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SHR Neuro Krebs Kardio Lipid Stoffw Microb

Schöffl, C; Haas, A; Herrmann, M; Aberer, W.
The crux of C1-INH testing in everyday lab work.
J Immunol Methods. 2021; 497:113109 Doi: 10.1016/j.jim.2021.113109
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Führende Autor*innen der Med Uni Graz
Schöffl Clemens
Co-Autor*innen der Med Uni Graz
Aberer Werner
Herrmann Markus

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BACKGROUND: The determination of functional C1-INH is complex and depends on methodology, sample transport, and storage conditions. In clinical practice, we encounter individuals with pathological values which then cannot be proved true, and HAE patients in whom the values were wrongly found to be normal under non-optimum conditions. We aimed to test realistic real-life sample processing conditions for accurate C1-INH determination. METHODS: We conducted two national inter-laboratory comparisons with optimal sample preparation but different dispatch conditions. We also investigated variations of temperature and time, and their influence on C1-INH. RESULTS: C1-INH levels showed a significantly wider dispersion under suboptimal transport conditions than under optimal conditions (p < 0.00001). Two putatively healthy patient samples turned out to be pathological. Contrary to our expectations, we found no significant trend in a specific direction when the variables of temperature, time and sample material were combined and varied under realistic conditions. However, the range of variation in [%] functionality was markedly greater in supposedly healthy volunteers. Thus, under experimental conditions we obtained false pathological results that were not far from reality. CONCLUSION: C1- INH determination is crucial for the diagnosis of HAE. Time, temperature, and sample handling have a significant impact on this laboratory value, sometimes leading to incorrect values, inaccurate diagnoses, and inappropriate therapies. This underlines the importance of proper handling of samples. If a patient has ambiguous C1-INH values despite optimized conditions, thus hindering a conclusive diagnosis of HAE, we recommend genetic testing.
Find related publications in this database (using NLM MeSH Indexing)
Angioedemas, Hereditary - blood, diagnosis, immunology
Biomarkers - blood
Case-Control Studies - administration & dosage
Centrifugation - administration & dosage
Complement C1 Inhibitor Protein - analysis
Humans - administration & dosage
Immunologic Techniques - administration & dosage
Laboratory Proficiency Testing - administration & dosage
Observer Variation - administration & dosage
Predictive Value of Tests - administration & dosage
Reproducibility of Results - administration & dosage
Specimen Handling - administration & dosage
Temperature - administration & dosage
Time Factors - administration & dosage

Find related publications in this database (Keywords)
Hereditary angioedema
Laboratory testing
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