Abstract

Background: Children with multicystic kidney disease (MCKD) are increasingly managed conservatively, and are followed up throughout childhood because of the risk of hypertension highlighted in some reports. With this risk still poorly defined, the strategy and the duration of follow up do not seem to be based on evidence.

Methods: Systematic review of the literature for all published cohort studies (prospective and retrospective) of children diagnosed to have unilateral MCKD and managed conservatively. Exclusion criteria were bilateral MCKD, and nephrectomy (not for hypertension) during the follow up period. For children with MCKD, the probability of developing hypertension during the follow up period was estimated.

Results: From 29 reviewed studies, six cases of hypertension developed in 1115 eligible children. The mean probability of a child with unilateral MCKD developing hypertension was therefore 5.4 per 1000 (95% CI estimated at 1.9 to 11.7 per 1000).

Conclusion: Although the risk of hypertension in MCKD is low, the results of this study do not allow firm recommendations on the frequency and duration of blood pressure measurement follow up for these children. Large prospective cohort studies with a very long duration of follow up are needed.