Peripheral Arterial Tonometry in Assessing Endothelial Dysfunction in Pediatric Sickle Cell Disease

Sivamurthy, Krupa M.; Dampier, Carlton; MacDermott, MaryLou; Meier, Maureen; Cahill, Michele; Hsu, Lewis L.

doi:10.3109/08880010903271689

Cited by 16 publications

(10 citation statements)

References 14 publications

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“…To assess the potential import of associations between vascular measures and functional capacity reported, it is relevant to understand how vascular measures in this Fontan cohort compared with those of adolescents and young adults without Fontan circulation or congenital heart disease who underwent evaluation of vascular health. Interestingly, percentage of FMD was higher in this Fontan cohort (10.2±5.5%) than in many other disease states, including familial hypercholesterolemia (1.2±0.4%), hypertension (4.5±4.0%), and obesity (6.6±2.3%), whereas PAT‐RHI (1.2 [range 0.2–4.8]) and Framingham Heart Study modification of RHI (0.17±0.44) were lower compared with participants with type 2 diabetes mellitus (T2DM; 1.6±0.5), obesity (1.5±0.4), and sickle cell disease (1.6) . It is reasonable to infer from these comparisons that endothelial function might be better preserved in conduit arteries than in the more distal peripheral arteries of Fontan patients.…”

Section: Discussionmentioning

confidence: 57%

“…Interestingly, percentage of FMD was higher in this Fontan cohort (10.2AE5.5%) than in many other disease states, including familial hypercholesterolemia (1.2AE0.4%), hypertension (4.5AE4.0%), and obesity (6.6AE2.3%), whereas PAT-RHI (1. disease (1.6). [28][29][30][31][32][33] It is reasonable to infer from these comparisons that endothelial function might be better preserved in conduit arteries than in the more distal peripheral arteries of Fontan patients. Laser flow Doppler was low in Fontan patients (1539 [range 1119-2213]) compared with both adolescent and young adult controls (2539AE1255) and those with T2DM (1870AE945).…”

Section: Discussionmentioning

confidence: 99%

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Endothelial Function and Arterial Stiffness Relate to Functional Outcomes in Adolescent and Young Adult Fontan Survivors

Goldstein

Urbina

Khoury

et al. 2016

JAHA

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Section: Discussionmentioning

confidence: 57%

Section: Discussionmentioning

confidence: 99%

Endothelial Function and Arterial Stiffness Relate to Functional Outcomes in Adolescent and Young Adult Fontan Survivors

Goldstein

Urbina

Khoury

et al. 2016

JAHA

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“…The correlation between RHI and the age of subjects was reported as not statistically significant (Hamburg et al 2008). Endothelial function by RH-PAT with EndoPAT has also been studied in the pediatric population (Sivamurthy et al 2009).…”

Section: Introductionmentioning

confidence: 99%

Glycosaminoglycan metabolism defects and atherosclerosis: frequent association of endothelial dysfunction in patients with Mucopolysaccharidosis

Yano

Moseley

Wong

et al. 2013

J of Inher Metab Disea

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Cardiovascular lesions, including coronary artery stenosis, are frequently associated and can cause sudden death in patients with genetic defects of glycosaminoglycan (GAG) metabolism. Early diagnosis of coronary artery lesions is difficult, although potentially lifesaving. Histopathological similarities between atherosclerotic changes in adults and in patients with genetic GAG metabolism defects have been known. Atherosclerosis is the result of a complex process involving metabolism of GAGs and proteoglycans preceded by endothelial dysfunction as a key event. Decreased nitric oxide (NO) bioavailability is considered the hallmark of endothelial dysfunction. Reduced NO synthase (NOS) has been reported in atherosclerotic arteries. Impairment in reactive hyperemia-digital peripheral arterial tonometry (RH-PAT) with EndoPAT has been validated to correlate coronary microvascular function in patients with atherosclerosis. RH-PAT is thought to reflect endothelial NO production. Immunohistological staining of endothelial NOS was performed in the stenotic lesions in the coronary artery of a 3-year-old patient with Mucopolysaccharidosis-I, showing decreased activities. This prompted a study to measure endothelial function in patients with GAG metabolism defects for early diagnosis of endothelial dysfunction in the coronary arteries as an early sign of coronary artery changes. Evaluation by RH-PAT in 30 patients with variable genetic defects in GAG metabolism revealed significantly decreased Reactive Hyperemia Indexes compared with 12 controls. Evaluation of endothelial function with RH-PAT in patients with GAG metabolism defects may detect coronary artery lesions that can be underdiagnosed by the other measures such as coronary angiography. Use of this method may prove vital in the management of patients with GAG metabolism defects.

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“…A reduction of flow-mediated dilation or RHI has been reported when hemoglobin is released into plasma, such as in sickle cell disease, 10 malaria, 11 or during hemodialysis 12 . A complementary approach to assess the fate of nitric oxide is by nitric oxide metabolite profiling in blood.…”

Section: Introductionmentioning

confidence: 99%

Transfusion of Stored Autologous Blood Does Not Alter Reactive Hyperemia Index in Healthy Volunteers

Coppadoro

Yu²,

Lei

et al. 2012

Anesthesiology

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Background Transfusion of human blood stored for over 2 weeks is associated with increased mortality and morbidity. During storage, packed erythrocytes progressively release hemoglobin, which avidly binds nitric oxide. We hypothesized that the nitric oxide mediated hyperemic response following ischemia would be reduced after transfusion of packed erythrocytes stored for 40 days. Methods and results We conducted a cross-over randomized interventional study, enrolling 10 healthy adults. Nine volunteers completed the study. Each volunteer received one unit of 40-day and one of 3-day stored autologous leukoreduced packed erythrocytes, on different study days according to a randomization scheme. Blood withdrawal and reactive hyperemia index measurements were performed before and 10 min, 1 h, 2 h, and 4 h after transfusion. The reactive hyperemia index during the first 4 h after transfusion of 40-day as compared to 3-day stored packed erythrocytes was unchanged. Plasma hemoglobin and bilirubin levels were higher after transfusion of 40-day than after 3-day stored packed erythrocytes (p = 0.02, [95% CI difference 10-114mg/l] and 0.001, [95% CI difference 0.6-1.5mg/dl], respectively). Plasma levels of potassium, Lactate dehydrogenase, haptoglobin, cytokines, as well as blood pressure, did not differ between the two transfusions and remained within the normal range. Plasma nitrite concentrations increased after transfusion of 40-day stored packed erythrocytes, but not after transfusion of 3-day stored packed erythrocytes (p = 0.01, [95% CI difference 0.446 - 0.66 μM]). Conclusions Transfusion of autologous packed erythrocytes stored for 40 days is associated with increased hemolysis, an unchanged reactive hyperemia index, and increased levels of plasma nitrite.

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Peripheral Arterial Tonometry in Assessing Endothelial Dysfunction in Pediatric Sickle Cell Disease

Cited by 16 publications

References 14 publications

Endothelial Function and Arterial Stiffness Relate to Functional Outcomes in Adolescent and Young Adult Fontan Survivors

Endothelial Function and Arterial Stiffness Relate to Functional Outcomes in Adolescent and Young Adult Fontan Survivors

Glycosaminoglycan metabolism defects and atherosclerosis: frequent association of endothelial dysfunction in patients with Mucopolysaccharidosis

Transfusion of Stored Autologous Blood Does Not Alter Reactive Hyperemia Index in Healthy Volunteers

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