RT Book, Section
A1 Shah, Shweta
A1 Braun, Michael C.
A2 Kline, Mark W.
SR Print(0)
ID 1182911859
T1 Renal Morphogenesis
T2 Rudolph's Pediatrics, 23e
YR 2018
FD 2018
PB McGraw-Hill Education
PP New York, NY
SN 9781259588594
LK accesspediatrics.mhmedical.com/content.aspx?aid=1182911859
RD 2024/04/20
AB Renal  morphogenesis  is  a  complex,  temporally  and  spatially  regulated  3-dimensional  stochastic  process  by  which  precursor  cells  develop  into  a  structurally  and  functionally  normal  kidney.  Abnormal  or  dysregulated  renal  morphogenesis  results  in  a  wide  range  of  renal  abnormalities  including  renal  agenesis,  dysplasia,  hypoplasia,  and  structural  anomalies  such  as  ectopic  kidneys,  fused  kidneys,  duplicated  collecting  systems,  and  vesicoureteral  reflux;  these  are  collectively  known  as  congenital  anomalies  of  the  kidney  and  urinary  tract  (CAKUT).  Together  they  comprise  the  most  common  cause  of  end-stage  renal  disease  (ESRD)  in  children.  It  is  likely  that  more  subtle  forms  of  abnormal  renal  morphogenesis  contribute  to  the  development  of  chronic  kidney  disease  (CKD),  ESRD,  and  “essential”  hypertension  in  the  adult  population,  although  this  hypothesis  remains  unproven.  Much  of  our  understanding  of  the  complexities  of  renal  morphogenesis  comes  from  either  experiments  of  nature,  ie,  naturally  occurring  genetic  mutations  linked  with  specific  forms  of  CAKUT  (see  Chapter  465),  or  from  decades  of  investigation  using  in  vitro  or  in  vivo  model  systems.  With  the  advent  of  modern  molecular  targeting  techniques,  murine  models  and  human  culture  systems  have  revolutionized  our  understanding  of  renal  morphogenesis  using  tissue,  cell,  and  temporal-specific  gene  modification.  The  regulation  of  renal  morphogenesis  spans  the  entire  spectrum  of  human  biology  from  transcriptional  regulation,  intracellular  signaling,  paracrine  and  autocrine  responses,  cell-cell  induction,  repression,  epigenetic  modification,  and  even  environmental  factors.