INTRODUCTION: Autosomal dominant tubulointerstitial kidney disease (ADTKD) is an increasingly recognized cause of chronic kidney disease. ADTKD pregnancy outcomes have not previously been described. METHODS: A cross-sectional survey was sent to women from ADTKD families. RESULTS: Information was obtained from 85 afffected women (164 term pregnancies) and 23 controls (50 pregnancies). Only 16.5% of genetically affected women knew they had ADTKD during pregnancy. Eighteen percent of ADTKD mothers had hypertension during pregnancy versus 12% in controls (p = 0.54) and >40% in comparative studies of chronic kidney disease in pregnancy. Eleven percent of births of ADTKD mothers were <37 weeks versus 0 in controls (p < 0.0001). Cesarean section occurred in 19% of pregnancies in affected women versus 38% of unaffected individuals (p = 0.06). Only 12% of babies required a neonatal intensive care unit stay. CONCLUSIONS: ADTKD pregnancies had lower rates of hypertension during pregnancy versus other forms of chronic kidney disease, which may have contributed to good maternal and fetal outcomes.

Division of Nephrology Department of Medicine London Health Sciences Centre London Ontario Canada

Division of Public Health Sciences Department of Biostatistics and Data Science Wake Forest School of Medicine Winston Salem NC USA

DNA Data Solutions LLC St Petersburg FL USA

Research Unit of Rare Diseases Department of Paediatrics and Inherited Metabolic Disorders 1st Faculty of Medicine Charles University Prague Czech Republic

Schulich School of Medicine and Dentistry University of Western Ontario ON Canada

Section on Nephrology Wake Forest School of Medicine Winston Salem NC USA

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