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Objectives: To determine whether autosomal dominant polycystic kidney disease (ADPKD) is associated with adverse fetal outcomes and maternal complications.Methods: We identified a cohort of 146 patients seen for pregnancy and cystic kidney disease at Mayo Clinic from 1975 to 2010. From this cohort, 54 patients met the ultrasound diagnostic criteria for ADPKD (ADPKD group), while the other 92 patients were diagnosed as "Simple Cyst" (control group). We compared the fetal and maternal outcomes of pregnancy and long-term maternal prognoses between these two groups.Results: Overall, the fetal complication rates were similar between the ADPKD and control groups. Rates of spontaneous abortion (15.1% versus 14%, p = 0.77) and premature birth (11.1% versus 6.8%, p = 0.44) were comparable between groups, while the rate of fetal distress (3.4% versus 0.7%, p < 0.01) was increased in the ADPKD group. The rate of preeclampsia in the patients with simple cysts (2%) was similar to that of the general population. In contrast, the pregnant ADPKD patients had higher risks for hypertension, proteinuria, edema, urinary tract infection, renal dysfunction and preeclampsia during their pregnancies.Conclusion: ADPKD is associated with increased maternal complications during pregnancy, but only has a slight potential of increased rates of fetal complications. [ABSTRACT FROM AUTHOR]