Fibrodysplasia ossificans progressiva: case report.
Fibrodysplasia ossificans progressiva (FOP) is a rare autosomal dominant disorder characterized by postnatal progressive heterotopic ossification of the connective tissue and congenital malformation of the big toes. We report on a nine-year-old girl with clinical and radiological features of FOP. She was born with bilateral hallux valgus and at the age of nine presented an indurate mass in the left cervical region that was painful. A significant decreased range of motion in all levels of the spine and shoulder girdle was found. The radiographs showed heterotopic ossification in the thoracic region. The patient had two outbreaks of the disease ("flare-ups") that were treated with prednisone 2 mg/kg/day for four days. After the "flare-ups", she had a continuous therapy with a Cox-2 inhibitor (25 mg/day) and a leukotriene inhibitor, montelukast (10 mg/day).
Gonçalves
Andre Leite
AL
Department of Neurology and Neurosurgery, Federal University of São Paulo, São Paulo, SP, Brazil. goncalvesnp@yahoo.com.br
Masruha
Marcelo Rodrigues
MR
de Campos
Carmelinda Correia
CC
Delai
Patricia Longo Ribeiro
PL
Vilanova
Luiz Celso Pereira
LC
D000085:Acetates / Q000627:therapeutic use*; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D002648:Child; D003521:Cyclopropanes; D005260:Female; D006801:Humans; D020024:Leukotriene Antagonists / Q000627:therapeutic use*; D009221:Myositis Ossificans / Q000000981:diagnostic imaging* / Q000188:drug therapy; D011804:Quinolines / Q000627:therapeutic use*; D013440:Sulfides; D014057:Tomography, X-Ray Computed
D000085:Acetates; D000305:Adrenal Cortex Hormones; D003521:Cyclopropanes; D020024:Leukotriene Antagonists; D011804:Quinolines; D013440:Sulfides; C093875:montelukast