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Wiedemann Rautenstrauch Syndrome

Abstract

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NORD is very grateful to Eniko Karman Pivnick, MD, professor at the University of Tennessee Health Science Center, for assistance in the preparation of this report.

Synonyms of Wiedemann Rautenstrauch Syndrome

neonatal progeroid syndrome
neonatal pseudo-hydrocephalic progeroid syndrome of WRS
Rautenstrauch-Wiedemann syndrome

Disorder Subdivisions

No subdivisions found.

General Discussion

Wiedemann-Rautenstrauch syndrome (WRS), also known as neonatal progeroid syndrome, is a very rare genetic disorder characterized by an aged appearance at birth (old man look) growth delays before and after birth (prenatal and postnatal growth retardation), and deficiency or absence of the layer of fat under the skin (subcutaneous lipoatrophy). It is anticipated that most individuals with WRS have decreased life expectancy. There are few individuals who have lived well in to the teens and afew still live in their 20s. WRS is inherited as an autosomal recessive trait, as several pairs of siblings have been reported in families with unaffected parents.

Organizations related to Wiedemann Rautenstrauch Syndrome

(Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder [progeroid appearance, neurological abnormalities, mental retardation, etc.].)

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