dictionary help| Clausen --> Dyggve-Melchior-Clausen syndrome | medical dictionary |
<syndrome>
An osteochondrodysplasia that clinically resembles Morquio's syndrome, but without excretion of mucopolysaccharides; characterised by mental retardation, short stature, progressive sternal bulging, flattening of vertebral bodies and iliac crests, shortening of metacarpals, and changes in long bones; autosomal recessive inheritance, but there is an X-linked form.
Dyskinesia, clearance of mucus is sluggish and bronchiectasis is prevalent and intractable. There is evidence that the defect lies in dynein, a protein in the cilia. The pattern of inheritance is apparently autosomal recessive however multiple versions may exist.
(05 Mar 2000)
dye laser, dyes, dyewood, Dyggve, Holger < Prev | Next > dyke-davidoff-masson syndrome, Dylan, Dylperl
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