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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Although microvascular occlusion has been considered a basis for pathophysiology of the myocardium during the crisis of sickle cell anemia, the status of the left ventricle in uncertain. To determine if left ventricular performance is affected by crisis, 11 patients were evaluated noninvasively by the systolic time interval method on the first day of crisis and serially until recovery. There were no significant differences in the time intervals over this period. In addition, since the serum CPK-MB isoenzyme was not elevated during crisis and evidence of acute injury was not present on ECG, myocardial necrosis appeared unlikely. Four patients on subsequent admission exhibited systolic time interval values similar to the earlier crisis. To determine if there were chronic changes in cardiac function, subjects with sickle cell hemoglobin were studied between crises. Those under 23 years of age were not dissimilar from a group of normals and a group of patients with chronic blood loss anemia A significant abnormality of the PEP/LVET ratio was observed in subjects over 23 years of age. Similar observations were made on echocardiography, with subjects over the age of 23 demonstrating an abnormal ejection fraction compared to the younger group, despite enhanced end-diastolic diameter. Thus, it is suggested that the chronic hemolytic process in subjects with sickle cell anemia may effect cumulative myocardial alterations, resulting in chronic cardiac malfunction in the apparent absence of acute ischemia during crises.
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PMID:Left ventricular performance during and after sickle cell crisis. 43 33

Recurrences of CNS infarction often lead to progressive neurologic disability in sickle cell anemia. To prevent such reccurrence, a periodic blood transfusion program was begun in 1969. Currently, 27 patients are on this regimen. Before inclusion in the program, 12 patients had had one to nine CNS recurrences each. Since the program was started, two patients have had transient CNS ischemia. There were no other recurrences and none of the patients have shown progression of neurologic abnormalities. In addition, there was a striking decrease in bacterial infection and pain. We conclude that periodic transfusions are effective in preventing recurrent CNS infarction in sickle cell anemia. The benefits must be weighed against the potentially serious problem of iron overload, as evidenced by moderately elevated serum ferritin values.
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PMID:Periodic transfusions for sickle cell anemia and CNS infarction. 51 76

Sickle-cell anemia may give heart troubles: heart insufficiency by chronic anemia, pseudo-rhumatic heart, chronic pulmonary heart, coronarian ischemia and cardiomyopathic syndrome. The homo or heterozygotic status and the age are important factors. A rather clear pathogenesis has been built up for these various troubles but therapy has only a limited efficiency.
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PMID:[Heart in sickle-cell anemia (author's transl)]. 72 64

A 30-year-old black woman with biopsy-proven sarcoidosis and sickle cell anemia had peripheral retinal neovascularization. Elevated fibrovascular proliferations and distal retinal ischemia with nonperfused ghost vessels passing through the area of neovascularization were found at the temporal periphery. No signs of intraocular inflammation were present. Both diseases occur in black patients and should always be considered in the presence of peripheral retinal neovascularization.
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PMID:Peripheral retinal neovascularization in sarcoidosis and sickle cell anemia. 84 45

Localized proton nuclear magnetic resonance spectroscopy (MRS), obtained with stimulated echo and spin echo sequences, MR imaging (MRI) and MR angiography (MRA) were used to study the brain in 13 children and adolescents with sickle cell disease. Regions of interest (ROI) studied by MRS included regions appearing normal on MRI as well as regions showing complications of sickle cell disease, including focal deep white matter areas of high signal intensity (deep white matter ischemia, DWMI) seen on long TR images, focal atrophic brain areas, and infarcts. The findings in these studies are summarized as follows: Normal-appearing regions on MRI have normal MRS. In ROI including small areas of DWMI, lactate elevation was not detected, but the levels of N-acetyl-aspartate (NAA) appeared slightly elevated. In areas of DWMI 1-2 cm in size, reduced blood flow could be seen on MRA and lactate elevation could be detected with MRS. When blood flow to a DWMI region was normal, NAA was reduced and there was little lactate elevation, as cell death had already occurred. ROI consisting of atrophic tissue had reduced NAA levels but total creatine levels were not changed. Sometimes lipids, presumably from broken cell membrane, could be detected. In regions of past massive stroke, all metabolites were absent except for small amounts of lactate or lipids.
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PMID:Investigation of stroke in sickle cell disease by 1H nuclear magnetic resonance spectroscopy. 128 41

Liver changes in patients with sickle cell anemia, for some authors, is a common finding and is hot in relation with the severity of the anemia. The grade of liner disfunction or malfunction is related with ischemia and, there exists probable, slowing of intrahepatic circulation secondary to sinusoidal obstruction due to masses of sickle cells and to the hypertrophy of Kupffer cells. In this paper, clinical morphologic and ultrastructural findings of 21 cases of SS and SA, hemoglobinopathies are presented. Sixty percent were females and forty percent were males with ages between 18 and 46 years. The most frequent microscopic findings were sinusoid distention followed by hypertrophy of Kupffer cells and inflammation. Areas of necrosis, fatty changes and iron deposits were also seen.
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PMID:[Sickle-cell anemia. The liver lesions. A clinical, morphological and ultrastructural study of 21 cases]. 134 Aug 23

We tested retinal vessel autoregulation in 16 sickle cell patients and 6 controls by computer-assisted measurements of constriction of the superior and inferior temporal veins and arteries after pure oxygen breathing. Compared to the controls and corrected for age, we found a decrease of vasoconstriction in three of the four vessels measured in the sickle cell group, which was statistically significant only for the superior temporal artery (P 0.009). The number of patients was too small to allow a separate analysis of possible contributory factors within the sickle cell group, such as sickle cell hemoglobin subtype and extent of retinal ischemia. Our findings indicate a factor not yet well known in the pathophysiology of sickle cell disease: an abnormality of local microvascular control.
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PMID:Retinal vessel autoregulation in sickle cell patients. 152 10

Advances in imaging hardware for positron emission tomography and single-photon emission CT, coupled with a wide variety of radiopharmaceutical agents, have allowed these techniques to be used in the evaluation of neoplasm, stroke, epilepsy, and dementia. Cerebral perfusion agents continue to be the mainstay of single-photon emission CT imaging but, in addition to the evaluation of ischemia, it has seen an increasing role in the study of dementia, neuropsychiatric disorders, and seizures. Positron emission tomography scanning has had similar applications but it is playing a greater part in the evaluation of neoplasms, including primary gliomas and pituitary adenomas. Stable-xenon CT has shown value in the study of ischemia associated with meningitis, sickle cell disease, chronic subdural hematomas, and cerebral arteriovenous malformations. MR diffusion imaging shows promise in the evaluation of white matter pathology and some tumors.
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PMID:Physiologic imaging of the brain. 173 6

A patient with long-standing bilateral circumferential lower extremity sickle cell ulcerations refractory to conservative management was successfully treated with bilateral free latissimus muscle transfers. This report confirms the value of free tissue transfer in the treatment of these difficult skin ulcerations. Exchange transfusions that brought the SS hemoglobin below 30% were crucial to the prevention of sickling in the microcirculation of the flap during its obligate period of ischemia. Furthermore, they protected the flap during a period of ischemia that exceeded 4 hours following a postoperative arterial thrombosis. In the presence of severe thrombocytosis associated with sickle cell disease, prophylactic treatment with aspirin may be of significant value.
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PMID:Bilateral lower limb salvage with free flaps in a patient with sickle cell ulcers. 179 45

Much of the morbidity and mortality in sickle cell disease (SCD) is caused by tissue ischemia and infarction resulting from vascular occlusion. Research in this area has been dominated by the hypothesis that vascular occlusion in SCD is due primarily to microvascular obstruction by sickle erythrocytes (SS RBC), yet there is no direct evidence that microvascular occlusion is responsible for any of the vasocclusive complications of SCD. In this paper an alternate hypothesis is proposed: that thrombotic occlusion of larger arteries and veins is an important factor in many of the vasocclusive complications of SCD. Large-vessel cerebral arterial disease (intimal hyperplasia with superimposed thrombosis) has clearly been established as the most important cause of stroke in SCD, and considerable evidence suggests that pulmonary arterial thrombosis/embolism is a major cause of pulmonary infarction and hypertension. The involvement of large-vessel thrombosis in painful crisis, aseptic necrosis of bone, priapism, leg ulcers, retinopathy, and miscarriage has not been adequately investigated. Large-vessel occlusion in SCD is probably a consequence of the abnormal adhesive and procoagulant properties of SS RBC, which produce endothelial damage, secondary intimal proliferation, and thrombosis. Techniques currently used to treat large-vessel occlusion in other disorders (antiplatelet and anticoagulant agents, thrombolytic therapy, angioplasty, endarterectomy, and vascular bypass surgery) should be considered in sickle cell subjects with large-vessel occlusion, especially in the cerebral vasculature.
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PMID:Large-vessel occlusion in sickle cell disease: pathogenesis, clinical consequences, and therapeutic implications. 189 Sep 82


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