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Hecht-Beals syndrome (Frederick Hecht)

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A relatively rare syndrome characterized by the inability to open the mouth completely (trismus) and short finger-flexor tendons with wrist extension (pseudocamptodactyly), various foot deformities, and less than normal stature.

Mabry et al proposed the eponym Dutch-Kentucky syndrome in 1974, after performing an extensive pedigree of a Kentucky family; the earliest affected family member that they were able to trace was a young Dutch girl who immigrated to southern United States in 1780. Inheritance is autosomal dominant.

Hecht and Beals in 1969 described the disease in a family. Mabry and collaborators coined the term trismus-pseudocamptodactyly in 1974.


  • F. Hecht, R. K. Beals:
    Inability to open the mouth fully: an autosomal dominant phenotype with facultative camptodactyly and short stature. Preliminary note.
    Birth Defects Original Article Series, New York, 1969, 5(3): 96-98.
  • R. V. Wilson, D. L. Gaines, A. Brooks, et al:
    Autosomal dominant inheritance of shortening of flexor profundus muscle-tendon unit with limitation of jaw excursion.
    Birth Defects Original Article Series, New York, 1969, 5(3): 99-102.
  • C. C. Mabry, I. S. Barnet, M. W. Hutcheson, H. W. Sorenson:
    Trismus pseudocamptodactyly syndrome: Dutch-Kentucky syndrome.
    Journal of Pediatrics, St. Louis, 1974, 85: 503-508.
  • M. Tsukahara, F. Shimozaki, T. Kajii:
    Trismus-pseudocamptodactyly syndrome in a Japanese family.
    Clinical Genetics, Copenhagen, 1985, 28: 247-250.

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