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fibrodysplasia ossificans progressiva
Fibrodysplasia ossificans progressiva (FOP) is an extremely rare connective tissue disease. The disease is caused by a mutation of the body's repair mechanism, which causes fibrous tissue (including muscle, tendon, and ligament) to be ossified spontaneously or when damaged. In many cases, injuries can cause joints to become permanently frozen in place. Surgical removal of the extra bone growths has been shown to cause the body to "repair" the affected area with more bone.
==Signs and symptoms ==
For unknown reasons, children born with FOP have deformed big toes, possibly missing a joint or simply presenting with a notable lump at the minor joint. The first "flare-up" that leads to the formation of FOP bones usually occurs before the age of 10. The bone growth progresses from the top downward, just as bones grow in fetuses. A child with FOP will typically develop bones starting at the neck, then on the shoulders, arms, chest area and finally on the feet.
Specifically, FOP involvement is typically seen first in the dorsal, axial, cranial and proximal regions of the body. Later the disease progresses in the ventral, appendicular, caudal and distal regions of the body.〔Fibrodysplasia ossificans progressiva. Frederick S. Kaplan, MD, Martine Le Merrer, MD, PhD, Professor of Genetics, David L. Glaser, MD, Robert J. Pignolo, MD, PhD, Robert Goldsby, MD, Joseph A. Kitterman, MD, Jay Groppe, PhD, and Eileen M. Shore, PhD〕 However, it does not necessarily occur in this order due to injury-caused flare-ups. Often, the tumor-like lumps that characterize the disease appear suddenly. This condition causes loss of mobility to affected joints, including inability to fully open the mouth limiting speech and eating. Extra bone formation around the rib cage restricts the expansion of lungs and diaphragm causing breathing complications.
Since the disease is so rare, the symptoms are often misdiagnosed as cancer or fibrosis. This leads physicians to order biopsies, which can actually exacerbate the growth of these lumps. However, those born with FOP tend to have malformed toes or thumbs which help distinguish this disorder from other skeletal problems.〔(【引用サイトリンク】url=http://ghr.nlm.nih.gov/condition/fibrodysplasia-ossificans-progressiva )〕
抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』
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