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Kidney Week

Abstract: FR-PO199

Renal Cortical Necrosis in Sickle Cell Trait

Session Information

Category: Acute Kidney Injury

  • 103 AKI: Mechanisms

Authors

  • Alnasrallah, Basil, Qatif Central Hospital, Qatif, Eastern, Saudi Arabia
  • Alfaraj, Shatha Abdulrazaq, Qatif Central Hospital, Qatif, Eastern, Saudi Arabia
  • Al Rubh, Jafar Mohammed, Qatif Central Hospital, Qatif, Eastern, Saudi Arabia
  • Alkhamis, Osama Abdelraof, Qatif Central Hospital, Qatif, Eastern, Saudi Arabia
  • Aljishi, Manaf, Saudi German Hospital Dammam, Dammam, Eastern, Saudi Arabia
  • Alzayer, Husam, Saudi Arabia Ministry of Health, Riyadh, Saudi Arabia
Introduction

Sickle cell disease (SCD) is a monogenic disorder characterized by an abnormal hemoglobin molecule due to a single nucleotide substitution in the β-globin gene. A homozygous inheritance causes sickling and hemolysis of erythrocytes and, subsequently, ischemia and end-organ damage. The heterozygous inheritance results in sickle cell trait (SCT) with a milder phenotypic presentation. We report a rare biopsy-proven renal cortical necrosis (RCN) in a patient with SCT.

Case Description

A 40-year-old male presented with severe, non-radiating lower abdominal pain and had lower abdominal tenderness on examination. His creatinine was 530 mmol/L. The urinalysis showed moderate pyuria and hematuria with negative urine culture. The urine protein:creatinine ratio was 1040 mg/g and his lactate dehydrogenase was 1209 units/L. The vasculitic and viral screens were negative, with normal complement levels. His hemoglobin electrophoresis showed a hemoglobin S of 31%. A kidney ultrasound showed normal size and morphology of both kidneys. A kidney biopsy showed diffuse extensive coagulative necrosis in the cortex, where the tubules and glomeruli were lined by pale ghost cells with pyknotic nuclei, there was marked interstitial inflammatory infiltrate composed predominantly of neutrophil ghosts. The immunofluorescence stains were negative. The patient's creatinine gradually improved over a 6-month period down to 176 mmol/L.

Discussion

Recurrent vaso-occlusive crises in SCD lead to many kidney manifestations, including RCN. Due to the relatively low levels of hemoglobin S, individuals with SCT rarely experience clinical manifestations. To the best of our knowledge, RCN has not been reported before in SCT.

H&E Stain showing necrotic areas with ghost outlines of glomeruli and tubules, and loss of cellular details