Unfortunately, there was an error in the first paragraph of the introduction on page 215. The PI ZZ prevalence for Western and Northern Europe should read 1:1,000–1:4,500 and not 1:1,000–1:145,000. The PI ZZ prevalence for Central Europe should read 1:4,500–1:10,000 and not 1:45,000–1:10,000. The corrected paragraph is produced in full below.
The αl‐antiprotease inhibitor (Pi), or αl‐antitrypsin (α1‐AT), is the principal serum inhibitor of lysosomal proteases, such as neutrophil elastase [1]. The αl‐AT is a polymorphic single chain glycoprotein of 52 kDa and 394 amino acids, synthesised in the liver and normally present in serum at 150–350 mg·dL−1 [2]. It displays >90 different genetically determined phenotypes [3]: phenotype M is the normal variant (90% of the population) and phenotypes S and Z are the two most frequent abnormal variants [3]. Calculated values of PI ZZ prevalence are approximately: 1:1,000–1:4,500 in Western and Northern Europe; 1:4,500–1:10,000 in Central Europe; and 1:10,000–1:90,000 in Eastern Europe and in the southernmost and northernmost areas of the continent. In the white population of USA, Canada, Australia and New Zealand, PI ZZ phenotype prevalence ranges from 1:2,000–1:7,000 individuals. In nonwhite populations αl‐AT deficiency is thought to be a rare or nonexistent disease [4, 5]. Homozygosity for the Z phenotype is the principal cause of α1‐AT deficiency. It typically leads to the development of diverse liver diseases in children and adults and to early adult onset emphysema, with plasma level of αl‐AT in homozygous PiZ individuals reaching only 10–15% of α1‐AT concentration observed in PiM individuals [6, 7]. Although individuals' MS or SS are unaffected, SZ subjects may be symptomatic. More recently, α1‐AT deficiency has been associated with asthma, bronchiectasis, vasculitis and panniculitis [8, 9].
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