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Tay's syndrome (Chong Hai Tay)

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A very rare syndrome characterized by brittle hair, growth and mental retardation, progeria-like facies, ichtyosiform erythroderma, dysplastic nails, spasticity, ataxia, and decreased fertility.

Description

A very rare syndrome affecting both sexes and present from birth. Long list of characteristic features include trichothiodystrophy (sulphur-deficient brittle hair), growth and mental retardation, progeria-like facies caused by lack of subcutaneous fat, ichtyosiform erythroderma, dysplastic nails, spasticity, ataxia, and decreased fertility. When photosensitivity is present, the syndrome is known as PIBIDS syndrome; without photosensitivity it is referred to as the IBIDS syndrome. Tay described the disturbance in 1971 in 3 children of consanguineous parents.

Bibliography

  • C. H. Tay:
    Ichtyosiform erythrodermia, hair shaft abnormalities, and mental and growth retardation. A new recessive disorder.
    Archives of Dermatology, Chicago, 1971, 104: 4-13.

  • R. Happle, H. Traupe, H. Grobe, G. Bonsmann:
    The Tay syndrome (congenital ichtyois with trichothyodytrophy).
    European Journal of Pediatrics, Berlin, 1984, 141: 147-152.

  • L. Porto, R. Weis, C. Schulz, P. Reichel, H. Lanfermann, F. E. Zanella:
    Tay's syndrome: MRI. Neuroradiology, November 2000, 42 (11): 849-851.

  • R. J. Motley, A. Y Finlay:
    A patient with Tay's syndrome.
    Pediatric Dermatology, Boston, September 1989, 6 (3): 202-205.

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