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Query: UMLS:C0019045 (hemoglobinopathies)
 2,704 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A study of 70 ophthalmologically asymptomatic patients with abnormal hemoglobinopathy (AS, SS, S Thal) is presented. A significant number of SS and S Thal group patients had visual acuity of less than 20/20. Retinal vein dilation and tortuosity was observed in 54% of S Thal and 83% of SS patients. Early stages of proliferative retinopathy were encountered in 7% of SS and S Thal patients in the study. The importance of routine ocular examination including meticulous binocular indirect ophthalmoscopy with scleral depression in asymptomatic patients with abnormal hemoglobin is stressed.
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PMID:Retinopathy in ophthalmologically asymptomatic patients with abnormal hemoglobins. 45 42

Two distinct episodes of posterior ciliary artery occlusion were studied in a 32-year-old man with hemoglobin SS disease and multiple episodes of amaurosis fugax. Although posterior ciliary artery occlusions have been observed following photocoagulation of sickle cell retinopathy, their spontaneous evolution in patients with sickling hemoglobinopathies has received little attention. The manifestations of posterior ciliar artery occlusion seen in this case and in other clinical and experimental situations are reviewed. Histopathologic examination of three additional eyes of patients with sickle hemoglobinopathies revealed changes which may have been the result of previous small posterior ciliary artery occlusions or small vessel occlusive disease related to the sickling hemoglobinopathies; these cases are also reported.
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PMID:Choroidal occlusive disease in sickle cell hemoglobinopathies. 46 63

The ocular manifestations of 76 sickle cell disease patients are reported. Our findings indicate that any type of sickle cell oculopathy may appear in conjunction with any type of hemoglobinopathy and that no single ocular finding is specific to any one type of sickle cell anomaly. Sickle cell oculopathy, particularly retinopathy, is found to be a slowly progressive condition. Treatment of retinal and vitreal neovascularization with photocoagulation seems to be indicated to stop the disease process. Acute retinal vascular occlusion seems to respond favorably to the intravenous administration of low molecular weight dextran and papaverine hydrochloride. Sickle cell patients suffering from retinal detachment do not respond favorably to conventional retinal detachment surgery.
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PMID:Some observations on ocular manifestation of sickle cell disease. 67 56

Sickle cell retinopathy, in all of its manifestations, represents the effects of arteriolar and capillary occlusions. Increased viscosity of circulating whole blood plus the microembolic action of individual sickled erythrocytes contribute to vasoocclusion. Decreased oxygenation and increased acidosis develop and lead to further sickling -- and further vaso-occlusion. The cycle of erythrostasis that characterizes sickling throughout the body is also applicable to the retina. The transparent media of the eye permit direct visualization of vaso-occlusions which occur preferentially in and about the macula and in the far periphery of the retina. Many of the occlusive episodes are transient. There dynamic events are simultaneously occurring elsewhere in the body but can only be visualized in the eye. The net effect in the retina is a remodeling of its vasculature, as some vessels close and others reopen. After the onset of arteriolar closure in the retina, affected blood vessels embark on a spontaneous, naturally evolving course of events leading to arteriolarvenular anastomoses, neovascular proliferations, vitreous hemorrhages, and retinal detachment. The advanced stages of proliferative sickle retinopathy are most commonly observed in SC disease and in Sthal, possibly because these two forms of sickling have significantly higher than normal whole blood viscosity. Retinal vaso-occlusions can also lead to blow-out hemorrhages which may evolve into salmon patches, iridescent spots, schisis cavities, and black sunbursts. In some respects sickle retinopathy is unique, but many of its manifestations are similar to those of retinopathies found in diabetes mellitus, AC hemoglobinopathy, Takayasu pulseless disease, sarcoidosis, chronic myelogenous leukemia, branch retinal vein occlusion, retrolental fibroplasia, and Eales disease.
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PMID:Retinal vaso-occlusion in sickling hemoglobinopathies. 78 1

Seven patients had sickle cell trait (hemoglobin AS) and vasoproliferative retinopathy. The retinal abnormalities in these seven patients were indistinguishable from those seen in patients with clinically significant sickling hemoglobinopathies (sickle cell-hemoglobin C disease, hemoglobin S-thalassemia disease, and sickle cell anemia). All seven patients also had some evidence of associated systemic disease such as diabetes, syphilis, tuberculosis, or sarcoidosis. In the presence of an associated systemic disease, marked retinopathy can occur in the ordinarily benign condition of sickle cell trait.
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PMID:Proliferative retinopathy in sickle cell trait. Report of seven cases. 84 50

Patients with sickle cell disease can have a distinct retinopathy in which the posterior pole shows abnormalities, including perifoveolar vascular abnormalities. The foveal avascular zone (FAZ) was examined using fluorescein angiography in patients with sickle cell disease and in healthy normal controls. The longest FAZ diameters of 38 patients (51 eyes) with sickle cell disease were compared with those of the 48 patients (60 eyes) in the control group. The average of the longest FAZ diameter in the patients with sickle cell disease was 1.00 mm compared with 0.61 mm for the controls. This difference was statistically significant (P less than .00001). Within the sickle cell group, there were no significant differences in the FAZ diameters with respect to degree of retinopathy, type of sickle hemoglobinopathy, or visual acuity. Thus, an enlarged FAZ diameter in patients with sickle cell disease is confirmed.
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PMID:Foveal avascular zone diameter and sickle cell disease. 204 68

We report a case of unilateral optociliary shunt vessels and sickle cell retinopathy in a patient with sickle cell trait. Sickle cell retinopathy has rarely been reported in patients with sickle cell trait hemoglobinopathy. To our knowledge, this is the first report of the association of sickle cell trait, unilateral sickle cell retinopathy, and ipsilateral optociliary shunt vessels.
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PMID:Optociliary shunts and sickle retinopathy in a woman with sickle cell trait. 231 55

The cases of 100 consecutive persons (156 eyes) seen with peripheral proliferative retinopathy were reviewed in a retrospective fashion. Associated systemic and ocular diseases included sickling hemoglobinopathies (49%), branch retinal vein obstruction (20%), diabetes mellitus (9%), sarcoidosis (4%), intravenous drug abuse (4%), the ocular ischemic syndrome (1%), pars planitis (1%), Coats' disease (1%) and retinitis pigmentosa/retinal detachment (1%). In ten (10%) patients no obvious cause for the development of new blood vessel growth could be ascertained.
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PMID:Peripheral proliferative retinopathies. 244 26

The authors have followed 140 patients with sickle cell disease anemia (101 cases) or related hemoglobinopathies (39 cases). Among them hip involvement was noted in 55 (104 hips). Forty three times the hip involvement occurred in childhood and twelve times in adult life. When the necrosis appeared in childhood (84 hips) the average age was twelve. The deformity involved the femoral head (coxa plana, coxa magna) as well as the neck (short neck, coxa vara). After an average follow-up of nineteen years, clinical and radiological examinations evidenced 64 functional impairments and 25 arthrosis, 10 of which have already been operated on. The necrosis appearing during adult life (20 hips) had the same outcome as idiopathic necroses, leading rapidly to arthrosis after collapse of the sequestrum. It seems that the etiology of the necroses is linked to rheologic disorders, the deformity of the red cells causing arteriolar thromboses. In this series the hip disease was correlated with sickle cell retinopathy as defined after angiography. On the contrary there was no correlation with the severity of anemia, its treatment, the ethnical origin of the patient.
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PMID:[Natural history of hip necrosis in sickle cell disease. Apropos of 104 necroses]. 269 46

Ten patients (11 eyes) with sickle-C hemoglobinopathy with complications of proliferative sickle retinopathy were treated using pars plana vitrectomy with or without the use of a scleral buckle. Postoperative visual acuity was improved in ten of 11 cases. Three cases of retinal detachment were managed by internal vitreoretinal techniques alone without the use of a scleral buckle. Although exchange transfusions were used in only five of the 11 cases, no cases of recognized anterior segment ischemia occurred during the postoperative course of these patients. Because of exchange transfusion risks and awareness of intraoperative and postoperative measures to reduce this complication, the use of exchange transfusions probably should be discontinued as prophylaxis for vitreoretinal surgery in these patients.
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PMID:Pars plana vitrectomy in the management of complications of proliferative sickle retinopathy. 319 May 40


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