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Can Alpha kids be lung affected?

This question does not have a clearcut answer. This is likely because not enough research has been done into the subject, and it’s really tough to distinguish what is and isn’t a result of Alpha-1 and what could be coincidental (home environment, other genetics, etc.)

The damage wrought by uninhibited neutrophil elastase in the lung takes many years to manifest itself clinically. The characteristic pathology seen in α-1 antitrypsin deficiency is emphysema, caused by loss of elastic recoil. Children and adolescents with α-1 antitrypsin deficiency have not been shown to have significant lung function abnormalities. Although a study of affected children with liver disease suggested a tendency to hyperinflation, this was not found in Sveger’s subsequent study of 150 adolescents. After the age of 30–35 years there is an accelerated decline in forced expiratory volume in one second (FEV1), which is considerably worsened by cigarette smoking. In a non-smoker, symptoms are generally seen at around 50 years of age, while smokers will be symptomatic by 30–40 years. Although life expectancy is more difficult to estimate with accuracy, a combination of three studies gives a mean age of death of 50 years in smokers, compared with 66 years in non-smokers. Interestingly, only 3% of adolescents with α-1 antitrypsin deficiency smoked in Sveger’s study, suggesting that health education may be effective in this group of children.

Paediatricians tend to include an α-1 antitrypsin phenotype in the panel of tests for unexplained pulmonary symptoms. There is little evidence in support of this, although it is theoretically possible that a coexisting inflammatory disease might be worsened by α-1 antitrypsin deficiency, even in childhood. In a study of adults with bronchiectasis there was no increase in the prevalence of α-1 antitrypsin deficiency alleles, but more emphysema if both diseases coexisted. If α-1 antitrypsin deficiency is found in a child with lung symptoms it should not therefore be accepted as the underlying cause of the problem, but it might be an exacerbating factor in disease progression.

Alpha-1 Antitrypsin DeficiencyBMJ Journals

Until recently, most physicians believed there was little risk for lung disease developing in Alpha-1 children. Furthermore, it was felt that children with Alpha-1 liver disease were less likely to develop Alpha-1 lung disease. It appears now that both of these beliefs are incorrect.

Recent studies indicate that children who are carriers of a single Alpha-1 gene and who have no symptoms of lung problems still appear to have decreased lung function test results when compared to children that have two normal Alpha-1 genes. There also is mounting evidence that there is an association between abnormal Alpha-1 phenotypes and childhood asthma. In addition, a suspected link between parental cigarette smoking during an Alpha’s early years, and the development of lung problems later in their lives, appears to exist.

Much remains to be learned about identifying the risk factors to which Alphas may be exposed during their childhoods and how these exposures might influence the development of disease later in life. Nevertheless, common sense dictates there are a number of preventive measures parents can take to help maintain the health of their children with Alpha-1.

Big Fat Reference Guide – AlphaNet