Refractory hypotension after bilateral nephrectomies in a Denys-Drash patient with phenylketonuria.

Amanda B Hassinger, Sudha Garimella

Index: Pediatr. Nephrol. 28(2) , 345-8, (2013)

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Abstract

Denys-Drash (DDS) syndrome is a rare genetic syndrome resulting from a mutation in the Wilms' tumor suppressor gene 1 (WT1), which presents with early onset nephrotic syndrome progressing rapidly to end-stage kidney disease (ESKD), pseudohermaphroditism, and high rates of Wilms' tumor.We present the case of an infant born with DDS and phenylketonuria with neonatal ESKD and dependence on peritoneal dialysis (PD). This patient developed refractory hypotension after elective bilateral nephrectomies at 10 months of age. Despite outpatient management with sodium supplements and changes in PD fluid removal, the patient was hospitalized for refractory post-prandial hypotension with concurrent lactic acidosis. Blood pressure control and feeding tolerance was achieved using intermittent doses of midodrine, an oral alpha-adrenergic agonist.We discuss this case to offer a therapeutic option for the rare occurrence of persistent post-nephrectomy hypotension.