Infarction In Sickle Cell Disease Research Articles

Scintigraphy and ultrasonography in differentiating osteomyelitis from bone infarction in sickle cell disease

Scintigraphy and ultrasonography in differentiating osteomyelitis from bone infarction in sickle cell disease

Cerebral infarction in sickle cell disease: transcranial Doppler US versus neurologic examination.

To evaluate flow velocity measurements in the middle cerebral artery (MCA) and/or neurologic examination for detection of cerebral infarction in sickle cell disease (SCD). Twenty-four pediatric patients aged 6 1/2-17 years with SCD underwent magnetic resonance (MR) imaging, MR angiography, neurologic examination, and transcranial Doppler ultrasonography (US). Transcranial Doppler studies were evaluated for maximum flow velocity in the right and left MCAs. Combinations of cut-off values were used to determine the sensitivity and specificity of transcranial Doppler US for detection of infarction. Neurologic examination had 58% sensitivity and 92% specificity for cerebral infarction. Maximal flow velocity > 200 cm/sec or < 100 cm/sec (including no flow) helped identify nine of 12 patients with infarcts proved at MR imaging, with only one false-positive result (sensitivity, 75%; specificity 92%). The combination of neurologic examination and transcranial Doppler US produced 92% sensitivity and 83% specificity for cerebral infarction. The combination of transcranial Doppler US and neurologic examination has potential as a screening technique for infarction in SCD.

Sickle Cell Disease and Stroke

The increased risk of stroke in sickle cell disease has been recognized for over 50 years. Recent reports have increased our understanding by better describing the clinical characteristics and presentation of sickle cell disease patients with stroke and provided relatively consistent estimates of prevalence applicable to younger cohorts. The experience from established clinics in Los Angeles,1 1 Philadelphia,2 and Kingston, Jamaica,3 indicate that clinically evident cerebrovascular disease has a prevalence of about 7% to 8% in cohorts followed for the first 2 decades of life. Less is known about stroke in older patients, but the Cooperative Study of Sickle Cell Disease4 is developing estimates of stroke incidence and prevalence data from adults as well as children with sickle cell disease. Studies using magnetic resonance imaging (MRI) or computed tomography (CT) have shown that the prevalence of cerebral infarction is higher than that of stroke. This is consistent with the definition that stroke is a clinical syndrome, and cerebral infarction is a pathologic entity detected with high sensitivity and specificity by MRI and, to a lesser extent, CT. The study by Glauser and colleagues5 supports prior observations that not all sickle cell disease patients with abnormal MRIs have a history or neurologic findings that go with the lesion(s). Their systematic study provides an estimate of silent infarction from their sample of 30 patients of 24% based on history and examination alone. This is higher than the 11% reported from a 55patient series from Columbia-Presbyterian Hospital6 and from the Cooperative Study of Sickle Cell Disease, in which 16% of 172 patients had abnormal MRI without a stroke history This suggests that only about half of the sickle cell disease patients with brain infarction report or develop symptoms sufficient to prompt recognition, at least early in life. Clinical surveillance alone misses a sizable number of patients with brain lesions. The rate of silent infarction in sickle cell disease is comparable to that reported in other groups at high risk for cerebrovascular disease. The Asymptomatic Carotid Atherosclerosis Study, which compared carotid endarterectomy with medical treatment in patients with 60% or greater cervical carotid stenosis, reported that 126 (15%) of 848 patients with no history of transient ischemic attacks or stroke had one or more CT lesions, primarily small, deep infarcts, at entry into the study.8 Ten percent of Framingham patients presenting with first-time stroke already had CT-evident lesions.9 In the presence of chronic atrial fibrillation, the reported prevalence of silent cerebral infarction has ranged from 11% to 48%.8 S

Transcranial Doppler correlation with cerebral angiography in sickle cell disease.

Cerebral infarction in sickle cell disease is associated with arterial narrowing or occlusions of intracranial arteries. Primary stroke prevention would be feasible if a noninvasive screening test could be developed to detect intracranial disease in patients before symptoms develop. To determine the sensitivity and specificity of transcranial Doppler in detecting significant (greater than or equal to 50% lumen diameter reduction) intracranial arterial lesions, we compared transcranial Doppler and cerebral angiography in a primarily young, symptomatic group of 33 patients (18 males and 15 females) with sickle cell disease. From a total of 34 examinations, transcranial Doppler detected significant abnormalities in 26 of 29 (sensitivity 90%, specificity 100%). Five were normal by both techniques. The transorbital examination detected abnormalities in two patients whose studies were otherwise unremarkable. Transcranial Doppler is sensitive and specific for the detection of arterial vasculopathy of sickle cell disease. Screening should include a transorbital examination of the distal internal carotid artery as well as examination using the transtemporal approach.

A modified transfusion program for prevention of stroke in sickle cell disease

Regular red blood cell transfusions reduce the rate of recurrent cerebral infarction in sickle cell disease but lead to accumulation of excessive iron. We studied the effect on the prevention of recurrent stroke and the volume of blood transfused of a modified transfusion program in which the pretransfusion percentage of hemoglobin S (HbS) was maintained at 50%, rather than the conventional 30%. Fifteen patients with sickle cell disease and cerebral infarction who had been free of recurrent stroke for at least 4 years during which the pretransfusion HbS was maintained below 30% were assigned to a transfusion program in which the HbS was allowed to increase to 50%. Transfusion regimens included simple transfusion and manual and automated partial exchange transfusion. The duration of follow-up was 14 to 130 months with a median duration of 84 months. None of the 15 patients had a recurrent cerebral infarction during 1,023 patient-months in which the target pretransfusion HbS was 50%. Analysis of this finding, using a binomial distribution, indicates that there is less than a 5% chance that the risk per patient of recurrent stroke in the first year of the modified transfusion program is greater than 18%. One 23-year-old patient had a fatal intraventricular hemorrhage when the HbS was 30% and a 21-year-old patient had a fatal subarachnoid hemorrhage in the 40th week of pregnancy when the HbS was 29%. Blood requirements with simple transfusions decreased by 17% to 48% (mean 31%) when the target pretransfusion HbS level was increased from 30% to 50% (P less than .001). Manual or automated partial exchange transfusions and a target HbS level of 50% in eight patients reduced blood requirements by 33% to 99% (mean 67%) in comparison with simple transfusion and a target HbS level of 30% (P less than .001). This study offers evidence that a target pretransfusion HbS level of 50% affords a continuing high rate of protection against recurrent cerebral infarction in sickle cell disease after 4 years of a conventional transfusion program. Increasing the target HbS level from 30% to 50% provides a major reduction in blood requirements and lowers the rate of iron accumulation.

A modified transfusion program for prevention of stroke in sickle cell disease

Regular red blood cell transfusions reduce the rate of recurrent cerebral infarction in sickle cell disease but lead to accumulation of excessive iron. We studied the effect on the prevention of recurrent stroke and the volume of blood transfused of a modified transfusion program in which the pretransfusion percentage of hemoglobin S (HbS) was maintained at 50%, rather than the conventional 30%. Fifteen patients with sickle cell disease and cerebral infarction who had been free of recurrent stroke for at least 4 years during which the pretransfusion HbS was maintained below 30% were assigned to a transfusion program in which the HbS was allowed to increase to 50%. Transfusion regimens included simple transfusion and manual and automated partial exchange transfusion. The duration of follow-up was 14 to 130 months with a median duration of 84 months. None of the 15 patients had a recurrent cerebral infarction during 1,023 patient- months in which the target pretransfusion HbS was 50%. Analysis of this finding, using a binomial distribution, indicates that there is less than a 5% chance that the risk per patient of recurrent stroke in the first year of the modified transfusion program is greater than 18%. One 23-year-old patient had a fatal intraventricular hemorrhage when the HbS was 30% and a 21-year-old patient had a fatal subarachnoid hemorrhage in the 40th week of pregnancy when the HbS was 29%. Blood requirements with simple transfusions decreased by 17% to 48% (mean 31%) when the target pretransfusion HbS level was increased from 30% to 50% (P less than .001). Manual or automated partial exchange transfusions and a target HbS level of 50% in eight patients reduced blood requirements by 33% to 99% (mean 67%) in comparison with simple transfusion and a target HbS level of 30% (P less than .001). This study offers evidence that a target pretransfusion HbS level of 50% affords a continuing high rate of protection against recurrent cerebral infarction in sickle cell disease after 4 years of a conventional transfusion program. Increasing the target HbS level from 30% to 50% provides a major reduction in blood requirements and lowers the rate of iron accumulation.

Bone Ischaemia and Infarction in Sickle Cell Disease

HomeRadiologyVol. 152, No. 3 PreviousNext ReviewsBone Ischaemia and Infarction in Sickle Cell DiseaseKenneth E. RobinsonKenneth E. RobinsonKenneth E. RobinsonPublished Online:Sep 1 1984https://doi.org/10.1148/radiology.152.3.600MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In Article HistoryPublished in print: Sept 1984 FiguresReferencesRelatedDetailsRecommended Articles RSNA Education Exhibits RSNA Case Collection Vol. 152, No. 3 Metrics Altmetric Score PDF download

Bone Ischaemia and Infarction in Sickle Cell Disease,

Vaso-occlusive crises with bone and joint pain, ischemia, and infarction are common and major clinical manifestations of sickle cell anemia and its variants, sickle cell hemoglobin C disease, and sickle cell-thalassemia. Well known to the pediatrician and the pediatric hematologist, bone infarction is amply and reasonably well documented in most available general and subspecialty textbooks. The main purpose of Dr Bohrer's monograph, the most complete and refreshing treatise on the subject to date, is to illustrate, describe, and discuss acute and chronic bone ischemia in sickle cell anemia and heterozygous sickle cell states. Dr Bohrer has devoted his career to the study of ischemic osteonecrosis, osteomyelitis, and infarctive bone necrosis in sickle cell anemia. He had extensive experience in Ibadan, Nigeria, from 1967 through 1977 and has compiled a magnificent collection of roentgenograms. As he clearly cautions the reader, sickle cell anemia in Nigerian children is clinically dissimilar to the

Book Reviews

Book Reviews in Thie Article:Leukemia Markers. Edited by W. Knapp.Bone Ischaemia and Infarction in Sickle Cell Disease. By Stanley P. Bohrer.The Red Cell: Fifth Ann Arbor Conference. Edited by G. Brewer.Fibrinolysis. By Neville Marsh.

Orbital Infarction in Sickle Cell Disease

Bone infarction is a common occurrence in sickle cell disease. Described are three cases in which frontal headache, proptosis, and lid edema were seen with infarction of the orbital bone. Radionuclide scanning was useful in distinguished bone infarction from orbital infection in one case.

Aspirin, hyperventilation, and cerebellar infarction in sickle cell disease

Aspirin, hyperventilation, and cerebellar infarction in sickle cell disease

Aspirin, Hyperventilation, and Cerebellar Infarction in Sickle Cell Disease

Aspirin ingestion was followed by hyperventilation, cerebellar signs, and fatal brain stem dysfunction in a patient with sickle cell disease. Autopsy showed a swollen, recently infarcted cerebellum with tonsillar herniation compressing the medulla. We propose that hypocapnea from aspirin-induced hyperventilation caused carotid artery constriction and focal cerebral hypoxia, resulting in cerebellar sickling nad infarction. Hypocapnea should be treated promptly to prevent brain damage in patients with sickle cell disease.