Priapism In Patients Research Articles

Klotho Polymorphisms and Priapism In Sickle Cell Disease.

AbstractAbstract 1653 Background:Sickle cell disease (SCD) is a monogenic disorder with phenotypic heterogeneity, possibly determined by polymorphisms (SNPs) in genes whose products modify the pathophysiology of the disease. Priapism is one of the most common vaso-occlusive complications of SCD, and it occurred in more than 30% of males. The Klotho (KL) gene appears to be associated with vascular function and nitric oxide biology and the presence of SNPs could affect its function. Association between KL and priapism in SCD patients was suggested by Nolan et al. in 2004. However, other authors could not confirm this finding (Elliot et al., 2007). Objective:We decided to evaluate the relevance of SNPs rs2249358, rs211234 and rs9536314 to the occurrence of priapism in patients with SCD followed at Outpatient clinic at Escola Paulista de Medicina/UNIFESP. Methods:Forty male patients with SCD were enrolled, 39 (97.5%) with sickle cell anemia (SS) and one (2.5%) SC hemoglobinopathy. The manifestation of priapism was identified through analyses of medical records. The SNP rs2249358 was identified by PCR followed by restriction with XbaI. The other SNPs, rs211234 and rs9536314, were analyzed by allele specific PCR. Statistical analysis: t test, Chi2 or Fisher. This study was approved by Ethical Committee, and all patients agreed in participate. Results:The median age of the patient was 28.5 years-old (20-68 y.o.). Fourteen out of 40 patients had priapism (35%), each one with SS disease. The group of patients with priapism were older (32.5 y.o., 25–68 y.o.) than the group without this manifestation (27.5 y.o., 20–56 y.o.) (p=0.03). There was no statistical difference in the distribution of the SNPs rs211234 and rs9536314 between the two groups of patients (p=0.51 and p= 0.09, respectively). Regarding the distribution of SNP rs2249358, the group with priapism presented 8 individuals (57.1%) with GG genotype, 5 (35.7%) with AA and 1 (7.17%) with AG, whereas in the group without priapism, the distribution was different: 5 (19.2%) with GG, 7 (26.9%) with AA and 14 (53.8%) with AG genotype (p=0.0212). When we compare the presence of at least one A allele (AA or A-) with the G allele in homozygosis (GG), we observed that the A allele has a protector effect (OR: 0.1786; CI: 0.04232–0.7535) (p=0.031). Conclusions:In a relatively small group of patients with SCD, it was observed a significant proportion of individuals with priapism, which reinforces the importance of this manifestation. We also observed correlation between SNP rs2249358 of KL gene and priapism, as suggested previously. Disclosures:No relevant conflicts of interest to declare.

The actual incidence of papaverine-induced priapism in patients with erectile dysfunction following penile colour Doppler ultrasonography

Penile color Doppler sonography is a valuable method for evaluating erectile dysfunction. However, there are some concerns about the safety of this method due to the intracorporeal pharmacological injection, which may cause priapism as a complication, resulting in penile fibrosis. To evaluate the actual incidence of papaverine-induced priapism in patients with erectile dysfunction (ED) who underwent penile colour Doppler sonography and to determine the safety of this diagnostic tool, a retrospective study was conducted using the database of our institution. A total of 672 men with ED underwent penile color Doppler ultrasonography with the intracorporeal injection of 60 mg papaverine hydrochloride. The patient characteristics of priapism cases were retrospectively evaluated. Priapism in 18 of the 672 patients (2.68%) was successfully treated with blood aspiration, irrigation and injection of an alpha-agonist medication, when needed. Patients with priapism were younger compared with those without priapism; mean age 45 +/- 12.51 (20-68) versus 50.93 +/- 12.04 (17-78) (P < 0.001). Penile Doppler ultrasound is a safe procedure in evaluating erectile dysfunction. The incidence of priapism, which is the most important complication of this procedure, is low and can be managed successfully with conservative approaches.

Priapism as the first manifestation of chronic myeloid leukemia

lettersPriapism as the first manifestation of chronic myeloid leukemia Ilias Tazi Ilias Tazi From the Department of Medicine, Hematology Unit, Centre Hospitalier Regional, Beni-Mellal, Morocco Search for more papers by this author Published Online::1 Oct 2009https://doi.org/10.4103/0256-4947.55176SectionsPDF ToolsAdd to favoritesDownload citationTrack citations ShareShare onFacebookTwitterLinked InRedditEmail AboutIntroductionTo The Editor: A previously healthy 33-year-old Moroccan man was referred to the local hospital for persistent painful erection of the penis that had lasted approximately 22 hours. His penis remained erect, painful, and swollen when he arrived at the emergency department. He denied recent intercourse, trauma, use of illicit drugs, use of medications, and radiation therapy. The patient also denied fever, sweats, and chills. The vital signs revealed a body temperature of 37.4 °C, pulse of 98 beats/minute, and blood pressure of 12/7 mm Hg, and respiration of 22 beats/minute. He was alert and oriented. The physical examination revealed that the spleen was palpable 4 cm below the left costal margin. His conjunctiva was pale but no jaundice. On examination, the penis was erect, firm, and tender with superficial venous engorgement. Urinalysis was normal. Laboratory data showed hemoglobin 10.53 g/dL, hematocrit 24%, white blood count 400000/mm3, and platelets 1200000/mm3. A peripheral blood smear demonstrated immature leukocytes in various stages of differentiation. Karyotype analysis revealed the Ph1 chromosome and myeloid hyperplasia in the bone marrow. A diagnosis of chronic myeloid leukemia (CML) was made. Treatment of the priapism was initially performed by cavernosa aspiration and epinephrine irrigation at emergency department under the impression of low flow-type priapism. The erection was relieved later by these procedures. For hyperleukocytosis, the patient was admitted to the hematological ward. He was started on hydroxyurea therapy (50 mg/kg/day) associated with intravenous fluid hydration and allopurinol (300 mg/day) for potential tumor lysis syndrome. After 15 days of chemotherapy with hydroxyurea, the patient's leukocyte and platelet counts were 8000/mm3 and 240000/mm3, respectively. Recurrent priapism had not happened to him during his admission period. The patient was treated with imatinib mesylate for his CML and continued to report to us without any erectile dysfunction until the date of writing.Priapism is a rare disease, characterized by prolonged, painful and irreducible erection, not resulting in ejaculation. It is an andrological emergency with a poor prognosis, as the risk of impotence is 50% despite appropriate management. Sickle cell anaemia, chronic myelogenous leukemia, chronic lymphocytic leukemia, and acute lymphoblastic leukemia are hematologic disorders that can be a cause of priapism.1 In adult leukemic patients, the incidence of priapism is estimated to be approximately 5%.2 Priapism as a result of hematologic malignancy is most likely caused by venous obstruction from microemboli/thrombi as well as hyperviscosity caused by the increased number of circulating leukocytes in mature and immature forms.3 In cases of hematologic malignancy, controversy has existed regarding the optimal treatment of leukemic priapism. Earlier series of case reports show successful detumescence with local radiation therapy, open surgical shunting, or combination of the two treatments.4 More recent literature has focused on the use of cytoreductive modalities such as chemotherapy or combination chemotherapy and leukapheresis.5 Because of the relatively rare occurrence of leukemic priapism and the small number of case series, there is no standard treatment protocol. Chemotherapy or radiotherapy may first be attempted. If detumescence is not achieved, then surgical shunting should be considered.ARTICLE REFERENCES:1. Schreibman SM, Gee TS, Grabstald H. "Management of priapism in patients with chronic granulocytic leukemia" . J Urol. 197406 111(6):786-8. Google Scholar2. Allué López M, García de Jalón Martínez A, Pascual Regueiro D, Mallén Mateo E, Villanueva Benedicto A, Rioja Sanz LA. "[Priapism as an initial presentation of chronic myeloid leukaemia]" . Actas Urol Esp. 200405 28(5):387-9. Google Scholar3. Mulhall JP, Honig SC. "Priapism: etiology and management" . Acad Emerg Med. 199608 3(8):810-6. Google Scholar4. Winter CC, McDowell G. "Experience with 105 patients with priapism: update review of all aspects" . J Urol. 198811 140(5):3. Google Scholar5. Kumar L, Sagar TG, Majhi U, Shanta V. "Priapism in leukaemia-report of three cases" . J Assoc Physicians India. 199003 38(3):229-30. Google Scholar Previous article Next article FiguresReferencesRelatedDetails Volume 29, Issue 5Sep–Oct 2009 Metrics History Published online1 October 2009 InformationCopyright © 2009, Annals of Saudi MedicineThis is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.PDF download

Treatment of Recurrent Priapism in Sickle Cell Anemia With Finasteride: A New Approach

To determine whether the use of finasteride controls recurrent priapism in patients with sickle cell anemia. Thirty-five patients with recurrent priapism because of sickle cell disease received finasteride during 120 days. The initial dose was decreased every 40 days, from 5 mg/d to 3 mg and then to 1 mg of finasteride until the end of 120 days. Five groups (G) were created based on priapism episodes in a month: G0, no episode; G1, 1-15 episodes; G2, 16-30; G3, 31-45; and G4, >45 episodes. Records on day 0: G0, no patient; G1, 7 (20%); G2, 21 (60%); G3, 4 (12%); and G4: 3 (8%). After 40 days of using 5 mg/d finasteride we found the following results: G0, 5 patients (14%); G1, 19 (55%); G2, 8 (23%); G3, 3 (8%); and G4, none. At the end of the 40-day period, using 3 mg/d finasteride, the findings were as follows: G0, 19 patients (55%); G1, 14 (39%); G2, 2 (6%); G3, none; and G4, none. The findings after 120 days with 1 mg/d finasteride for the last 40 days were as follows: 16 patients (46%) and G1, 16 (46%). In 1 patient, the dose was increased to 3 mg and in 2 patients, to 5 mg, so as to achieve remission. To our knowledge, this is the first study demonstrating that the use of finasteride could decrease and control the number of priapism recurrences in patients with sickle cell anemia, with fewer side effects than other drugs currently used.

Increased Red Blood Cell Adhesion Is Associated with Priapism in Sickle Cell Disease.

The enhanced adhesion of sickle RBCs likely plays a role in the pathogenesis of vaso-occlusive complications in patients with sickle cell disease (SCD). In preliminary studies, we have previously found that RBCs from patients with overt stroke associated with large vessel disease have an abnormally enhanced adhesive phenotype. Priapism, another common vaso-occlusive complication that occurs in approximately 30% of males with SCD, has been associated with the development of stroke as well as pulmonary hypertension. The exact pathophysiology of priapism in the context of SCD is not completely understood. Recently, a potential mechanism of hemolysis-associated NO resistance, endothelial dysfunction and end-organ vasculopathy, including priapism, has been described. In this study, we hypothesized that increased RBC adhesion will also be linked to the development of priapism in patients with SCD. After obtaining informed consent, citrated blood for RBC adhesion analysis was obtained from greater than 3 year old males with homozygous HbSS disease during routine clinic visits (steady state) to the Wisconsin Sickle Cell Comprehensive Center. Female patients, those less than 3 years of age, or those with a history of stroke or recent blood transfusion were excluded. Washed RBCs were perfused through flow chambers previously coated with thrombospondin (TSP) (2μg/cm2) at a wall shear stress of 1 dyne/cm2 that mimics the forces in post-capillary venules and optimizes sickle RBC adhesion. After rinsing, adherent RBCs per unit area were counted in four random fields in each of two duplicate wells by direct microscopic visualization. Mean adhesion levels were compared between patients with a history of priapism and those patients without a history of priapism using an independent samples t-test. We studied 27 male patients who met the above inclusion criteria. The priapism group contained 8 patients and the control group 19 patients. The priapism group was older (18.7 vs. 12.9 years). The two groups had comparable laboratory data (Hb, retic, LDH), except for a trend toward a higher total bilirubin in the priapism group (6.4 vs. 3.1 μg/dL), which may be a marker of increased hemolysis. The level of adhesion to TSP for RBCs from patients with priapism was significantly higher compared to the patients without priapism (1336 ± 65 vs. 672 ± 93 RBCs/mm2; mean ± SEM; p= <.0001). Of the 8 patients with priapism, 2 were taking hydroxyurea (HU) at the time of adhesion analysis. Because HU has been shown to decrease sickle cell adhesion, we also analyzed our data excluding all patients taking HU at the time of the adhesion assay. After excluding patients on HU, we still found increased levels of adhesion for RBCs from patients with priapism compared to RBCs from patients without priapism (p= <.0001). In summary, sickle RBCs from patients who develop priapism have increased adhesion to TSP when compared to patients who have not developed priapism. Enhanced RBC adhesion may be an important marker of male patients at increased risk for priapism and perhaps for other severe vaso-occlusive complications associated with SCD.

Hematological parameters in sickle cell anemia patients with and without priapism.

BACKGROUNDPriapism was associated with certain hematological parameters in sickle cell anemia (SCA) patients in one report but not in another. We studied differences in haematological parameters between SCA patients with and without priapism.PATIENTS AND METHODSEighteen patients with SCA who presented with acute priapism during the years 2001–2004 were compared with age- and sex-matched SCA patients without priapism with respect to hematocrit, reticulocyte count, level of irreversibly sickled cells (ISC), percentage of haemoglobin F (Hb F), total leukocyte and platelet counts.RESULTSSCA patients with priapism had a mean hematocrit of 0.28 L/L, which was significantly higher than the mean hematocrit value of 0.24 L/L (P<0.05) in patients without priapism. The mean reticulocyte count of 8% in patients with priapism was significantly lower than mean reticulocyte count of 12% (P<0.05) in patients without priapism. The level of ISC of 3% in patients with priapism was significantly lower than the level of 6.5% (P<0.05) in patients without priapism. There was no statistically significant difference in the mean levels of Hb F (7% vs. 6%). Patients with priapism had a mean leukocyte count and mean platelet count that did not significantly differ from values in patients without priapism.CONCLUSIONSSCA patients with priapism had a lower rate of hemolysis, resulting in a higher hematocrit and greater blood viscosity, which increased the risk of corpora cavernosal sickling and blockade. Hence, a relatively high hematocrit is a risk factor for the development priapism in patients with sickle cell anemia.

Uso do dietil-estilbestrol no tratamento do priaspismo em pacientes com doença falciforme - Relato de dois casos e uma a revisão da literatura

Priapismo é a ereção dolorosa e persistente do pênis, associado ou não ao estímulo sexual. A prevalência de priapismo em pacientes com doença falciforme é de 2% a 11%, embora estudos retrospectivos tenham demonstrado que 30% a 45% dos pacientes relatam um ou mais episódios de priapismo. Priapismo prolongado é uma emergência urológica que requer intervenção urgente a fim de evitar fibrose irreversível do pênis e impotência. Diversos procedimentos terapêuticos, incluindo: hidratação, analgesia, eritrocitaférese, vasodilatadores, hidroxiuréia, hormônios (dietil-estilbestrol), aspiração do corpo cavernoso e administração local de drogas vasoativas, e tratamento cirúrgico (fístula caverno-esponjosa) têm sido propostos no tratamento do priapismo em pacientes com doença falciforme. O objetivo deste relato é descrever dois casos clínicos de pacientes com doença falciforme e priapismo que não responderam ao tratamento clínico e hemoterápico, mas obtiveram resolução completa dos episódios agudos com o uso do dietil-estilbestrol.

Efficacy of exchange blood transfusion in the management of priapism in patients with sickle cell disease in northeast Nigeria

Background: Thirteen cases of priapism seen in adult patients with sickle cell disease (SCD) were treated by exchange blood transfusion (EBT) at the University of Maiduguri Teaching Hospital during a five-year period (1996-2000). Each patient was scheduled to receive a total of 12-unit exchange, but due to voluntary donor blood scarcity and differences in affordability of commercial donors only 1 (7.7%) patient could receive 10 units, 3 (23.1%) patients had 8 units each, and 9 (69.2%) patients received 7 units each. Method: EBT was carried out manually in all cases and the mean number of units exchanged, the mean levels of Hb S and the mean levels of haematocrit were determined before the start of EBT (pre-EBT) and at the time of achieving complete detumescence. Result: The exchange procedures were started with mean pre- EBT levels of 95% of Hb S and 0.24L/L of haematocrit; complete detumescence was achieved in all cases after an average of 6 units were exchanged by which time the mean Hb S level had fallen to 50.3% and the haematocrit had risen to a mean level of 0.28L/L. Conclusion: EBT was successful in achieving complete detumescence and at much higher Hb S level (50.3%) than was earlier recommended. EBT is therefore effective in treating priapism in SCD and should be offered to such patients in order to avoid surgery, the risks of general anaesthesia in SCD and the possibility of postoperative impotence. Keywords: Sickle cell disease, Priapism, Exchange blood transfusion. Nig. J. of Surgical Research Vol.4(1-2) 2002: 22-27

THE PREDICTION OF PAPAVERINE INDUCED PRIAPISM BY COLOR DOPPLER SONOGRAPHY

We identified color Doppler sonography findings that are useful for predicting priapism in patients after the intracavernous injection of diagnostic papaverine. We evaluated 72 men with erectile dysfunction by color Doppler sonography after the diagnostic injection of papaverine. Cavernous artery waveforms were recorded, and peak systolic and diastolic velocity of the recorded waveforms was measured. In cases of priapism color Doppler sonography findings were retrospectively evaluated to identify any finding that would predict priapism. Priapism was observed in 8 of the 72 patients (11.1%). In 6 of 8 priapism cases there was no blood flow in the cavernous artery during or after adequate erection, including after minutes 5, 10 and 20 in 3, 1 and 2, respectively. This absence of encoding was not relieved even after all stimuli were removed. In 2 patients who later presented with complaints of priapism the Doppler study was completed normally and all parameters were acquired without any cessation of blood flow in the cavernous artery. The persistent disappearance of color and pulse encoding in the cavernous artery predicted priapism with 75% sensitivity, 100% specificity, a positive and negative predictive value of 100% and 96.9%, respectively, and 97% accuracy. The persistence of absent blood flow in the cavernous artery even after the elimination of all stimuli during penile color Doppler ultrasound is a reliable predictor of priapism. The finding is accurate enough to initiate treatment for priapism to avoid further delay.

Do automated red cell exchanges relieve priapism in patients with sickle cell anemia?

Priapism is a dramatic, painful complication for some men afflicted by sickle cell anemia. Although the natural history remains unclear, many believe replacing the patient's abnormal red blood cells (RBCs) with normal RBCs by apheresis is effective. However, no controlled trials have demonstrated its effectiveness. We exchanged 7 men after medical management failed. All procedures reduced sickle hemoglobin levels to < 30%. Two patients underwent emergency automated red cell exchanges without any detumescence or reduction of pain. The remaining 5 patients were exchanged non-emergently; 4 experienced no detumescence or relief of pain. One adult experienced resolution 8 h postexchange. However, he had a history of "stuttering" priapism. All required decompression procedures. Automated RBC exchanges were not effective in achieving detumescence or reducing pain.

High Flow Priapism Associated with Sickle Cell Disease

Priapism associated with sickle cell disease is classically described as a low flow state. We report 2 cases of high flow priapism associated with sickle cell disease. High flow priapism has previously been reported almost exclusively in patients with traumatic rupture of the cavernous artery. Neither of our patients had historical or radiographic findings consistent with injury to the penile vasculature. One patient was treated unsuccessfully with intracorporeal injection of methylene blue and 1 underwent successfully bilateral pudendal artery embolization. The pathophysiological mechanism(s) responsible for the production of high flow priapism in patients with sickle cell disease is not known.

Priapism in patients treated with total parenteral nutrition: B. Ekstrom and A.M. Olsson. Br J Urol 59:170–171, (February), 1987

Priapism in patients treated with total parenteral nutrition: B. Ekstrom and A.M. Olsson. Br J Urol 59:170–171, (February), 1987

Priapism in Patients Treated With Total Parenteral Nutrition

No AccessJournal of Urology1 Sep 1987Priapism in Patients Treated With Total Parenteral Nutrition B. Ekström, and A.M. Olsson B. EkströmB. Ekström More articles by this author , and A.M. OlssonA.M. Olsson More articles by this author View All Author Informationhttps://doi.org/10.1016/S0022-5347(17)43333-7AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail "Priapism in Patients Treated With Total Parenteral Nutrition." The Journal of Urology, 138(3), p. 690 © 1987 by The American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 138Issue 3September 1987Page: 690 Advertisement Copyright & Permissions© 1987 by The American Urological Association Education and Research, Inc.MetricsAuthor Information B. Ekström More articles by this author A.M. Olsson More articles by this author Expand All Advertisement Loading ...

Management of priapism in patients with chronic granulocytic leukemia.

Management of priapism in patients with chronic granulocytic leukemia.