Pulmonary Alveolar Proteinosis (PAP) is a rare disease in which the air sacs of the lungs (alveoli) become plugged with a protein-rich fluid. The exact cause of this respiratory disorder is currently unknown.

Primary Alveolar Hypoventilation Syndrome is a breathing disorder that usually gets worse during sleep. Patients often stop breathing for short time, during their nightly episodes of apnea.

This disease primarily occurs in men between 20 to 60 years of age. This condition is often seen in those suffering from obesity hypoventilation syndrome.

Signs and Symptoms of Primary Alveolar Hypoventilation Syndrome

Considering that the transfer of oxygen to the blood from the lungs is severely impaired for these patients, most people with pulmonary alveolar proteinosis experience shortness of breath when they exert themselves. The following symptoms are also often present:

• Severe difficulty in breathing, even while at rest,
• Non-Productive Cough,
• Severely disabled due to breathing difficulties,
• Lethargy,
• Awakening from sleep unrested,
• Awakening many times at night, and
• Periodic Fever.

Diagnosis of Primary Alveolar Hypoventilation Syndrome

Diagnosis of this respiratory disorder may involve the following:

• Blood gases,
• Dense white patches evident on chest X-Ray,
• Chest X-ray often shows white patches centrally located near the heart,
• chest X-ray rules out other diseases,
• CT scan shows similar results,
• Pulmonary function tests indicative of very small lung capacity,
• Low Oxygen levels in the blood,
• Examination of alveoli fluid, and
• Lung Biopsy.

Primary Alveolar Hypoventilation Syndrome Treatment

People who do not have severe symptoms generally do not require any treatment. For those with disabling symptoms, however, the protein-rich fluid in the alveoli can be washed out with a salt solution during a procedue called bronchoscopy.

In case of patients suffering from severe respiratory impairment, an entire lung can be washed while the patient is resting comfortably under general anesthesia. Few days later, the other lung is then washed, again with the person resting under general anesthesia. For most patients, one washing is usually enough. However, in some cases, lung washings may need to be repeated every 6 to 12 months for several years. In spite of repeated lung washings, however, some patients may still continue to experience shortness of breath.

Additionally, the following may also be considered:

• Oxygen Therapy,
• Breathing devices, and
• Medications to stimulate the respiratory system.

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