Alpha -1 Antitrypsin Deficiency

Ira Shah
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Alpha -1 Antitrypsin Deficiency - Treatment
At present, the only treatment for these patients is replacement therapy with Human alpha-1 antitrypsin, which is derived from pooled human blood plasma. Replacement therapy is given as IV infusion and is recommended every weekly. It is given in the dose of 60mg/kg. It is indicated in persons with PiZZ, PiZ(Null) or Pi(Null)(Null) and those who have early evidence of panacinar emphysema. Patients should be vaccinated with Hepatitis B and Hepatitis A vaccine preferably before starting therapy. In patients with emphysema, anti inflammatory steroids and bronchodilators are the treatment of choice. Oxygen therapy may be required in patients' with:
- Resting PaO2 < 55m Hg or O2 saturation <85%
- Cor pulmonale.
- Resting PaO2 >55mHg or O2 saturation >85% who desaturate to PO2 <55mHg or O2 saturation <85% with exercise.

Surgery in the form of lung volume reduction by removing damaged portions of the lung is recommended in patients unresponsive to medical treatment. However, there is a high mortality with this surgery. There are also chances of air leaks and infection developing after the surgery. If this surgery works, it may help for a year.

Lung transplant: Patients with end stage lung disease (with at least 18 months expected survival) may be candidates for a lung transplant either unilateral or bilateral. Generally single lung transplant is done in patients under 60-65 years of age and double lung transplant is done in patients under 50-55 year of age. The one-year survival rate for single lung transplant is greater than 90% and one year survival rate for double lung transplant is 85%. The most common cause of death for transplant patients is infection.For patients with liver disease, Phenobarbital or cholestyramine may be beneficial for cholestasis. Diuretics may be required for treating ascitis. For patients with severe liver disease, liver transplant is the only option. Panniculitis may be treated with dapsone and these patients may require long term alpha-l-antitrypsin replacement.

Most patients with alpha-1 antitrypsin deficiency live a long and healthy life with little or on sign of lung damage. The chance of avoiding liver damage is also fairly high. However, for those affected, prognosis depends on several factors namely, the pulmonary function tests, the medical treatment given and liver involvement.


Alpha -1 Antitrypsin Deficiency Alpha -1 Antitrypsin Deficiency 03/05/2001
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