The Role of Angiotensin-II Infusion in an Infant With Autosomal Recessive Polycystic Kidney Disease Postbilateral Nephrectomies and Refractory Hypotension in the Neonatal Period.

Abstract

Autosomal recessive polycystic kidney disease (ARPKD) is a form of hereditary cystic disease with a highly variable phenotypic expression that ultimately leads to chronic kidney disease. Severe cases may warrant surgical intervention with unilateral or bilateral nephrectomy to alleviate thoracic and abdominal compression from massive nephromegaly. Hypotension has been identified as a potential complication following nephrectomy in pediatric patients. We present the case of an infant with end-stage kidney disease secondary to ARPKD who developed refractory hypotension following elective bilateral nephrectomies. We describe the use of angiotensin-II infusion with a significant increase in mean arterial blood pressure and successful reduction in other inotropic and vasopressor support. This case suggests that angiotensin-II may represent another valuable therapeutic agent in the treatment of refractory hypotension in anephric infants.