Fibrodysplasia ossificans progressiva. An 11-year-old boy treated with a diphosphonate.
Fibrodysplasia ossificans progressiva (FOP) is a severe, rare, autosomal dominant, ectopic ossifying condition, with primary involvement of the skeletal muscles associated with skeletal abnormalities. This report concerns an 11-year-old boy suffering from FOP, who presented significant modification of the musculoskeletal structure of the thorax and problems with articular movements. The patient showed progress after treatment with ethane-1-hydroxy-1,1-diphosphonate (EHDP). In fact, using a scintiscanner we were able to observe a significant improvement in symptoms and a recovery of some of the active sites of ossification.
Bruni
L
L
Department of Paediatrics, University of Rome, La Sapienza, Italy.
Giammaria
P
P
Tozzi
M C
MC
Camparcola
D
D
Scopinaro
F
F
Imperato
C
C
D002648:Child; D012968:Etidronic Acid / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D009132:Muscles / Q000000981:diagnostic imaging; D009221:Myositis Ossificans / Q000000981:diagnostic imaging / Q000188:drug therapy*; D011877:Radionuclide Imaging
D012968:Etidronic Acid