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Hecht-Beals syndrome (Frederick Hecht)
Also known as:
Beals-Hecht syndrome
Hecht's syndrome
Hecht-Beals-Wilson syndrome
Dutch-Kentucky syndrome
Associated persons:
Rodney Kenneth Beals
Family Dutch-Kentucky
Frederick Hecht
Ralph V. Wilson
Description:
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.
Bibliography:
- 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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