Cases reported "Sickle Cell Trait"

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1/15. Transcorneal oxygen therapy for glaucoma associated with sickle cell hyphema.

    PURPOSE: To study three patients with glaucoma caused by sickle cell hyphema who were successfully treated with transcorneal oxygen therapy. methods: case reports. Three patients with increased intraocular pressure caused by sickle cell hyphema were administered transcorneal oxygen therapy using humidified oxygen at a flow rate that ranged from 1 to 3 l/minute. RESULTS: All three patients had a dramatic reduction in their intraocular pressure within hours of receiving oxygen therapy. No complications were associated with the oxygen therapy. CONCLUSION: Transcorneal oxygen therapy can reduce intraocular pressure in patients with glaucoma from sickle cell hyphema. Further study is warranted to evaluate this new therapy.
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2/15. Latent proliferative sickle cell retinopathy in sickle cell trait.

    PURPOSE: To describe a patient with sickle cell trait who developed latent proliferative sickle cell retinopathy after mild blunt trauma. METHOD: Case Report. A 20-year-old man with unilateral Stage 3 sickle retinopathy associated with an ischaemic ridge presenting three years after the initial mild blunt ocular trauma. RESULTS: Fundus examination of the left eye showed an ischaemic ridge delineating avascular from vascular retina. fluorescein angiography of the left eye showed an avascular peripheral retina and multiple sea fan neovascularization. Blood studies showed him to be Hb AS. CONCLUSIONS: In our patient the proliferative changes were the result of his initial mild trauma associated with an increase in the intraocular pressure. The latent development of the sea-fan neovascularization associated with an ischaemic ridge is unusual. Advice about potential complications to patients with Hb AS after ocular trauma is advocated.
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3/15. An unusual case of uveitis-glaucoma-hyphema syndrome.

    PURPOSE: To report a case of uveitis-glaucoma-hyphema (UGH) syndrome in which anterior chamber paracentesis led to the diagnosis of sickle cell trait. DESIGN: Observational case report. methods: A 43-year-old Cuban pseudophakic male was seen multiple times over a 3-year period complaining of floaters and blurry vision in his left eye. He was noted to have an inferotemporally displaced posterior chamber intraocular lens and recurrent microhyphemas with elevated intraocular pressure (IOP) readings between 29 and 46 mm Hg with each episode. He was diagnosed with UGH syndrome. Posterior chamber intraocular lens explantation and anterior chamber washout was performed. The aqueous fluid was submitted for cytopathologic examination. RESULTS: Postoperatively, the patient's symptoms resolved and he had no further hemorrhages or elevated IOP readings. Cytopathology of the aspirate revealed sickled red blood cells. CONCLUSIONS: Microscopic examination of aqueous fluid can be a valuable tool in diagnosing ophthalmic manifestations of systemic disease.
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4/15. Migratory white-without-pressure retinal lesions.

    Nine patients had white-without-pressure retinal lesions. These lesions were seen in areas of vitreoretinal adhesions. The configuration and location of these lesions changed over variable periods of time. The cause of this pecular migration is unknown, but may be related to separation and re-creation of vitreoretinal adhesions. The migratory nature of white-without-pressure areas has not, to our knowledge, previously been reported in the literature.
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5/15. Hemoglobin jamaica plain--a sickling hemoglobin with reduced oxygen affinity.

    A baby girl presented with symptomatic sickle cell disease exacerbated by mild hypoxemia, despite a newborn-screening diagnosis of sickle cell trait. dna sequencing of the beta globin gene revealed that her maternal beta globin allele was normal. Her paternal allele had not only the expected sickle-trait mutation, betaGlu6Val, but also a second, charge-neutral mutation, betaLeu68Phe. Analysis of the patient's hemoglobin revealed that the double-mutant protein, which we called "hemoglobin jamaica Plain," had severely reduced oxygen affinity. Structural modeling suggested destabilization of the oxy conformation as a molecular mechanism for sickling in a heterozygote at an ambient partial pressure of oxygen.
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6/15. lung isolation in a child with unilateral necrotizing clostridium perfringens pneumonia.

    OBJECTIVE: To describe lung isolation and the selective application of continuous positive airway pressure using an endobronchial blocker in a patient with sickle cell disease and unilateral necrotizing clostridium perfringens pneumonia. DESIGN: Case report. SETTING: Pediatric intensive care unit. PATIENT: A 12-yr-old male with sickle cell disease developed persistent necrotizing pneumonia of the left lung following exchange transfusion for acute chest syndrome and hyper-hemolytic syndrome. INTERVENTIONS: An endobronchial blocker was placed into the left main stem bronchus for lung isolation and application of continuous positive airway pressure to the left lung for 48 hrs. MEASUREMENTS AND MAIN RESULTS: After 14 days of persistent atelectasis of the left lung despite thorascopic decortication and multiple bronchoscopies, our patient had substantial lung aeration within 48 hrs of continuous positive airway pressure applied via the endobronchial blocker. lung resection was avoided and the patient was successfully extubated 2 days after removal of the blocker. CONCLUSIONS: This case report demonstrates a therapeutic application of prolonged lung isolation and differential ventilation in a patient with an airway too small for commercially available double-lumen endotracheal tubes. The apparent success of this intervention suggests the feasibility of selective ventilation in pediatric patients and highlights a novel application of the bronchial blocker.
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7/15. Complicated hyphaema: think sickle.

    Two cases of complicated hyphaema associated with sickle cell trait are presented. The pathophysiology, diagnosis and management of raised intraocular pressure in sickle cell trait are discussed.
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8/15. hypertension in sickle cell disease.

    We report a young male with sickle cell trait who developed severe hypertension and splenic infarction soon after travel to a high altitude. hypertension persisted for three days after a diagnostic laparotomy. His blood pressure then continued to be normal over the next one and a half years. Red cell sludging in the small vessels of the kidney possibly activated the renin angiotensin system.
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9/15. Superior sagittal sinus thrombosis with infarction in sickle cell trait.

    An adolescent female with sickle cell trait presented with acute neurologic deterioration during treatment for pseudotumor cerebri. Cranial computed tomography, initially normal, subsequently revealed multiple hemorrhagic infarctions. Suspected superior sagittal sinus thrombosis was confirmed by cerebral angiography. Superior sagittal sinus thrombosis associated with sickle cell trait is exceedingly rare, and the accompanying increased intracranial pressure may require aggressive management.
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10/15. Sickle cell hyphema with secondary glaucoma in a non-black patient.

    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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