The Role of Red Blood Cell Exchange Transfusion in the Treatment and Prevention of Complications of Sickle Cell Disease

Danielson, Constance

doi:10.1046/j.1526-0968.2002.00396.x

Cited by 66 publications

(46 citation statements)

References 122 publications

(115 reference statements)

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“…12 It is known that during prolonged, normal erection, the static blood within the corporal sinuses gradually loses oxygen tension and pH. 13 In this relative hypoxic and acidic environment, the erythrocytes of the sicklemic patients become more sickled, further sludge the outflowing veins, and thus worsen the hypoxic condition.…”

Section: S9mentioning

confidence: 99%

Tissue expression, distribution, and regulation of PDE5

Lin

2004

Int J Impot Res

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Phosphodiesterase 5 (PDE5) has been identified in many species. The three isoforms, PDE5A1, PDE5A2, and PDE5A3, differ only in their N-terminal sequence. PDE5A1 and PDE5A2 are ubiquitous, but PDE5A3 is specific to smooth muscle. The initial three exons (A1-A3-A2) of PDE5A, located on chromosome 4q26, are alternative first exons encoding the isoform-specific sequences. The PDE5A promoter overlaps with the A1-specific exon, while the PDE5A2 promoter is located between the A3-and A2-specific exons. Both respond to cyclic guanosine monophosphate (cGMP) or cyclic adenosine monophosphate (cAMP) stimulation. The PDE5A2 promoter contains an Sp1-binding sequence critical for basal and cGMP/cAMP-inducible promoter activities. PDE5A is induced by adjacent sequences (enhancers) containing Sp1-binding sites. The potential role of PDE5A promoters in the tachyphylaxis effect of PDE5 inhibitors is currently being investigated. Preliminary data suggest that hypoxia might down-regulate PDE5A promoters, implying an involvement of PDE5 in stuttering priapism.

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Section: S9mentioning

confidence: 99%

Tissue expression, distribution, and regulation of PDE5

Lin

2004

Int J Impot Res

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“…In addition, the efficiency of this treatment is controversial [26,27], and exchange transfusion has been associated with neurologic events [28][29][30].…”

Section: Introductionmentioning

confidence: 99%

Follow‐up of sickle cell disease patients with priapism treated by hydroxyurea

Saad¹,

Lajolo²,

Gilli³

et al. 2004

American J Hematol

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Hydroxyurea is one of the most successfully used therapies for sickle cell disease. Results of many clinical trials point to hydroxyurea administration for patients with frequent painful crises and acute chest syndrome. Priapism is one of the complications that could be prevented by hydroxyurea, but there are few reports demonstrating the results. Since November 1993, hydroxyurea has been used in our clinic for preventing priapism in patients with stuttering or major attacks who are still capable of achieving intercourse on demand. Five patients were enrolled in the study, and 4 cases benefited by this treatment. After the initial treatment for the acute attack, all five patients developed stuttering priapism. Hydroxyurea was then introduced at the initial dose of 10 mg/kg, and as the hydroxyurea dosage increased, the number or length of priapism episodes decreased. One to two months after the maximal dose (20-35 mg/kg) was introduced, the episodes disappeared. In two patients, we were forced to administer over 30 mg hydroxyurea/kg to abort the episodes, and, in another patient, 25 mg/kg was necessary. All patients present normal sexual activity. Hydroxyurea was discontinued in two patients, but stuttering priapism reappeared. Hydroxyurea was then re-introduced, and priapism disappeared. One patient, using 20 mg hydroxyurea/kg, had a 6-year remission of priapism after hydroxyurea administration; however, he experienced stuttering priapism, 1 month before a major attack, that progressed to impotence. During that month, he did not seek medical attention. In conclusion, the data here presented suggests that hydroxyurea may prevent priapism attacks in sickle cell disease, probably at higher doses than usually prescribed for painful crisis prevention. Am.

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“…28,78,80,81 Consequently, a recent expert panel recommended against the use of transfusion therapy for the treatment of priapism. 82 …”

Section: -53mentioning

confidence: 99%

How I treat priapism

Anele

Resar

et al. 2015

Blood

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Priapism is a disorder of persistent penile erection unrelated to sexual interest or desire. This pathologic condition, specifically the ischemic variant, is often associated with devastating complications, notably erectile dysfunction. Because priapism demonstrates high prevalence in patients with hematologic disorders, most commonly sickle cell disease (SCD), there is significant concern for its sequelae in this affected population. Thus, timely diagnosis and management are critical for the prevention or at least reduction of cavernosal tissue ischemia and potential damage consequent to each episode. Current guidelines and management strategies focus primarily on reactive treatments. However, an increasing understanding of the molecular pathophysiology of SCD-associated priapism has led to the identification of new potential therapeutic targets. Future agents are being developed and explored for use in the prevention of priapism.

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The Role of Red Blood Cell Exchange Transfusion in the Treatment and Prevention of Complications of Sickle Cell Disease

Cited by 66 publications

References 122 publications

Tissue expression, distribution, and regulation of PDE5

Tissue expression, distribution, and regulation of PDE5

Follow‐up of sickle cell disease patients with priapism treated by hydroxyurea

How I treat priapism

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