Doppler ultrasonographic evaluation of celiac and mesenteric arteries in subjects with sickle cell disease.

Vasculopathy, as occurring in sickle cell disease (SCD), can affect celiac and mesenteric arteries and result in stenosis, with elevated peak systolic velocity (PSV) on Doppler ultrasonography. In six subjects with confirmed SCD in steady state, routine Doppler ultrasonographic examination discovered features of celiac artery (CA) or superior mesenteric artery (SMA) stenosis with CA PSV >200 cm/s (median = 222.8 cm/s; range = 201.5-427.1 cm/s) and/or SMA PSV >275 cm/s (median 183.2 cm/s; range = 87.8-289.3 cm/s). Among the six subjects, five had elevated soluble P-selectin values (median 72.55 ng/mL), while all six (100%) had elevated cystatin C levels (median 4.15 mg/L). Peripheral oxygen saturation was suboptimal in five subjects. All subjects had low hemoglobin concentration levels (median 8.5 g/dL) while four had elevated white blood cell count. Although vaso-occlusive crises result from microvessel occlusion, these findings at the macrovascular level suggest that SCD patients may also be vulnerable to mesenteric ischemic injury, especially in the setting of anemic heart failure from hemolysis.

Intima-media thickness of the common femoral artery as a marker of leg ulceration in sickle cell disease patients.

Leg ulceration is a debilitating chronic complication of sickle cell disease (SCD) the pathogenesis of which is yet to be fully elucidated. We hypothesized that SCD patients with histories of previous leg ulcers would have intima hyperplasia of the common femoral artery (CFA). We enrolled 44 SCD patients and 33 age-matched and sex-matched controls with hemoglobin AA. Anthropometric measurements, biochemical parameters, and sonographic intima-media thickness (IMT) of the CFA were determined. The median CFA IMT in SCD limbs with history of leg ulcers (SWLU) was 1.0 mm, whereas it was 0.7 mm in SCD limbs with no history of leg ulcer (SNLU) and 0.60 mm in controls (P < .001). Among the SNLU, 70.3% had CFA IMT <0.9 mm, whereas only 29.7% had CFA IMT ≥0.9 mm. Conversely, only 20.8% of SWLU had CFA IMT <0.9 mm, whereas the remaining 79.2% had CFA IMT ≥0.9 mm. All the controls had CFA IMT <0.9 mm. Binary logistic regression to determine the odds of having leg ulcer among SCD limbs with CFA IMT of ≥0.9 mm yielded an odds ratio of 9, indicating that SCD limbs with CFA IMT ≥0.9 mm had a 9 times greater risk of having leg ulcer compared with those with CFA IMT <0.9 mm. There is a significant increase in the CFA IMT of SCD limbs with ulcer compared with controls and SCD limbs without ulcer, suggesting that arterial vasculopathy plays a major role in the formation of these ulcers.