TY - CHAP M1 - Book, Section TI - Sickle Cell Nephropathy A1 - T. Pham, Phuong-Mai A1 - Qaqish, Shaker S. A1 - T. Pham, Phuong-Thu A2 - Lerma, Edgar V. A2 - Rosner, Mitchell H. A2 - Perazella, Mark A. Y1 - 2017 N1 - T2 - CURRENT Diagnosis & Treatment: Nephrology & Hypertension, 2e AB - Sickle cell disease (SCD) is an autosomal recessive hemoglobin disorder arising from the substitution of glutamate for valine at the sixth amino acid of the β-globin chain. The mutation results in poorly soluble hemoglobin S (HbS) tetramers that aggregate during cellular or tissue hypoxia, dehydration, or oxidative stress. Such aggregation can lead to red blood cell sickling deformity, premature destruction of erythrocytes, and widespread vaso-occlusive episodes, potentially leading to multiorgan damage. Various kidney complications including hematuria, renal papillary necrosis, renal tubular disorders, acute and chronic kidney injury, sickle cell glomerulopathy, and renal medullary carcinoma are reviewed in the current chapter. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accessmedicine.mhmedical.com/content.aspx?aid=1149116211 ER -