Kocher Madison, Martin Iris, Twombley Katherine, Kanter Julie, Jackson Sherron, Self Sally, Hill Jeanne
Department of Pediatric Radiology, Medical University of South Carolina, 96 Jonathan Lucas Street, Suite 210, MSC 323, Charleston, SC 29425 USA.
Department of Pathology and Laboratory Medicine, Medical University of South Carolina, 171 Ashley Avenue, Charleston, SC 29425 USA.
Radiol Case Rep. 2019 Nov 8;15(1):26-30. doi: 10.1016/j.radcr.2019.10.002. eCollection 2020 Jan.
Sickle cell disease is the most common inherited blood disorder in the United States. The primary driver of pathology is microvascular occlusion which affects multiple organ systems including the kidney. The renal pathology usually manifests as hematuria, proteinuria, or microalbuminuria, and up to 10% of individuals with homozygous sickle cell disease (HbSS) develop renal failure over their lifetime. At ultrasound, the most common finding is increased size with mild variation in echogenicity of the renal parenchyma. We report the ultrasound appearance of a case of acute sickle cell nephropathy with markedly abnormal, enlarged, and echogenic kidneys due to intravascular hemolysis and hemosiderosis, confirmed by biopsy. Knowledge of this potential presentation of sickle cell nephropathy may help aid in earlier diagnosis of renal complications and avoidance of unnecessary renal biopsies.
镰状细胞病是美国最常见的遗传性血液疾病。病理的主要驱动因素是微血管阻塞,这会影响包括肾脏在内的多个器官系统。肾脏病理通常表现为血尿、蛋白尿或微量白蛋白尿,高达10%的纯合子镰状细胞病(HbSS)患者在其一生中会发展为肾衰竭。在超声检查中,最常见的发现是肾脏大小增加,肾实质回声略有变化。我们报告了一例急性镰状细胞肾病的超声表现,由于血管内溶血和含铁血黄素沉着症,肾脏明显异常、肿大且回声增强,活检证实了这一点。了解镰状细胞肾病的这种潜在表现可能有助于早期诊断肾脏并发症并避免不必要的肾活检。