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.
Case-Diagnosis/Treatment
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.
This is a preview of subscription content, log in via an institution to check access.
References
Niaudet P, Gubler MC (2006) WT1 and glomerular diseases. Pediatr Nephrol 21:1653–1660
Auber F, Jeanpierre C, Denamur E, Jaubert F, Schleiermacher G, Patte C, Cabrol S, Leverger G, Nihoul-Fekete C, Sarnaki S (2009) Management of Wilms tumors in Drash and Frasier syndromes. Pediatr Blood Cancer 52:55–59
van Lieburg AF, Monnens LA (2001) Kidney insufficiency and persistent hypotension following compromise of the renin-angiotensin system in a fetus and a young infant. Ned Tijdschr Geneeskd 145:695–698
Masutani S, Senzaki H, Ishido H, Taketazu M, Matsunaga T, Kobayashi T, Sasaki N, Asano H, Kyo S, Yokote Y (2005) Vasopressin in the treatment of vasodilatory shock in children. Pediatr Int 47:132–136
Chen L, Wang L, Sun J, Qin J, Tang C, Jin H, Du J (2011) Midodrine hydrochloride is effective in the treatment of children with postural orthostatic tachycardia syndrome. Circ J 75:927–931
Nawaz A, Mohammed I, Ashan K, Karakurum A, Hadjiyane C, Pellecchia A (1998) Clostridium difficile colitis associated with treatment of Helicobacter pylori infection. Am J Gastroenterol 93:1175–1176
Shupakova AN (2006) Chronic abdominal ischemia—relation to Helicobacter infection. Kardiologiia 46:65–66
Lapeyraque AL, Haddad E, Andre JL, Bremond-Gignac D, Taylor M, Rianthavorn P, Salusky IB, Loirat C (2003) Sudden blindness caused by anterior ischemic optic neuropathy in 5 children on continuous peritoneal dialysis. Am J Kidney Dis 42:E3–E9
Hijazi R, Abitbol CL, Chandar J, Seeherunvong W, Freundlich M, Zilleruelo G (2009) Twenty-five years of infant dialysis: a single center experience. J Pediatr 155:111–117
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Hassinger, A.B., Garimella, S. Refractory hypotension after bilateral nephrectomies in a Denys–Drash patient with phenylketonuria. Pediatr Nephrol 28, 345–348 (2013). https://doi.org/10.1007/s00467-012-2311-9
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00467-012-2311-9