic
state in which
both normal and pathologic
tissues coexist.
1:22
Endocrine system.
Gonads, thyroid, pituitary,
and adrenal glands are
the most frequently
affected with
the overproduction of
their specific hormones.
1:36
Skin.
Melanocytes are overstimulated
with the formation of
cafe-au-lait skin macules.
Muscle.
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