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Background  Maternal kidney disease (acute kidney injury (AKI), advanced chronic kidney disease (CKD), dependence on dialysis or a kidney transplant) has a substantial impact on pregnancy, with risks of significant perinatal morbidity. These pregnancies require integrated multidisciplinary care to manage a complex and often challenging clinical situation. The ability to deliver optimal care is currently hindered by a lack of understanding around prevalence, management and outcomes in Australia. This study aims to expand an evidence base to improve clinical care of women with serious kidney impairment in pregnancy.    Methods/design  The “Kidney Disease in Pregnancy Study” is a national prospective cohort study of women with stage 3b-5 CKD (including dialysis and transplant) and severe AKI in pregnancy, using the Australasian Maternity Outcomes Surveillance System (AMOSS). AMOSS incorporates Australian maternity units with > 50 births/year ( n  = 260), capturing approximately 96% of Australian births. We will identify women meeting the inclusion criteria who give birth in Australia between 1st August 2017 and 31st July 2018. Case identification will occur via monthly review of all births in Australian AMOSS sites and prospective notification to AMOSS via renal or obstetric clinics. AMOSS data collectors will capture key clinical data via a web-based data collection tool. The data collected will focus on the prevalence, medical and obstetric clinical care, and maternal and fetal outcomes of these high-risk pregnancies.    Discussion  This study will increase awareness of the issue of serious renal impairment in pregnancy through engagement of 260 maternity units and obstetric and renal healthcare providers across the country. The study results will provide an evidence base for pre-pregnancy counselling and development of models of optimal clinical care, clinical guideline and policy development in Australia. Understanding current practices, gaps in care and areas for intervention will improve the care of women with serious renal impairment, women with high-risk pregnancies, their babies and their families.    Electronic supplementary material  The online version of this article (10.1186/s12882-019-1393-z) contains supplementary material, which is available to authorized users.

Serious kidney disease in pregnancy: an Australian national cohort study protocol