title: Targeting renin-angiotensin system in malignant hypertension in atypical hemolytic uremic syndrome.
abstract: Hypertension is common in hemolytic uremic syndrome (HUS) and often difficult to control. Local renin-angiotensin activation is believed to be an important part of thrombotic microangiopathy, leading to a vicious cycle of progressive renal injury and intractable hypertension. This has been demonstratedin vitrovia enhanced tissue factor expression on glomerular endothelial cells which is enhanced by angiotensin II. We report two pediatric cases of atypical HUS with severe refractory malignant hypertension, in which we targeted the renin-angiotensin system by using intravenous (IV) enalaprilat, oral aliskiren, and oral enalapril with quick and dramatic response of blood pressure. Both drugs, aliskiren and IV enalaprilat, were effective in controlling hypertension refractory to multiple antihypertensive medications. These appear to be promising alternatives in the treatment of severe atypical HUS-induced hypertension and hypertensive emergency.
authors.affiliation: Pediatric Critical Care Unit, Medanta - The Medicity, Gurgaon, Haryana, India.
authors.forename: V
authors.initials: V
authors.lastname: Raghunathan
authors.affiliation: Kidney Institute, Medanta - The Medicity, Gurgaon, Haryana, India.
authors.forename: S K
authors.initials: SK
authors.lastname: Sethi
authors.affiliation: Department of Immunology, Georges Pompidou European Hospital, APHP, Paris, France.
authors.forename: M A
authors.initials: MA
authors.lastname: Dragon-Durey
authors.affiliation: Pediatric Critical Care Unit, Medanta - The Medicity, Gurgaon, Haryana, India.
authors.forename: M
authors.initials: M
authors.lastname: Dhaliwal
authors.affiliation: Department of Pediatric Nephrology, Akron Children's Hospital, Akron, Ohio, USA.
authors.forename: R
authors.initials: R
authors.lastname: Raina
authors.affiliation: Kidney Institute, Medanta - The Medicity, Gurgaon, Haryana, India.
authors.forename: P
authors.initials: P
authors.lastname: Jha
authors.affiliation: Kidney Institute, Medanta - The Medicity, Gurgaon, Haryana, India.
authors.forename: S B
authors.initials: SB
authors.lastname: Bansal
authors.affiliation: Kidney Institute, Medanta - The Medicity, Gurgaon, Haryana, India.
authors.forename: V
authors.initials: V
authors.lastname: Kher
keywords: Enalapril; enalaprilat; hemolytic uremic syndrome; malignant hypertension; renin