Alpha 1 Antitrypsin Deficiency Life Expectancy, Symptoms, Causes, Treatment
Alpha 1 antitrypsin deficiency is a genetic problem in which band of alpha 1 protein is missing in the serum. It is a common disease which occur genetically and usually it is undiagnosed. Alpha 1 antitrypsin deficiency is the predisposing factor of liver and lung diseases. it is estimated that it Is commonly inherited in white people. Almost 1 to 5 percent of patients with chronic obstructive pulmonary disease will definitely have alpha 1 antitrypsin deficiency. Rarely emphysema is observed in the patients with alpha 1 antitrypsin deficiency. As the time passes and as the patient becomes aged, the chances of liver diseases increases.
The disease and death rates of this disease are not specific. It always depends on the complications patient face due to alpha 1 antitrypsin deficiency. It is not necessary that every patient develop cirrhosis and symptomatic emphysema. So the chances of mortality varies from patient to patient. It is estimated that the life expectancy is increased in case of symptomatic patients. The patients who does not experience symptoms have less chances of diagnosis and it may prove fatal for patient. The median time observed between the diagnosis and observation of symptoms is estimated about 8 years.
Alpha 1 antitrypsin deficiency is the genetic disease. It is not a rare disease but still uncommon. It is still under study. It usually inherited in white people. Basically alpha 1 antitrypsin molecule is responsible for inhibition of various neutrophil derived proteases. Hepatocytes are involved in the formation of alpha 1 antitrypsin molecule. After formation, this molecule diffuses into blood and start acting against proteases. Its main function is to inactivate neutrophil elastase which in turn regulates phagocytosis. It is released during normal phagocytosis process.
It is an uncommon genetic disease which is responsible for various other lung and liver diseases. .So, the symptoms are according to the complications patient facing. Usually patient develops symptomatic emphysema which shows some characteristic signs like airflow obstruction, respiratory work and hyperinflation. The increased load on lungs causes tachypnea, scalene and intercostal muscle retraction. Airway obstruction shows symptoms like wheezing, pursed lip breathing and pulsus paradoxus. Likewise hyperinflation show symptoms like decreased breathe sound intensity, distant heart sounds, barrel chest and increased percussion note. Patients having emphysema does not show any abnormal findings upon examination.
The complications of alpha 1 antitrypsin deficiency includes liver and lung diseases. so the treatment plans include medical care of lungs and liver diseases. The medications are selected according to the disease and some precautions also helps to reduce symptoms. Avoiding smoking and curing asthma and respiratory infections helps to cover up this disease. Emphysema requires treatment, if it is left untreated, then it may prove fatal for patient. There are some points regarding treatment which should be guided to patient.
Alpha 1 Antitrypsin Deficiency Life Expectancy
The disease and death rates of this disease are not specific. It always depends on the complications patient face due to alpha 1 antitrypsin deficiency. It is not necessary that every patient develop cirrhosis and symptomatic emphysema. So the chances of mortality varies from patient to patient. It is estimated that the life expectancy is increased in case of symptomatic patients. The patients who does not experience symptoms have less chances of diagnosis and it may prove fatal for patient. The median time observed between the diagnosis and observation of symptoms is estimated about 8 years.
Alpha 1 Antitrypsin Deficiency Causes
Alpha 1 antitrypsin deficiency is the genetic disease. It is not a rare disease but still uncommon. It is still under study. It usually inherited in white people. Basically alpha 1 antitrypsin molecule is responsible for inhibition of various neutrophil derived proteases. Hepatocytes are involved in the formation of alpha 1 antitrypsin molecule. After formation, this molecule diffuses into blood and start acting against proteases. Its main function is to inactivate neutrophil elastase which in turn regulates phagocytosis. It is released during normal phagocytosis process.
Alpha 1 Antitrypsin Deficiency Symptoms
It is an uncommon genetic disease which is responsible for various other lung and liver diseases. .So, the symptoms are according to the complications patient facing. Usually patient develops symptomatic emphysema which shows some characteristic signs like airflow obstruction, respiratory work and hyperinflation. The increased load on lungs causes tachypnea, scalene and intercostal muscle retraction. Airway obstruction shows symptoms like wheezing, pursed lip breathing and pulsus paradoxus. Likewise hyperinflation show symptoms like decreased breathe sound intensity, distant heart sounds, barrel chest and increased percussion note. Patients having emphysema does not show any abnormal findings upon examination.
Alpha 1 Antitrypsin Deficiency Treatment
The complications of alpha 1 antitrypsin deficiency includes liver and lung diseases. so the treatment plans include medical care of lungs and liver diseases. The medications are selected according to the disease and some precautions also helps to reduce symptoms. Avoiding smoking and curing asthma and respiratory infections helps to cover up this disease. Emphysema requires treatment, if it is left untreated, then it may prove fatal for patient. There are some points regarding treatment which should be guided to patient.
- Ask about smoking habits.
- Arrange follow up
- Advice about health effects.
- Assist patient to replace nicotine.
Alpha 1 Antitrypsin Deficiency Life Expectancy, Symptoms, Causes, Treatment
Reviewed by Simon Albert
on
February 05, 2019
Rating:
Reviewed by Simon Albert
on
February 05, 2019
Rating:
