Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.1.4.1 (phosphodiesterase)
 18,767 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Priapism due to sickle cell disease is a common but less well characterized complication of the disorder. It represents a "medical emergency" with the key determinant of outcome being the duration of penile ischaemia and time to detumescence of <4 h associated with a successful treatment outcome. Management can be outpatient-based and consists of pre-emptive strategies for early stuttering attacks based on prior health education of the association between the 2 disorders, non pharmacological management, outpatient penile aspiration and irrigation with or without instillation of alpha and beta adrenergic agonists for acute episodes and secondary prophylaxis to prevent the high rates of recurrences. The evidence to recommend medical prophylaxis is sparse but based on a consensus of experts and small phase 2 or III clinical trials. A clearer understanding of the molecular mechanism(s) involving normal and dysregulated erectile physiology, scavenger haemolysis and nitric oxide pathway paves way for the use of phosphodiesterase type 5 inhibitors in medical prophylaxis of stuttering attacks. These agents will need to be studied in multi-centre randomized phase III trials before they become standard of care. A multidisciplinary team approach is required to enhance "sexual wellness" and prevent erectile dysfunction in this sexually vulnerable group.
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PMID:How I manage priapism due to sickle cell disease. 2329 42

Recurrent ischaemic priapism also known as stuttering priapism is an uncommon form of ischaemic priapism, and its treatment is not yet clearly defined. If left untreated, it may evolve into classic form of acute ischaemic priapism and lead to erectile dysfunction due to fibrosis of corpora cavernosa. Several drugs have been proposed with variable results and only supported with level three or four of evidence. Hormonal therapy such as cyproterone acetate, oestrogen, bicalutamide or Lh-Rh agonist are often effective but can cause side effects such as hypogonadal state and infertility. Other medical options are 5-alpha-reductase and phosphodiesterase-5 inhibitors, ketoconazole, baclofen, digoxin, gabapentin and beta-2-agonist terbutaline. We report the first case of stuttering priapism treated with beta-2-agonist salbutamol.
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PMID:Idiopathic stuttering priapism treated with salbutamol orally: a case report. 2603 21