Background Severe congenital renal anomalies can cause significant mortality and morbidity especially when associated with pulmonary hypoplasia. Information about outcome would be useful for counselling.
Objectives To identify prevalence and outcome at 1 year of life for severe congenital renal anomalies.
Methods Information was obtained from the regional maternity survey office. It covers an area with population of approximately 3 million people and estimated 35 000 deliveries each year. We evaluated data on all neonates diagnosed with severe congenital renal anomaly during 1989–2009.
Results We identified 985 cases. 230 (23%) were classified into severe renal congenital anomalies: bilateral renal agenesis (BRA), bilateral multicystic kidney disease (MCKD), bilateral renal hypoplasia (BRH) and polycystic kidneys (autosomal dominant polycystic kidney disease (ADPKD); autosomal recessive polycystic kidney disease (ARPKD)). The results of prevalence and survival at one year are summarised in table 1.
Conclusion Survival at 1 year for ADPKD was very good. Three quarters of all neonates born with BRH survived at 1 year. To our knowledge, this is the first population based study looking at outcome at 1 year of life for severe congenital renal anomalies.
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