Please use this identifier to cite or link to this item: http://repositorio.unicamp.br/jspui/handle/REPOSIP/66916
Type: Artigo de periódico
Title: Fibrodysplasia ossificans progressiva
Author: Nucci, A
Queiroz, LD
Santos, AD
Camargo, EE
Moura-Ribeiro, MVL
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 valgous and ventricular septal defect, corrected by transsternal 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 waif. 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: fibrodysplasia ossificans progressiva
myositis ossificans
muscle biopsy
rigid spine
Tc-99m-MDP scan
etidronate treatment
Country: Brasil
Editor: Assoc Arquivos De Neuro- Psiquiatria
Rights: aberto
Identifier DOI: 10.1590/S0004-282X2000000200023
Date Issue: 2000
Appears in Collections:Unicamp - Artigos e Outros Documentos

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