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

Four patients with sickle cell hemoglobinopathies (one sickle cell hemoglobin C disease (SC); three sickle cell trait (AS)) and hyphemas had a higher percentage of erythrocytes sickled in their anterior chambers than in their circulating venous blood. Intraocular pressure (IOP) was severely increased, despite relatively small amounts of intracameral blood. Systemic hypotensive agents were not always successful in reducing IOP, and in patients with sickle cell hemoglobinopathy, are probably contraindicated in high or repeated dose regimens. Moderate increase of IOP in sickle cell hemoglobinopathy patients may produced rapid deterioration of visual function, because of a greater than usual effect on vascular perfusion in the central retinal artery and optic nerve. Early anterior chamber paracentesis may be the best treatment for this type of hyphema-induced secondary glaucoma.
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PMID:The diagnosis and treatment of secondary glaucoma after hyphema in sickle cell patients. 43 52

Four patients with sickle cell hemoglobinopathies (one SC, three AS) and hyphemas were found to have more erythrocytes sickled in their anterior chambers than in their circulating venous blood. Intraocular pressure was severely elevated, despite relatively small amounts of intracameral blood. Systemic hypotensive agents were not always successful in reducing IOP, and in patients with sickle cell hemoglobinopathy, are probably contraindicated in high or repeated dose regimens. Moderate elevation of IOP in sickle cell hemoglobinopathy patients may produce rapid deterioration of visual function, perhaps because of a greater than usual effect on vascular perfusion in the central retinal artery and optic nerve. Early anterior chamber paracentesis may be the best treatment for this type of hyphema-induced secondary glaucoma.
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PMID:Sickled erythrocytes, hyphema, and secondary glaucoma: I. The diagnosis and treatment of sickled erythrocytes in human hyphemas. 46 Aug 14

Four patients with sickle cell hemoglobinopathies (one SC; three AS) and hyphemas were found to have more erythrocytes sickled in their anterior chambers than in their circulating venous blood. Intraocular pressure was severely elevated, despite relatively small amounts of intracameral blood. Systemic hypotensive agents were not always successful in reducing IOP, and in patients with sickle cell hemoglobinopathy, are probably contraindicated in high or repeated dose regimens. Moderate elevation of IOP in sickle cell hemoglobinopathy patients may produce rapid deterioration of visual function, perhaps because of a greater effect on vascular perfusion in the CRA and optic nerve. Early anterior chamber paracentesis may be the best treatment for this type of hyphema-induced secondary glaucoma.
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PMID:The diagnosis and treatment of sickled erythrocytes in human hyphemas. 75 80

Sickle cell retinopathy in its advanced form is complicated by preretinal neovascularization, vitreous hemorrhage, and retinal detachment. Treatment of neovascularization can be performed with photocoagulation. Complications such as retinal breaks, retinal detachments, and choroidally fed neovascularization may result from such treatment. The risks vs. the benefits of various types of photocoagulation are currently being evaluated. Cryotherapy also may be used to treat neovascularization. It is currently being used in eyes with media that are too hazy to permit photocoagulation. It is used commonly during scleral buckling and vitrectomy procedures. In eyes with decreased visual acuity secondary to prolonged vitreous hemorrhage, pars plana vitrectomy can be utilized to produce optically clear media. Complications (including erythrocyte-induced glaucoma), however, may be severe. Retinal detachment can be treated by scleral buckling, but the markedly increased risk of anterior segment ischemia in patients with sickle cell hemoglobin necessitates preoperative, intraoperative, and postoperative prophylactic measures to minimize the risk of this potentially devastating complication. In eyes with retinal detachment with cloudy media and severe vitreous traction, combined scleral buckling and vitrectomy may be necessary. These eyes are extremely fragile, and a successful result is currently obtained in only about 50% of such cases. Hyphemas in patients with sickle cell hemoglobinopathies, whether traumatically or surgically induced, may have devastating effects on the eye. If elevated IOP results decreased vascular perfusion of the eye may cause irreversible damage to the retina and optic nerve. Most antiglaucoma medications, when used in the sickle cell patient, have a narrow margin of safety. Therefore, early surgical intervention for the treatment of sickle cell hyphemas is currently being evaluated.
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PMID:Diagnosis and management of ocular complications of sickle hemoglobinopathies: Part V. 242 44

A 5-year-old Mexican-American boy developed marked elevation of intraocular pressure in association with a small traumatic hyphema. Laboratory studies prompted by this clinical presentation revealed that the child had sickle trait. Pressure returned to normal after surgical evacuation of blood from the anterior chamber. Sickle cell hemoglobinopathy should be considered in the differential diagnosis of secondary glaucoma following blunt ocular trauma even in non-black patients.
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PMID:Sickle cell hyphema with secondary glaucoma in a non-black patient. 399 Nov 13

Eighteen patients (19 eyes) with sickling hemoglobinopathies underwent vitrectomy or scleral buckling operations. The indications for surgery were tractional or rhegmatogenous retinal detachment or vitreous hemorrhage. The retina was reattached in all four patients with rhegmatogenous detachments requiring scleral buckling surgery but no vitrectomy. All had excellent visual acuity postoperatively. Four of the five patients with vitreous hemorrhage requiring vitrectomy without buckling had substantial improvement in visual acuity. In ten patients with vitreous hemorrhage with either preexisting or iatrogenic retinal breaks, rhegmatogenous detachment, or tractional detachment, the rate of improvement in visual acuity was only 50%. Problems associated with vitreous or retinal surgery in patients with sickling hemoglobinopathies include iatrogenic retinal breaks, intraocular bleeding and secondary glaucoma, anterior segment ischemia, and systemic sickling.
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PMID:An update on vitrectomy surgery and retinal detachment repair in sickle cell disease. 707 71

During the past year, there has been increased understanding of the ocular manifestations of various cardiovascular and hematologic disorders. Carotid and vertebral artery lesions may lead to significant and varied ophthalmic pathology. Disorders of blood pressure may influence the intraocular pressure and play a role in the progression of glaucoma. Cardiovascular risk factors such as smoking, hyperlipidemia, hypertension, and diabetes mellitus, may also play a role in the development of anterior ischemic optic neuropathy. Several cardiac anomalies as well as the cardiac use of streptokinase have been reported to have secondary ocular involvement. Both benign and malignant hematologic disorders may result in serious ocular morbidity. Recent publications have focused on the secondary ophthalmic complications from the hemoglobinopathies, problems with blood viscosity, the lymphomas, the leukemias, and bone marrow transplantation.
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PMID:Ocular manifestations of cardiovascular and hematologic disorders. 1016 Apr 27