Clinical Rheumatology

, Volume 6, Issue 2, pp 185–191 | Cite as

Tricho-rhino-phalangeal syndrome type I in a Belgian family

  • L. A. Verbruggen
  • C. Van Laere
  • J. Lamoureux
  • R. Van Tiggelen
Originals

Summary

We report three cases of tricho-rhino-phalangeal syndrome (TRPS) type I in a Belgian family. They presented typical characteristics such as a pear-shaped nose, and short, deformed fingers with cone-shaped epiphyses of some middle phalanges of the hands. Hair growth was practically normal in our patients, except for some narrowing of the lateral part of the eyebrows. Perthes-like hip dysplasia was documented in two of our cases. The proband presented at the age of 31 with Kienböch's disease of the right wrist. Blood and urine analysis showed no clear anomalies. In this patients, echography revealed a renal cyst containing a stone. The relationship of these findings to TRPS is discussed.

Key Words

Tricho-Rhino-Phalangeal Syndrome Pertes-Like Hip Dysplasia Kienböch Disease Cone-Shaped Epiphyses Brachymetacarpia Brachyphalangia 

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Copyright information

© Acta Medica Belgica 1987

Authors and Affiliations

  • L. A. Verbruggen
    • 1
  • C. Van Laere
    • 1
  • J. Lamoureux
    • 3
  • R. Van Tiggelen
    • 2
  1. 1.Rheumatology UnitAkademisch Ziekenhuis Vrije Universiteit BrusselBrusselsBelgium
  2. 2.Department of RadiologyAkademisch Ziekenhuis Vrije Universiteit BrusselBrusselsBelgium
  3. 3.Department of Pediatric SurgeryUniversitary Hospital BrugmannBrusselsBelgium

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