1/45. Large cerebral vessel disease in sickle cell anaemia.An 18 year old male with documented sickle cell disease was admitted to the hospital for the final time in coma. cerebral angiography revealed multiple stenotic lesions of the large cerebral vessels. The pathology of this large vessel involvement is demonstrated and the potential contribution of large as opposed to small cerebral vessel disease in the neurological manifestations of sickle cell anaemia is discussed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/45. Coil embolization of cerebral aneurysms in patients with sickling disorders.In patients with sickle cell disease cerebral aneurysm formation is thought to be a complication of recurrent red cell sickling, and multiple aneurysms have been reported in these patients. Management of patients with suspected cerebral aneurysm has traditionally involved cerebral vessel angiography followed by craniotomy and aneurysmal clipping. In patients without sickle cell disease, non-operative intervention in the form of endovascular coil embolization is increasingly being used to ablate aneurysms, but has not thus far been reported in patients with sickle cell disease. We report two patients with sickling disorders who have undergone coil embolization of cerebral aneurysms with good functional and radiological outcomes. These patients illustrate that endovascular coiling is useful in the treatment of cerebral aneurysms associated with sickling disorders, although, as with surgical intervention, preparation with exchange transfusion is appropriate.- - - - - - - - - - ranking = 0.14285714285714keywords = vessel (Clic here for more details about this article) |
3/45. bone marrow necrosis in sickle cell disease: a description of three cases and a review of the literature.bone marrow necrosis (BMN) ranges from a localized to a widespread generalized process. Most often seen in patients with leukemia and other malignant conditions, generalized BMN has also been observed in patients with sickle cell disease (SCD), where it is almost certainly a consequence of blood vessel occlusion. Activation of the clotting system seems to play a role in this clinical setting. Systemic fat embolism and acute multi-organ failure syndrome can also complicate BMN in patients with SCD. We describe here 3 cases of BMN associated with SCD. Each patient exhibited an unusually severe vaso-occlusive crisis accompanied by persistent fever, a high level of serum lactate dehydrogenase, leukoerythroblastosis, and large numbers of nucleated red cells. Despite such suggestive clinical features, diagnosis of BMN still requires a bone marrow biopsy. Particularly in patients with SCD, the early institution of transfusion therapy can be life-saving. The ominous prognosis ascribed to generalized BMN seems to reflect the poor outcome of such underlying conditions as leukemia; however, the prognosis of generalized BMN is not so poor in association with SCD and other nonmalignant states.- - - - - - - - - - ranking = 0.14285714285714keywords = vessel (Clic here for more details about this article) |
4/45. Sickle cell disease with orbital infarction and epidural hematoma.Although bone infarction is a common feature in sickle cell disease, the involvement of the orbit is an unusual complication. Intracranial bleeding is another uncommon and serious complication. Few cases of orbital infarction alone have been reported. We report imaging findings (CT, bone scan, MRI) in a 16-year-old boy with sickle cell disease with orbital infarction and epidural hematoma. The precise cause of epidural hematoma is not well known, but it is probably related to vaso-occlusive episodes and the tearing of small vessels.- - - - - - - - - - ranking = 0.14285714285714keywords = vessel (Clic here for more details about this article) |
5/45. Spontaneous regression (autoinfarction) of proliferative sickle retinopathy.Of 45 patients with proliferative sickle retinopathy in stages III, IV, and V, nine patients (eight with hemoglobin sc disease, one with sickle cell thalassemia) showed spontaneous regression (autoinfarction) of retinal sea fans. One mechanism involved in autoinfarction of neovascular tissue is progressive, centripetal retraction of the anterior vascular arcade of the peripheral retina. In addition, vitreous traction on feeder vessels may result in sluggish blood flow and occlusion of these vessels, or may tear the sea fan completely away from its feeder vessels. In view of the many incidences of vitreous hemorrhages that occur in patients with proliferative retinopathy, however, we recommend treatment of neovascularization rather than prolonged observation.- - - - - - - - - - ranking = 0.42857142857143keywords = vessel (Clic here for more details about this article) |
6/45. Facial swelling and gingival enlargement in a patient with sickle cell disease.Sickle cell anemia is a frequent hemoglobinopathy in the Caribbean. While vaso-occlusion induced tissue injury in sickle cell anemia is common in various organs, orofacial lesions are rare. A 14-year-old Afro-Trinidadian boy suffering from sickle cell anemia developed an acute facial swelling, mimicking facial cellulitis of dental origin, which was caused by sickle cell-related hemorrhage. He also exhibited gingival enlargement, considered to be an outcome of repeated hemorrhagic episodes and fibrous repair. A new finding is the presence of erythrocyte-filled intraepithelial blood vessels in the gingival epithelium. We hypothesize this phenomena is a tissue response to hypoxia that occurs in sickle cell disease.- - - - - - - - - - ranking = 0.14285714285714keywords = vessel (Clic here for more details about this article) |
7/45. bone marrow scan evaluation of arthropathy in sickle cell disorders.Twelve patients with sickle cell hemoglobinopathies and arthropathy were studied, using technetium tc 99m sulfur colloid bone marrow scans. Eight of 12 had decreased marrow radionuclide activity adjacent to painful joints, suggestion obliteration of vessels supplying bone marrow. Four patients without marrow defects on scanning had causes other than infarction for their joint symptoms, viz, small fractures, postinfectious synovitis, degenerative arthritis, and osteochondromas. Roentgenograms never showed bony abnormalities in five patients with marrow infarctions, and, in three others, showed defects several months later than did the marrow scans. bone marrow scans offer a sensitive and early diagnostic aid in sickle cell hemoglobinopathies with arthropathy.- - - - - - - - - - ranking = 0.14285714285714keywords = vessel (Clic here for more details about this article) |
8/45. Acute pancreatitis during sickle cell vaso-occlusive painful crisis.Sickle cell disease is characterized by chronic hemolytic anemia and vaso-occlusive painful crisis. The vascular occlusion in sickle cell disease is a complex process and accounts for the majority of the clinical manifestations of the disease. abdominal pain is an important component of vaso-occlusive painful crisis and may mimic diseases such as acute appendicitis and cholecystitis. Acute pancreatitis is rarely included as a cause of abdominal pain in patients with sickle cell disease. When it occurs it may result form biliary obstruction, but in other instances it might be a consequence of microvessel occlusion causing ischemia. In this series we describe four cases of acute pancreatitis in patients with sickle cell disease apparently due to microvascular occlusion and ischemic injury to the pancreas. All patients responded to conservative management. Acute pancreatitis should be considered in the differential diagnosis of abdominal pain in patients with sickle cell disease.- - - - - - - - - - ranking = 0.14285714285714keywords = vessel (Clic here for more details about this article) |
9/45. Autologous immune complex nephritis associated with sickle cell trait: diagnosis of the haemoglobinopathy after renal structural and immunological studies.A renal tubular epithelial antigen (RTE)--anti-RTE autologous immune complex nephritis associated with sickle cell anaemia (SS) has been reported, but immune complex nephritis has never been described in patients with sickle cell trait (SA). During investigation of a child with "asymptomatic proteinuria" cryoprecipitable complexes of RTE-anti-RTE were detected in the serum and granular deposits of RTE, immunoglobulins, and complement localised on the glomerular basement membranes. Morphological and ultrastructural studies showed increased mesangial matrix, sickled red blood cells in the glomeruli and vessels, and tubular and interstitial abnormalities. These findings prompted haemoglobin electrophoretic studies, which showed previously undiagnosed haemoglobin SA in this patient and her family. These observations suggest that nephritis mediated by similar immunopathogenic mechanisms may be associated with SS and SA haemoglobinopathy. Under some conditions patients with sickle cell trait may experience haemodynamic and oxygenation abnormalities, which may be aetiological factors in the immune complex nephritis associated with SS disease.- - - - - - - - - - ranking = 0.14285714285714keywords = vessel (Clic here for more details about this article) |
10/45. stroke and elevated blood flow velocity in the anterior cerebral artery in sickle cell disease.Transcranial Doppler ultrasonography provides a noninvasive method of predicting stroke risk in children with sickle cell disease. Elevated cerebral blood flow velocity in the terminal internal carotid or middle cerebral artery is associated with an increased stroke risk, but the clinical significance of elevated velocities in the other large intracranial vessels is unknown. The authors report stroke in two children with sickle cell disease and high blood flow velocity limited to the anterior cerebral artery. This suggests that elevated velocity in this vessel may be associated with an increased risk of stroke.- - - - - - - - - - ranking = 0.28571428571429keywords = vessel (Clic here for more details about this article) |
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