Muscular System
Definition- A disorder in which muscle tissue and connective tissue such as tendons and ligaments are gradually replaced by bone, forming bone outside the skeleton that constrains movement.
This process generally becomes noticeable in early childhood, starting with the neck and shoulders and proceeding down the body and into the limbs.
Symptoms-Extra-skeletal bone formation causes progressive loss of mobility as the joints become affected. Inability to fully open the mouth may cause difficulty in speaking and eating. Over time, people with this disorder may experience malnutrition due to their eating problems. They may also have breathing difficulties as a result of extra bone formation around the rib cage that restricts expansion of the lungs.
Fibrodysplasia ossificans progressiva is a very rare disorder, believed to occur in approximately 1 in 2 million people worldwide. Several hundred cases have been reported.
Treatment-Systemic steroids are sometimes used for acute flare-ups. Iontophoresis with steroids or acetic acid may improve diminished range of motion.
Diagnosis- Little is known about this disease so diagnosis is unclear yet biopsies, as well as permanent complications from medical interventions, including loss of mobility may the case.
Cause-
History- The earliest documented cases date back to the 17th and 18th centuries. In 1692, French physician Guy Patin met with a patient who had FOP and mentioned the encounter in his writings. In 1736, British physician John Freke described at length an adolescent whose diagnosis included swellings throughout his back.
This process generally becomes noticeable in early childhood, starting with the neck and shoulders and proceeding down the body and into the limbs.
Symptoms-Extra-skeletal bone formation causes progressive loss of mobility as the joints become affected. Inability to fully open the mouth may cause difficulty in speaking and eating. Over time, people with this disorder may experience malnutrition due to their eating problems. They may also have breathing difficulties as a result of extra bone formation around the rib cage that restricts expansion of the lungs.
Fibrodysplasia ossificans progressiva is a very rare disorder, believed to occur in approximately 1 in 2 million people worldwide. Several hundred cases have been reported.
Treatment-Systemic steroids are sometimes used for acute flare-ups. Iontophoresis with steroids or acetic acid may improve diminished range of motion.
Diagnosis- Little is known about this disease so diagnosis is unclear yet biopsies, as well as permanent complications from medical interventions, including loss of mobility may the case.
Cause-
History- The earliest documented cases date back to the 17th and 18th centuries. In 1692, French physician Guy Patin met with a patient who had FOP and mentioned the encounter in his writings. In 1736, British physician John Freke described at length an adolescent whose diagnosis included swellings throughout his back.