Maroteaux-Lamy syndrome IV

Related people

A familial type of bone dysplasia with the principal symptoms ofshort-trunk dwarfism, back pain, pain in the hips, and limitation of joint movement.

Description

A familial type of bone dysplasia marked by short-trunk dwarfism, back pain, pain in the hips, and limitation of joint movement. May be associated with a large number of anomalies. Onset between the ages of 5 and 10 years. Inheritance is X-linked recessive.

Bibliography

  • A. W. Jacobsen:
    Hereditary osteochondrodystrophia deformans. A family with twenty members affected in 5 generations.
    Journal of the American Medical Association, Chicago, 1939, 113: 121-124.
  • P. Maroteaux, M. Lamy, J. Bernard:
    La dysplasie spondylo-epiphysaire tardive.
    La presse médicale, Paris, 1957, 65: 1205-1208.

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