Published online Jan 9, 2022. doi: 10.5409/wjcp.v11.i1.14
Peer-review started: February 28, 2021
First decision: July 30, 2021
Revised: August 12, 2021
Accepted: November 15, 2021
Article in press: November 15, 2021
Published online: January 9, 2022
Processing time: 312 Days and 23 Hours
Whether the underlying mutations are homozygous, heterozygous, or co-inherited with other hemoglobinopathies, sickle cell disease is known to afflict the kidneys, leading to the clinical entity known as sickle cell nephropathy (SCN). Although common, SCN remains diagnostically elusive. Conventional studies performed in the context of renal disorders often fail to detect early stage SCN. This makes the quest for early diagnosis and treatment more challenging, and it increases the burden of chronic kidney disease-related morbidity among patients. Novel diagnostic tools have been employed to overcome this limitation. In this study, we discuss various biomarkers of SCN, including those employed in clinical practice and others recently identified in experimental settings, such as markers of vascular injury, endothelial dysfunction, tubulo-glomerular damage, and oxidative stress. These include kidney injury molecule-1, monocyte chemoattractant protein-1, N-acetyl-B-D-glucosaminidase, ceruloplasmin, orosomucoid, nephrin, and cation channels, among others. Furthermore, we explore the potential of novel biomarkers for refining diagnostic and therapeutic approaches and describe some obstacles that still need to be overcome. We highlight the importance of a collaborative approach to standardize the use of promising new biomarkers. Finally, we outline the limitations of conventional markers of renal damage as extensions of the pathogenic process occurring at the level of the organ and its functional subunits, with a discussion of the expected pattern of clinical and biochemical progression among patients with SCN.
Core Tip: This study discusses the expected clinical and biochemical progression among patients with sickle cell nephropathy, the utility of various biomarkers, and the limitations of conventional biomarkers. Novel biomarkers used in combination have been demonstrated to have a higher diagnostic yield as compared to that of individual markers, necessitating a collaborative approach in the standardization and utilization of promising biomarkers such as kidney injury molecule-1, monocyte chemoattractant protein-1, N-acetyl-B-D-glucosaminidase, ceruloplasmin, orosomucoid, nephrin, cation channels, and endothelial dysfunction.