Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two acute phase reactants (C-reactive protein: CRP and alpha 1-acid glycoprotein: alpha 1-AGP) and transferrin (Tf) levels change were measured in order to evaluate their clinical or prognostic value during the vaso-occlusive crisis. Measurements were performed in normal controls, painful crisis with or without treatment and during steady state. Immunochemical determinations indicate that markedly acute inflammation occurred in sickle cell painful crisis, as revealed CRP and alpha 1-AGP levels. Compared to controls, during periods of steady state in the absence of any intercurrent condition, a lesser but significative increase of alpha 1-GPA was observed. Transferrin level remained lower and did not sufficient to discriminate between crisis and steady state. Compared to the vasodilatator drug effect, non steroidal anti-inflammatory drug showed more efficiency in the sickle cell disease inflammatory process. Taken together, these findings indicate that serial determinations of CRP, alpha 1-GPA and Tf may be helpful in monitoring the course of sickle cell disease and response to treatment. Then, non steroidal anti-inflammatory drugs appear necessary to reduce the length of the sickle cell crisis.
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PMID:[Clinical value of C-reactive protein, alpha 1-glycoprotein acid and transferrin assay in homozygous sickle cell disease]. 829 21

Painful vaso-occlusive crisis (VOC) is the most common complication of sickle cell disease (SCD). Increasing evidence suggests that vaso-occlusion is initiated by increased adherence of sickle red blood cells (RBCs) to the vascular endothelium. Thus, the mechanisms that remove endothelial-attached sickle RBCs from the microvasculature are expected to be critical for optimal blood flow and prevention of VOC in SCD. We hypothesized that patrolling monocytes (PMos), which protect against vascular damage by scavenging cellular debris, could remove endothelial-adherent sickle RBCs and ameliorate VOC in SCD. We detected RBC (GPA+)-engulfed material in circulating PMos of patients with SCD, and their frequency was further increased during acute crisis. RBC uptake by PMos was specific to endothelial-attached sickle, but not control, RBCs and occurred mostly through ICAM-1, CD11a, and CD18. Heme oxygenase 1 induction, by counteracting the cytotoxic effects of engulfed RBC breakdown products, increased PMo viability. In addition, transfusions, by lowering sickle RBC uptake, improved PMo survival. Selective depletion of PMos in Townes sickle mice exacerbated vascular stasis and tissue damage, whereas treatment with muramyl dipeptide (NOD2 ligand), which increases PMo mass, reduced stasis and SCD associated organ damage. Altogether, these data demonstrate a novel mechanism for removal of endothelial attached sickle RBCs mediated by PMos that can protect against VOC pathogenesis, further supporting PMos as a promising therapeutic target in SCD VOC.
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PMID:Patrolling monocytes scavenge endothelial-adherent sickle RBCs: a novel mechanism of inhibition of vaso-occlusion in SCD. 3107 43