RT Book, Section
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.
SR Print(0)
ID 1149116211
T1 Sickle Cell Nephropathy
T2 CURRENT Diagnosis & Treatment: Nephrology & Hypertension, 2e
YR 2017
FD 2017
PB McGraw-Hill Education
PP New York, NY
SN 9781259861055
LK accessmedicine.mhmedical.com/content.aspx?aid=1149116211
RD 2024/04/24
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.