Arterial Blood Pressure in Adults With Sickle Cell Disease

Johnson, Cage S.; Giorgio, Anthony J.

doi:10.1001/archinte.1981.00340070071015

Cited by 78 publications

(17 citation statements)

References 13 publications

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“…3,40,41 In contrast to an earlier report, 42 we found intact baroreflex cardiovascular control and no signs of cardiovascular autonomic dysfunction in the SCD group. Thus, integrity of cardiovascular control contrasts to impairment of dynamic cerebrovascular control in patients with SCD.…”

Section: Discussioncontrasting

confidence: 99%

Dynamic Cerebral Autoregulation in Homozygous Sickle Cell Disease

Kim

Nur²,

Beers³

et al. 2009

Stroke

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Background and Purpose-Sickle cell disease (SCD) is associated with cerebral hyperperfusion and an increased risk of stroke. Also, both recurrent microvascular obstruction and chronic hemolysis affect endothelial function, potentially interfering with systemic and cerebral blood flow control. We addressed the question whether cerebrovascular control in patients with SCD is affected and related to hemolysis. Methods-Systemic and cerebrovascular control were studied in 18 patients with SCD and 10 healthy subjects. Dynamic cerebral autoregulation was evaluated by transfer function analysis assessing the relationship between mean cerebral blood flow velocity and mean arterial pressure. Results-Normal baroreflex sensitivity and postural cardiovascular reflex responses indicated integrity of systemic cardiovascular control. In the low-(0.07 to 0.15 Hz) frequency region, mean arterial pressure variability was comparable for both groups, but a larger mean cerebral blood flow velocity variability in SCD (6.

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Section: Discussioncontrasting

confidence: 99%

Dynamic Cerebral Autoregulation in Homozygous Sickle Cell Disease

Kim

Nur²,

Beers³

et al. 2009

Stroke

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“…We found that systemic BP levels in patients with SCA are lower than that of African American control subjects, as reported previously (17,18). The novel finding of our study is that systemic BP does not increase in a majority of patients with SS when they develop proteinuria or renal insufficiency.…”

Section: Discussionsupporting

confidence: 85%

Glomerular Involvement in Adults with Sickle Cell Hemoglobinopathies

Guasch

Navarrete

Nass

et al. 2006

Journal of the American Society of Nephrology

228

207

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Patients with sickle cell anemia (SCA) may develop a glomerulopathy with proteinuria and progressive renal insufficiency, leading to ESRD. Albuminuria is a sensitive marker of glomerular damage in this population and precedes the development of renal insufficiency. For determination of the prevalence of glomerular damage in SCA and the clinical correlates of renal insufficiency, 300 adult patients with SCA were studied (hemoglobin SS ‫؍‬ 184; and 116 with other sickling hemoglobinopathies: SC, SD, and S-␤ thalassemia); albumin excretion rates (AER) and renal function (Cockroft-Gault formula) were determined, and clinical and hematologic evaluations were conducted. In hemoglobin SS disease, increased AER (micro-and macroalbuminuria) occurred in 68% of adult patients, and macroalbuminuria occurred in 26%. In other sickling disorders, increased AER occurs in 32% of adults, and macroalbuminuria occurs in 10%. The development of graded albuminuria was age dependent, so at 40 yr, 40% of patients with SS disease had macroalbuminuria. There were no differences in hematologic parameters (hemoglobin levels, white blood cell count, percentage of reticulocytes, platelet counts, or lactate dehydrogenase levels) between patients with normoalbuminuria and those with micro-or macroalbuminuria. By multivariate analysis, albuminuria correlated with age and serum creatinine in SS disease but not with BP or hemoglobin levels. In other sickling disorders, albuminuria tended to be associated with age but not with hemoglobin or BP levels. The diastolic BP was lower in patients with SCA than in African American control subjects, and the development of renal insufficiency, which was present in 21% of adults with SS disease, was not accompanied by significant hypertension. It is concluded that glomerular damage in adults with SCA is very common, and a majority of patients with SS disease are at risk for the development of progressive renal failure. The development of micro-and macroalbuminuria is not related to the degree of anemia, suggesting that sickle cell glomerulopathy is not solely related to hemodynamic adaptations to chronic anemia. In contrast to other glomerulopathies, the development of systemic hypertension is uncommon in SS disease with renal insufficiency.

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“…It is unlikely to be secondary to hypertensive effects; there were no difference in the blood pressures between younger and older patients. This is in agreement with previous data that have shown that blood pressures in patients with sickle cell disease did not demonstrate the expected rise in advancing age [20]. Because cardiomegaly in our study was assessed by X ray and not by echocardiography, it is not clear how many of these represent left ventricular failure with decreased ejection fractions.…”

Section: Cardiomegalysupporting

confidence: 91%

The older sickle cell patient

2004

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The lifespan of sickle cell patients has increased substantially. We chose to compare the attributes of older sickle cell patients with younger ones within the confines of a single institutional study serving the local Bronx population. Laboratory and clinical assessments from 40 patients with sickle cell disease over 40 years of age were compared to 40 patients under 30 years of age. When the older group was compared to the younger group, hemoglobin, indirect bilirubin, and platelet counts were significantly lower. Creatinine clearances were lower and BUN levels were higher in the older group. Although the prevalence of radiographically measured cardiomegaly was significantly higher in the older group, systolic and diastolic blood pressures remained unchanged. The number of crises and number of admissions were higher in the younger group than in the older, but there was wide variation, and no significant differences could be detected; days spent in hospital over the previous 2 years did not differ. No statistically significant differences were observed for absolute reticulocyte count, MCV, % HbF, white cell count, liver function tests, or oxygen saturation. Comparing genders within age groups, younger males had higher hemoglobin levels and absolute reticulocyte counts than younger females, while only the older females had higher HbF levels than the corresponding males. Lower levels of hemoglobin associated with older age may reflect decreased hematopoietic potential that may in part be due to decreased renal function. Lower platelet counts in older sickle cell patients may be a consequence of this decreased hematopoietic potential but may also represent a secondary survival benefit effect in sickle cell anemia. Am.

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Arterial Blood Pressure in Adults With Sickle Cell Disease

Cited by 78 publications

References 13 publications

Dynamic Cerebral Autoregulation in Homozygous Sickle Cell Disease

Dynamic Cerebral Autoregulation in Homozygous Sickle Cell Disease

Glomerular Involvement in Adults with Sickle Cell Hemoglobinopathies

The older sickle cell patient

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