296 Estimation of the Glomerular Filtration Rate in Children with Haemophilia

Abstract

<h3></h3> Haemophilia is rare, inherited and severe bleeding disorder characterised with factor VIII or factor IX deficiency. The estimated glomerular filtration rate (eGFR) is one of the best-performing methods to evaluate kidney function. Glomerular filtration rate cannot be directly measured; however, it can be determined by measuring the clearance of an ideal filtration marker or estimated using predictive formulas. The aim of this preliminary study was to calculate eGFR of paediatric haemophilia patients treated in our centre and asses the correlation of eGFR calculated by creatinine-based and cystatin C-based equations. In our study, we included 36 boys with moderate or severe haemophilia. Out of a total of 36 patients, 27 had haemophilia A and 9 had haemophilia B. Their mean age was 11.2±4.31 years, with a range from 3 to 18 years. We investigated the correlation and agreement between two eGFR equations (creatinine-based ‘Bedside Schwartz’ equation and cystatin-C based equation). Along with applying correlation and linear regression tests, the Bland Altman test was performed to assess the agreement of the results. Statistically significant differences were found between the mean eGFR values (p&lt;0.001). No significant correlation was found between the two methods (p=0.07). Bland-Altman analysis results showed higher mean eGFR values of bedside Schwartz equation compared to the cystatin-C based formula, meaning that a significant disagreement was found between those two equations. However, within the group of haemophilia B patients, statistically significant positive correlations between the two methods were found, although still a disagreement was observed Due to the observed disagreements between eGFR within haemophilia patients, further research is needed to find the optimal measure of eGFR. We suggest extending this study on a larger cohort of patients and include other possible eGFR equations.