Diffuse Cortical Necrosis of Native Kidneys in Sickle Cell Trait: A Case Report

镰状细胞性状患者原发性肾脏弥漫性皮质坏死:病例报告

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Abstract

INTRODUCTION: Sickle cell disorders are the most common hereditary hematological disorders; sickle cell trait (SCT) is largely benign with mild clinical manifestations, if any. Renal cortical necrosis (RCN) is a rare and severe form of kidney injury and, to our knowledge, has not been previously reported to affect the native kidneys of patients with SCT. CASE PRESENTATION: We describe a case of a 41-year-old male with a background of SCT who presented with acute abdominal pain and lower abdominal tenderness. He had rapidly rising creatinine over 48 h from 229 to 526 µmol/L, as well as elevated lactate dehydrogenase and total bilirubin at 2,606 U/L and 31 µmol/L, respectively. His toxicology, viral, and autoimmune profiles were negative, with a normal kidney ultrasound scan. The kidney biopsy revealed diffuse RCN. The patient was managed conservatively and had partial recovery of his kidney function to a baseline creatinine of 176 µmol/L 6 months later. CONCLUSION: Although SCT has long been considered a benign condition, growing evidence suggests that vaso-occlusive manifestations can occur, especially in the context of physiological stressors. This is the first described case of diffuse RCN affecting the native kidneys of a patient with SCT without an identifiable stressor, highlighting the need for vigilance in managing SCT and its potential severe kidney manifestations.

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