Abstract
Although the clinical features in childhood are well recognised, little is recorded about later development. 15 patients aged 15-40 years have been reviewed. All have the recognised features of the syndrome in childhood. Salient features in adulthood are an insatiable appetite, an IQ of 50-85, a generally placid temperament but a tendency to tantrums. Extreme obesity (mean weight 87.5 kg; range 50.0 kg to 174.6 kg) is associated with somnolence. Eleven have scoliosis clinical or radiological. All males are hypogonadal. In females menses are uncommon but one started regularly at age 29 years. Six have diabetes or pre-diabetes; general illnesses are rare. In addition 6 patients have died (7 to 26 years) but in the only patient who had a post mortem the brain was considered normal.
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Laurance, B., Brito, A. & Wilkinson, J. Prognosis of the Prader-Willi Syndrome. Pediatr Res 12, 152 (1978). https://doi.org/10.1203/00006450-197802000-00038
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DOI: https://doi.org/10.1203/00006450-197802000-00038