Pyogenic bacterial infections in humans with MyD88 deficiency.

@article{Bernuth2003PyogenicBI,
  title={Pyogenic bacterial infections in humans with MyD88 deficiency.},
  author={Horst von Bernuth and Capucine Picard and Zhongbo Jin and Rungnapa Pankla and Hui Xiao and Cheng-Lung Ku and Maya Chrabieh and Imen Ben Mustapha and Pegah Ghandil and Yildiz Camcioglu and Julia Santos Vasconcelos and Nicolas Sirvent and M. Carmo M. Guedes and Artur Bonito V{\'i}tor and Mar{\'i}a Jos{\'e} Herrero-Mata and Juan I Ar{\'o}stegui and Carlos Rodrigo and Laia Alsina and Est{\'i}baliz Ru{\'i}z-Ortiz and Manel Juan and C. Maeso Fortuny and Jordi Yag{\"u}e and Jordi Garcia Ant{\'o}n and Mariona Pascal and Huey-Hsuan Chang and Lucile Janni{\`e}re and Yoann Rose and Ben-zion Garty and Helen M. Chapel and Andrew C. Issekutz and L{\'a}szl{\'o} Mar{\'o}di and Carlos Rodr{\'i}guez-Gallego and Jacques Banchereau and Laurent Abel and Xiaoxia Li and Damien Chaussabel and Anne Puel and Jean-laurent Casanova},
  journal={Science},
  year={2003},
  volume={321 5889},
  pages={
          691-6
        }
}
MyD88 is a key downstream adapter for most Toll-like receptors (TLRs) and interleukin-1 receptors (IL-1Rs). MyD88 deficiency in mice leads to susceptibility to a broad range of pathogens in experimental settings of infection. We describe a distinct situation in a natural setting of human infection. Nine children with autosomal recessive MyD88 deficiency suffered from life-threatening, often recurrent pyogenic bacterial infections, including invasive pneumococcal disease. However, these patients… CONTINUE READING
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