A new study published in Pediatric Nephrology in May 2021 has uncovered risk factors that help identify which children born with only one functioning kidney may go on to develop chronic kidney disease later on in life. The results highlight previously underappreciated differences between children with multicystic dysplastic kidney (MCDK) disease compared to those with unilateral renal agenesis (URA), and could lead to more individualized care.
Both MCDK and URA are congenital conditions whereby a child is born with only one functioning kidney. MCDK is when one kidney is abnormal, and shrinks over time before disappearing completely. URA is when a second kidney never develops in the first place.
Traditionally, these two conditions have been treated similarly. But the new study done by the Pediatric Nephrology Clinical Pathway Development Team at BC Children’s Hospital will change the way kids are assessed and delivered long-term care at a local level – and perhaps even internationally.
The research team retrospectively followed 230 children who were diagnosed with either MCDK or URA. They looked at data capturing outcomes of hypertension, proteinuria, or chronic kidney disease over the course of five years.
In general, URA was more often associated with an increased risk of all three of these outcomes. As well, children with URA were found to have a higher prevalence of coinciding genetic syndromes, for example affecting the heart, eyes or other parts of the body. Regardless of whether a child had URA or MCDK, the presence of other genetic syndromes was associated with more health complications later in life.
“Taken together, we’ve identified a number of different variables early on that would suggest higher risk versus lower risk of developing long-term complications,” says Dr. Douglas Matsell, a pediatric nephrologist at BC Children’s Hospital who was involved in the study. “So not all solitary functioning kidneys have a perfect outcome, and there are some kids that require longer term follow up.”
Based on the results of this study, Matsell and his colleagues have created a new risk assessment tool that will help identify children at higher risk of developing chronic kidney disease. “If that risk score is above or below a certain point, we’ll suggest whether they should be followed long term in our clinic or can be discharged back to their family doctor,” explains Matsell.
This new approach will be beneficial in several ways. Notably, it has the potential to decrease the anxiety of patients and their families who are at low risk of long-term complications. As well, it will ensure that those who are at higher risk can be followed and treated more closely by their kidney team.
In an editorial commentary accompanying the study, researchers based in Holland note that these “interesting results reported by Matsell et al. provide another step towards further personalizing the care for patients with a solitary functioning kidney… However, collaborative efforts of research groups will be needed to create and validate prediction models that are applicable in different centers and healthcare settings.”
Such plans are already underway. Next year Matsell will be taking a sabbatical to expand upon this risk assessment tool in partnership with researchers at Ohio State University in Columbus, Ohio. The goal is to make sure the risk assessment tool is widely applicable to diverse populations and will be an effective means for improving the long-term care of children born with a single functioning kidney.
