Fibrodysplasia Ossificans Progressiva

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FIBRODYSPLASIA OSSIFICANS PROGRESSIVE (FOP)
Ghislaine M Charbonier
Florida Technical College
Med 1000
Professor Kind-Adams
March 25, 2013

FOP or Fibrodysplasia ossificans progressiva means “soft connective tissue that progressively turns to bone.” The earliest documented cases dated back to 17th and 18th centuries. The disease became known as myositis ossificans progressive, which means “muscle turns progressively to bone.” The name was modified in the 1970’s by Dr. Victor McKusick of Johns Hopkins University School of Medicine,who is considered the father of Medical Genetics.

Fibrodysplasia ossificans progressiva (FOP) is a rare and disabling genetic condition of congenital skeletal malformation and progressive heterotopic ossification (HO), is the most catastrophic disorder of (HO) in humans. FOP causes immobility through progressive metamorphosis of skeletal muscle and soft connective tissue into a second skeleton of heterotopic bone. FOP is extremely rare with a worldwide prevalence of approximately one in a two million. There appears to be no ethnic, racial, gender or geographic predisposition. When observed, genetic transmission is autosomal dominant and can be inherited from either mother or father.

Clinical features that define FOP patients are malformation of the great toes; and progressive HO in specific spatial patterns. There are two different type of FOP atypical and classic. FOP patients appear normal at the time of birth except for the characteristics malformation of the great toes which are present in all affected patients.

During the first decade of life, children with FOP develop painful and highly inflammatory soft tissue swellings (or flare-ups) that transform soft connective tissues, including aponeuroses, fascia, ligaments, tendons and skeletal muscles, into an armament like encasement of bone. Ribbons, sheets and plates of heterotopic bone replace skeletal muscles and connective tissues through a...
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