RT Book, Section
A1 Dirkx, Tonja C.
A1 Woodell, Tyler B.
A2 Papadakis, Maxine A.
A2 McPhee, Stephen J.
A2 Rabow, Michael W.
A2 McQuaid, Kenneth R.
SR Print(0)
ID 1184183511
T1 Sickle Cell Disease
T2 Current Medical Diagnosis & Treatment 2022
YR 2022
FD 2022
PB McGraw-Hill Education
PP New York, NY
SN 9781264269389
LK accessmedicine.mhmedical.com/content.aspx?aid=1184183511
RD 2024/04/18
AB Kidney  dysfunction  associated  with  sickle  cell  disease  is  most  commonly  due  to  sickling  of  red  blood  cells  in  the  renal  medulla  because  of  low  oxygen  tension  and  hypertonicity.  Congestion  and  stasis  lead  to  hemorrhage,  interstitial  inflammation,  and  papillary  infarcts  with  resultant  necrosis.  Clinically,  hematuria  is  common,  and  proteinuria  can  be  present  as  well,  portending  a  poorer  prognosis.  Damage  to  renal  capillaries  also  leads  to  diminished  concentrating  ability.  Isosthenuria  (urine  osmolality  equal  to  that  of  serum)  is  routine,  and  patients  can  easily  become  dehydrated.  These  abnormalities  are  also  encountered  in  patients  with  sickle  cell  trait.  Sickle  cell  glomerulopathy  is  less  common  but  inexorably  progresses  to  ESKD.  Its  primary  clinical  manifestation  is  proteinuria.  Optimal  treatment  requires  adequate  hydration  and  control  of  the  sickle  cell  disease.