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Simple steps to achieve harmonisation and standardisation of dried blood spot phenylalanine measurements and facilitate consistent management of patients with phenylketonuria
Journal article   Peer reviewed

Simple steps to achieve harmonisation and standardisation of dried blood spot phenylalanine measurements and facilitate consistent management of patients with phenylketonuria

Rachel S. Carling, Zoe Barclay, Nathan Cantley, Nana Ghansah, Sarah L. Hogg, Alistair Horman, Stuart J. Moat, Simon Cowen, Chris Hopley, Chloe Deaves, …
Clinical chemistry and laboratory medicine, Vol.63(7), pp.1336-1343
26/06/2025
PMID: 39895045

Abstract

Life Sciences & Biomedicine Medical Laboratory Technology Science & Technology
Objectives: Management of phenylketonuria (PKU) relies upon life-long monitoring of phenylalanine (Phe) in dried blood spots (DBS), thus comparability of measurements is important. The lack of harmonisation and standardisation between laboratories, combined with the variable quality of patient-collected DBS specimens, are currently preventing this from being achieved. A traceable, matrix-matched Phe certified reference material, common methodology and means to ensure patient collected DBS specimens are of consistent quality would improve comparability between laboratories. Methods: Baseline inter-laboratory (n=15) variation of DBS Phe was determined by triplicate measurement of four DBS materials, on three days. Laboratories prepared and analysed these samples using their routine method of analysis. A sub-set of laboratories (n=5) repeated the process using a common sample preparation and instrument methodology (LC-MS/MS), and three different calibration approaches. Samples prepared on dried blood spot microsampling cards (DBS-MCs) from whole blood, value assigned for Phe concentration by National Measurement Laboratories (NML), were then analysed using the harmonised methodology. Results: Inter-laboratory co-efficient of variation (CV) differed with calibration approach; internal calibration 27.7 %; in-house aqueous calibration 4.7 %; centrally distributed aqueous calibration, 2.1 %. Inter-laboratory CV was reduced from 8.7 to 2.1 % by using common sample preparation and LC-MS/MS methodology. No significant difference was observed between consensus and assigned values for Phe in the four materials (p>0.05). Conclusions: This study demonstrates a simple approach to harmonising and standardising DBS Phe measurements, traceable to value assigned materials. Combined with the introduction of DBS-MCs to ensure specimen quality, clinical laboratories can achieve comparability of patient results over time.
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https://doi.org/10.1515/cclm-2024-1367View
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