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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 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