Osteíte fibrosa cística

Osteíte fibrosa cística
Osteíte fibrosa cística
Escoliose causada por recklinghausen
Especialidade endocrinologia
Classificação e recursos externos
CID-10 E21.0
CID-9 252.01
CID-11 111150122
OMIM 603233
DiseasesDB 30721
MedlinePlus 001252
MeSH D010002
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Osteíte fibrosa cística, osteodistrofia fibrosa ou Doença óssea de von Recklinghausen (não confundir com a Doença de von Recklinghausen dos nervos) é uma doença endócrina rara caracterizada por uma perda de massa óssea é progressivamente substituída por tecido fibroso (como uma cicatriz) e pela formação de tumores císticos marrons ao redor dos ossos. É uma complicação do hiperparatireoidismo avançado.

A excessiva produção de hormônio da paratireóide(PTH) causa liberação dos depósitos de cálcio dos ossos e estimula a proliferação das células que produzem tecido fibroso (fibroblastos). Nesta patologia, existe uma tentativa por parte dos osteoclastos de reparar a destruição óssea, elevando, assim, o nível de fosfatase alcalina. [1]

Causa

O hiperparatireoidismo geralemnte é desencadeado por um adenoma da paratireoide e fatores hereditários, raramente por carcinoma de paratireoide ou por osteodistrofia renal. A causa mais frequente (80-85% dos casos) é um adenoma secretor de PTH. [2]. Nos casos genéticos a localização do principal gene defeituoso responsável é em 17q12-q22 de transmissão autossômica dominante.

Sinais e sintomas

Pode causar lesões císticas nos ossos, geralmente danificando os metacarpos, falanges, ossos do quadril, fêmur e coluna vertebral. Quando causa compressão medular pode resultar em paraplegia [3] Foi descrita por von Recklinghausen em 1882.

Seus princiais sintomas são: manchas e bolhas por toda a pele, tumores neurofibromatosos que percorrem os nervos, dor osteócopa e lesões dos ossos longos. Com a proliferação de tecido fibroblástico há aumento de osteoblastos e osteoclastos e hemorragia focal com depósitos de hemossiderina.

Tratamento

Quando o problema é causado por tumor da paratiroide, remover parte da glândula, e injeções de vitamina D ativada são curativos. A fibrose e cistos regridem lentamente ao longo de meses ou anos.[4] As deformidades também podem precisar de cirúrgia, que podem incluir transplantes de ossos cadavéricos.[5]

Ver também

Referências

  1. ↑ JORGE ALBERTO FRISCHENBRUDER, GERALDO AYALA PEREIRA (1996) Hiperparatiroidismo: relato de um caso. Revista Brasileira de Ortopedia janeiro de 1996
  2. ↑ Kearns, A. E.; Thompson, G. B. (2002). "Medical and Surgical Management of Hyperparathyroidism". Mayo Clinic Proceedings. 77 (1): 87–91. doi:10.4065/77.1.87. PMID 11794462
  3. ↑ Lázaro Bruno Borges Silva, Letícia Maria Coelho, Sávio de Moraes, Henrique Borges da Silva, Sindeval José da Silva. (2008) Osteíte fibrosa cística causando paraplegia crural. Rev. Bras. Cir. Cabeça Pescoço, v. 37, nº 3, p. 177 -178, julho / agosto / setembro 2008
  4. ↑ Arabi, A. (2006). "Regression of Skeletal Manifestations of Hyperparathyroidism with Oral Vitamin D". Journal of Clinical Endocrinology & Metabolism. 91 (7): 2480–3. doi:10.1210/jc.2005-2518.
  5. ↑ Brand, Richard A. (2008). "Follow-Up Study of the Use of Refrigerated Homogenous Bone Transplants in Orthopaedic Operations". Clinical Orthopaedics and Related Research. 466 (1): 22–36. doi:10.1007/s11999-007-0030-5. PMC 2505281. PMID 18196370.
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