Clinical Disease Caused by Klebsiella in 2 Unrelated Patients With Interleukin 12 Receptor β1 Deficiency

with interleukin 12 (IL-12)p40 or IL-12 receptor 1 (IL12R1) deficiencies are prone to develop infections caused by mycobacteria and salmonella; other infections have only been rarely observed. In this report we describe 2 unrelated patients with complete autosomal re- cessive IL12R1 deficiency who suffered from sepsis attributable to Klebsiella pneumoniae. A Mexican boy suffered from disseminated ba- cille Calmette-Guerin disease and infections caused by K pneumoniae and Candida albicans and had a fatal outcome. A Turkish girl living in France suffered from disseminated Nocardia nova infection and K pneumoniae sepsis. Therefore, Klebsiella infections should be consid- ered in patients with IL12R1 deficiency. Conversely, IL12R1 defi- ciency should be considered in patients with unexplained klebsiellosis. Pediatrics 2010;126:e971-e976