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Therapeutic Advances in Urology 2017Stuttering priapism is rare and under-investigated clinical entity. Although it shares similarities with ischaemic priapism, by definition, stuttering priapism has... (Review)
Review
Stuttering priapism is rare and under-investigated clinical entity. Although it shares similarities with ischaemic priapism, by definition, stuttering priapism has distinct characteristics that advocate for a different management in the clinical setting. Therefore, the management of stuttering priapism aims primarily to prevent recurrence rather than the resolution of spontaneous attacks. A multimodal approach and the individualization of each case are essential because of the diversity of the condition and the plethora of proposed therapeutic strategies. Understanding the underlying pathophysiology and familiarity with contemporary, past and emerging future agents and therapeutic options are required in order to provide an optimal solution for each patient. In addition, patient counselling and the option to combine therapeutic strategies and challenge second-line therapies are essential weapons in the armament of the urologist. Although further clinical trials and studies are mandatory in order to obtain solid data and provide recommendations, all therapeutic options are analysed, with specific interest in the potential advantages and disadvantages. A structured evaluation procedure is also described.
PubMed: 28932276
DOI: 10.1177/1756287217717913 -
Journal of Clinical Medicine Jul 2023Pediatric chronic myeloid leukemia (CML) is a very rare malignancy (age-related incidence 0.1/100,000) typically presenting with leucocyte counts >100,000/µL. However,... (Review)
Review
Pediatric chronic myeloid leukemia (CML) is a very rare malignancy (age-related incidence 0.1/100,000) typically presenting with leucocyte counts >100,000/µL. However, clinical signs of leukostasis are observed at diagnosis in only approximately 10% of all cases and among these, priapism is infrequent. Here, we analyze data from pediatric CML registries on the occurrence of priapism heralding diagnosis of CML in 16/491 (3.2%) boys (median age 13.5 years, range 4-18) with pediatric CML. In the cohort investigated, duration of priapism resulting in a diagnosis of CML was not reported in 5 patients, and in the remaining 11 patients, occurred as stuttering priapism over 3 months ( = 1), over 6 weeks ( = 1), over 1-2 weeks ( = 2), over several days ( = 2), or 24 h ( = 1), while the remaining 4 boys reported continuous erection lasting over 11-12 h. All patients exhibited splenomegaly and massive leukocytosis (median WBC 470,000/µL, range 236,700-899,000). Interventions to treat priapism were unknown in 5 patients, and in the remaining cohort, comprised intravenous fluids ± heparin ( = 2), penile puncture ( = 5) ± injection of sympathomimetics ( = 4) ± intracavernous shunt operation ( = 1) paralleled by leukocyte-reductive measures. Management without penile puncture by leukapheresis or exchange transfusion was performed in 3 boys. In total, 7 out 15 (47%) long-term survivors (median age 20 years, range 19-25) responded to a questionnaire. All had maintained full erectile function; however, 5/7 had presented with stuttering priapism while in the remaining 2 patients priapism had lasted <12 h until intervention. At its extreme, low-flow priapism lasting for longer than 24 h may result in partial or total impotence by erectile dysfunction. This physical disability can exert a large psychological impact on patients' lives. In a narrative review fashion, we analyzed the literature on priapism in boys with CML which is by categorization stuttering or persisting as mostly painful, ischemic (low-flow) priapism. Details on the pathophysiology are discussed on the background of the different blood rheology of hyperleukocytosis in acute and chronic leukemias. In addition to the data collected, instructive case vignettes demonstrate the diagnostic and treatment approaches and the outcome of boys presenting with priapism. An algorithm for management of priapism in a stepwise fashion is presented. All approaches must be performed in parallel with cytoreductive treatment of leukostasis in CML which comprises leukapheresis and exchange transfusions ± cytotoxic chemotherapy.
PubMed: 37510891
DOI: 10.3390/jcm12144776 -
Urologia Internationalis 2021Infection by COVID-19, being a respiratory disease caused by SARS-CoV-2, can predispose to arterial and venous thrombotic disease, in response to excessive inflammation,...
Infection by COVID-19, being a respiratory disease caused by SARS-CoV-2, can predispose to arterial and venous thrombotic disease, in response to excessive inflammation, platelet activation, endothelial dysfunction, and venous stasis. During the COVID-19 pandemic period, the technological and resource availability for the care of these patients with thrombotic disease is critical, marking a factor of morbidity and poor prognosis in these cases. We describe a case of priapism in a patient with COVID-19, during the course of systemic inflammatory response syndrome and respiratory distress syndrome with a procoagulant state, seeking to relate the pathophysiological factors of ischemic priapism in patients with infection with SARS-Cov-2.
Topics: Adult; COVID-19; Fatal Outcome; Humans; Ischemia; Male; Penile Erection; Penis; Priapism; Regional Blood Flow
PubMed: 34107472
DOI: 10.1159/000514421 -
PloS One 2021Priapism is a urologic emergency characterized by an uncontrolled, persistent and painful erection in the absence of sexual stimulation, which can lead to penile...
Priapism is a urologic emergency characterized by an uncontrolled, persistent and painful erection in the absence of sexual stimulation, which can lead to penile fibrosis and impotence. It is highly frequent in sickle cell disease (SCD) associated with hemolytic episodes. Our aim was to investigate molecules that may participate in the regulation of vascular tone. Eighty eight individuals with SCD were included, of whom thirty-seven reported a history of priapism. Priapism was found to be associated with alterations in laboratory biomarkers, as well as lower levels of HbF. Patients with sickle cell anemia using hydroxyurea and those who received blood products seemed to be less affected by priapism. Multivariate analysis suggested that low HbF and NOm were independently associated with priapism. The frequency of polymorphisms in genes NOS3 and EDN1 was not statistically significant between the studied groups, and the presence of the variant allele was not associated with alterations in NOm and ET-1 levels in patients with SCD. The presence of the variant allele in the polymorphisms investigated did not reveal any influence on the occurrence priapism. Future studies involving larger samples, as well as investigations including patients in priapism crisis, could contribute to an enhanced understanding of the development of priapism in SCD.
Topics: Adolescent; Anemia, Sickle Cell; Case-Control Studies; Child; Endothelin-1; Fetal Hemoglobin; Genetic Association Studies; Humans; Male; Multivariate Analysis; Nitric Oxide; Nitric Oxide Synthase Type III; Polymorphism, Single Nucleotide; Priapism
PubMed: 33539452
DOI: 10.1371/journal.pone.0246067 -
BMJ Case Reports Apr 2022Priapism is an urgent urological condition with varied aetiology that may be classified as low flow (ischaemic) or high flow (non-ischaemic). Diagnosis requires detailed...
Priapism is an urgent urological condition with varied aetiology that may be classified as low flow (ischaemic) or high flow (non-ischaemic). Diagnosis requires detailed clinical history and examination combined with appropriate investigations such as cavernosal blood gas sampling and penile Doppler ultrasound. In the case of high-flow priapism CT angiography can identify sources of abnormal arterial blood flow and cases may be managed conservatively, with surgery or through arterial embolisation. We detail a case of a young man presented 2 weeks after perineal trauma with high-flow priapism with an equivocal penile Doppler ultrasound. Cavernosal blood gas sampling was consistent with arterial blood and CT angiography was performed showing an arteriovenous fistula. The patient was then successfully managed with arterial embolisation resulting in detumescence and preserving sexual function.
Topics: Angiography; Arteriovenous Fistula; Embolization, Therapeutic; Humans; Male; Penis; Priapism
PubMed: 35440436
DOI: 10.1136/bcr-2022-249513 -
Diseases (Basel, Switzerland) Feb 2023Priapism is a very rare complication of malignancy and is usually accompanied by locally advanced or widely metastatic disease. We describe a case of priapism arising in...
BACKGROUND
Priapism is a very rare complication of malignancy and is usually accompanied by locally advanced or widely metastatic disease. We describe a case of priapism arising in a 46-year-old male with localised rectal cancer that was responding to therapy.
CASE PRESENTATION
This patient had just completed two weeks of neoadjuvant, long-course chemoradiation when he presented with persistent painful penile erection. Assessment and diagnosis were delayed for more than 60 h, and although a cause could not be determined from imaging, a near complete radiological response of the primary rectal cancer was seen. His symptoms were refractory to urologic intervention and were associated with extreme psychological distress. He re-presented shortly thereafter with extensively metastatic disease in the lungs, liver, pelvis, scrotum, and penis; additionally, multiple venous thromboses were identified, including in the dorsal penile veins. His priapism was not reversible and was associated with a considerable symptom burden for the remainder of his life. His malignancy did not respond to first-line palliative chemotherapy or radiation, and his clinical course was further complicated by obstructive nephropathy, ileus, and genital skin breakdown with a suspected infection. We initiated comfort measures, and he ultimately died in hospital less than five months after his initial presentation.
CONCLUSION
Priapism in cancer is usually related to tumour infiltration of the penis and corporal bodies resulting in poor venous and lymphatic drainage. The management is palliative and can include chemotherapy, radiation, surgical shunting, and potentially penectomy; however, conservative penis-sparing therapy may be reasonable in patients with limited life expectancy.
PubMed: 36810548
DOI: 10.3390/diseases11010034 -
Urology Case Reports Jan 2022Priapism is defined as a whole or partial penile erection that persists for more than 4 hours without stimulation. Hematologic disorders are a significant risk factor...
Priapism is defined as a whole or partial penile erection that persists for more than 4 hours without stimulation. Hematologic disorders are a significant risk factor for ischemic priapism. Here, we report a case of priapism due to chronic myelocytic leukemia. This is rare considering that the epidemiology of priapism in hematologic malignancies includes only 1% of patients with chronic myelocytic leukemia. Priapism has a relatively high complication rate, and management generally focuses on prompt treatment. Hematologic screening should be performed to rule out the possibility of these disorders that could underlie priapism.
PubMed: 34840959
DOI: 10.1016/j.eucr.2021.101946 -
Medical Principles and Practice :... 2021Sickle cell disease (SCD) is phenotypically heterogeneous. One major genetic modifying factor is the patient's fetal hemoglobin (HbF) level. The latter is determined by... (Review)
Review
Sickle cell disease (SCD) is phenotypically heterogeneous. One major genetic modifying factor is the patient's fetal hemoglobin (HbF) level. The latter is determined by the patient's β-globin gene cluster haplotype and cis- and trans-acting single nucleotide polymorphisms (SNPs) at other distant quantitative trait loci (QTL). The Arab/India haplotype is associated with persistently high HbF levels and also a relatively mild phenotype. This haplotype carries the Xmn1 (C/T) SNP, rs7482144, in the HBG2 locus. The major identified trans-acting QTL contain SNPs residing in the BCL11A on chromosome 2 and the HMIP locus on chromosome 6. These collectively account for 15-30% of HbF expression in different world populations and in patients with SCD or β-thalassemia. Patients with SCD in Kuwait and Eastern Saudi Arabia uniformly carry the Arab/India haplotype, but despite this, the HbF and clinical phenotypes show considerable heterogeneity. Pain episodes and avascular necrosis of the femoral head are particularly common, but severe bacterial infections, stroke, priapism, and leg ulcers are uncommon. Moreover, the HbF modifiers appear to be different; the reported BCL11A and HMIP SNPs appear to play insignificant roles. There are probably novel modifiers to be discovered in this population. This review examines the common clinical phenotypes in Kuwaiti patients with elevated HbF and the available information on HbF modifiers. The response of the patients to hydroxyurea is discussed. The presentation of patients with other sickle compound heterozygotes (Sβthal and HbSD), vis-à-vis their HbF levels, is also addressed critically.
Topics: Anemia, Sickle Cell; Antisickling Agents; Fetal Hemoglobin; Haplotypes; Humans; Hydroxyurea; Kuwait; Polymorphism, Single Nucleotide; beta-Globins
PubMed: 32892201
DOI: 10.1159/000511342 -
Federal Practitioner : For the Health... Jul 2022Priapism is a disorder that occurs when the penis maintains a prolonged erection in the absence of appropriate stimulation. Conditions that result in hypercoagulable...
BACKGROUND
Priapism is a disorder that occurs when the penis maintains a prolonged erection in the absence of appropriate stimulation. Conditions that result in hypercoagulable states and hyperviscosity are associated with ischemic priapism. COVID-19 is increasingly associated with coagulopathy. To date, there are 6 reported cases of priapism occurring in patients with COVID-19, 5 occurring in the setting of critical illness.
CASE PRESENTATION
We present a case of ischemic priapism which we suspect resulted from COVID-19-associated coagulopathy in a patient without severe COVID-19 presentation.
CONCLUSIONS
Although there have been only a handful of reported cases of COVID-19-associated coagulopathy leading to ischemic priapism, it is possible that the true incidence is much higher. While our case highlights the importance of considering COVID-19 infection in the differential diagnosis of ischemic priapism, more research is needed to understand incidence and definitively establish a causative relationship.
PubMed: 36425347
DOI: 10.12788/fp.0286 -
Neuropsychiatric Disease and Treatment 2015Clozapine (CLZ) is the drug of choice for the treatment of resistant schizophrenia; however, its suitable use is limited by the complex adverse effects' profile. The... (Review)
Review
Clozapine (CLZ) is the drug of choice for the treatment of resistant schizophrenia; however, its suitable use is limited by the complex adverse effects' profile. The best-described adverse effects in the literature are represented by agranulocytosis, myocarditis, sedation, weight gain, hypotension, and drooling; nevertheless, there are other known adverse effects that psychiatrists should readily recognize and manage. This review covers the "rare" and "very rare" known adverse effects of CLZ, which have been accurately described in literature. An extensive search on the basis of predefined criteria was made using CLZ and its combination with adverse effects as keywords in electronic databases. Data show the association between the use of CLZ and uncommon adverse effects, including ischemic colitis, paralytic ileus, hematemesis, gastroesophageal reflux disease, priapism, urinary incontinence, pityriasis rosea, intertriginous erythema, pulmonary thromboembolism, pseudo-pheochromocytoma, periorbital edema, and parotitis, which are influenced by other variables including age, early diagnosis, and previous/current pharmacological therapies. Some of these adverse effects, although unpredictable, are often manageable if promptly recognized and treated. Others are serious and potentially life-threatening. However, an adequate knowledge of the drug, clinical vigilance, and rapid intervention can drastically reduce the morbidity and mortality related to CLZ treatment.
PubMed: 26273202
DOI: 10.2147/NDT.S83989