Cases reported "Anemia, Sickle Cell"

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1/29. Extensive spontaneous retroperitoneal hemorrhage: an unusual complication of heparin anticoagulation during pregnancy.

    A 27-year-old patient at 13 weeks' gestation maintained on subcutaneous heparinization due to hemoglobin S and hemoglobin c (SC) sickle cell disease and previous splenic vein thrombosis presented with spontaneous acute onset of severe left lower abdominal and groin pain. The pain, which radiated to the anterior aspect of the thigh, was associated with nausea and vomiting and was exacerbated by extension of the left lower extremity. The patient was hemodynamically stable, yet during the first 24 h of hospitalization a marked decrease in hematocrit from 29% to 22% occurred. Contrast computed tomography (CT) revealed an extensive abdominal-pelvic, retroperitoneal hematoma extending approximately 15 cm in length from above L5 cephalad to below the greater trochanter of the left femur caudally. The retroperitoneal hemorrhage self-tamponaded and did not require surgical management. The dosage of heparin was decreased and maintained with appropriate activated partial prothrombin (aPTT) levels. To our knowledge, this is the first report of a spontaneous retroperitoneal hemorrhage complicating heparin anticoagulation in pregnancy. Unusual hemorrhagic complications of anticoagulation therapy are discussed.
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keywords = vein
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2/29. venous thromboembolism, factor v Leiden, and methylenetetrahydrofolate reductase in a sickle cell anemia patient.

    Vaso-occlusive crisis is the most common cause of morbidity in patients with sickle cell anemia (SCA). central nervous system involvement that leads to hemiplegia is the most frequent neurological complication in those patients. Peripheral deep venous thromboembolism was not reported in SCA patients. activated protein c resistance is associated with an increased risk of thrombophilia. The authors report an SCA patient with recurrent cerebrovascular accident and deep venous thrombosis. activated protein c resistance due to factor v Leiden heterozygous and heterozygocity for the methylenetetrahydrofolate reductase were diagnosed and suspected to be the risk factors that contribute to the development of the deep vein thrombosis in this SCA patient.
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keywords = vein
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3/29. spinal cord compression by extramedullary hemopoietic tissue in sickle cell anemia. Case report.

    The authors report a patient with sickle cell anemia who suffered from paraplegia of 18 months duration due to spinal cord compression by a hemopoietic mass. Recovery following removal of the mass was complete.
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ranking = 171.33607796823
keywords = compression
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4/29. Orbital involvement in sickle cell disease: a report of five cases and review literature.

    PURPOSE: To present five cases of orbital infarction in sickle cell disease and review relevant literature. METHOD: We reviewed the hospital records of 5 patients with sickle cell disease who developed a periorbital swelling during a vaso-occlusive crisis and were managed at our hospital between April 1992 and June 2000. RESULTS: The 5 patients (4 with homozygous sickle cell disease and 1 with sickle cell-beta-thalassaemia disease) were aged 6-15 years with a history of multiple admissions for vaso-occlusive crises. The periorbital swelling spread to the orbit in 4 cases and resulted in proptosis (2 cases), restriction of ocular motility and visual impairment. In all 4 cases, computed tomography and/or magnetic resonance imaging of the orbits showed a mass adjacent to the orbital wall. In 2 cases the mass was identified as a haematoma. Orbital wall infarction was demonstrated in 3 cases by bone/bone marrow scintigraphy. Epidural haematomas were detected by computed tomography in one case. All patients received intravenous fluids, analgesics, broad spectrum antibiotics and steroids, as well as simple or exchange transfusion, and responded well to medical management. CONCLUSIONS: infarction of orbital bones during vaso-occlusive crises in sickle cell disease presents acutely with a rapidly progressive periorbital swelling. Haematomas frequently complicate the condition and, along with the inflammatory swelling, may lead to orbital compression syndrome. The condition is therefore sight-threatening, and necessitates prompt diagnosis and appropriate management for resolution without adverse sequelae. Imaging techniques are invaluable in the evaluation of patients. The majority of cases resolve with conservative treatment that includes steps to combat the vaso-occlusive crisis and use of systemic steroids under antibiotic cover.
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ranking = 58819.303735917
keywords = compression syndrome, compression
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5/29. Vertebral bone collapse in sickle cell disease: a report of two cases.

    We describe two female children both nine years of age with sickle cell anaemia and compression deformity of three successive lumbar vertebrae in one child and collapse of one lumbar vertebra in the other. Management for the two children included analgesics, antibiotics and application of a lumbar jacket for stabilisation of the spine. In both patients the vertebral bodies remodelling with re-generation of the tissue. This report is being made to highlight the improved chances of response with early detection and adequate management.
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ranking = 34.267215593647
keywords = compression
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6/29. An unusual etiology of hypertension in a 5-year-old boy.

    Ganglioneuromas are rare benign tumors of neural crest origin, arising from ganglia of the sympathetic nervous system and adrenal medulla. These masses are usually detected during the first 2 decades of life and are generally discovered incidentally. We present a 5-year-old boy with sickle beta-thalassemia whose hypertension is caused by a perihilar ganglioneuroma encasing the right renal artery and distorting the right renal vein. The tumor was resected and the child's blood pressure subsequently normalized.
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keywords = vein
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7/29. Central retinal vein occlusion in sickle cell disease.

    Central retinal vein occlusion has not been reported previously in patients with sickle cell anemia. We describe the case of a 31-year-old man with sickle cell anemia who developed this complication. The search for risk factors for central retinal vein occlusion in this young patient revealed protein s deficiency and a history of iron deficiency. He was treated with anticoagulation therapy, and his vision improved gradually.
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ranking = 6
keywords = vein
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8/29. Sickle beta-thalassemia presenting as orbital compression syndrome.

    Orbital compression syndrome is caused by disorders of varying etiologies involving the orbit and presents with ocular and extraocular dysfunction. Ocular involvement of sickle cell disease is uncommon. A 17-year-old male presented with low backache and proptosis of both eyes of 5 days duration without past history of pain crisis or transfusion. Examination revealed pallor, icterus, bilateral proptosis, conjuctival chemosis, and symmetrical restriction of ocular movements with preserved visual acuity. He was drowsy with no other focal deficits. The fundus showed macular edema, venous engorgement, and no hemorrhage. His peripheral smear showed presence of sickle cells. Computed tomography (CT) scan of the orbit revealed orbital subperiosteal hematomas. CT head images showed epidural hematoma in the frontal lobe. High-performance liquid chromatography (HPLC) and mutation studies revealed sickle beta-thalassemia in the patient. He was managed with supportive care, transfusions to keep hemoglobin above 10 g/dl, and hydroxyurea. The patient recovered fully and remained well during follow-up of 12 months. Our case was unique for presenting as orbital compression syndrome without any history of vaso-occlusive crisis.
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ranking = 352915.8224155
keywords = compression syndrome, compression
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9/29. Are spontaneous epidural haematoma in sickle cell disease a rare complication? A report of two new cases.

    BACKGROUND: Sickle cell anaemia, an autosomal recessive disease relatively common among the black races, gives rise sometimes to neurological complications. Among these, spontaneous epidural haematoma constitutes a rare event that is not always easy to treat in the Third world conditions. methods:Two new cases are described and their pathology is compared with the five already described cases in the literature. A vaso-occlusive pathological process as in the orbital compression syndrome is thought to be implicated in the generation of the spontaneous epidural haematoma. RESULTS:When facing an epidural haematoma as a complication of sickle cell disease in a hospital of the Third world conditions, a cautious attitude towards surgery should be observed because of the high complication rate. If the relation between the haematoma and the anaemia is not immediately apparent, we are in favour of starting treatment with antibiotics.
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ranking = 58819.303735917
keywords = compression syndrome, compression
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10/29. portal vein thrombosis following laparoscopic surgery in a patient with sickle cell disease.

    We report a case of portal vein thrombosis following prolonged laparoscopic intervention in a patient with Sickle cell disease. The operation combined a laparoscopic splenectomy, cholecystectomy, appendectomy. Presentation was insidious with vague abdominal pain and persistent postoperative pyrexia. The literature is reviewed, possible pathogeneses are discussed in the light of currently available data, and a strategy is suggested to recognize, avoid, and prevent this rare but potentially lethal complication.
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ranking = 5
keywords = vein
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