Sickle cell disease (SCD) affects multiple organ systems. Complications of SCD such as pulmonary hypertension (PHT) and sickle cell nephropathy (SCN) are associated with an increased mortality. Both PHT and SCN have some common risk factors. In adults, PHT has been found to be associated with albuminuria, a manifestation of SCN. There is no data on this association in children. We conducted a study to determine if elevated pulmonary artery systolic pressures estimated on echocardiogram was associated with proteinuria in children with SCD.

A detailed retrospective chart review was conducted on a cohort of sickle cell patients screened with echocardiograms from June 2005 to July 2010. Patients who had an initial screening echocardiogram and urine analysis within 1 year were included. Longitudinal data from all subsequent echocardiograms and urine analyses were collected.

Eighty‐five patients were included. On initial echocardiograms 32.9% had an elevated tricuspid regurgitant jet velocity (TRV) ≥2.5 m/second. On follow up, in the 28 patients with elevated TRV, 49.27% of all repeat echocardiograms showed persistent elevation. In the 57 patients with normal baseline TRV, 73.6% of all repeat echocardiograms continued to have normal TRV. On initial screening 7.14% of patients with elevated TRV had proteinuria compared to 1.75% without elevated TRV. On follow up, 19.08% of repeat urinalysis had proteinuria in patients with elevated baseline TRV compared to 12.35% in patients with normal baseline TRV (P = 0.04).

Elevated TRV ≥ 2.5 m/second is significantly associated with proteinuria on longitudinal follow up in children with SCD. Pediatr Blood Cancer 2012; 58: 937–940. © 2011 Wiley Periodicals, Inc.