Table 1

Clinical and radiological features in nine DMC families

Families
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N: normal; ?: not available; L: Lebanon; M: Morocco; P: Portugal; T: Tunisia.
Ethnic originMMMMPMTML
Consanguinity1/161/161/161/641/161/161/1600
Clinical features
    Affected children321311221
    Growth retardation<−3 SD−4 SD<−3 SD−4 SD<−3 SD<−3 SD<−3 SD<−3 SD<−3 SD
    Microcephaly++++++++
    Facial dysmorphism++++++
    Thoracic anomalies+++++++++
    Scoliosis++++++++
    Rhizomelic dwarfism+++++++++
    Mental retardation++++++++++++++++
Radiological features
    Platyspondyly+++++++++
    Epiphyseal dysplasia+++++++++
    Metaphyseal dysplasia+++++++++
    Lacy appearance of the iliac crests+++++++++
Urinary keratan sulphate excretion?N???NNNN