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Type: Artigo de periódico
Title: Fibrodysplasia Ossificans Progressiva: Case Report.
Author: Nucci, A
Queiroz, L D
Santos, A D
Camargo, E E
Moura-Ribeiro, M V
Abstract: Fibrodysplasia ossificans progressiva is a rare genetic disease characterized by widespread soft tissue ossification and congenital stigmata of the extremities. We report on a male child followed for ten years since the age of 3 years and 9 months, when the diagnosis was made. He was born with bilateral hypoplasic hallux valgus and ventricular septal defect, corrected by trans-sternal approach when 32 months old. Restriction of neck mobility followed and foci of ectopic ossification appeared. Four crises of disease exacerbation were treated with oral prednisone and/or other antiinflammatory drugs. Sodium etidronate 5 to 10 mg/kg/day was prescribed intermittently during about six years but was discontinued due to osteopenia. The disease course has been relentless, with severe movement restriction including the chest wall. A review showed few similar case reports in the Brazilian literature. We revisit the criteria for diagnosis and the essentials of management and treatment.
Subject: Adolescent
Child, Preschool
Etidronic Acid
Follow-up Studies
Myositis Ossificans
Technetium Tc 99m Medronate
Citation: Arquivos De Neuro-psiquiatria. v. 58, n. 2A, p. 342-7, 2000-Jun.
Rights: aberto
Date Issue: 2000
Appears in Collections:Unicamp - Artigos e Outros Documentos

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