Low Blood Pressure, Decreased Incidence of Hypertension, and Renal Cardiac, and Autonomic Nervous System Functions in Patients with Sickle Cell Syndromes

Karayaylalı, İbrahim; Önal, Münif; Yildizer, Kasım; Seyrek, Neslihan; Paydaş, Saime; Akoğlu, Emel; Gürçay, Ali A.; Birand, Ahmet; Sağlıker, Yahya

doi:10.1159/000064306

Cited by 7 publications

(7 citation statements)

References 12 publications

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“…Further examination of this phenomenon in a large group of adolescents in Jamaica [15], showed that the blood pressure effect was present in patients younger than age 20 who had sickle cell anemia but not sickle C disease, and that the blood pressure difference could be explained by differences in weight. Similar findings have been reported from Turkey and Nigeria [16,17]. The coinheritance of α-thalassemia with sickle cell disease appears to reduce blood pressure in sickle cell disease, as well as possibly protect against proteinuria.…”

Section: Blood Pressuresupporting

confidence: 87%

Arterial Blood Pressure and Hyperviscosity in Sickle Cell Disease

Johnson¹

2005

Hematology/Oncology Clinics of North America

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Section: Blood Pressuresupporting

confidence: 87%

Arterial Blood Pressure and Hyperviscosity in Sickle Cell Disease

Johnson¹

2005

Hematology/Oncology Clinics of North America

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“…Impaired NO bioavailability in this disorder is demonstrated by the blunted response to endothelium-dependent vasodilators in sickle cell mouse models [104, 105], as well as by a reduced flow-mediated vasodilation in human patients with SCD [102, 106, 107]. NO metabolites are elevated in patients with SCD at steady-state compared to normal controls [108], while peripheral vascular resistance [109] and resting blood pressures are low [110, 111]. Adding to this paradox, a “relative hypertension” occurs in SCD as blood pressures, while lower than in normal subjects, are higher in some patients [112, 113].…”

Section: Nitric Oxide Scavenging In Sickle Cell Diseasementioning

confidence: 99%

Nitric Oxide and Arginine Dysregulation: A Novel Pathway to Pulmonary Hypertension in Hemolytic Disorders

Morris¹,

Gladwin²,

Kato³

2008

CMM

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Secondary pulmonary hypertension (PH) is emerging as one of the leading causes of mortality and morbidity in patients with hemolytic anemias such as sickle cell disease (SCD) and thalassemia. Impaired nitric oxide (NO) bioavailability represents the central feature of endothelial dysfunction, and is a major factor in the pathophysiology of PH. Inactivation of NO correlates with hemolytic rate and is associated with the erythrocyte release of cell-free hemoglobin, which consumes NO directly, and the simultaneous release of the arginine-metabolizing enzyme arginase, which limits bioavailability of the NO synthase substrate arginine during the process of intravascular hemolysis. Rapid consumption of NO is accelerated by oxygen radicals that exists in both SCD and thalassemia. A dysregulation of arginine metabolism contributes to endothelial dysfunction and PH in SCD, and is strongly associated with prospective patient mortality. The central mechanism responsible for this metabolic disorder is enhanced arginine turnover, occurring secondary to enhanced plasma arginase activity. This is consistent with a growing appreciation of the role of excessive arginase activity in human diseases, including asthma and pulmonary arterial hypertension. New treatments aimed at improving arginine and NO bioavailability through arginase inhibition, suppression of hemolytic rate, oral arginine supplementation, or use of NO donors represent potential therapeutic strategies for this common pulmonary complication of hemolytic disorders.

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“…The number of patients with PEM in our sample was small, which reduces analytical power. Several studies (6)(7)(8)(9)(10) with adults found that SCD is associated with lower BP values and lower incidence of HT than among the general population, which may be a beneficial factor, because HT is a risk factor for sickling crises, pulmonary hypertension and strokes (11) .…”

Section: Discussionmentioning

confidence: 99%

“…About one third of all adolescents and young adults with SCD have nephropathies and renal abnormalities, such as impaired urinary concentration ability, defects in urinary acidification and potassium excretion, as well as glomerular disorders, such as glomerular hyperfiltration and proteinuria (3)(4)(5) Hypertension (HT), although frequent in African ethnicities, has not been frequently diagnosed in patients with SCD (3) . Studies with adults with SCD showed that they had a lower incidence of HT and lower blood pressure (BP) values than patients without hemoglobinopathy S, but these findings have not been confirmed in pediatric populations (6)(7)(8)(9)(10) . Gordeuk et al (11) found that patients with SCD have a higher risk of pulmonary hypertension and renal failure when their blood pressure was elevated.…”

Section: Introductionmentioning

confidence: 99%

Pressão arterial em crianças portadoras de doença falciforme

Hsien

Carvalhaes

Braga

2012

Rev. paul. pediatr.

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OBJETIVO: Avaliar os valores da pressão arterial (PA) em crianças portadoras de doença falciforme (DF). MÉTODOS: Estudo observacional unicêntrico descritivo de 70 crianças portadoras de DF. Os valores da PA obtidos foram classificados conforme as V Diretrizes Brasileiras de Hipertensão Arterial. Os pacientes foram distribuídos segundo o genótipo em grupo HbSS e HbSC e segundo a faixa etária: grupo I (três anos a quatro anos e 11 meses), grupo II (cinco anos a oito anos e 11 meses) e grupo III (nove anos a 13 anos e 11 meses). Na análise estatística, aplicou-se o teste t de Student e a ANOVA, sendo significante p<0,05. RESULTADOS: A média e o desvio padrão (DP) das medidas da PA sistólica (PAS) (mmHg) foram 95,9±11,45 e da PA diastólica (PAD) 62,6±7,78. As médias da PA por faixa etária foram: grupo I, PAS 91,2±5,78 e PAD 61,5±7,15; grupo II, PAS 97,3±10,86 e PAD 64,4±7,89; e grupo III, PAS 100,0±9,88 e PAD 61,5±4,94. Observou-se que 5,7% dos pacientes apresentavam hipertensão arterial (HA) e 8,6% eram pré-hipertensos. A média dos valores da PAS e PAD entre os pacientes HbSS e HbSC não diferiu. CONCLUSÕES: Novos estudos devem ser realizados para avaliar PA em pacientes com DF e detectar as possíveis causas de HA nesses pacientes.

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Low Blood Pressure, Decreased Incidence of Hypertension, and Renal Cardiac, and Autonomic Nervous System Functions in Patients with Sickle Cell Syndromes

Cited by 7 publications

References 12 publications

Arterial Blood Pressure and Hyperviscosity in Sickle Cell Disease

Arterial Blood Pressure and Hyperviscosity in Sickle Cell Disease

Nitric Oxide and Arginine Dysregulation: A Novel Pathway to Pulmonary Hypertension in Hemolytic Disorders

Pressão arterial em crianças portadoras de doença falciforme

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