Oral phosphodiesterase type 5 inhibitors and priapism: A VigiBase analysis.

PURPOSE: To explore the differences of priapism events among a diverse cohort taking erectogenic medicines (i.e., phosphodiesterase type 5 inhibitors [PDE5i] and intracavernousal drugs). METHODS: We queried the World Health Organization global database of individual case safety reports (VigiBase) for records of the adverse drug reactions (ADR) with sildenafil, tadalafil, avanafil, vardenafil, papaverine, and alprostadil. Disproportionality analyses (case/non-case approach) were performed to assess the reporting odds ratio (ROR) of priapism reporting in PDE5i consumers compared to intracavernousal drug recipients. RESULTS: From a total of 133 819 ADR events for erectogenic medications, 632 were priapism (PDE5is: n = 550, 0.41%; intracavernousal drugs: n = 82, 9.92%). Priapism disproportionality signals from intracavernousal drugs were 25 times stronger than PDE5is (ROR = 34.7; confidence interval [CI] 95%: 27.12-43.94 vs. ROR = 1.38; 95% CI: 1.24-1.54). For all PDE5i agents, the 12-17 years age group had the highest ROR (9.49, 95% CI: 3.76-19.93) followed by 2-11 years (4.31, 95% CI: 1.57-9.4). Disproportionality signals for consumers under 18 for both all PDE5is as a whole (ROR = 4.57, 95% CI: 2.48-7.73) and sildenafil (ROR = 4.89, 95% CI: 2.51-8.62) were stronger than individuals 18 or older (ROR = 1.06, 95% CI: 0.93-1.21 and ROR = 1.08, 95% CI: 0.91-1.26, respectively). CONCLUSIONS: PDE5i use shows disproportionate priapism signals which are higher in young patients.

Association between priapism and HIV disease and treatment.

BACKGROUND: Priapism, a urologic emergency, has known associations with certain medical conditions. Many cases are idiopathic, suggesting an opportunity to identify novel risk factors. AIM: We sought to identify medical conditions and pharmaceutical treatments that are associated with priapism using data-mining techniques. METHODS: Using deidentified data in a large insurance claims database, we identified all men (age ≥20 years) with a diagnosis of priapism from 2003 to 2020 and matched them to cohorts of men with other diseases of male genitalia: erectile dysfunction, Peyronie disease, and premature ejaculation. All medical diagnoses and prescriptions used prior to first disease diagnosis were examined. Predictors were selected by random forest, and conditional multivariate logistic regressions were applied to assess the risks of each predictor. OUTCOMES: We identified novel relationships of HIV and some HIV treatments with priapism and confirmed existing associations. RESULTS: An overall 10 459 men with priapism were identified and matched 1:1 to the 3 control groups. After multivariable adjustment, men with priapism had high associations of hereditary anemias (odds ratio [OR], 3.99; 95% CI, 2.73-5.82), use of vasodilating agents (OR, 2.45; 95% CI, 2.01-2.98), use of HIV medications (OR, 1.95; 95% CI, 1.36-2.79), and use of antipsychotic medications (OR, 1.90; 95% CI, 1.52-2.38) as compared with erectile dysfunction controls. Similar patterns were noted when compared with premature ejaculation and Peyronie disease controls. CLINICAL IMPLICATIONS: HIV and its treatment are associated with priapism, which may affect patient counseling. STRENGTHS AND LIMITATIONS: To our knowledge, this is the first study to identify risk factors for priapism utilizing machine learning. All men in our series were commercially insured, which limits the generalizability of our findings. CONCLUSION: Using data-mining techniques, we confirmed existing associations with priapism (eg, hemolytic anemias, antipsychotics) and identified novel relationships (eg, HIV disease and treatment).

Regimented Phosphodiesterase Type 5 Inhibitor Use Reduces Emergency Department Visits for Recurrent Ischemic Priapism.

PURPOSE: We evaluated the real-world effectiveness of regimented phosphodiesterase type 5 inhibitor dosing on recurrent ischemic priapism outcomes using emergency department visits as a proxy for therapeutic control of the disorder. MATERIALS AND METHODS: We performed a retrospective chart review of patients with recurrent ischemic priapism who were started on regimented phosphodiesterase type 5 inhibitor therapy from May 2006 to January 2020. We compared the number of emergency department visits per month during a 6-month period before treatment, during treatment and after treatment discontinuation. We extracted and categorized priapism outcomes such as priapism frequency and duration. RESULTS: Of 216 patients identified with all cause priapism 114 were diagnosed with recurrent ischemic priapism and 42 were initiated on regimented phosphodiesterase type 5 inhibitor therapy. Treatment effectiveness was analyzed for 24 evaluable patients. Priapism etiology was idiopathic in 12 patients (50%), sickle cell disease in 11 (46%) and drug-induced in 1 (4%). The median length of regimented phosphodiesterase type 5 inhibitor use was 3 months (IQR 2-7). Treatment decreased emergency department visits per month by 4.4-fold (p <0.001), priapism duration tiers (p <0.001) and priapism frequency tiers (p <0.001). Of 24 patients 22 (92%) reported improvement in priapism outcomes, 9 of whom reported resolution of recurrent ischemic priapism episodes. A subgroup analysis of 17 patients with recurrent ischemic priapism relapse after treatment discontinuation showed a significant increase in priapism duration (p <0.001) and frequency (p <0.001) but no significant change in emergency department visits per month (p=0.91). CONCLUSIONS: Regimented phosphodiesterase type 5 inhibitor therapy was an impactful treatment in managing recurrent ischemic priapism according to objective and subjective parameters. This study provides further support for the use of regimented phosphodiesterase type 5 inhibitor dosing as a preventive strategy for recurrent ischemic priapism.

Prevalence and Characteristics of Priapism in Sickle Cell Disease.

Priapism is a pathological condition of persistent penile erection in the absence of sexual arousal or desire. It is an urological emergency and its identification is important as lack of prompt treatment can result in erectile dysfunction. The aim of this study was to estimate and describe the characteristics (number of episodes, duration, time of occurrence and evolution) of priapism in patients with sickle cell disease. A bibliographical research was carried out in PubMed, searching for papers published in the last 5 years. Thirteen scientific articles were included in this review. The main results were: 1) the highest prevalence of priapism in males reported was 48.0% and the lowest 0.67%; 2) six studies were carried out on the African Continent (46.1%), three in America (23.1%), two in Europe (15.4%) and two in Asia (15.4%); 3) the main goal of ∼50.0% of the studies was to determine the rate of priapism in patients with sickle cell disease; 4) there was predominance of sickle cell anemia patients [homozygous Hb S (HBB: c.20A>T) genotype]; 5) the minimum age of patients with priapism was 7 years old and the maximum 30 years. In general, the episodes of priapism occurred during sleep, were recurrent and had variable duration. The prevalence of priapism are not real and the explanations include underreporting by patients, lack of awareness by physicians and lack of proper prospective studies. Priapism is a complication that deserves close attention due to its significant impact on the life of the patient with sickle cell disease and, therefore, should be further clarified.

Risk Factors for Priapism Readmission.

INTRODUCTION: Priapism is a urologic emergency with a tendency to recur in some patients. The frequency of, time to, and risk factors for priapism recurrence have not been well characterized. AIM: To identify predictors of priapism readmission. METHODS: We used the New York Statewide Planning and Research Cooperative System database to identify patients presenting to emergency departments with priapism from 2005 through 2014. Patients were tracked up to 12 months after initial presentation. Proportional hazards regression was used to identify risk factors for priapism readmission. MAIN OUTCOME MEASURES: Readmissions for priapism. RESULTS: The analytic cohort included 3,372 men with a diagnosis of priapism. The average age at first presentation was 39 ± 18 years and 40% were black. Within 1 year, 24% of patients were readmitted for recurrent priapism, 68% of whom were readmitted within 60 days. On multivariate analysis, sickle cell disease (hazard ratio [HR] = 2.5, 95% CI = 2.0-3.0), drug abuse or psychiatric disease (HR = 1.9, 95% CI = 1.6-2.2), erectile dysfunction history (HR = 1.9, 95% CI = 1.5-2.3), other than commercial medical insurance (HR = 1.2, 95% CI = 1.0-1.4), and inpatient admission for initial priapism event (HR = 0.5, 95% CI = 0.4-0.6) were significant risk factors for readmission. CONCLUSION: Nearly one fourth of patients with priapism were readmitted for recurrent priapism within 1 year of initial presentation. Most readmissions were within 60 days. Future research should focus on strategies to decrease recurrences in high-risk patients.

Ischemic priapism in South-East Nigeria: Presentation, management challenges, and aftermath issues.

CONTEXT: Ischemic priapism is the more common variety of priapism and often presents late. Outcome is largely dependent on the duration of ischemia. AIMS: To determine the etiology, presentation, management, and outcome of ischemic priapism. SETTINGS AND DESIGN: Retrospective analysis of consecutive cases presenting to three hospitals offering specialist urological services in South-East Nigeria from January 2000 to December 2010. PATIENTS AND METHODS: Fifteen patients were assessed for clinical data and outcome. STATISTICAL ANALYSIS USED: The data were analyzed descriptively and inferentially using Statistical Package for Social Sciences (SPSS version 16, SPSS Inc., Chicago IL, USA) with P < 0.05. RESULTS: Mean age was 30.5 years (standard deviation [SD] =1.63), range: 14-79 years. Onset to presentation interval ranged from 6 h to 28 days. Eight patients (53.3%) had sickle cell disease (SCD). Four patients (26.7%) had unidentified causes. The 8 SCD patients had stuttering priapism on several occasions previously. Six patients (40%) had taken oral herbal medications as treatment prior to presentation. Initial resuscitative measures were intravenous hydration, aspiration, and irrigation with normal saline in 13 patients. Glanulo-cavernous shunt (Al-Ghorab) was performed in all the patients. Detumescence was immediate in 14 and delayed in 1 patient. Three patients had transient recurrence of tumescence, while one had to be reshunted. Erectile dysfunction (ED) occurred in 7 patients (46.7%). Occurrence of ED increased significantly in patients presenting 24 h after onset of symptoms ([P = 0.032] Fishers exact test). Mean duration of follow-up was 21.9 weeks (SD = 4.1), range: 3-156 weeks. CONCLUSIONS: Low flow priapism is common in our environment, and approximately half will occur in SCD patients who have had stuttering priapism previously. Timely diagnosis and treatment will reduce the probability of severe ED. In our experience, the Al-Ghorab shunt provides rapid relief. Enlightenment is vital in reducing ischemia time. Emphasis on preventive measures in SCD patients is vital.

Erectile dysfunction after sickle cell disease-associated recurrent ischemic priapism: profile and risk factors.

INTRODUCTION: Risk factors associated with erectile dysfunction (ED) that results from recurrent ischemic priapism (RIP) in sickle cell disease (SCD) are incompletely defined. AIM: This study aims to determine and compare ED risk factors associated with SCD and non-SCD-related "minor" RIP, defined as having ≥2 episodes of ischemic priapism within the past 6 months, with the majority (>75%) of episodes lasting <5 hours. METHODS: We performed a retrospective study of RIP in SCD and non-SCD patients presenting from June 2004 to March 2014 using the International Index of Erectile Function (IIEF), IIEF-5, and priapism-specific questionnaires. MAIN OUTCOME MEASURES: Prevalence rates and risk factor correlations for ED associated with RIP. RESULTS: The study was comprised of 59 patients (40 SCD [mean age 28.2 ± 8.9 years] and 19 non-SCD [15 idiopathic and four drug-related etiologies] [mean age 32.6 ± 11.7 years]). Nineteen of 40 (47.5%) SCD patients vs. four of 19 (21.1%) non-SCD patients (39% overall) had ED (IIEF <26 or IIEF-5 <22) (P = 0.052). SCD patients had a longer mean time-length with RIP than non-SCD patients (P = 0.004). Thirty of 40 (75%) SCD patients vs. 10 of 19 (52.6%) non-SCD patients (P = 0.14) had "very minor" RIP (episodes regularly lasting ≤2 hours). Twenty-eight of 40 (70%) SCD patients vs. 14 of 19 (73.7%) non-SCD patients had weekly or more frequent episodes (P = 1). Of all patients with very minor RIP, ED was found among 14 of 30 (46.7%) SCD patients vs. none of 10 (0%) non-SCD patients (P = 0.008). Using logistic regression analysis, the odds ratio for developing ED was 4.7 for SCD patients, when controlling for RIP variables (95% confidence interval: 1.1-21.0). CONCLUSIONS: ED is associated with RIP, occurring in nearly 40% of affected individuals overall. SCD patients are more likely to experience ED in the setting of "very minor" RIP episodes and are five times more likely to develop ED compared with non-SCD patients.

External validation of a prediction model for penile prosthesis implantation for erectile dysfunction management.

INTRODUCTION: Penile prosthesis implantation (PPI) is the definitive surgical treatment for erectile dysfunction (ED), yet it is often delayed for a variety of reasons. From commercial and Medicare claims data, we previously developed a tool for determining a patient's likelihood of eventually receiving PPI. We validated this instrument's utility by comparing cohorts receiving surgical (PPI) versus non-surgical ED management at a single institution. MATERIAL AND METHODS: The prediction model was based on a logistic regression incorporating claims data on demographics, comorbidities and ED therapy. A risk score is calculated from the model as the product of relative risks for the individual variables. The current validation was a retrospective analysis of ED patients seen at this institution from January to December 2012. Inclusion criteria included ED diagnosis and either first-time PPI or non-surgical treatment (controls). Risk scores for patients receiving PPI were compared to those of non-surgical controls. RESULTS: We established a cohort of 60 PPI patients (mean age 54.4 ± 9.5) and compared them with 120 non-PPI patients (mean age 53.4 ± 11.2 years). The median score of the PPI cohort was 5.7 (IQR 2.8-9.9) versus the non-PPI cohort's 1.8 (IQR 0.9-5.5) (p < 0.0001). The area under the receiver operator characteristic curve for predicting eventual PPI was 0.72 (95% CI, 0.64-0.79) (p < 0.0001). CONCLUSION: The prediction model risk-stratified men who ultimately underwent PPI compared to non-surgically managed controls. This external validation study suggests that the prediction model may be used on an individual patient basis to support a recommendation of PPI for managing ED.

Priapism in homozygous sickle cell patients: important clinical and laboratory associations.

OBJECTIVE: To evaluate the relationship between the occurrence of priapism and important steady-state clinical and laboratory parameters in homozygous sickle cell disease (SCD). SUBJECTS AND METHODS: Steady-state clinical and laboratory data were obtained from the medical records of 126 male patients seen in the clinic over a 7-year period. Estimated prevalence rates, correlation coefficients and independent t tests were calculated to assess the relationship between priapism and several important clinical and laboratory indices. Patient data on age, haemoglobin concentrations, the frequency of crises per annum, leucocyte counts, platelet counts, serum bilirubin and aspartate transaminase were evaluated. RESULTS: The prevalence of priapism was determined to be 21.4%, and 22.2% of those affected had erectile dysfunction. There was a significant positive correlation between priapism and older age (p = 0.049) and lower leucocyte counts (p = 0.008). There was no significant relationship with other clinical or laboratory indices. CONCLUSION: About 1 in 4 of all homozygous older SCD patients had priapism, and an approximately similar ratio developed erectile dysfunction; they also had lower steady-state leucocyte counts. Other clinical and laboratory indicators of disease severity in SCD did not positively correlate with the occurrence of priapism, and this may imply an alternative pathogenetic mechanism.

[Priapism in children and adolescents with homozygous sickle cell disease in Brazzaville]. / Priapisme chez l'enfant et l'adolescent drépanocytaire homozygote à Brazzaville.

OBJECTIVE: To determine the prevalence of priapism, assess knowledge and appreciate its characteristics in childhood sickle cell disease. METHODOLOGY: A case-control study was conducted at the University Hospital of Brazzaville (Department of Pediatrics, Hematology and Clinical Urology). The cases consisted of 202 sickle cell anemia who are at least 5 years. Witnesses consisted of 112 children with sickle cell disease not of the same age from the same family as the previous. RESULTS: Priapism was found in 68 (34%) affected children, divided into 54 cases (79.4%) of chronic intermittent priapism and 14 cases (20.6%) of acute priapism. In the control group no cases were observed (p=0.001). Priapism was known by six (3%) patients in the group of children with sickle cell disease. In the control group, it was known by 25 (22.3%) children. It was seen in the group of sickle cell disease as any: 113 children (56%), a natural phenomenon that can occur in life: 57 children (28%), a complication of sickle cell disease: 26 children (13%). In the control group, it was considered a natural phenomenon that can occur in life: 60 children (53.6%), a complication of sickle cell disease: 52 children (46.4%). The average age of priapism occurred in the first episode was 10.4±9.5 years. CONCLUSION: The importance of the prevalence of priapism, and insufficient knowledge needed strengthening information, education and communication with children and their parents.

Clinical Practice Patterns for Surgical Shunts and Penile Prosthesis Placement in Men With Priapism: A Retrospective Large Claims Database Analysis.

INTRODUCTION: The objective of this study was to assess the rates of surgical shunting and prosthesis placement for acute ischemic priapism using a large multi-institutional claims database. METHODS: A US claims database network (TriNetX Diamond Network) was queried from 2010 to 2020. We constructed a cohort of men ages ≥ 16 years who (1) had a diagnosis of priapism and (2) underwent an irrigation of the corpora cavernosa for priapism. We assessed the number of men who then had a surgical penile shunt or penile prosthesis placement. Demographics, time to surgical procedure, and order of procedures were collected. RESULTS: A total of 6392 men were identified with the diagnosis of priapism and the procedure of corpora cavernosal irrigation. Of these men, 693 (11%) proceeded to surgical shunt. One hundred forty-four men (2%) underwent initial penile prosthesis placement. Of the men undergoing initial penile prosthesis, only 17 of 144 (12%) cases occurred within the first month of corpora cavernosal irrigation. Finally, when assessing choice of initial shunts vs initial penile prosthesis before and after 2015, overall rates of initial shunt (10.0% vs 8.5%, P < .0001) and initial prosthesis (3.1% vs 2.1%, P < .0001) were lower after 2015 when compared with rates prior to 2015. CONCLUSIONS: In this US claims-based analysis of men presenting with ischemic priapism and treated with initial irrigation, a small percentage (11%) of men went on to receive surgical shunting, and only 2% received an initial prosthesis. Men receiving initial prostheses were more likely to have more comorbidities, and overall surgical management of priapism has decreased over time.

Incidence of priapism in emergency departments in the United States.

PURPOSE: Priapism is a complex medical emergency that often requires prompt management. In this study, we examine the incidence of this condition in a United States population based setting, and assess patient and emergency department attributes associated with an increased likelihood of hospitalization. MATERIALS AND METHODS: Emergency department visits with a primary diagnosis of priapism between 2006 and 2009 were abstracted from the Nationwide Emergency Department Sample. Univariable and multivariable analyses were performed of patient and hospital characteristics of those admitted with priapism. RESULTS: Between 2006 and 2009 a weighted estimate of 32,462 visits to the emergency department for priapism was recorded in the United States, which represents a national incidence of 5.34 per 100,000 male subjects per year. The incidence of emergency department visits increased by 31.4% during the summer compared to the winter months. Overall 4,320 visits (13.3%) resulted in hospitalization/admission for further management. On multivariable analyses independent predictors of admission included Charlson comorbidity index score 3 or greater (OR 5.67, p <0.001), insurance status (Medicaid vs private OR 1.60, p = 0.001), hospital location (rural vs urban nonteaching OR 0.32, p <0.001), median ZIP code income (very high OR 0.65, p = 0.005), emergency department volume (very high vs very low OR 1.61, p = 0.004), sickle cell disease (OR 2.22, p <0.001) and drug abuse (OR 5.47, p <0.001). CONCLUSIONS: Emergency department visits for priapism are relatively uncommon and occur more frequently during the summer months. The majority of patients are treated and released expediently. Predictors of hospital admission included comorbidity profile, insurance, hospital location and emergency department volume.

Testosterone replacement with 1% testosterone gel and priapism: no definite risk relationship.

INTRODUCTION: Although testosterone replacement therapy (TRT) is the preferred treatment for hypogonadism, information for patients using testosterone includes too frequent or prolonged erections as a potential side effect. AIM: To assess the frequency and risk of priapism or related adverse events (AEs) in hypogonadal men treated with a 1% testosterone gel. METHODS: Safety and tolerability data for AndroGel 1% were assessed, including three randomized, controlled clinical trials in varying populations of hypogonadal or near hypogonadal men. Study 1 was a Phase 3 trial of AndroGel 1% 5 g, 7.5 g, or 10 g once daily for 6 months (N = 227). Study 2 was a Phase 2 trial of AndroGel 1% 7.5 g once daily titrated as needed vs. placebo for 26 weeks in men with type 2 diabetes (N = 180). Study 3 was a Phase 4 trial of AndroGel 1% 5 g once daily vs. placebo for 12 weeks in men previously unresponsive to sildenafil 100 mg monotherapy and receiving concomitant sildenafil 100 mg (N = 75). Postmarketing AndroGel pharmacovigilance reporting data from 2001 to 2011 was searched for events coded as priapism. MAIN OUTCOME MEASURES: The incidence of priapism and/or related symptoms reported as urogenital or reproductive system AEs. RESULTS: In the 283 men exposed to AndroGel 1% over the three trials, mean exposure ranged from 84 days to 149 days. No AEs described as priapism or related symptoms were reported in the three trials. In the postmarketing data, representing 40 million units sold, eight cases described as priapism were reported. Of the six cases with accompanying data, all were judged as possibly related to AndroGel. CONCLUSIONS: Safety data from the clinical trials for AndroGel 1% did not report any cases of priapism, and its incidence in the postmarketing pharmacovigilance data is extremely low, indicating a minimal risk of inducing priapism.

Nationwide emergency department visits for priapism in the United States.

INTRODUCTION: The epidemiology of priapism is not well characterized. A small number of studies based on inpatient data or small population samples have estimated the incidence to range from 0.34 to 1.5 cases per 100,000 males. AIM: To estimate the current epidemiology and impact on resource utilization of priapism in the United States (US). MAIN OUTCOME MEASURES: Rate of emergency department encounters for priapism in the US. METHODS: Emergency department (ED) visits for priapism were analyzed using discharge data from the Nationwide Emergency Department Sample (NEDS), Healthcare Cost and Utilization Project (HCUP). Priapism encounters were identified by ICD9 code. Priapism encounters were analyzed for patient and hospital characteristics, associated diagnoses, and hospital charge. Established weighting in the sample was used to calculate nationwide estimates. RESULTS: A total of 8,738 ED encounters for priapism were identified between 2006 and 2009 in the NEDS. This translated to an estimated 39,964 encounters out of a total of 496,195,793 ED visits, or 8.05 per 100,000 ED visits (95% confidence interval [CI] 7.59-8.51). 21.1% of patients had a concurrent diagnosis of sickle cell disease (SCD). 72.1% of all patients were discharged home from the ED, while only 49.6% of patients with SCD were discharged home. A concurrent diagnosis of SCD was associated with an odds ratio (OR) of 3.84 (95% CI 3.65-4.05) for admission to the hospital when controlling for age, region, hospital and payer type. The mean hospital charge was $1,778 per encounter if discharged home and $41,909 per encounter if admitted. The estimated mean total annual charge for priapism was $123,860,432 with 86.8% of charges attributed to inpatient admissions. CONCLUSIONS: Our estimate of the rate of ED visits for priapism was significantly higher than prior estimates with a SCD concurrence rate lower than previously estimated.

[Priapisms]. / Priapismes.

OBJECTIVES: Priapism is a rare condition for which urgent diagnosis and treatment is required. This paper reviews the literature regarding ischaemic, non-ischaemic and stuttering priapism in order to provide management recommendations. METHODS: A Medline search was carried out to identify all relevant papers with management guidelines for priapism and combined with expert opinion of the authors. RESULTS: Ischaemic priapism represents a compartment syndrome of the penis and urgent intervention is required to decrease the risk of erectile dysfunction. First line treatment is medical and associate cavernosal blood aspiration and sympathomimetic intracavernosal injection. Second line treatment is surgical by creating a cavernospongious shunt. Non-ischaemic priapism is not a medical emergency; however, it may need embolization of the arteriocavernosal fistula and result in erectile dysfunction. The treatment objective for stuttering priapism is to decrease episodes of prolonged erections with systemic treatments, while treating each acute episode as an emergency. CONCLUSIONS: Priapism is a potentially severe condition that requires urgent diagnosis and well-defined sequential management to prevent treatment delay, complications and irreversible erectile dysfunction.

Socioeconomic Disparities and Risk Factors in Patients Presenting With Ischemic Priapism: A Multi-Institutional Study.

OBJECTIVES: To evaluate contemporary clinical presentations of priapism, their association with socioeconomic characteristics, and the role of prescribing providers in priapism episodes in a large cohort of patients managed at 3 major academic health systems. METHODS: We identified all consecutive patients presenting with ischemic priapism to the emergency departments of three major academic health systems (2014 -2019). Demographic characteristics, priapism etiologies, and clinical management were evaluated. Univariable and multivariable analyses were used to assess the contribution of socioeconomic characteristics and the role of prescribing providers in priapism episodes. RESULTS: We identified 102 individuals with a total of 181 priapism encounters. Hispanic race, lower income quartile, sickle-cell disease, and illicit drug use were associated with increased risk of recurrent episodes. Of ICI users, 57% received their prescriptions from non-urological medical professionals (NUMPs); the proportion with recurrent episodes was higher for NUMPs compared to urologists (24% vs 0%, P = 0.06) with no demographic differences identified between patients treated by either group. CONCLUSION: Socioeconomic disparities exist among patients presenting with recurrent episodes of priapism, potentially highlighting systemic issues with access to care and patient education. With most patients who developed ischemic priapism from ICI being prescribed these medications by NUMPs, further investigation is required to elucidate the prescribing and counseling patterns of these providers. Increased awareness of disparities and complications may improve patient safety.

Epidemiology and treatment of priapism in sickle cell disease.

Ischemic priapism is a common but underrecognized morbidity affecting about 33% of adult men with sickle cell disease (SCD). The onset of priapism occurs in the prepubertal period and tends to be recurrent with increasing age. Significantly, priapism is associated with an unrecognized high burden of mental duress and sexual dysfunctions. The diagnosis of priapism is clinical. Many episodes of priapism will resolve spontaneously, but when an episode lasts longer than 4 hours, the episode is considered a urologic emergency requiring quick intervention with either corporal aspiration or shunt surgery. Only 3 randomized clinical trials (stilbesterol, ephedrine or etilefrine, and sildenafil) have been conducted for secondary priapism prevention in SCD. All 3 trials were limited with small sample sizes, selection biases, and inconclusive results after completion. The current molecular understanding of the pathobiology of priapism suggests a relative nitric oxide (NO) deficiency secondary to chronic hemolysis in SCD and associated phosphodiesterase type 5 dysregulation. We posit an increase in NO levels will restore the normal homeostatic relationship between voluntary erection and detumescence. Currently, 2 randomized phase 2 trials (1 double-blind, placebo-controlled trial and 1 open-label, single-arm intervention) are being conducted for secondary priapism prevention in men at high risk for recurrent priapism (NCT03938454 and NCT05142254). We review the epidemiology and pathobiology of priapism, along with mechanistic therapeutic approaches for secondary prevention of priapism in SCD.

Clinical Vignettes Part I.

Patients with sickle cell disease and/or (rarely) trait are at increased risk for developing recurrent episodes of priapism, also known as stuttering priapism, and major ischemic priapism. Treatment of acute ischemic priapism is reactive; whereas ideal management consists of preventative approaches to ultimately promote the best improvement in patient's quality of life. Leg ulcers in patients with sickle cell disease (SCD) are quite common, with ∼20 % of patients with HBSS reporting either having an active or a past ucler. They can be confused with venous ulcers, with lower extremity hyperpigmentation confounding further the diagnosis. Several factors believed to contribute to the development of leg ulcers in patients with SCD are discussed in this article. Sickle cell liver disease (SCLD) occurs because of a wide variety of insults to the liver that happen during the lifetime of these patients. SCLD includes a range of complications of the hepatobiliary system and is increasing in prevalence with the aging adult sickle population. Liver nodular regenerative hyperplasia (NRH) is more common than realized and underappreciated as a diagnosis and requires liver biopsy with reticulin staining. Undiagnosed, the insidious damage from liver NRH can lead to noncirrhotic portal hypertension or cirrhosis.

Priapism in Patients with Chronic Myeloid Leukemia (CML): A Systematic Review.

BACKGROUND: Priapism is defined as a penile erection that persists four or more hours and is unrelated to sexual stimulation. Priapism resulting from hematologic malignancy is most likely caused by venous obstruction from microemboli/thrombi and hyperviscosity caused by the increased number of circulating leukocytes in mature and immature forms. In patients with leukemia, 50% of cases of priapism are due to Chronic Myeloid Leukemia (CML). We present a systematic review of priapism in CML. Acquisition of evidence: An extensive literature research was carried out in PubMed, Google Scholar, SCOPUS, and Science Citation Index databases... The search included cases up to 4th August 2020. Synthesis of evidence: A total of 68 articles were found and included in our review, including 3 reviews from three different centers.  We found 68 articles (102 patients; figure 1) and several case reports on priapism in CML. Priapism was noticed in some patients at the first presentation of CML. However, it was infrequently reported during the start of treatment, following the stop of medication and post-splenectomy. The mean age at presentation was 27.4 years, and the mean time from onset of priapism to the time to get medical attention (presentation) was 78.2 hours. The mean white blood cell count associated with priapism was 321.29x109/L, and the mean platelet count was 569 x10 9/L. The chronic phase of CML was the most common phase where priapism occurred. Most patients were Asian (>50%). Nearly a quarter of patients (27.4%) developed permanent erectile dysfunction. CONCLUSIONS: Priapism is a urological emergency requiring urgent multidisciplinary management to prevent erectile dysfunction. Because of the relatively rare occurrence of priapism in CML patients, there is no standard treatment protocol.