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Wiedemann-Rautenstrauch syndrome

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A syndrome characterised by intrauterine growth retardation, low birth weight, failure to thrive, short stature, widely open cranial sutures, a progeria-like appearance, hypotonia, pseudohydrocephalus, lipoatrophy, and mental retardation.

Description

A congenital syndrome characterised by intrauterine growth retardation, low birth weight, failure to thrive, short stature, widely open cranial sutures, a progeria-like appearance, hypotonia, pseudohydrocephalus, lipoatrophy, and mental retardation. A peculiar facial appearance is characterized by a triangular old-looking face, sparse hair, low-set ears and eyes, scanty eyebrows, facial bone hypoplasia, prominent scalp veins, micromaxilla, beaked nose, relative prognathism, and a subcutaneous lipodystrophy. Death occurs before six years of age. It is probably transmitted as an autosomal recessive trait.

The syndrome was first described as a new entity in 1979 by Wiedemann, who had previously described 2 cases, in 1966 and 1977. In 1977 Rautenstreich described it as progeria in 2 cases of siblings.

Bibliography

  • H.-R. Wiedemann:
    Über die Greisenhaftigkeit im Kindesalter, insbesondere die Gilford’sche Progerie: Zugleich ein Beitrag zum Bereich der mesodermalen Dysplasie.
    Zeitschrift für Kinderheilkunde, Berlin, 1948, 65: 670. Über einige progeroide Krankheitsbilder und deren diagnostische Einordnung.
    Zeitschrift für Kinderheilkunde, Berlin, 1969, 107: 91-106.
    An unidentified neonatal progeroid syndrome.
    European Journal of Pediatrics, Berlin, 1979, 130: 65-70.
  • T. Rautenstrauch, F. Snigula, T. Krieg, S. Gay, P. K. Müller:
    Progeria: A cell culture study and clinical report of familial incidence.
    European Journal of Pediatrics, Berlin, 1977, 124: 101-111.

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