When does obesity become life threatening? Clinical findings in patients with Prader-Willi Syndrome experiencing cardio-respiratory complications from obesity

Linda M. Gourash and James E. Hanchett

Pittsburgh, Pennsylvania 15217 

There has been recent concern over deaths of children who had recently begun treatment with recombinant human growth hormone (rhGH). The temporal relationship with the use of rhGH does not indicate a causal relationship to rhGH.  Other causes must be examined but cannot be assumed. Lacking from the discussion has been clinical data and clinical description of critically and seriously ill obese children and adolescents with PWS (not on rhGH).

The Children’s Institute is a tertiary referral center for children and adults with Prader-Willi syndrome.  Admissions are in response to behavioral, medical and psychiatric crises.  Referrals come from all over the USA and Canada providing 60 PWS admissions per year.

The insidious nature of cardiopulmonary deterioration in persons with PWS whose weight has reached the obese range is evident by the fact that cor pulmonale and hypoxia are frequently undiagnosed at the time of referral.  Numerous case histories demonstrate that cardiorespiratory decompensation often occurs seemingly without warning in patients who have appeared to be tolerating their obesity without imminent risk.

This talk will review clinical descriptions, clinical data, anthropometric measurements and of well, to seriously ill, hospitalized patients with PWS with an emphasis on children and adolescents who had critical care admissions near the time of referral.

June 2004