Advertisement

Diaphyseal Dysplasia (Camurati-Engelmann Disease)

  • Peter Beighton
  • Bryan J. Cremin

Abstract

Camurati (1922) described a seven-year-old boy with painful lower limbs and diaphyseal cortical thickening in the long bones of the legs. The 55-year-old father and eight of their family members in four generations were thought to be affected, and the condition was termed ‘hereditary symmetrical osteitis’.

Keywords

Painful Lower Limb Radiographic Manifestation Cortical Hyperostosis Proximal Femoral Shaft Optic Nerve Decompression 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Allen DT, Saunders AM, Northway WH JR, Williams GF, Schafer IA (1970) Corticosteroids in the treatment of Engelmann’s disease: Progressive diaphyseal dysplasia. Pediatries 46: 523Google Scholar
  2. Anderson FG (1953) Engelmann’s disease. Br J Radiol 26: 603PubMedCrossRefGoogle Scholar
  3. Camurati M (1922) Di un raro caso di osteite simmetrica ereditaria degli arti inferiori. Chir Organi Mov 6: 662Google Scholar
  4. Clawson DK, Loop JW (1964) Progressive diaphyseal dysplasia (Engelmann’s disease). J Bone Joint Surg [Am] 46: 143Google Scholar
  5. Engelmann G (1929) Ein Fall von Osteopathia Hyperostica (sclerotisans) Multiplex Infantalis. Fortschr Roentgenstr 39: 1101Google Scholar
  6. Girdany BR, Sane S, Graham CB (1973) Engelmann’s disease. In: Kaufmann HJ (ed) Intrinsic diseases of bones. Basel, Karger (Progress in pediatric radiology, vol 4, p 414 )Google Scholar
  7. Griffiths DL (1956) Engelmann’s disease. J Bone Joint Surg 38: 312Google Scholar
  8. Hundley JD, Wilson FC (1973) Progressive diaphyseal dysplasia. J Bone Joint Surg [Am] 55: 461Google Scholar
  9. Neuhauser EB, Schwachman H, Wittenborg M, Cohen J (1948) Progressive diaphyseal dysplasia. Radiology 51: 11PubMedGoogle Scholar
  10. Paul LW (1953) Hereditary multiple diaphyseal sclerosis (Ribbing). Radiology 60: 412PubMedGoogle Scholar
  11. Ribbing S (1949) Hereditary multiple diaphyseal sclerosis. Acta Radiol (Stockh) 31: 522Google Scholar
  12. Sear HR (1948) Engelmann’s disease: Osteopathia hyperostotica sclerotisans multiplex infantilis. Report of a case. Br J Radiol 21: 236PubMedCrossRefGoogle Scholar
  13. Singleton EB, Thomas JR, Worthington WW, Hild JR (1956) Progressive diaphyseal dysplasia (Engelmann’s disease). Radiology 67: 233PubMedGoogle Scholar
  14. Smith R, Walton RJ, Corner BD, Gordon 1RS (1977) Clinical and biochemical studies in Engelmann’s disease (Progressive diaphyseal dysplasia). Q J Med 46: 273PubMedGoogle Scholar
  15. Sparkes RS, Graham CB (1972) Camurati-Engelmann disease. Genetics and clinical manifestations. J Med Genet 9: 73PubMedCrossRefGoogle Scholar
  16. Sty JR, Babbitt DP, Starshak RJ (1978) Bone scintigraphy demonstrating Engelmann’s disease. Clin Nucl Med 3: 69PubMedCrossRefGoogle Scholar
  17. Yen JK, Bourke RS, Popp AJ, Wirth CR (1978) Camurati-Engelmann disease (Progressive hereditary craniodiaphyseal dysplasia): case report. J Neurosurg 48: 138PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1980

Authors and Affiliations

  • Peter Beighton
    • 1
  • Bryan J. Cremin
    • 2
  1. 1.Department of Human Genetics, Medical School and Groote Schuur HospitalUniversity of Cape TownSouth Africa
  2. 2.Department of Radiology, Groote Schuur and Red Cross Children’s HospitalUniversity of Cape TownSouth Africa

Personalised recommendations