Disseminated histoplasmosis and pulmonary cavitation in an infant with alpha-1-antitrypsin deficiency

Since Laurell and Eriksson's1 initial description in 1963, the association of alpha-1-antitrypsin deficiency with childhood liver disease and adult emphysema has been well-established. While no specific immunodeficiency has been described in children with this disorder, there appears to be an increased risk of pulmonary infection.2 There are three case reports in the current literature of children with alpha-l-antitrypsin deficiency and cavitary pulmonary lesions.2-4 We describe a 4-month-old male with alpha-l-antitrypsin deficiency who developed fatal disseminated histoplasmosis with pulmonary cavitation.