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Primary alveolar hypoventilation
Alternative namesOndine's curse
DefinitionA rare disorder of unknown cause that leads to inadequate breathing despite normal physiologic make-up.
Causes, incidence, and risk factors
The cause of this disease is unknown. Current research is looking at how the brain of these patients may be less responsive to carbon dioxide.
The condition is usually worse during sleep, and periods of apnea (where the breathing stops) are usually present. Patients with this disease are extremely sensitive to even small doses of sedatives or narcotics, which can make their inadequate breathing much worse. The disease primarily affects men 20 to 50 years old. It can also be present in male children.
This condition is commonly seen in those with obesity hypoventilation syndrome .
Signs and tests
This disease may also alter the results of the following tests:
TreatmentMedications that stimulate the respiratory system may be used. Mechanical devices that assist breathing, particularly at night, may be helpful in some patients. Oxygen therapy may be helpful in some patients, but may produce worse night symptoms in others.
Expectations (prognosis)Response to treatment varies.
ComplicationsA possible complication is cor pulmonale (right-sided heart failure).
Calling your health care providerCall for an appointment with your health care provider if symptoms of this disorder develop. Bluish skin ( cyanosis ) may indicate an urgent need for treatment.
PreventionThere is no known prevention.
Update Date: 2/6/2003David A. Kaufman, M.D., Pulmonary & Critical Care Medicine, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.
Last updated: Tue, 06 Jan 2009 00:20:03 GMT