Cases reported "Anemia, Sickle Cell"

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1/26. Megalophallus as a sequela of priapism in sickle cell anemia: use of blood oxygen level-dependent magnetic resonance imaging.

    priapism is a common complication of sickle cell anemia. We report a little known sequela of priapism: painless megalophallus, with significant penile enlargement. The patient had had an intense episode of priapism 9 years previously and his penis remained enlarged. Blood oxygen level-dependent magnetic resonance imaging revealed enlarged, hypoxic corpora cavernosa. Megalophallus probably resulted from permanent loss of elasticity of the tunica albuginea due to severe engorgement during the episode of priapism. This sequela needs to be recognized by physicians because no intervention is necessary and sexual function seems to remain intact.
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2/26. priapism of ambiguous classification in a sickle cell patient.

    We report a case of recurrent priapism in a sickle cell patient who had undergone physiological transformation from a low-flow state to a mixed high-flow/low-flow picture. An oral alpha-adrenergic agonist/histamine-1 antagonist combination maintained a balance of potency without stutter in the latter state, whereas an oral alpha-adrenergic agonist in the early part of his course did not prevent priapic progression. Concomitant use of a non-steroidal anti-androgenic agent rendered him impotent despite good libido. Other etiologies should be considered in the setting of recurrent priapism and failed standard therapies; duplex ultrasound is useful in order to delineate the hemodynamics. This case supports contentions that priapism is a spectrum phenomenon, and raises questions regarding the mechanism by which alpha-adrenergic agents prevent priapic progression.
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ranking = 0.42857142857143
keywords = priapism
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3/26. Epidural analgesia in a child with sickle cell disease complicated by acute abdominal pain and priapism.

    We describe a case of a 9-yr-old child with sickle cell disease complicated by abdominal vaso-occlusive crisis and priapism. Both complications were successfully treated with a combination of epidural local anesthetics and morphine.
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keywords = priapism
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4/26. priapism associated with asplenic state.

    priapism may be primary (idiopathic) or secondary to sickle cell anemia, trauma, leukemia, drugs, venous thromboembolic diseases, and other less common disorders. This study concerns 21 patients with priapism treated during a period of ten years. Nine patients (43%) had sickle cell anemia. Of the 12 individuals (57%) classified as idiopathic, 3 (25%) had previously undergone surgical splenectomy for benign conditions. Considering the propensity for this unusual condition to develop in patients with hemoglobinopathy-induced hyposplenism, the possibility of a relationship between the asplenic state and priapism is considered.
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ranking = 0.28571428571429
keywords = priapism
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5/26. Paediatric priapism--treatment conundrum.

    priapism is a rare condition in childhood. The majority of reported cases are boys with sickle cell disease, in whom nonoperative management may be successful when patients present early. We report a 14-year-old boy with sickle cell disease who presented with priapism of 72-h duration and was successfully treated by bilateral saphenocorporal shunts.
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ranking = 0.71428571428571
keywords = priapism
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6/26. Neurologic events after partial exchange transfusion for priapism in sickle cell disease.

    We describe six boys with homozygous sickle cell disease, aged 7 to 13 years, in whom acute, severe neurologic abnormalities developed 1 to 11 days after partial exchange transfusion was performed to treat priapism that was unresponsive to more conservative therapy. Hemoglobin levels were 10.5 to 13.4 gm/dl (mean 12.1 gm/dl), and hemoglobin S levels were 18% to 33% (mean 27%) before the onset of neurologic complications. Severe headache was the initial finding in five patients, four of whom had increased intracranial pressure and three of whom required tracheal intubation and hyperventilation. Four patients had seizures; three had focal neurologic deficits for more than 24 hours. Cerebral arteriography demonstrated vascular abnormalities, including irregularity, stenosis, and complete occlusion of vessels. patients treated with regular erythrocyte transfusions had no recurrence of neurologic signs or symptoms when hemoglobin S levels were kept at 30% to 50%. The occurrence of serious neurologic complications after partial exchange transfusion in patients with homozygous sickle cell disease from three centers indicates the possibility of a causal relationship between the events. Early and thorough investigation of neurologic symptoms, especially severe headache, is warranted in this clinical setting.
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ranking = 0.71428571428571
keywords = priapism
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7/26. Long-term oral phosphodiesterase 5 inhibitor therapy alleviates recurrent priapism.

    OBJECTIVES: Recurrent ischemic priapism describes a disorder of repeated episodes of prolonged penile erection that frequently leads to devastating complications of erectile tissue damage and erectile dysfunction. A mechanistic role for dysregulated phosphodiesterase 5 (PDE5) in the deranged smooth muscle response of the corpus cavernosum of the penis offers new understanding about the pathogenesis of the disorder and suggests that PDE5 may serve as a molecular target for its treatment and prevention. We explored the use of PDE5 inhibitors to treat recurrent priapism, based on the hypothesis that the erection regulatory function of PDE5 would be regularized by this treatment and protect against further episodes. methods: We administered PDE5 inhibitors using a long-term therapeutic regimen to 3 men with sickle cell disease-associated priapism recurrences and 1 man with idiopathic priapism recurrences. RESULTS: Long-term PDE5 inhibitor treatment alleviated priapism recurrences. CONCLUSIONS: These observations support the hypothesis that PDE5 dysregulation exerts a pathogenic role for priapism associated with hematologic dyscrasias, as well as idiopathic priapism. Although these preliminary findings suggest that continuous, long-term PDE5 inhibitor therapy may be useful as a preventative strategy for priapism, additional evaluation in the form of a controlled clinical trial is needed.
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ranking = 1.7142857142857
keywords = priapism
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8/26. priapism in sickle cell anaemia: a case report.

    priapism is an unusual and distressing complication of sickle cell anaemia and its management has been varied and generally unsatisfactory. We report priapism in a Libyan boy with sickle cell anaemia, managed successfully by blood transfusion.
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ranking = 0.14285714285714
keywords = priapism
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9/26. 'priapism' of the turbinates: a cause of nasal obstruction in sickle cell anaemia.

    A cause of nasal obstruction in sickle cell anaemia not previously described in the literature is presented. The pathophysiology is the same as in priapism because of the similarities in the vascular arrangement of the turbinate and the penis. Various treatment options are considered but the best results were achieved by surgical reduction of the inferior turbinates.
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ranking = 0.14285714285714
keywords = priapism
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10/26. Corporectomy for intractable sickle-associated priapism.

    priapism is a complication of sickle cell disease, and for those with severe prolonged attacks, it is serious, often resulting in permanent sexual impairment. Treatment is frustrating, and psychological consequences may be profound. A 20-year-old sickle cell patient with intractable priapism associated with intense pain and penile gigantism underwent multiple but unsuccessful medical and surgical treatment regimens. Finally, corpora cavernosa corporectomy was performed. The pathologic findings of extensive vascular thrombosis and stromal fibrosis underscore the irreversibility of this process and explain the inevitable impotence. Pain and sexual impairment were associated with serious psychological difficulties and suicide attempts. counseling and close follow-up have improved his outlook considerably. He has been pain-free for 1 year, and future management includes consideration of placement of a penile prosthesis.
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ranking = 0.71428571428571
keywords = priapism
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