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François' syndrome II
Also known as:
Jules François' syndrome
Jensen’s syndrome
Associated persons:
Jules François
Edmund Zeuthen Jensen
Description:
A familial syndrome, characterized by corneal dystrophy with minute punctate opacities in all layers, and osteochondral dystrophy of the extremities. Both sexes affected; normal at birth, onset at 1 to 2 years of age. Osteochondral deformities at hands and feet; ulnar deviation with micromelia, clawhand, and short pes cavus. Concomitant development of xanthomalike, small, yellowish, hard nodules in the skin. Nephropathy and proteinuria may occur. Etiology unknown. Inheritance is autosomal recessive.
Bibliography:
- J. François:
Dystrophie dermo-chondro-cornéenne familiale.
Annales d’oculistique, Paris, 1949, 182: 409-422.
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