Keywords
priapism
emergency
epidemiology
mechanism
diagnosis
treatment
How to Cite
Abstract
Priapism is a frequent complication in patients with sickle cell disease, putting their sexual and reproductive health at risk. This systematic review aims to analyze existing studies on priapism in this context comprehensively. The results show that priapism, particularly the ischaemic type, is prevalent in men with sickle cell disease, especially those carrying HbSS genotypes. Risk factors include vaso-occlusive crises, severe anemia, infections, and dehydration. Pathophysiological mechanisms involve endothelial dysfunction, vasoconstriction, local hypoxia, and inflammation. Diagnosis is based on clinical assessment and investigations such as penile Doppler ultrasound. Treatments include conservative measures, aspiration of the corpora cavernosa, intracavernous injections of vasoconstrictors, and surgical shunts. Prevention involves specific management of sickle cell anemia, with regular monitoring and raising awareness among the general public. This review highlights the clinical challenges of priapism in sickle cell disease and underlines the importance of a multidisciplinary approach to improve care and quality of life for affected patients.
References
Broderick GA, Kadioglu A, Bivalacqua TJ, Ghanem H, Nehra A, Shamloul R. Priapism: pathogenesis, epidemiology, and management. J Sex Med 2010;7:476–500. https://doi.org/10.1111/j.1743-6109.2009.01625.x
K R, M MA, Fg M, F KR, F NM, A D, et al. Les Complications Uro-Andrologiques de la Drépanocytose au Service d’Urologie du Centre Hospitalier Universitaire de Référence Nationale de N’Djaména.: Complications uro-andrologiques de la drépanocytose. HEALTH SCIENCES AND DISEASE 2021;22. https://doi.org/10.5281/hsd.v22i9.2957
Makani J, Williams TN, Marsh K. Sickle cell disease in Africa: burden and research priorities. Ann Trop Med Parasitol. 2007 Jan;101(1):3-14. https://doi.org/10.1179/136485907x154638
Piel FB, Hay SI, Gupta S, Weatherall DJ, Williams TN. Global burden of sickle cell anaemia in children under five, 2010-2050: modelling based on demographics, excess mortality, and interventions. PLoS Med. 2013;10(7):e1001484. https://doi.org/10.1371/journal.pmed.1001484
Dodo R, Zohoun A, Baglo T, Mehou J, Anani L. Urgences drépanocytaires au Service des Maladies du Sang du Centre National Hospitalier Universitaire-Hubert Koutoukou Maga de Cotonou, Benin. Pan Afr Med J. 2018; 30: 192. https://doi.org/10.11604%2Fpamj.2018.30.192.15931
Russo G, De Franceschi L, Colombatti R, Rigano P, Perrotta S, Voi V, et al. Current challenges in the management of patients with sickle cell disease - A report of the Italian experience. Orphanet J Rare Dis 2019;14:120. https://doi.org/10.1186/s13023-019-1099-0
Adeyoju AB, Olujohungbe ABK, Morris J, Yardumian A, Bareford D, Akenova A, et al. Priapism in sickle-cell disease; incidence, risk factors and complications - an international multicentre study. BJU Int 2002;90:898–902. https://doi.org/10.1046/j.1464-410x.2002.03022.x
Priapism and impotence in homozygous sickle cell disease - PubMed n.d. https://pubmed.ncbi.nlm.nih.gov/6159833/ (accessed April 28, 2024).
Priapism in Homozygous Sickle Cell Disease: A 40-year Study of the Natural History - PubMed n.d. https://pubmed.ncbi.nlm.nih.gov/26426165/ (accessed April 28, 2024).
Bouya PA, Odzébé AWS, Koumou Moritoua RD, Elira Dokékias A. Priapisme aigu drépanocytaire: analyse de 20 cas traités au CHU de Brazzaville. Basic Clin Androl 2011;21:34–7. https://doi.org/10.1007/s12610-010-0110-3
Gaye O, Seck M, Gueye S, Jalloh M, Ndiaye M, Niang L, et al. Évaluation de la connaissance du priapisme chez les drépanocytaires sénégalais. Progrès En Urologie - FMC 2023;33:S31–2. https://doi.org/10.1016/j.fpurol.2023.07.045
NCT05142254. A Trial for Prevention of Recurrent Ischemic Priapism in Men With Sickle Cell Disease: a Pilot Study. Https://ClinicaltrialsGov/Show/NCT05142254 2022. https://doi.org/10.1002/central/CN-02353419
Okoko AR, Odzébé ASW, Moyen E, Ekouya Bowassa G, Oko APG, Mbika-Cardorelle A, et al. Priapisme chez l’enfant et l’adolescent drépanocytaire homozygote à Brazzaville. Progrès En Urologie 2014;24:57–61. https://doi.org/10.1016/j.purol.2013.04.021
Habou O, Adamou H, Amadou Magagi I, Amadou S, Magagi A, Malam Bade A, et al. Prise en charge du priapisme ischémique aigu à l’Hôpital National de Lamordé de Niamey. African Journal of Urology 2017;23:338–41. https://doi.org/10.1016/j.afju.2017.03.003
Gopalakrishnan A, Valerio M, Grobet-Jeandin E. Le priapisme, une urgence urologique: définition et prise en charge. Urol Prax 2023;25:109–14. https://doi.org/10.1007/s41973-023-00225-1
Epidemiology and treatment of priapism in sickle cell disease | Cochrane Library n.d. https://doi.org/10.1002/central/CN-02505523
Jin YC, Gam SC, Jung JH, Hyun JS, Chang KC, Hyun JS. Expression and Activity of Heme Oxygenase‐1 in Artificially Induced Low‐Flow Priapism in Rat Penile Tissues. The Journal of Sexual Medicine 2008;5:1876–82. https://doi.org/10.1111/j.1743-6109.2008.00886.x
Droupy S, Giuliano F. Priapismes. Progrès En Urologie 2013;23:638–46. https://doi.org/10.1016/j.purol.2013.01.026
Dodo R, Zohoun A, Baglo T, Mehou J, Anani L. Urgences drépanocytaires au Service des Maladies du Sang du Centre National Hospitalier Universitaire-Hubert Koutoukou Maga de Cotonou, Benin. Pan African Medical Journal 2018;30.
Hématurie massive chez le drépanocytaire hétérozygote : a propos de deux cas n.d. https://www.clinical-medicine.panafrican-med-journal.com/content/article/5/6/full/ (accessed April 8, 2024).
PRIAPISM - Uroweb. Uroweb - European Association of Urology n.d. https://uroweb.org/guidelines/sexual-and-reproductive-health/chapter/priapism (accessed May 26, 2024).
Diagnostic et prise en charge du priapisme : lignes directrices AUA/SMSNA (2022) - American Urological Association n.d. https://www.auanet.org/guidelines-and-quality/guidelines/diagnosis-and-management-of-priapism-aua/smsna-guideline-(2022)#x16579 (accessed May 26, 2024).
Intracavernosal Irrigation by Cold Saline as a Simple Method of Treating Iatrogenic Prolonged Erection | The Journal of Sexual Medicine | Oxford Academic n.d. https://academic.oup.com/jsm/article-abstract/2/2/248/6863082 (accessed April 8, 2024).
Bondil P, Descottes J-L, Salti A, Sabbagh R, Hamza T. Conservative treatment of venous priapism based on a series of 46 cases: Puncture, pharmacological detumescence or penile cooling? Progres en Urologie 1997;7:433–41.
Clinical Outcomes of Periprocedural Antithrombotic Therapy in Ischemic Priapism Management | The Journal of Sexual Medicine | Oxford Academic n.d. https://academic.oup.com/jsm/article-abstract/17/11/2260/6973816 (accessed April 8, 2024).
Salem EA, El Aasser O. Management of ischemic priapism by penile prosthesis insertion: prevention of distal erosion. J Urol 2010;183:2300–3. https://doi.org/10.1016/j.juro.2010.02.014
Upadhyay J, Shekarriz B, Dhabuwala CB. Penile implant for intractable priapism associated with sickle cell disease. Urology 1998;51:638–9. https://doi.org/10.1016/s0090-4295(97)00704-8
Ralph DJ, Garaffa G, Muneer A, Freeman A, Rees R, Christopher AN, et al. The immediate insertion of a penile prosthesis for acute ischaemic priapism. Eur Urol 2009;56:1033–8. https://doi.org/10.1016/j.eururo.2008.09.044
Zacharakis E, Garaffa G, Raheem AA, Christopher AN, Muneer A, Ralph DJ. Penile prosthesis insertion in patients with refractory ischaemic priapism: early vs delayed implantation. BJU Int 2014;114:576–81. https://doi.org/10.1111/bju.12686
Johnson MJ, Kristinsson S, Ralph O, Chiriaco G, Ralph D. The surgical management of ischaemic priapism. Int J Impot Res 2020;32:81–8. https://doi.org/10.1038/s41443-019-0197-9
Broderick GA. Priapism and sickle-cell anemia: diagnosis and nonsurgical therapy. J Sex Med 2012;9:88–103. https://doi.org/10.1111/j.1743-6109.2011.02317.x
Burnett AL, Bivalacqua TJ. Priapism: Current Principles and Practice. Urologic Clinics of North America 2007;34:631–42. https://doi.org/10.1016/j.ucl.2007.08.006
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