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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Abnormalities of the cardiovascular system are common in patients with sickle cell anemia (SS). Noninvasive testing to document left ventricular dysfunction has yielded conflicting results. Left ventricular performance was evaluated in 27 patients with SS by M-mode and 2-D echocardiography, and systolic time intervals. Comparisons were made to 25 normal controls, and to 22 patients with chronic aortic regurgitation. Left ventricular diastolic diameter (LVDD) and cardiac index (CI) were significantly greater in the patients with SS than in controls (LVDD 5.3 +/- 0.4 vs. 4.7 +/- 0.5 cm; CI 4.2 +/- 1.3 vs. 3.1 +/- 0.8 liters/min/m2; both p less than 0.001). Left ventricular ejection fraction (EF) was slightly, but significantly less (62.9 +/- 7.3 vs. 67.0 +/- 5.4; p less than 0.05). In comparison to the patients with chronic aortic regurgitation, the LVDD in the patients with SS was slightly, but significantly lower (LVDD 5.3 +/- 0.4 vs. 5.9 +/- 0.6 cm; p less than 0.05). There was no significant difference between the patients in EF or CI (EF 62.9 +/- 7.3 vs. 63.3 +/- 4.4; CI 4.2 +/- 1.3 vs. 5.0 +/- 1.0 liters/min/m2; NS). Left ventricular EF was below 55 in three patients who also had hypertension at the time of examination. We conclude that patients with SS have resting left ventricular performance consistent with a high output state. Significant left ventricular dysfunction related to sickle cell disease alone was not demonstrated in this population, although the addition of hypertension appears to deleteriously affect resting left ventricular performance.
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PMID:A noninvasive comparison of left ventricular performance in sickle cell anemia and chronic aortic regurgitation. 664 Oct 39

A 20-year-old black man with known sickle cell anemia was admitted to the hospital with the signs and symptoms of right middle lobe pneumonia. Because of a hemoglobin value of 3.9 g/dL, he received a transfusion of 3 units of packed erythrocytes. Seven days later, hypertension, headache, and one grand mal seizure occurred. Evaluation of his condition gave normal findings. Similar events, some terminating in cerebral hemorrhage and death, have been reported in patients with hemolytic anemia after they received multiple transfusions. To our knowledge, this patient represents the first adult with sickle cell anemia who had this syndrome. Prompt antihypertensive therapy may have been of benefit.
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PMID:Hypertension and a seizure following transfusion in an adult with sickle cell anemia. 670 31

Three of five siblings developed a steroid-resistant nephrotic syndrome with focal segmental glomerulosclerosis within a four-month period. Two of the siblings with nephrotic syndrome (Patients 1 and 2) also have sickle cell anemia; the third (Patient 3) carries the thalassemia trait. The dizygotic twin brother of Patient 2 has sickle cell anemia, but does not have the nephrotic syndrome. The nephrotic syndrome of patient 1 was resistant to corticosteroid and cyclophosphamide therapy and she developed severe renal failure 14 months after onset. The nephrotic syndrome of Patients 2 and 3 was steroid resistant but was partially responsive to cyclophosphamide therapy. They have persistent proteinuria with mild elevation of serum creatinine concentration and hypertension 5 1/2 years after diagnosis. In this family, the nephrotic syndrome appeared unrelated to the specific hemoglobinopathy, HLA type or mixed lymphocyte culture responsiveness despite the similarity of the renal disease.
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PMID:Familial nephrotic syndrome and focal segmental glomerulosclerosis. 719 18

Arterial blood pressures (BPs) in 187 adult patients with sickle cell disease, casually recorded during hospitalization or clinic visits, were compared with BPs from age- and sex-matched populations of black Americans. The BPs in those with sickle cell disease were significantly lower than those in the control populations in all ages and did not demonstrate the expected rise with advancing age. In these patients, there was no difference between BP and sex, degree of anemia, or hemoglobin genotype. Four patients had diastolic and two had systolic hypertension. The prevalence of hypertension was significantly less than that in the block population. These BP findings in sickle cell disease may be due to the renal tubular defect responsible for increased sodium and water excretion, which may blunt the plasma volume expansion necessary for sustained hypertension and thus promote lower arterial pressures, similar to that situation observed in patients with salt-losing nephritis.
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PMID:Arterial blood pressure in adults with sickle cell disease. 723 8

Choosing the proper method of contraception for patients with sickle cell disease is an empiric exercise. In this manuscript, the commonly used contraceptive methods are examined for their risk-benefit ratio. However, because of its extensive use and effectiveness, steroid contraception is examined in greatest depth. Physiologic and pathologic changes induced by oral steroid contraceptives are evaluated, and their potential effects on patients with sickle cell disease are considered. Physiologic alterations evaluated are carbohydrate metabolism, blood lipid levels, hepatic function, thyroid function, and pigment changes. Pathologic changes reviewed are hypertension, thromboembolic phenomena, myocardial infarction, amenorrhea, and emotional changes. The choice of sterilization procedures is also considered for these patients.
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PMID:Contraceptives in sickle cell disease. 724 8

Long-term study of 21 PNH patients revealed an unexpectedly high incidence of functional and anatomic renal abnormalities. Most patients demonstrated varying degrees of hematuria and proteinuria distinct from hemoglobinuria. Evaluation of renal function revealed hyposthenuria, abnormal tubular function, and declining creatinine clearance. Radiologically these patients had enlarged kidneys, cortical infarcts, cortical thinning, and papillary necrosis which were confirmed by autopsy studies. Hypertension developed in eight patients. Urinary tract infection was uncommon. The renal findings bear striking similarity to those of sickle cell anemia. Contrary to the usual opinion, out studies clearly showed evidence of widespread renal pathology in PNH most likely due to repeated microvascular thrombosis similar to the venous thrombosis involving other organs in this disorder.
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PMID:The kidneys in paroxysmal nocturnal hemoglobinuria. 744 17

The acute chest syndrome is a clinical entity appearing in patients suffering from sickle cell anaemia. It presents with pleuritic pain, fever, leucocytosis and pulmonary infiltrates in the thoracic radiology. The etiological diagnosis is difficult, and it is necessary to distinguish between pneumonia and pulmonary infarction. This syndrome is quite frequent among the patients at risk, and can be lethal according to the severity and the etiology of the event. A case of acute chest syndrome due to a S. pneumoniae sepsis is presented. The interest of the case lies in the rareness of this disease in our population and the peculiar evolutive clinical features of this case, with the development of intracranial hypertension and death.
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PMID:[Acute thoracic syndrome]. 798 60

Coronary artery disease is the leading cause of death in the United States. Serum cholesterol is a widely used screening test to detect persons at high risk for coronary artery disease, including those with familial hypercholesterolemia. However, universal screening of currently healthy persons is not without risk. Previous experience in screening for sickle cell anemia and hypertension has shown that these risks include misunderstanding of test results, misdiagnosis, labeling, stigmatization, and decreased psychological well-being. Results of screening programs may be misused by industry or insurance companies to exclude individuals from positions or benefits. Consideration of these harms suggests that screening should not be implemented until certain safeguards are in place. Physicians and the public should be educated about the potential risks and benefits of screening. Screening tests should be accurate, reliable, valid, and of demonstrated sensitivity. Informed consent for screening should be obtained. Follow-up surveillance and recommended treatments, including dietary counseling and drug therapy, should be available to all individuals identified as being at high risk regardless of their socioeconomic status. Finally, procedures to protect the right to privacy of individuals and their families should be implemented well in advance of the actual screening.
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PMID:Psychological and ethical considerations in screening for disease. 821

The case histories of two patients with sickle cell disease and gram negative sepsis complicated by encephalopathy and hypertension is presented. The first patient had 2 episodes of "hypertensive encephalopathy" before control of her blood pressure was achieved while the second patient had only one. The occurrence, though apparently rare, can have serious implications. Possible mechanisms are discussed and the need to monitor the blood pressure of children with sickle cell disease is stressed.
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PMID:Acute encephalopathy, hypertension and gram negative sepsis in sickle cell disease. 831 20

Supine arterial blood pressure measurements of 89 patients with homozygous sickle cell disease and normal renal function were compared with those of an age-, race-, and sex-matched normal population and with those of individuals who had similar levels of anemia due to beta thalassemia. Consistent with previous reports, sickle cell patients had significantly lower blood pressure than the normal population. However, within most age groups, sickle cell patients tended to have higher than expected blood pressure than individuals with similar or less severe degrees of anemia. Furthermore, the authors have found an association between cerebrovascular accident and elevated blood pressure in men, even in a range of systolic and diastolic pressures that would be considered normal by conventional standards. These results reiterate the intricate relationship that exists between factors governing red cell rheology and microvascular tone. They also raise the possibility that "relative" hypertension may be associated with other vaso-occlusive manifestations of the sickle cell syndromes.
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PMID:Is "relative" hypertension a risk factor for vaso-occlusive complications in sickle cell disease? 844 34


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