Postnatal management of congenital bilateral renal hypodysplasia.
Renal hypodysplasia (RHD) is a congenital disorder, characterized by an abnormally developed kidney. Mutations in genes such as PAX2, HNF1-beta, TCF2, EYA1, that encode factors critical in early renal development, are being found. RHD is the leading cause of chronic renal failure in childhood, with or without associated urologic abnormalities such as vesicoureteric reflux and urinary tract obstruction. Antenatal detection has improved understanding of this disorder, resulting in enhanced outcomes through earlier intervention, including peritoneal dialysis. Management requires a multidisciplinary team approach that commences prior to the birth of the child.
La Scola
Claudio
C
Nephrology and Dialysis Unit, Pediatric Department, Azienda Ospedaliera di Bologna, Italy.
Hewitt
Ian
I
Pasini
Andrea
A
Pugliese
Fabrizio
F
Montini
Giovanni
G
D003937:Diagnosis, Differential; D006801:Humans; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*; D007668:Kidney / Q000002:abnormalities* / Q000473:pathology; D007674:Kidney Diseases / Q000151:congenital* / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*; D011379:Prognosis