Assessment of renal function in Indian patients with sickle cell disease

Abstract

Sickle cell disease (SCD) and its variants are genetic disorders resulting from the presence of a mutated form of hemoglobin. Renal disease is one of the most frequent complications, and kidney damage starts very early and progresses throughout life causing severe complications. The present study is aimed to analyze creatinine-based estimated glomerular filtration rate (eGFR) in 616 SCD patients (507 HbSS and 109 HbSB+), receiving medical care at outpatient wing of Sickle Cell Institute, Chhattisgarh. Glomerular filtration rate (GFR) estimated using the Modification of Diet in Renal Disease (MDRD), Cockcroft-Gault, chronic kidney disease epidemiology collaboration (CKD-EPI) (<17 years analyzed with Schwartz), and SCD specific Jamaica Sickle Cell Cohort Study (JSCCS)-GFR equations were compared. Further, eGFR calculated using the CKD-EPI and Schwartz equations was used to define various stages of kidney function and compared with clinical and hematological variables. The mean age of patients was 15.8 years. Comparison of eGFR using various formulas revealed that MDRD and JSCCS formulas overestimated the GFR. Among SCD patients, prevalence of glomerular hyperfiltration (GHF) is high followed by renal insufficiency (RI) and renal failure (RF). However, no differences were found in hematological profiling among different functional stages of kidney. Age and body surface area are significantly more in SCD individuals with normal kidney function and GHF. Participants with RF showed a higher level of blood urea and fetal hemoglobin. In summary, this is the first study to analyze different functional stages of kidney among SCD patients of India. Our study revealed that the GHF and RI are the important indicators of kidney damage.

Ellagic Acid Exerts Dual Action to Curb the Pathophysiological Manifestations of Sickle Cell Disease and Attenuate the Hydroxyurea-Induced Myelosuppression in Berkeley Mice

“…SCD is associated with liver ischemia 41 and kidney impairment. 42 The elevated levels of urea and creatinine in SCD and the effect of HU on these levels are in line with the reported literature. 3,43 Current results indicate that EA had minimal effect for any further improvement in the HU effect on serum glutamic pyruvic transaminase (SGPT), serum glutamic oxaloacetic transaminase (SGOT), and urea levels (Figure S8).…”

IL1RN VNTR Polymorphism and kidney damage in sickle cell anemia patients

“…One of our previous studies demonstrated that most SCA patients exhibit GHF followed by renal insufficiency. Moreover, this study showed that the SCA patients with kidney damage have a higher level of HbF (25). This study revealed that the IL1RN VNTR polymorphism was not found to be associated with kidney damage in SCA patients.…”

Investigations of Kidney Dysfunction-Related Gene Variants in Sickle Cell Disease Patients in Cameroon (Sub-Saharan Africa)

“…HbF is a major SCD modifier, which is known to modulate the SCD phenotype (Coleman and Inusa, 2007), to ameliorates pathophysiological and clinical manifestations of the sickling process (Adekile, 2020). There is accumulating evidence indicating that this major disease modifier is influenced by β-globin gene cluster haplotypes (Lakkakula et al, 2017;Piel et al, 2017;Adekile, 2020) and (α-globin gene deletions (Piel et al, 2017). Thus, we have looked at distribution of fetal haemoglobin levels vs. representative β-globin gene cluster haplotypes and α-globin gene deletions and results are shown in Figure 4.…”