Priapismo y leucemia mieloide crónica en adolescente. Debut poco frecuente. Reporte de caso / Priapism and chronic myeloid leukemia in an adolescent. Rare debut presentation. A case report

El priapismo es una erección dolorosa y persistente acompañada o no de estímulo sexual. Una causa poco frecuente de esta anormalidad es la leucemia mieloide crónica. Se han reportado pocos casos de priapismo como manifestación inicial de una leucemia de este tipo en pacientes adolescentes. A continuación, se informa el caso de un paciente de 16 años de edad que presentó priapismo como manifestación inicial de una leucemia mieloide crónica. Durante su evolución, no se realizó aspiración de los cuerpos cavernosos. Se inició tratamiento hematológico específico y, ante la persistencia del priapismo, fue necesario realizar un shunt de cuerpos cavernosos en dos ocasiones, tratamiento a pesar del cual existen altas probabilidades de secuelas.

Priapism is a painful and persistent erection, with or without sexual stimulation. A rare cause of such abnormality is chronic myeloid leukemia. Few cases of priapism as an initial manifestation of this type of leukemia have been reported in adolescent patients. Here we describe the case of a 16-year-old patient who presented with priapism as the initial manifestation of chronic myeloid leukemia. No cavernosal aspiration was performed. A specific hematological treatment was started and, given the persistence of priapism, the patient required 2 corpora cavernosa shunt procedures; despite this treatment, there is a high probability of sequelae.

Priapism and chronic myeloid leukemia in an adolescent. Rare debut presentation. A case report. / Priapismo y leucemia mieloide crónica en adolescente. Debut poco frecuente. Reporte de caso.

Priapism is a painful and persistent erection, with or without sexual stimulation. A rare cause of such abnormality is chronic myeloid leukemia. Few cases of priapism as an initial manifestation of this type of leukemia have been reported in adolescent patients. Here we describe the case of a 16-year-old patient who presented with priapism as the initial manifestation of chronic myeloid leukemia. No cavernosal aspiration was performed. A specific hematological treatment was started and, given the persistence of priapism, the patient required 2 corpora cavernosa shunt procedures; despite this treatment, there is a high probability of sequelae.

El priapismo es una erección dolorosa y persistente acompañada o no de estímulo sexual. Una causa poco frecuente de esta anormalidad es la leucemia mieloide crónica. Se han reportado pocos casos de priapismo como manifestación inicial de una leucemia de este tipo en pacientes adolescentes. A continuación, se informa el caso de un paciente de 16 años de edad que presentó priapismo como manifestación inicial de una leucemia mieloide crónica. Durante su evolución, no se realizó aspiración de los cuerpos cavernosos. Se inició tratamiento hematológico específico y, ante la persistencia del priapismo, fue necesario realizar un shunt de cuerpos cavernosos en dos ocasiones, tratamiento a pesar del cual existen altas probabilidades de secuelas.

Corpus cavernosum abscess after Al-Ghorab shunt with Snake Maneuver for ischemic priapism treatment: Clinical and surgical treatments.

Corpus cavernosum abscess is a rare condition that can lead to permanent and debilitating consequences. This case reports a 58-year-old man who developed erectile dysfunction with no response to oral and intracavernous medications after the surgical treatment of a penile abscess.

Systematic review: interventions in sickle cell disease-related priapism in reducing overall morbidity, recurrence, and side effects / Revisión sistemática: intervenciones en priapismo relacionado con enfermedad de células falciformes (ECF), en reducción de morbilidad, recurrencia y efectos secundarios

Pediatric priapism is a rare, underreported urological emergency. Sickle cell disease (SCD)-related priapism cases should be assessed considering specific pathophysiologic characteristics. Chronic control of the disease has an impact on a patient's quality of life. A systematic review in different databases was done, for defining if management in All-type priapism in SCD patients was equally effective in reducing overall morbidity, disability and comorbidities, recurrence, and therapy-related side effects. Qualitative analysis was performed. Nine studies were included in the study; the majority were case series, and 300 patients were included in the analysis. The interventions and outcomes were clinically heterogeneous but displayed perspectives for further studies. This is the first analysis approach for a consensus for SCD-related priapism treatment. Studies with internal validity and causality designs are needed, to evaluate further causality relationships and establish evidence-based approaches. Etilefrine, Pseudoephedrine/etilefrine-5 inhibitors, and finasteride are promising strategies. Quality of life scores should be applied to these patients, and molecular studies should be developed for pharmacological designs.

El priapismo pediátrico es una entidad poco común, sin embargo, es una urgencia urológica infranotificada. Los casos de priapismo relacionados con enfermedad de células falciformes (ECF) deben evaluarse teniendo en cuenta características fisiopatológicas específicas. El control crónico de la enfermedad tiene impacto en la calidad de vida del paciente. Se realizó una revisión sistemática en diferentes bases de datos, para definir si las intervenciones en pacientes con priapismo de todo tipo relacionado con ECF fueron igualmente efectivas en la reducción de morbilidad general, discapacidad, comorbilidades, recurrencia y efectos secundarios relacionados con la terapia. Se realizó un análisis cualitativo de nueve estudios; la mayoría series de casos, para un total de 300 pacientes. Las intervenciones y los resultados fueron clínicamente heterogéneos, pero mostraron perspectivas para futuros estudios. Este es el primer análisis de información para llegar a un consenso para el tratamiento del priapismo relacionado con la ECF. La perspectiva principal es la necesidad de estudios con validez interna y diseños elaborados para profundizar las relaciones de causalidad y el abordaje basado en la evidencia de esta condición. La etilefrina, los inhibidores de la PDE-5 y la finasterida son estrategias prometedoras. Se deben aplicar puntajes de calidad de vida a estos pacientes y se deben desarrollar estudios moleculares para diseños farmacológicos.

Corpus cavernosum abscess: A case report.

Corpus cavernosum abscess is a rare infection of the genitourinary tract. In this case report, we present a 42-year-old patient admitted with pain and edema in the penile shaft twenty-four days after drainage by puncture of the cavernous body due to ischemic priapism. Al-Ghorab procedure together snake-maneuver and broad-spectrum antibiotic therapy were performed. Despite manifesting erectile dysfunction, he evolved without penile deviations.

Association between partial thrombosis of the corpus cavernosum, partial priapism, and hard flaccid syndrome: A scoping review.

AIM: To describe the association between partial thrombosis of the corpus cavernosum, partial priapism, and hard flaccid syndrome. METHODS: A scoping review was performed according to the recommendations of the Joanna Briggs Institute. Moreover, we performed a search strategy using the MEDLINE, EMBASE, and CENTRAL databases. We included the available information, evaluating the conditions of partial thrombosis of the corpus cavernosum, partial priapism, and hard flaccid syndrome, and their molecular and physiological mechanisms and clinical presentation. RESULTS: We identified 207 articles and chose eight studies published between 2001 and 2021. The total number of patients was 34, and their mean age was 28.2 years. Moreover, in 84% of the studies, the pathophysiology of the events was related to microtrauma or prolonged perineal compression. Additionally, 94.2% of the patients had some degree of erectile dysfunction. In addition, out of all patients, 94% underwent magnetic resonance imaging (MRI). However, patients with hard flaccid syndrome did not show relevant findings in these studies. Conversely, MRI showed asymmetry in the proximal corpora cavernosa, thrombosed corpus cavernosum segments, and mainly cavernous fibrous septum in patients with partial cavernous thrombosis and partial priapism. CONCLUSION: Partial thrombosis of the corpus cavernosum, partial priapism, and hard flaccid syndrome occurred more frequently in young patients, possibly related to microtraumas that generate cavernous fibrosis and trigger alterations in the erection of the distal portion of the penis. Additionally, they cause proximal hardening of the pelvis, perineal pain, painful ejaculations, and cavernous asymmetry. Moreover, the imaging characteristics are similar in patients with partial priapism and partial cavernous thrombosis.

Priapism in Sickle Cell Disease: An Evaluation of the Knowledge of an at Risk Population in Jamaica.

INTRODUCTION: Ischemic priapism is characterized by painful erections that may lead to erectile dysfunction. Men with sickle cell disease (SCD) are particularly prone to this condition, however, the knowledge among this population with respect to possible complications is not well known. AIM: The objective of this study was to evaluate the knowledge of males with SCD about priapism and its possible consequences. METHODS: A cross-sectional study was carried out among consecutive consenting males aged 12 years and older with SCD presenting to the Sickle Cell Unit between September 2018 and August 2019. All participants completed a questionnaire detailing knowledge on the definition of priapism, its association with SCD, consequences of untreated priapism and treatment strategies. The responses were used to generate a total priapism knowledge score for each of the participants. MAIN OUTCOME MEASURES: Main outcomes included knowledge of the term priapism, its association with SCD as well as the total priapism knowledge score. RESULTS: 219 patients of mean age 29.8 ± 13 years completed the questionnaire. 38.4% of patients were familiar with the term priapism and of these 68.8% were aware of the association between SCD and priapism. There was a significant association between knowledge of association of priapism with SCD and increasing educational level (P = .036) and history of prior priapism episodes (P = .02). There was a significant association between knowledge of the term "priapism" and history of priapism (P = .002). The mean total priapism knowledge score among the participants was 5.16 out of a maximum score of 12, with 70.8% of participants being categorized as having "poor knowledge." CONCLUSION: There is a general lack of knowledge among patients with SCD with respect to the term priapism. Education campaigns geared toward addressing the identified knowledge gaps may prove beneficial in increasing awareness among this population and potentially decrease the risk of complications. Whyte N, Morrison-Blidgen B, Asnani M, Priapism in Sickle Cell Disease: An Evaluation of the Knowledge of an at Risk Population in Jamaica. Sex Med 2021;9:100339.

Priapism in a patient on hemodialysis and with COVID-19. Case report / Priapismo en paciente en diálisis y con COVID-19. Reporte de caso

Abstract Introduction: Priapism is rare in dialysis patients. It is associated with several risk factors inherent to therapy and kidney disease. This condition has been reported in severe COVID-19 cases, probably caused by coagulopathy secondary to the infection. However, it has not been reported in patients with mild COVID-19. Case presentation: A 65-year-old patient on hemodialysis and with mild COVID-19 presented with a 30-hour painful penile erection. Physical examination revealed an erection, but no other significant findings were observed. Based on the clinical history, physical examination and laboratory test results, the patient was diagnosed with ischemic priapism. Corpora cavernosa drainage was performed and two injections of epinephrine (each with a dose of 2mL of epinephrine solution 1/100 000) were administered, achieving complete resolution of the symptoms. Conclusions: At the time of writing this case report, there is no information available on cases of priapism in patients with mild manifestations of COVID-19, nor is it clear how the risk of thrombosis should be assessed in this group of patients. From a pathophysiological point of view, factors related to dialysis and kidney disease could have predisposed this patient to priapism, although the role of SARS-CoV-2 infection is uncertain. Therefore, further studies are required to confirm or rule out the association between COVID-19 and priapism in dialysis patients.

Resumen Introducción. El priapismo es una condición infrecuente en pacientes en diálisis que se puede presentar por factores inherentes a esta terapia y a la enfermedad renal. Por otra parte, es una entidad que se ha reportado en pacientes con síntomas severos de COVID-19 y se ha asociado a coagulopatía secundaria a la infección, pero de la que no hay registro en casos leves de esta enfermedad. Presentación de caso. Paciente masculino de 65 años en hemodiálisis y con infección por SARS-CoV-2 con síntomas leves, quien consultó al servicio de urgencias de un hospital de cuarto nivel por erección peneana dolorosa de 30 horas de evolución. Al examen físico se evidenció la erección, sin otros hallazgos relevantes. Con base en la historia clínica, el examen físico y los resultados de laboratorio, se diagnosticó priapismo isquémico y se realizó drenaje de senos cavernosos y aplicación de dos inyecciones de epinefrina (cada una con una dosis de 2mL de solución de epinefrina 1/100 000), lográndose resolución completa de la condición. Conclusiones. Hasta el momento de la elaboración del presente reporte de caso no hay información disponible de casos de priapismo en pacientes con manifestaciones leves de COVID-19, y tampoco hay claridad de cómo se debe evaluar el riesgo de trombosis en este grupo de pacientes. Desde el punto de vista fisiopatológico, factores relacionados con la diálisis y la enfermedad renal pudieron predisponer a este paciente al priapismo, aunque no es claro el rol de la infección por SARS-CoV-2. Por tanto, se requieren estudios que permitan confirmar o descartar la asociación entre COVID-19 y priapismo en pacientes en diálisis.

Alpha thalassemia, but not ßS-globin haplotypes, influence sickle cell anemia clinical outcome in a large, single-center Brazilian cohort.

Alpha thalassemia and beta-globin haplotype are considered classical genetic disease modifiers in sickle cell anemia (SCA) causing clinical heterogeneity. Nevertheless, their functional impact on SCA disease emergence and progression remains elusive. To better understand the role of alpha thalassemia and beta-globin haplotype in SCA, we performed a retrospective study evaluating the clinical manifestations of 614 patients. The univariate analysis showed that the presence of alpha-thalassemia -3.7-kb mutation (αα/-α and -α/-α) decreased the risk of stroke development (p = 0.046), priapism (p = 0.033), and cholelithiasis (p = 0.021). Furthermore, the cumulative incidence of stroke (p = 0.023) and cholelithiasis (p = 0.006) was also significantly lower for patients carrying the alpha thalassemia -3.7-kb mutation. No clinical effects were associated with the beta-globin haplotype analysis, which could be explained by the relatively homogeneous haplotype composition in our cohort. Our results reinforce that alpha thalassemia can provide protective functions against hemolysis-related symptoms in SCA. Although, several genetic modifiers can impact the inflammatory state of SCA patients, the alpha thalassemia mutation remains one of the most recurrent genetic aberration and should therefore always be considered first.

Prevalence of priapism in individuals with sickle cell disease and implications on male sexual function / Prevalência de priapismo em indivíduos com doença falciforme e suas implicações na função sexual masculina

ABSTRACT Objective To evaluate epidemiological aspects of priapism in patients with sickle cell disease, and these aspects impact on adult sexual function. Methods This was a cross-sectional study including individuals with sickle cell disease who were evaluated at a reference center for sickle cell. Participants completed a structured questionnaire about their sociodemographic characteristics and priapism events. Sexual function was assessed using validated two instruments, the Erection Hardness Score and one about the sex life satisfaction. Results Sixty-four individuals with median aged of 12 (7 to 28) years were interviewed. The prevalence of priapism was 35.9% (23/64). The earliest priapism episode occurred at 2 years of age and the latest at 42 years. The statistical projection was that 71.1% of individuals of the study would have at least one episode of priapism throughout life. Patients with episodes of priapism (10/23) had significantly worse erectile function Erection Hardness Score of 2 [1-3]; p=0.01 and were less satisfied with sexual life 3 [3-5]; p=0.02. Conclusion Priapism is usually present in childhood, and severe episodes are associated with cavernous damage, impairment in the quality of the erection, and lower sexual satisfaction.

RESUMO Objetivo Avaliar aspectos epidemiológicos do priapismo em pacientes com doença falciforme e o impacto desses aspectos na função sexual de adultos. Métodos Trata-se de estudo transversal, que incluiu indivíduos com doença falciforme acompanhados em um centro de referência. Os participantes responderam a um questionário estruturado acerca das características sociodemográficas e eventos de priapismo. A função sexual foi avaliada por meio de dois instrumentos validados, a Escala de Rigidez de Ereção e um sobre satisfação com a vida sexual. Resultados Foram entrevistados 64 indivíduos com média de idade de 12 (7-28) anos. A prevalência de priapismo foi de 35,9% (23/64). O episódio mais precoce ocorreu aos 2 anos de idade e o mais tardio, aos 42 anos. A projeção estatística foi de que 71,1% desses sujeitos teriam pelo menos um episódio de priapismo ao longo da vida. Pacientes adultos com episódios de priapismo (10/23) apresentaram função erétil significativamente pior Escala de Rigidez de Ereção de 2 [1-3]; p=0,01 e estavam menos satisfeitos com a vida sexual 3 [3-5]; p=0,02. Conclusão O priapismo manifesta-se desde a infância, e episódios graves estão associados a dano cavernoso, prejuízo na qualidade da ereção e menor satisfação sexual.

Clinical and Genetic Predictors of Priapism in Sickle Cell Disease: Results from the Recipient Epidemiology and Donor Evaluation Study III Brazil Cohort Study.

INTRODUCTION: Priapism is the persistent and painful erection of the penis and is a common sickle cell disease (SCD) complication. AIM: The goal of this study was to characterize clinical and genetic factors associated with priapism within a large multi-center SCD cohort in Brazil. METHODS: Cases with priapism were compared to SCD type-matched controls within defined age strata to identify clinical outcomes associated with priapism. Whole blood single nucleotide polymorphism genotyping was performed using a customized array, and a genome-wide association study (GWAS) was conducted to identify single nucleotide polymorphisms associated with priapism. MAIN OUTCOME MEASURE: Of the 1,314 male patients in the cohort, 188 experienced priapism (14.3%). RESULTS: Priapism was more common among older patients (P = .006) and more severe SCD genotypes such as homozygous SS (P < .0001). In the genotype- and age-matched analyses, associations with priapism were found for pulmonary hypertension (P = .05) and avascular necrosis (P = .01). The GWAS suggested replication of a previously reported candidate gene association of priapism for the gene transforming growth factor beta receptor 3 (TGFBR3) (P = 2 × 10-4). CLINICAL IMPLICATIONS: Older patients with more severe genotypes are at higher risk of priapism, and there is a lack of consensus on standard treatment strategies for priapism in SCD. STRENGTHS & LIMITATIONS: This study characterizes SCD patients with any history of priapism from a large multi-center cohort. Replication of the GWAS in an independent cohort is required to validate the results. CONCLUSION: These findings extend the understanding of risk factors associated with priapism in SCD and identify genetic markers to be investigated in future studies to further elucidate priapism pathophysiology. Ozahata M, Page GP, Guo Y, et al. Clinical and Genetic Predictors of Priapism in Sickle Cell Disease: Results from the Recipient Epidemiology and Donor Evaluation Study III Brazil Cohort Study. J Sex Med 2019;16:1988-1999.

Access of men with sickle cell disease and priapism in emergency services / Acesso de homens com doença falciforme e priapismo nos serviços de emergência

ABSTRACT BACKGROUND AND OBJECTIVES: Priapism is one of the complications of sickle cell disease characterized by a persistent and painful erection, which can lead to erectile dysfunction and sexual impotence. The objective of this study was to understand how men with sickle cell disease and priapism access emergency care. METHODS: A qualitative study conducted in a reference healthcare unit to people with sickle cell disease in the second largest city in Bahia. Seven adult men with sickle cell disease who had experienced priapism participated in the study. The data were collected by semi-structured interview and thematic story designs and submitted to content analysis. RESULTS: Priapism is seen as a lack of genital health. Participants use strategies to manage it at home to avoid embarrassment, which ends up in cocooning. Access to emergency services is motivated by persistent and relentless pain; and limited by the fear of priapism being mistaken for sexual deviance, lack of knowledge about the complication as a urologic emergency and financial shortfall, which confers a worse prognosis about erectile function. Men are embarrassed and discriminated by healthcare and support professionals, which discourages them from accessing these services in the future. CONCLUSION: This study emphasizes the importance of early diagnosis of sickle cell disease, the orientation of family members and the need for healthcare professionals to educate young boys and men with sickle cell disease and their caregivers about priapism in advance to allow adequate self-care and prevent complications.

RESUMO JUSTIFICATIVA E OBJETIVOS: O priapismo é uma das complicações da doença falciforme caracterizada por ereção persistente e dolorosa, podendo levar à disfunção erétil e impotência sexual. O objetivo deste estudo foi compreender como os homens com doença falciforme e priapismo acessam os cuidados nos serviços de emergência. MÉTODOS: Estudo qualitativo realizado em unidade de saúde referência para pessoas com doença falciforme no segundo maior município baiano. Participaram do estudo 7 homens adultos com doença falciforme que já vivenciaram priapismo. Utilizou-se entrevista semiestruturada e desenhos-história com o tema, analisados por análise de conteúdo. RESULTADOS: O priapismo é visto como uma falta de saúde genital. Os participantes usam estratégias para seu manuseio em domicílio para evitar constrangimentos, o que acaba isolando-os socialmente. O acesso aos serviços de emergência é motivado pela dor persistente e irredutível; e limitado pelo temor do priapismo ser confundido como resultado de desvio sexual, desconhecimento da complicação como emergência urológica e carência financeira, o que confere pior prognóstico sobre a função erétil. Os homens sofrem constrangimento e discriminação pelos profissionais de saúde e de apoio das unidades, o que os desmotiva a acessar esses serviços no futuro. CONCLUSÃO: Este estudo ressalta a importância do diagnóstico precoce da doença falciforme, da orientação de familiares e da necessidade de os profissionais de saúde educarem os meninos/homens jovens com doença falciforme e seus cuidadores sobre o priapismo de forma prévia, para permitir o adequado autocuidado futuro e prevenção de complicações.

Self-care of men with priapism and sickle cell disease / Autocuidado de hombres con priapismo y enfermedad falciforme / Autocuidado de homens com priapismo e doença falciforme

ABSTRACT Objective: To identify self-care demands of men with sickle cell disease and priapism and describe self-care measures in light of Orem's Self-Care Theory. Method: This is a descriptive exploratory study with qualitative approach conducted with nine men with a history of sickle cell disease and priapism. Data were analyzed using Orem's Self-Care Theory. Results: Some demands were identified: from universal self-care - difficulty in social interaction and solitude, changes in self-image, self-esteem and sexual activity; from development - the experience with priapism and little knowledge about the pathophysiology of the disease; regarding health deviations - pain crises. Conclusion: Orem's theory allowed to identify self-care demands, which are essential for the nursing care provided for men with priapism. Nursing has an essential role in the measures for the different demands presented.

RESUMEN Objetivo: Identificar las demandas sobre autocuidado de hombres con enfermedad falciforme y priapismo y describir las medidas del autocuidado a la luz de la Teoría de Orem. Método: Se trata de una investigación descriptiva, exploratoria, de abordaje cualitativo, realizada entre nueve hombres con historia clínica de enfermedad falciforme y priapismo. El análisis de los datos se llevó a cabo según la Teoría del Autocuidado de Orem. Resultados: se identificaron algunas demandas: del autocuidado universal: dificultad de interacción social y soledad, alteraciones de la autoimagen y autoestima y actividad sexual; del desarrollo: la experiencia con el priapismo y el conocimiento insuficiente sobre la fisiopatología de la enfermedad; sobre desviaciones de la salud: crisis de dolor. Conclusión: La teoría de Orem permitió identificar las demandas del autocuidado, esenciales para la atención de enfermería en hombres con priapismo y resaltó la importancia de la enfermería acerca de las medidas a ser tomadas en las diferentes demandas.

RESUMO Objetivo: Identificar as demandas de autocuidado de homens com doença falciforme e priapismo e descrever as medidas de autocuidado à luz da Teoria do Autocuidado de Orem. Método: Trata-se de uma pesquisa descritiva, exploratória, de abordagem qualitativa, com nove homens com história clínica de doença falciforme e priapismo. A análise dos dados foi realizada por meio da Teoria do Autocuidado de Orem. Resultados: Foram identificadas algumas demandas: de autocuidado universal - dificuldade de interação social e solidão, alterações na autoimagem e autoestima e atividade sexual; de desenvolvimento - a experiência com o priapismo o e pouco conhecimento sobre a fisiopatologia da doença; em relação a desvios de saúde - crises de dor. Conclusão: A teoria de Orem possibilitou identificar as demandas de autocuidado, que são essenciais para cuidado de enfermagem a homens com priapismo, e a importância da enfermagem frente às medidas para diferentes demandas apresentadas.

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.

Priapismo en la drepanocitosis. Diagnóstico y opciones terapéuticas / Priapism in sickle cell disease. Diagnosis and therapeutics options

El priapismo es una complicación de la anemia drepanocítica y se define como una erección prolongada, dolorosa y persistente del pene de más de 4 horas de duración sin estimulación sexual asociada. El 95 por ciento de las crisis de priapismo en estos pacientes es de tipo isquémico o de bajo flujo y constituyen una emergencia médica que, de no diagnosticarse y tratarse adecuadamente, provoca necrosis del tejido y disfunción eréctil. En este trabajo se revisan el diagnóstico y las opciones terapéuticas actuales y futuras de esta grave complicación(AU)

Priapism is a common complication of sickle cell disease and it is characterized by a prolonged, painful and persistent erection of the penis lasting more than 4 hours without associated sexual stimulation. The 95 percent of priapism crisis in these patients is ischemic type and represents a medical emergency that can provoke erectile tissue necrosis and erectile dysfunction if not treated properly. In this paper we reviewed the diagnosisand the current and perspectives therapeutic options of this severe complication(AU)

Daily use of phosphodiesterase type 5 inhibitors as prevention for recurrent priapism / Prevenção do priapismo recorrente com a utilização diária de inibidores da fosfodiesterase tipo 5

Summary Objective: The pathogenesis of recurrent priapism is currently being investigated based on the regulation of the phosphodiesterase 5 (PDE5) enzyme. We explored the daily use of PDE5 inhibitors to treat and prevent priapism recurrences. Method: We administered PDE5 inhibitors using a long-term therapeutic regimen in seven men with recurrent priapism, with a mean age of 29.2 years (range 21 to 35 years). Six men (85.7%) had idiopathic priapism recurrences and one man (24.3%) had sickle cell disease-associated priapism recurrences. Tadalafil 5 mg was administered daily. The mean follow-up was 6.6 months (range 3 to 12 months). Results: Daily long-term oral PDE5 inhibitor therapy alleviated priapism recurrences in all patients. Five (71.4%) had no episodes of priapism and two (28.6%) referred decrease in their episodes of priapism. All patients referred improvement in erectile function. Conclusion: These findings suggest the hypothesis that PDE5 dysregulation exerts a pathogenic role for both sickle cell disease-associated priapism and for idiopathic priapism, and that it offers a molecular target for the therapeutic management of priapism. These preliminary observations suggest that continuous long-term oral PDE5 inhibitor therapy may treat and prevent recurrent priapism.

Resumo Objetivo: Uma das teorias propostas para explicar a etiologia do priapismo recorrente está baseada no mecanismo de regulação da fosfodiesterase tipo 5. Estudamos o uso diário dos inibidores de fosfodiesterase tipo 5 no tratamento e na prevenção do priapismo recorrente. Método: Sete homens com diagnóstico de priapismo recorrente, com idade média de 29,5 anos (21 a 35 anos), utilizaram inibidor de fosfodiesterase tipo 5 em dose diária (tadalafila 5 mg/dia) por período prolongado. Seis homens (85,7%) apresentavam priapismo recorrente de etiologia idiopática, e um homem (24,3%), de etiologia associada à anemia falciforme. O seguimento médio foi de 6,6 meses (3 a 12 meses). Resultados: Todos os pacientes se beneficiaram com a utilização de inibidores de fosfodiesterase tipo 5. Cinco (71,4%) não apresentaram nenhum episódio de priapismo e dois (28,6%) relataram diminuição dos episódios. Todos os pacientes relataram melhora da função erétil. Conclusão: Estes achados sugerem que a hipótese do mecanismo de regulação da fosfodiesterase tipo 5 exerce papel importante na patogenia do priapismo recorrente. O uso contínuo e diário de inibidores da fosfodiesterase tipo 5 pode ser uma opção no tratamento do priapismo recorrente.

High-flow priapism treated with superselective transcatheter embolization using polyvinyl alcohol particles.

OBJECTIVES: Priapism is a persistent erection of the penis not associated with sexual stimulation. High-flow priapism is caused by unregulated arterial inflow, usually preceded by perineal or penile blunt trauma and formation of an arterial-lacunar fistula. We present a case of high-flow priapism in a 13-year-old patient managed with polyvinyl alcohol particles. METHODS: After obtaining informed consent of the parents of the minor, diagnosis was made with penile Color Doppler Ultrasound and confirmed with flush angiography. Selective arterial embolization was performed with the use of polyvinyl alcohol particles. RESULTS: Complete detumescence was achieved without compromising the patient's erectile function. CONCLUSIONS: The use of permanent occlusive agents like polyvinyl alcohol particles for embolization shows good occlusion rates compared to temporary agents. More studies are needed to find the safer and better agent for the treatment of high flow priapism without compromising erectile function.

Fisiopatología del priapismo en el paciente con anemia drepanocítica / Physiopathology of priapism in sickle cell anaemia patients

La anemia drepanocítica es una enfermedad con un alto índice de morbilidad que afecta la calidad de vida de los pacientes que la padecen. El priapismo es una complicación frecuente de la enfermedad y se define como una erección dolorosa, prolongada y persistente del pene de más de 4 horas de duración,sin estimulación sexual asociada. El 95 por ciento de las crisis de priapismo en el paciente con anemia drepanocítica son de tipo isquémico o de bajo flujo. En este trabajo se revisan los nuevos mecanismos moleculares que explican la patogenia del priapismo isquémico y que son la base de futuras posibles dianas terapéuticas para su tratamiento(AU)

Sickle cell anaemia (SCD) is a disorder with a high index of morbidity, affecting the quality of life of these patients. Priapism is a common complication of SCD and it is characterized by a prolonged and persistent erection of the penis lasting more than 4 hours without associated sexual stimulation. The 95 percent of priapism crisis in SCD patients are ischemic type. In this paper we review the new molecular mechanisms implicated in the pathogenesis of ischemic priapism and provide the basis for potential future therapies(AU)

Recurring priapism may be a symptom of voiding dysfunction – case report and literature review

ABSTRACT Recurring priapism is rare in pre-pubertal children and may be attributed to multiple causes. We propose that voiding dysfunction (VD) may also justify this symptom and detail a clinical case of recurring stuttering priapism associated to overactive bladder that completely resolved after usage of anticholinergics and urotherapy. Sacral parasympathetic activity is responsible for detrusor contraction and for spontaneous erections and a relationship between erections and bladder status has been proved in healthy subjects (morning erections) and models of medullar trauma. High bladder pressures and/or volumes, voiding incoordination and posterior urethritis can potentially trigger reflex erections.