Renal Tubular Dysfunction in Sickle Cell Disease

Silva, Geraldo Bezerra da; Vieira, Ana Patrícia Freitas; Couto, Amanda X.; Alves, Marília P.; Meneses, Gdayllon Cavalcante; Martins, Alice Maria Costa; Sanches, Talita Rojas; Andrade, Lúcia; Seguro, Antônio Carlos; Libório, Alexandre Braga; Daher, Elizabeth F.

doi:10.1159/000355748

Cited by 8 publications

(7 citation statements)

References 33 publications

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“…The serum level of sodium was significantly lower in the SCD patients, compared with the controls. This is in line with other studies 7-11 conducted elsewhere, which reported low serum levels of sodium in the sickle cell patients, compared with their ‘healthy’ counterparts. The reduced serum level of sodium in the SCD patients may be due to at least, in part, a possible loss of some body fluids and electrolytes, as well as inflammation and the underlying chronic condition.…”

Section: Discussionsupporting

confidence: 93%

“…The result from this current study highlights the potential use of electrolyte levels as biomarkers of SCD. Imbalance homeostasis of electrolytes including sodium, potassium, and chloride has been described in relation with sickling and increased dehydration in sickle cell patients outside Ghana, 7,8,11 with very few studies in Africa. 9,10 The sodium-potassium ATPase activity has been shown to be more active in sickle cell and contributes to cell dehydration.…”

Section: Discussionmentioning

confidence: 99%

“…3 These processes may consequently lead to increased cell dehydration in the sickle cell patients, with aggravated clinical complications. Previous studies have been conducted mainly to determine the levels of electrolytes in patients with sickle cell anaemia (HbSS), 7-10 as well as other sickle cell genotypes other than HbSC. 11 In Ghana, no single study has been done to determine and to compare the relative levels of electrolytes in patients with HbSS and HbSC genotypes.…”

Section: Introductionmentioning

confidence: 99%

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Serum Potassium, Sodium, and Chloride Levels in Sickle Cell Disease Patients and Healthy Controls: A Case-Control Study at Korle-Bu Teaching Hospital, Accra

et al. 2019

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The activity of Na+-K+ ATPase is altered in sickle cell disease (SCD), which affects serum electrolyte levels. This alteration is associated with several complications in sickle cell patients. This study evaluated the serum levels of sodium, potassium, and chloride in patients with SCD. The study was a case-control cross-sectional study involving 120 SCD patients in the steady state and 48 ‘healthy’ controls. The SCD patients were made up of 69 HbSS patients and 41 HbSC patients. Serum electrolyte levels (Na+, K+, and Cl−) were measured using a Flame Atomic Absorption Spectrometer (Variant 240FS; Varian Australia Pty Ltd). Serum sodium levels were significantly lower in the sickle cell patients, compared with their ‘healthy’ counterparts ( P = .0001). Although the study found significantly higher serum levels of potassium in the SCD patients ( P = .0001), there was no significant difference in serum chloride levels between patients with SCD and the controls ( P = .098). Serum sodium and chloride levels were not significantly different in both HbSS and HbSC patients ( P = .197 and P = .553, respectively). The level of serum potassium in the HbSS patients was, however, significantly higher compared with those with the HbSC genotype ( P = .0001). There is higher efflux of K+ from the intracellular into the extracellular space in HbSS patients, which may lead to red cell membrane dysfunction and associated complications.

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Section: Discussionsupporting

confidence: 93%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Serum Potassium, Sodium, and Chloride Levels in Sickle Cell Disease Patients and Healthy Controls: A Case-Control Study at Korle-Bu Teaching Hospital, Accra

et al. 2019

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“…Mechanism of increased GFR and renal plasma flow in patients with SCD is related to compensatory hypersecretion of vasodilator prostaglandins in response to sickling (5,11,12). During adolescence both GFR and RPF are normal, but are frequently subnormal after the age of 40.…”

Section: Discussionmentioning

confidence: 99%

Renal Complications of Sickle Cell Syndrome in Southern Iran

et al. 2017

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Background: In spite of many reports on sickle cell disease, to our knowledge frequencies of nephrologic complications are not well defined especially in sickle thalassemia. The aim of this study was to assess the nephrologic complications of sickle cell syndrome. Methods: This is a cross sectional study, including all of the patients with sickle cell syndromes (97 cases) in hematology-oncology clinics of Shiraz University of Medical Sciences. A complete history taken and physical examination was performed. Blood and urine samples were sent for different tests including micro albuminuria and Cystatin C to measure glomerular filtration rate.

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“…The prevalence of metabolic acidosis in patients with moderate SCD genotype is less clear. Etiologies for metabolic acidosis in SCD may include impaired ammonium availability, hyporeninemic hypoaldesteronism, or impaired distal tubular acidification capacity (5,6,25,27). Consistent with the literature, we observed a higher prevalence of metabolic acidosis in patients with severe SCD genotype (21%) and a higher prevalence of metabolic acidosis in patients with moderate SCD genotypes (11%) compared with African Americans from the NHANES (5%).…”

Section: Discussionmentioning

confidence: 99%

Hyperkalemia and Metabolic Acidosis Occur at a Higher eGFR in Sickle Cell Disease

et al. 2022

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Background: People with sickle cell disease (SCD) have an elevated estimated glomerular filtration rate (eGFR) compared to the general population and this may alter the usual creatinine-based eGFR cutoffs for which physiologic evidence of kidney dysfunction is apparent. This study aimed to identify eGFR thresholds for hyperkalemia and metabolic acidosis in patients with SCD Methods: This was a cross-sectional analysis of 733 patients with severe (hemoglobin SS or Sβ0-thalassemia) SCD genotype, 238 patients with moderate (hemoglobin SC or Sβ+-thalassemia) SCD genotype, and 1,333 age- and sex-matched African Americans from the National Health and Nutrition Examination Survey (NHANES). The prevalence of hyperkalemia and metabolic acidosis were compared by eGFR category. Cutoffs for hyperkalemia and metabolic acidosis were determined using generalized additive models. Results: Hyperkalemia and metabolic acidosis were more common in those with severe SCD genotype (13% and 21%, respectively) compared to in NHANES (0.3% and 5%, respectively); the prevalence rates in the moderate SCD genotype were intermediate for hyperkalemia (3%) and metabolic acidosis (11%). The proportion of patients with hyperkalemia and metabolic acidosis progressively increased with lower eGFR category in both SCD genotype groups. The eGFR thresholds for hyperkalemia and metabolic acidosis were higher in the severe (85 and 91 mL/min/1.73m2, respectively) and moderate (52 and 102 mL/min/1.73m2, respectively) SCD genotypes compared with NHANES (34 and 46 mL/min/1.73m2). Conclusions: We demonstrate that hyperkalemia and metabolic acidosis are more common and occur at higher eGFR values in patients with SCD compared to age- and sex-matched African Americans, including in eGFR ranges considered to be normal. Future studies using redefined creatinine-based eGFR thresholds for abnormal kidney function may identify high-risk patients for earlier intervention strategies and referral for specialized renal care in SCD.

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Renal Tubular Dysfunction in Sickle Cell Disease

Cited by 8 publications

References 33 publications

Serum Potassium, Sodium, and Chloride Levels in Sickle Cell Disease Patients and Healthy Controls: A Case-Control Study at Korle-Bu Teaching Hospital, Accra

Serum Potassium, Sodium, and Chloride Levels in Sickle Cell Disease Patients and Healthy Controls: A Case-Control Study at Korle-Bu Teaching Hospital, Accra

Renal Complications of Sickle Cell Syndrome in Southern Iran

Hyperkalemia and Metabolic Acidosis Occur at a Higher eGFR in Sickle Cell Disease

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