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→‎Paget disease of the bone: split out
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==Paget disease of the bone==
==Paget disease of the bone==
===General===
{{Main|Paget's disease of the bone}}
*Benign - unlike [[Paget disease of the breast]].
*Afflicts ~ 3% of population > 55 years old.<ref name=pmid22396921/>
*Leading cause of ''secondary [[osteosarcoma]]''.
*Uncommonly associated with [[giant cell tumour of bone]].<ref name=pmid17437100>{{Cite journal  | last1 = Hoch | first1 = B. | last2 = Hermann | first2 = G. | last3 = Klein | first3 = MJ. | last4 = Abdelwahab | first4 = IF. | last5 = Springfield | first5 = D. | title = Giant cell tumor complicating Paget disease of long bone. | journal = Skeletal Radiol | volume = 36 | issue = 10 | pages = 973-8 | month = Oct | year = 2007 | doi = 10.1007/s00256-007-0310-x | PMID = 17437100 }}</ref><ref name=pmid21319038>{{Cite journal  | last1 = Karakida | first1 = K. | last2 = Ota | first2 = Y. | last3 = Aoki | first3 = T. | last4 = Akamatsu | first4 = T. | last5 = Kajiwara | first5 = H. | last6 = Hirabayashi | first6 = K. | title = Multiple giant cell tumors in maxilla and skull complicating Paget's disease of bone. | journal = Tokai J Exp Clin Med | volume = 35 | issue = 3 | pages = 112-7 | month = Sep | year = 2010 | doi =  | PMID = 21319038 }}</ref>
*[[Diagnosis]] based on radiology.<ref name=pmid22396921/>
*Genetic component - several genes implicated<ref name=omim602080>{{OMIM|602080}}</ref> including ''TNFRSF11A'' ([[AKA]] PDB2)<ref name=omim603499>{{OMIM|603499}}</ref> and ''PDB4''.<ref name=omim606263>{{OMIM|606263}}</ref>


====Clinical====
Presentation:<ref name=pmid22396921>{{Cite journal  | last1 = Britton | first1 = C. | last2 = Walsh | first2 = J. | title = Paget disease of bone - an update. | journal = Aust Fam Physician | volume = 41 | issue = 3 | pages = 100-3 | month = Mar | year = 2012 | doi =  | PMID = 22396921 | URL = http://www.racgp.org.au/afp/201203/201203britton.pdf }}</ref>
*[[Fracture of bone|Fracture]].
*Bone pain.
*Bony deformity.
*Deafness.
*Incidental finding - radiologic or biochemical.
Serology:
*Elevated ALP.
Clinical features - mnemonic ''PANICS'':<ref>URL: [http://www.medicalgeek.com/orthopedics/2743-orthopedics-mnemonics.html http://www.medicalgeek.com/orthopedics/2743-orthopedics-mnemonics.html]. Accessed on: 30 April 2012.</ref>
*'''P'''ain (bone).
*'''A'''rthralgia and '''A'''LP elevated.
*'''N'''erve compression - deafness.
*'''I'''ncreased bone density.
*'''C'''ardiac failure (high output) - due to AVM formation in bone.
*'''S'''unburst skull on X-ray.
====Stage====
Classically divided into three phases:<ref name=emed_paget>URL: [http://emedicine.medscape.com/article/311688-overview http://emedicine.medscape.com/article/311688-overview]. Accessed on: 25 December 2010.</ref><ref>URL: [http://radiopaedia.org/articles/paget-disease-of-bone-1 http://radiopaedia.org/articles/paget-disease-of-bone-1]. Accessed on: 25 December 2010.</ref>
#Lytic (predominantly osteoclasts).
#Mixed lytic (osteoclastic) and blastic (osteoblastic).
#Sclerotic (burned-out).
===Microscopic===
Features:<ref name=emed_paget>URL: [http://emedicine.medscape.com/article/311688-overview http://emedicine.medscape.com/article/311688-overview]. Accessed on: 25 December 2010.</ref>
*Bone matrix has jigsaw-puzzle like pattern.
**Jigsaw-puzzle pieces each ~ 100-500 micrometres in size (largest dimension).
*Increased osteoclast activity.
**Osteoclast = macrophage that reabsorbs bone matrix.
====Images====
<gallery>
Image:Paget_disease_of_bone_-_high_mag.jpg | Paget disease - bone - high mag. (WC/Nephron)
Image:Paget_disease_of_bone_-_intermed_mag.jpg | Paget disease - bone - intermed. mag. (WC/Nephron)
</gallery>
==Fibrous dysplasia==
==Fibrous dysplasia==
*[[AKA]] ''osteitis fibrosa''.
*[[AKA]] ''osteitis fibrosa''.
Michael
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