How Does Autosomal Recessive Polycystic Kidney Disease Progress to Kidney Failure?

Decline in glomerular filtration rate in children with ARPKD was comparable with declines in other pediatric chronic kidney diseases.

Autosomal recessive polycystic kidney disease (ARPKD) can cause death in the neonatal period; however, the majority of children with this condition now survive infancy. This improvement in survival necessitates a better understanding of the natural history of ARPKD to guide care of survivors.

In a prospective study, researchers compared glomerular filtration rate (GFR), blood pressure, left ventricular hypertrophy, and proteinuria in 22 children with ARPKD surviving infancy and two cohorts of children with other renal diseases, including 44 children with aplasia/hypoplasia/dysplasia and 44 with obstructive uropathy. All children were diagnosed during the neonatal period. Results were as follows:

Children with ARPKD had an annual decline in GF…

Reviewing Author

F. Bruder Stapleton, MD

Disclosures

Consultant/Advisory BoardCareer Physician

Editorial BoardsUpToDate

Leadership Positions in Professional SocietiesAmerican Society of Pediatric Nephrology Foundation (Chair)

F. Bruder Stapleton, MD

Disclosures

Consultant/Advisory BoardCareer Physician

Editorial BoardsUpToDate

Leadership Positions in Professional SocietiesAmerican Society of Pediatric Nephrology Foundation (Chair)

How Does Autosomal Recessive Polycystic Kidney Disease Progress to Kidney Failure?

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Reviewing Author