V. Tizon et al., Alveolar hypoventilation and Willi-Prader syndrome: contribution of bilevel pressure nasal ventilation, REV MAL RES, 17(4), 2000, pp. 889-892
Willi-Prader syndrome is a genetic disease characterized by obesity and beh
avioral disorders. Prognosis is related to the development of respiratory f
ailure. We report the cases of two adults hospitalized for acute respirator
y failure with alveolar hypoventilation. Bilevel pressure nasal ventilation
was successful in improving the clinical presentation and blood gases. Com
pliance, tolerance and efficacy were satisfactory, even at long term, despi
te the difficulties encountered in these Willi-Prader patients with behavio
ral disorders who have difficulty in learning. This simple effective treatm
ent demonstrates the importance of assessing respiratory function early in
these patients.