Fibrodysplasia ossificans progressiva
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Transcript of Fibrodysplasia ossificans progressiva
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Fibrodysplasia ossificans progressivaMyositis ossificans progressiva
Big toe malformationCongenital
Heterotopic enchondral ossificationLater
Ligaments
Fasciae
Striated MusclesWith predictable pattern
Autosomal dominantBone morphogenetic protein ???
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First ray shortening Delta shape proximal phalanx
I.P.fusion Hallux Valgus
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Usually around 5 yr.
Shortly after birth Second decade
Spontaneous or Blunt trauma
Some nodules regress but usually progress to form mature bone
Axial to appendicularCranial to caudalProximal to distal
Dorsal to ventral
PainErythematicWarmthTenderness
Like infectionAfter several weeksSubside but Induration increases
Intermediate lesion12 weeksX Ray evident hard painless mass
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Advanced Subcutneous Ossification
Spontaneous fusion along post.elements & Lat. masses
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Short thumb metacarpal
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TreatmentSupportive measures
Avoiding trauma Padding
Nutrition
Research
Inhibition of Enchondral ossification & Angiogenic factors
BMP 4 inhibitors