Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Query: UMLS:C0519030 (
Klebsiella
)
21,988
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
X-linked agammaglobulinemia (XLA) is a primary immunodeficiency disease caused by mutations in the
Bruton's tyrosine kinase
(
BTK
) gene. XLA can also present in combination with juvenile idiopathic arthritis (JIA), the major chronic rheumatologic disease in children. We report herein the first known case of a juvenile patient diagnosed with XLA combined with JIA that later developed into invasive
Klebsiella
pneumoniae polyarticular septic polyarthritis. An additional comprehensive review of XLA combined with JIA and invasive K. pneumoniae septic arthritis is also presented. XLA was identified by the detection of
BTK
mutations while the diagnosis of JIA was established by clinical and laboratory assessments. Septic arthritis caused by invasive K. pneumoniae was confirmed by culturing of the synovia and gene detection of the isolates. Invasive K. pneumoniae infections can not only result in liver abscesses but also septic arthritis, although this is rare. XLA combined with JIA may contribute to invasive K. pneumoniae infection.
...
PMID:X-linked agammaglobulinemia combined with juvenile idiopathic arthritis and invasive Klebsiella pneumoniae polyarticular septic arthritis. 2456 39
Platelet
Bruton's tyrosine kinase
(
Btk
) is an essential signalling protein for the collagen receptor glycoprotein VI (GPVI) and podoplanin receptor C-type-lectin-like receptor-2, which are platelet receptors implicated in the maintenance of vascular integrity during inflammation. Moreover, platelets, platelet GPVI and
Btk
are important for host defence during murine bacterial pneumosepsis. The aim of this study was to determine the role of platelet
Btk
in vascular integrity and host defence during murine pneumosepsis caused by the common human pathogens
Streptococcus pneumoniae
and
Klebsiella
pneumoniae
. Using the Cre-loxP system, male platelet-specific
Btk
-deficient mice (PF4creBtk
fl
/Y) were created. Similar to platelets from total
Btk
-deficient mice, platelets from PF4creBtk
fl
/Y mice showed abrogated aggregation and P-selectin expression when stimulated with the GPVI ligand cross-linked collagen-related peptide. Upon infection with
S. pneumoniae
, PF4creBtk
fl
/Y mice showed increased lung bleeding, but unimpaired anti-bacterial defence. During pneumosepsis evoked by
K. pneumoniae
, platelet
Btk
deficiency was not associated with lung bleeding and did not impact on host defence, even when platelet function was further compromised by blocking secondary platelet activation by the P2Y
12
receptor antagonist clopidogrel. Together, these data indicate that, while platelet
Btk
is not important for anti-bacterial defence in pneumosepsis, its role in maintaining vascular integrity in the lung depends on the causative pathogen.
...
PMID:Platelet Btk is Required for Maintaining Lung Vascular Integrity during Murine Pneumococcal Pneumosepsis. 3087 67
