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August 1968

Prader-Willi Syndrome

Author Affiliations

New Haven, Conn
From the Department of Pediatrics, Yale University School of Medicine, and the Yale-New Haven Hospital, New Haven, Conn.

Am J Dis Child. 1968;116(2):211-217. doi:10.1001/archpedi.1968.02100020213017

IN 1956 Prader et al1 first reported nine cases of a new syndrome. The symptom complex evolves to its full expression over a number of years. The prenatal course is usually uneventful, except for decreased fetal activity. Infants are born at term with a low birth weight. Shortly after birth, extreme weakness with generalized muscular hypotonia, poor suck, and weak cry become evident. The period is marked by difficult feeding and episodes of hypoxia. After several weeks to months of failure to thrive, there is gradual improvement in strength accompanied by more vigorous feeding and accelerated weight gain. By 2 years of age obesity has supervened, while delay in psychomotor development is apparent. Subsequently, there develops extraordinary obesity with hyperphagia, and, in addition, short stature, hypoplastic genital organs, and delayed puberty. By this time the patients present a striking clinical picture, with extreme adiposity distributed predominantly over the lower

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