Hemolysis scavenger proteins and renal function marker in children with sickle cell disease at steady state: A cross-sectional study

Fatima A Fordjour; Alexander Kwarteng; Vivian Paintsil; Ernest Amanor; Ezekiel B Ackah; Evans X Amuzu; David S Sackey; Alex Osei Yaw Akoto

doi:10.1002/hsr2.1177

Hemolysis scavenger proteins and renal function marker in children with sickle cell disease at steady state: A cross-sectional study

Health Sci Rep. 2023 Mar 30;6(4):e1177. doi: 10.1002/hsr2.1177. eCollection 2023 Apr.

Authors

Fatima A Fordjour^{1

2}, Alexander Kwarteng^{2

3}, Vivian Paintsil^{4

5}, Ernest Amanor², Ezekiel B Ackah⁶, Evans X Amuzu⁴, David S Sackey⁷, Alex Osei Yaw Akoto⁴

Affiliations

¹ Department of Microbiology University for Development Studies Tamale Ghana.
² Department of Biochemistry and Biotechnology, College of Science Kwame Nkrumah University of Science and Technology Kumasi Ghana.
³ Kumasi Centre for Collaborative Research in Tropical Medicine Kumasi Ghana.
⁴ Child Health Directorate Komfo Anokye Teaching Hospital Kumasi Ghana.
⁵ College of Health Sciences Kwame Nkrumah University of Science and Technology Kumasi Ghana.
⁶ School of Public Health Kwame Nkrumah University of Science and Technology Kumasi Ghana.
⁷ Haematology Unit Komfo Anokye Teaching Hospital Kumasi Ghana.

Abstract

Background and aims: Hemolysis is a fundamental feature of sickle cell disease (SCD) contributing to the vaso-occlusive crisis of patients. The objectives of the study were to assess the link between hemolysis proteins and hematological parameters, and to validate cystatin C (CYS C) as a potent renal marker in diagnoising SCD.

Method: Here, a cross-sectional study carried out at the pediatric SCD clinic of the Komfo Anokye Teaching Hospital comprised 90 SCD children (HbSC, HbSF, and HbSS). ANOVA, t-test, and Spearman's rank correlation analysis were done. Elevated proteins levels were compared to standard values; alpha-1 microglobulin (A1M) (1.8-65 µg/L), CYS C (0.1-4.5 µmol/L), and haemopexin (HPX) (500-1500 µg/mL).

Results: The mean (standard deviation) age of participants was 9.830 (±0.3217) years, and 46% of them were males. From simple descriptive analysis, we observed that all but one patient had their HPX level below the reference range (<500 µg/mL). Here, A1M levels were shown to be within the appropriate reference range for all the patients except few patients. CYS C levels were also all within the required reference values. A Spearman's rank correlation test between full blood count and HPX generally suggested a weak but positive correlation; RBC (coef. = 0.2448; p = 0.0248), HGB (coef. = 0.2310; p = 0.030), hematocrit (coef. = 0.2509; p = 0.020), and platelet (coef. = 0.1545; p = 0.160). Mean corpuscular volume (coef. = -0.5645; p = 0.610) had a stronger but negative correlation with HPX. This study depicts a positive and stronger association between CYS C and HPX levels (coef. = 0.9996; p < 0.0001), validating the use of CYS C as a useful marker of renal function in persons with SCDs.

Conclusion: In the present study, we show that A1M levels were normal for most of the patients, hence CYS C levels are not alarming in this study. Further, there exists a correlation between hemolysis scavenger proteins and hematological parameters.

Keywords: crisis; hematolgy; hemolysis; proteins; renal/kidney; scavenger; sickle cell.

Grants and funding

U24 HL135881/HL/NHLBI NIH HHS/United States