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

A 7 year-old girl, with homozygous sickle cell disease experienced two cerebrovascular strokes over an 8-month period. The diagnostic investigations were performed with highly sensitive imaging methods (MRI, angiography). Such a complication raises problems in curative and prophylactic treatment. New diagnostic methods are being evaluated.
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PMID:[Cerebrovascular accidents and sickle cell disease]. 133 31

Twenty-four patients presenting an acute stroke with watershed cerebral infarct on CT scan or MRI were included in this retrospective study. Age was 63 +/- 14 years (mean +/- SD), and sex ratio was 2 men for 1 woman. Main clinical features were: in anterior location, lower limb weakness and frontal syndrome with transcortical motor aphasia in left lesions or spatial dyscalculia in right ones; in posterior location, brachiofacial weakness with constant quadranopsia and hypoesthesia, and Gerstmann syndrome in left lesion. There was no distinctive feature for subcortical and multiple infarcts. In bilateral infarcts, there were one pseudobulbar syndrome, and 2 pseudo brainstem syndromes with neuropsychological signs. Aetiologies were severe carotid artery disease in 14 cases, severe cardiopathy in 6, isolated cerebral angiitis in 1, essential thrombocythemia in 1, protein C deficiency with sickle cell disease in 1, and cholesterol emboli in 1 anatomical case. CBF performed in carotid artery occlusions or tight stenoses showed evidence of haemodynamic changes. Microembolic process can be proposed in the case with cholesterol emboli. Preventive treatment is discussed.
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PMID:Watershed cerebral infarcts: retrospective study of 24 cases. 135

We reviewed the prevalence of avascular necrosis (AVN) in a series of patients with sickle cell disease, using radiography and magnetic resonance imaging. We found AVN of at least one hip in 11 of 27 patients (41%). This is a significantly greater prevalence than reported. MRI was not as helpful in patients with sickle cell disease as it is in patients with AVN from other causes; it detected no more cases than radiography.
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PMID:Sickle cell disease and silent avascular necrosis of the hip. 195 42

Five cases of tumour-like extramedullary haemopoiesis (EH) causing spinal cord compression or back pain are reported. Three patients were suffering from thalassaemia major, one from sickle cell anaemia and one from thalassaemia intermedia. CT findings included soft tissue masses in the epidural space of the spinal canal, spinal cord displacement and involvement of the underlying bone. MRI studies showed masses compressing the spinal cord. On T1-weighted images there was a signal of slightly higher intensity compared to that of the adjacent marrow. CT suggested the diagnosis in four cases (in the fifth it was not performed), while MRI was positive in all five. There was good correlation between the MRI findings and the CT appearance of the EH masses. Our results suggest that MRI may eliminate the need for other diagnostic examinations in the investigation of patients with EH.
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PMID:CT and MRI of symptomatic spinal involvement by extramedullary haemopoiesis. 214 24

To investigate cerebral hemodynamics in sickle cell disease (SCD), we used the 133Xenon inhalation technique of quantifying cerebral blood flow (CBF) in 67 patients. Clinical examinations and cerebral magnetic resonance imaging also were performed in all patients. Compared with age-matched healthy controls, CBF was elevated by 68% in patients, and inversely related to hematocrit. An experimental index of cerebral blood volume, pr4, was also elevated in the patients in a similar manner. Cerebral blood volume was positively correlated to CBF in SCD patients but not in controls. History of stroke and current neurologic symptoms were associated with lower flow and higher cerebral blood volume. Transfusion therapy reduced the hyperemia, the reduction being greater than expected by hematocrit elevation alone. These findings document a vasodilatory hyperemia in SCD. This dilatation may be a risk factor for ischemic distal-field infarctions, as visualized by MRI, due to a limitation of cerebrovascular reserve capacity.
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PMID:Cerebral hyperemia, stroke, and transfusion in sickle cell disease. 292 41

We studied 25 patients with sickle cell anemia and cerebral infarction. We classified lesions as to probable mechanism (large versus small vessel disease) based on the CT/MRI appearance of established infarction. Most patients had CT/MRI patterns of major cerebral vessel occlusion (41%) or border-zone (distal insufficiency) infarcts (31%) best explained by large cerebral vessel vasculopathy. Seven of 25 (28%) had either isolated subcortical (12%) or small cortical branch occlusion (16%) consistent with other mechanisms such as small vessel occlusion or embolism. These results suggest that most clinically recognized cerebral infarctions in sickle cell anemia are caused by large vessel disease, but this mechanism may not account for symptoms of cerebral ischemia in all cases.
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PMID:Cerebral infarction in sickle cell anemia: mechanism based on CT and MRI. 338 16

Eleven patients with sickle cell disease and neurological symptoms underwent MRI examination. Cerebral infarcts of two types were found, those in the vascular distribution of the middle cerebral artery and those in the deep white matter. In the patient whose hydration and whose oxygenation of erythrocytes has been treated, MRI offers diagnostic advantages over arteriography and CT.
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PMID:MRI of sickle cell cerebral infarction. 361 18

Bone marrow transplantation (BMT) is the only curative therapy available for hemoglobinopathies. BMT was performed on a young child with sickle cell anemia (SCA) after approximately 9 months of transfusion therapy following her initial stroke. The patient received a matched sibling donor (sickle trait) BMT. The conditioning regimen consisted of busulfan 4 mg/kg/day x 4, cyclophosphamide 50 mg/kg/day x 4. Graft vs. host disease prophylaxis was daily cyclosporine for 6 months. There were no significant complications during BMT. Engraftment occurred on day +17 and the patient was transfusion independent since day +45. Pre-BMT cerebral arteriography showed multiple stenotic cerebral vessels and a moya-moya pattern. Perfusion MRI demonstrated reduced capillary perfusion. Approximately 170 days after BMT the patient experienced episodes of transient left-sided weakness and speech problems. Neuroimaging revealed progression of large vessel pathology by angiography despite significant improvement in cortical perfusion (MR perfusion scan). Molecular analysis by PCR and DNA fingerprinting confirmed absence of mixed mosaicism. Rheologic evaluation showed normal corrected bulk viscosity. It is possible that progression of large vessel pathology and return of clinical symptoms in the face of normal rheologic parameters may be due to worsening of the already damaged cerebral vessels by the BMT conditioning regimen. Further evaluations of patients with SCA undergoing BMT after a stroke are needed to answer this question.
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PMID:Bone marrow transplantation in a young child with sickle cell anemia. 771 75

In order to evaluate the role of gadolinium-DOTA enhanced MRI in the management of painful osseous crises in children with sickle cell anemia (SCA), nine children with SCA underwent MRI, bone scans and ultrasonographic studies during 11 osseous crises. Imaging findings were compared with the final diagnosis: three acute osteomyelitis (AO) and 16 acute infarcts (AI). MRI could not differentiate AO from AI. The appearance of severe AI was very misleading and was similar to the usual appearance of AO, including soft tissue changes, periosteal reaction and patterns of enhancement. Gadolinium-DOTA enhanced MRI was useful for determining the anatomic site and extent of AO or AI and for distinguishing between necrotic material, fluid collection and vascularized inflammatory tissue. It can also help to guide the aspiration of intraosseous, subperiosteal and soft tissue fluid collections.
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PMID:Gadolinium-DOTA enhanced MRI of painful osseous crises in children with sickle cell anemia. 807 30

We report 9 cases of febrile aseptic arthritis which occurred in 6 adults presenting with major sickle cell disease. The monoarticular arthritis affected the knee in 8 cases and the ankle in 1 case. Body temperature ranged from 38 degrees C to 39.5 degrees C in 7 cases, and neutrophilia (12.7 to 24 x 10(9)/l) was noted on six occasions. The always sterile synovial fluid contained 100 to 1700 cells/mm3 in 6 cases and 5200 to 12,500 cell/mm3 in 3 cases. Uricacidaemia was normal, and a search for crystals in the synovial fluid gave negative results. Bone scintigraphy and magnetic resonance imaging, performed in 3 patients, revealed the presence of a juxta-articular cortical bone infarct, whereas standard radiography was normal. In all patients, a brief immobilization and treatment with non-steroidal anti-inflammatory drugs resulted in complete cure in less than 10 days. These cases illustrate the characteristics of sickle cell arthritis, a rare complication of major sickle cell disease. Clinically, the condition resembles septic arthritis, being febrile and showing granulocytosis, but articular needle aspiration corrects the diagnosis. The mechanisms underlying these disorders have not yet been elucidated, but the bone scintigraphy and MRI performed in our patients are in favour of a reaction to a juxta-articular bone infarct.
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PMID:[Acute aseptic arthritis in major sickle cell syndromes]. 836 39


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