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The antimicrobial propeptide hCAP-18 plasma levels in neutropenia of various
aetiologies: a prospective study
#MMPMID26119962
Ye Y
; Carlsson G
; Karlsson-Sjöberg JM
; Borregaard N
; Modéer TU
; Andersson ML
; Pütsep KL
Sci Rep
2015[Jun]; 5
(?): 11685
PMID26119962
show ga
The underlying cause of neutropenia may be difficult to determine due to similar
clinical presentation in many neutropenic conditions. The neutrophil protein
hCAP-18 (pro-LL-37) is a major component of neutrophil secondary granules and in
this prospective study we assessed the use of hCAP-18 levels in blood plasma for
differential diagnosis of neutropenic patients (n = 133) of various aetiologies.
Plasma levels of hCAP-18 were determined using immunoblot and ELISA. Patients
with severe congenital neutropenia (n = 23) presented with the lowest levels of
plasma hCAP-18 and differential diagnostic accuracy revealed high sensitivity
(100%) and specificity (98.8%) for hCAP-18 ELISA. The correlation coefficient of
the hCAP-18 ELISA versus immunoblotting was (R = 0.831) and that of the peptide
LL-37 ELISA versus immunoblotting was (R = 0.405) (P < 0.001). Plasma hCAP-18
levels thus displayed high diagnostic value in differential diagnosis of chronic
neutropenia. Neutropenic patients with Shwachman-Diamond syndrome, Barth
syndrome, Cohen syndrome, acute myeloid leukaemia and specific granule deficiency
presented with reduced plasma hCAP-18 levels as well. The blood plasma level of
hCAP-18 was thus low in conditions in which the neutrophil antibacterial
propeptide hCAP-18 is deficient, i.e. severe congenital neutropenia and
neutrophil-specific granule deficiency, and in conditions in which bone marrow
myelopoiesis is negatively affected.
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