1- Unidade de Imunodeficiências Primária, Área Departamental de Pediatria Médica, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central
2- National Institute of Allergy and Infectious Diseases, NIH, Bethesda, MD, USA.
3- Unidade de Infeciologia, Área Departamental de Pediatria Médica, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central
- 16 thBiennial meeting og the European Society fo Immunodeficiencies, Praga, Novembro 2014
- Apresentação em poster e publicação sob a forma de resumo
Granulibacter bethesdensis (Gb) is a Gram-negative bacteria that belongs to the Acetobacteraceae family. First described in 2006, it has been shown to cause infection in Chronic Granulomatous Disease (CGD) patients.
In June 2012 a boy with XL-CGD was admitted for deep cervical abscess and treated with an appropriate 6 week IV antibiotic regimen, followed by oral therapy. He had complete clinical and radiological resolution. In October 2012 he was readmitted for pneumonia and treated with IV antibiotics and voriconazole with rapid resolution. No microorganism was identified in either episode, despite agressive research. In December 2012 he presented with fever and vomiting and a diagnosis of meningitis was made. He was treated with IV antibiotics (including anti-mycobacterial) and antifungals but his condition worsened and he ultimately passed away in April 2013. The CSF analysis led to the identification of Granulibacter bethesdensis by 16S rRNA sequencing, resistant to all tested antibiotics.
To our knowledge, 12 CGD patients with Gb infections have been identified. The 10 US patients had relatively mild infections and none died. The 2 European patients have both succumbed to their Gb infections. Interestingly, both European Gb are genetically similar and different from the US Gb. Mouse experiments performed in both gp91 KO and p47 KO showed higher lethality in those inoculated with the European Gb. Previously known as causing mild infections in CGD patients, Gb should also be suspected in life-threatening infections in CGD patients, especially in Europe.
Palavras Chave: Granulibacter bethesdensis, Chronic Granulomatous Disease, meningitis