title: Refractory hypotension after bilateral nephrectomies in a Denys-Drash patient with phenylketonuria.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  We discuss this case to offer a therapeutic option for the rare occurrence of persistent post-nephrectomy hypotension.
authors.affiliation: Department of Pediatrics, Division of Critical Care Medicine, Women and Children's Hospital of Buffalo, 219 Bryant Street, Buffalo, NY 14222, USA. ahassinger@upa.chob.edu
authors.forename: Amanda B
authors.initials: AB
authors.lastname: Hassinger
authors.forename: Sudha
authors.initials: S
authors.lastname: Garimella
mesh_terms: D058646:Adrenergic alpha-1 Receptor Agonists / Q000627:therapeutic use; D030321:Denys-Drash Syndrome / Q000150:complications* / Q000601:surgery; D005260:Female; D006801:Humans; D007022:Hypotension / Q000188:drug therapy* / Q000209:etiology*; D007223:Infant; D007231:Infant, Newborn; D007676:Kidney Failure, Chronic / Q000209:etiology; D008879:Midodrine / Q000627:therapeutic use; D009392:Nephrectomy / Q000009:adverse effects*; D010661:Phenylketonurias / Q000150:complications; D019518:Postprandial Period
chemical_list: D058646:Adrenergic alpha-1 Receptor Agonists; D008879:Midodrine