Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We analyzed 22 sickle cell trait hyphemas. Fourteen of the 22 eyes were adequately controlled with medical therapy alone, ie, the intraocular pressure averaged less than 25 mm Hg during consecutive 24-hour periods and there were no repeated transient rises of IOP above 30 mm Hg. Surgery was performed in eight eyes, because of inadequate medical control. Thirteen of the 14 medically treated eyes had acceptable levels of IOP within the first 24 hours; only one of the eight surgically treated eyes had adequate control during the first 24 hours. This suggests that control during the first 24 hours portends a good prognosis, while lack of control during that period predicts continued difficulty managing the IOP. This seems to be important in view of previous experience that suggests that these eyes do not tolerate minimal to moderate elevations in IOP as well as those of patients without sickle cell anemia.
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PMID:Indications for surgical management of hyphema in patients with sickle cell trait. 670 13

Hyphema is a potentially sight-threatening sequela of blunt trauma. Delayed healing time, poor visual outcome, and complications such as corneal blood staining, anterior and posterior synechiae, increased intraocular pressure, and glaucomatous optic atrophy are most often associated with hemorrhage filling more than one half of the anterior chamber. Rebleeds are most likely 3 to 5 days following injury, in children, in blacks (particularly if they have sickle cell disease), and in persons who have ingested aspirin or other antiplatelet compounds. Treatment of hyphema is controversial, and medical therapy (antibiotics, cycloplegics, steroids, aminocaproic acid) should be tailored to suit the needs of each case. Intraocular pressure-reducing medications may be required if there is significant elevation of IOP. The affected eye should be protected with a shield, and follow-up examinations should be conducted as necessary. Both the anterior and posterior eye should receive careful assessment.
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PMID:Hyphema. 826 94

Hyphema (blood in the anterior chamber) can occur after blunt or lacerating trauma, after intraocular surgery, spontaneously (e.g., in conditions such as rubeosis iridis, juvenile xanthogranuloma, iris melanoma, myotonic dystrophy, keratouveitis (e.g., herpes zoster), leukemia, hemophilia, von Willebrand disease, and in association with the use of substances that alter platelet or thrombin function (e.g., ethanol, aspirin, warfarin). The purpose of this review is to consider the management of hyphemas that occur after closed globe trauma. Complications of traumatic hyphema include increased intraocular pressure, peripheral anterior synechiae, optic atrophy, corneal bloodstaining, secondary hemorrhage, and accommodative impairment. The reported incidence of secondary anterior chamber hemorrhage, that is, rebleeding, in the setting of traumatic hyphema ranges from 0% to 38%. The risk of secondary hemorrhage may be higher in African-Americans than in whites. Secondary hemorrhage is generally thought to convey a worse visual prognosis, although the outcome may depend more directly on the size of the hyphema and the severity of associated ocular injuries. Some issues involved in managing a patient with hyphema are: use of various medications (e.g., cycloplegics, systemic or topical steroids, antifibrinolytic agents, analgesics, and antiglaucoma medications); the patient's activity level; use of a patch and shield; outpatient vs. inpatient management; and medical vs. surgical management. Special considerations obtain in managing children, patients with hemoglobin S, and patients with hemophilia. It is important to identify and treat associated ocular injuries, which often accompany traumatic hyphema. We consider each of these management issues and refer to the pertinent literature in formulating the following recommendations. We advise routine use of topical cycloplegics and corticosteroids, systemic antifibrinolytic agents or corticosteroids, and a rigid shield. We recommend activity restriction (quiet ambulation) and interdiction of non-steroidal anti-inflammatory agents. If there is no concern regarding compliance (with medication use or activity restrictions), follow-up, or increased risk for complications (e.g., history of sickle cell disease, hemophilia), outpatient management can be offered. Indications for surgical intervention include the presence of corneal blood staining or dangerously increased intraocular pressure despite maximum tolerated medical therapy, among others.
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PMID:Management of traumatic hyphema. 1268 17

Material occurring in the anterior chamber as a result of trauma may be of little or major significance. The most common finding requiring treatment is hyphema. Close observation and (often surgical) treatment is especially important in patients at high risk: those with sickle cell disease, rebleeding, and elevated intraocular pressure. Cataract is a common complication in eyes sustaining serious trauma, although its presence may be difficult to confirm during the initial repair. The diagnosis is especially crucial because of the significantly increased risk of endophthalmitis. If the surgeon is able to determine that cataract is indeed present and hinders visualization of the posterior segment, or may lead to rapid elevation of the intraocular pressure, primary lens removal should be considered because vitreoretinal injuries are expected in approximately one-half of eyes, and an early retinal examination is mandatory in all eyes with lens trauma. Because one out of two eyes have posterior capsule injury, vitrectomy methods of lens removal are commonly required. Preservation of the posterior capsule is less important than avoiding traction on the anterior vitreous, because alternative methods of intraocular lens placement offer similar functional results.
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PMID:Anterior chamber abnormalities and cataract. 1222 36

Intracameral tissue plasminogen activator (t-PA) application in a child with previously unrecognized sickle cell anemia, post-traumatic hyphema, thrombosis in trabecular mashwork and consecutive acute glaucoma showed positive results. Thirteen year-old boy, son of African father and Caucasian mother, was admitted to hospital, with symptoms of acute glaucoma and partial hyphema after right eye trauma. Visual acuity of affected eye was 0.5 and intraocular pressure (IOP) 46 mm Hg. Despite a common therapy three days later clinical condition of patient's right eye was getting worst. Visual acuity was only hand motion (HM) and IOP 53 mmHg. At this point rose suspicion of sickle cell disease (SCD) and decision about injecting t-PA (20 microg) into anterior chamber was made. Cytological examination of aqueous humor revealed 10% sickled erythrocytes. Hemoglobin electrophoresis discovered hemoglobin S so that diagnosis of SCD was confirmed. Intraocular application of t-PA showed excellent results in post-traumatic hyphema with trabecular mashwork thrombosis in the patient with sickle cell anemia. Two-years follow up confirmed permanent normalisation of IOP and visual acuity. Successful outcome with anterior chamber paracentesis and intracameral injection of t-PA is promising novel approach, which we recommend in treatment of post-traumatic hyphema in SCD.
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PMID:Treatment of post-traumatic trabecular mashwork thrombosis and secondary glaucoma with intracameral tissue plasminogen activator in previously unrecognized sickle cell anemia. 1619 93

Sickle cell trait is usually considered as a benign condition. However under certain adverse circumstances, it can give rise to vaso-occlusive features as in sickle cell disease. We present here two cases, both involving healthy young males, who developed retinal vaso-occlusive features following blunt ocular trauma. There was a rapid progression of the retinopathy with the development of proliferative changes in both patients and also vitreous hemorrhage in one patient, within 2 months of the trauma. The development of retinopathy was independent of raised intraocular pressure. Both patients were found to have sickle cell trait.
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PMID:Unusual presentation of ocular trauma in sickle cell trait. 2663 33

Clinical case The case concerns a 10-year-old boy of African origin, who suffered a mild ocular trauma to the left eye. Upon examination, the best visual acuity was 0.2 using the Snellen scale, with a 1mm height hyphema, intraocular pressure (IOP) of 12mmHg on left eye, with an increase up to 20mmHg within 72h. With a positive test for sickle cell disease, it was decided to treat medically with transcorneal oxygen therapy. Clearing of the anterior chamber was achieved, with and improvement in the best visual acuity to 0.8, and lowering of IOP to 8mmHg.
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PMID:Transcorneal oxygen therapy for persistent hyphema in a patient with sickle cell disease. 2990 49