Factor XIII--an under diagnosed deficiency--are we using the right assays?

摘要: Summary. Background:  The clot solubility test is the most widely used method for detection of factor (F)XIII deficiency. However, it will only detect severe deficiencies; consequently mild deficiencies and heterozygous states are probably under diagnosed. Objective: As an alternative first-line screening test, we assessed automated quantitative ammonia release assay (QARA). Patients/methods: Inter-assay imprecision was evaluated with commercial normal pathological control plasmas (10 replicates on each 5 days). Using QARA other assays a comparative assessment congenital (FXIII range < 1–70 u dL−1, n = 9) acquired (n = 43) made. We also investigated prevalence in hospitalized patients using residual samples from adult (n = 1004) paediatric intensive care unit (ICU, n = 56). Results: Assay acceptably low (normal control: mean 86.6 u dL−1; cv = 2.0%; 27.5 u dL−1; cv = 3.8%). iodoacetamide blanking procedure, results < 1–70 u dL−1) exhibited close agreement those immuno-turbidometric FXIII A-subunit (FXIII-A) method. There good correlation (R2 ≥ 0.89) between (range 20–180 u dL−1), second chromogenic assay, FXIII-A A+B-subunit ELISA. found that 21% had levels < 70 u dL−1 (mean normal ± 2 SD 73–161 u dL−1) 6% < 50 u dL−1. Within ICU samples, 52% were < 70 u dL−1, Conclusions: Our data demonstrates sensitive, highly reproducible clinical suggest deficiency relatively common phenomenon hospital after surgery ICU.