Endothelin-1 Antagonist in the Management of Refractory Systemic Hypertension in Children with End Stage Kidney Disease: A Case Series

Uncontrolled hypertension is a significant problem in children with end stage kidney disease. We studied 10 children on chronic hemodialysis who received the endothelin-1 receptor antagonist, bosentan as adjunctive therapy in refractory systemic hypertension. Data were retrospectively analyzed on systolic (SBP) and diastolic blood pressure (DBP), antihypertensive medications and hospitalization days per patient per year, at baseline and 3 months after initiation of bosentan. The standard deviation (Z) score of BP for age, height and gender was calculated. Mean age and weight of these children were 11.4 ± 4.8 years (range 1 - 17.5 years) and 32.9 ± 14.4 kg (range 7.4 - 57 kg) respectively and 90% were African American. Seven were on hemodialysis ≥4 times weekly. The average inter-dialytic weight gain was 4.7% ± 1.8%. The pre-dialysis SBP and DBP Z scores were 4.9 ± 1.8 and 4.0 ± 1.0 at baseline and decreased to 1.3 ± 0.4 and 1.3 ± 0.8, 3 months after initiation of bosentan (p < 0.0001). Similarly, anti-hypertensive medications were decreased from 5 ± 1.5 to 2.1 ± 2.6 (p < 0.01). Hospitalization days decreased from 35.6 ± 52.4 days to 1.5 ± 3.3 days (p < 0.0001). This preliminary report indicates bosentan may be beneficial in the management of children with severe refractory hypertension.