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 1193127749
T1 Sickle Cell Disease
T2 Current Medical Diagnosis & Treatment 2023
YR 2023
FD 2023
PB McGraw-Hill Education
PP New York, NY
SN 9781264687343
LK accessmedicine.mhmedical.com/content.aspx?aid=1193127749
RD 2024/04/23
AB Kidney  disease  associated  with  sickle  cell  disease  is  most  commonly  due  to  sickling  of  RBCs  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;  both  sickle  cell  trait  and  disease  are  associated  with  a  faster  decline  in  eGFR  compared  to  those  without  the  trait  or  disease.  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.  Sickle  cell  glomerulopathy  is  less  common  but  inexorably  progresses  to  ESKD.  Its  primary  clinical  manifestation  is  proteinuria.  Treatment  centers  on  control  of  sickle  cell  disease  and  ensuring  adequate  fluid  intake.