TY  - CHAP
M1  - Book, Section
TI  - Sickle Nephropathy
A1  - Saraf, Santosh L.
A1  - Derebail, Vimal K.
A1  - Gordeuk, Victor R.
A1  - Little, Jane A.
A2  - Gladwin, Mark T.
A2  - Kato, Gregory J.
A2  - Novelli, Enrico M.
Y1  - 2021
N1  - 
T2  - Sickle Cell Disease
AB  - Polymerization  of  deoxygenated  sickle  hemoglobin  (HbS),  resulting  in  fragile  red  blood  cells  (RBCs)  and  anemia,  is  the  first  step  in  a  pathophysiologic  cascade  that  involves  the  kidney.  Damage  arises  from  the  cumulative  impact  of  abnormal  red  cells,  hemolysis,  and  microvascular  occlusion.  These  insults  include  hyperfiltration  in  response  to  anemia,  cellular  toxicity  from  plasma  and  urine  cell-free  hemoglobin,  oxidative  stress,  inflammation,  and  potentiation  of  erythrocyte  sickling  in  the  hypoxic  and  acidotic  milieu  of  the  renal  medulla.  Genetic  modifiers  of  renal  disease  in  sickle  cell  disease  (SCD)  include  sickle  genotype,  mutations  that  modulate  HbS  polymerization  such  as  α  thalassemia  and  hereditary  persistence  of  fetal  hemoglobin  (HbF),  and  haplotypes  such  as  APOL1  G1  and  G2  that  increase  the  risk  of  kidney  disease  in  the  background  population.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/18
UR  - hemonc.mhmedical.com/content.aspx?aid=1179344360
ER  -