Priapism in patients with hemolytic disorders: a nationwide retrospective cohort study.

Priapism is a persistent, painful erection, which can lead to permanent penile damage and reduced quality of life. Patients with sickle cell disease have an increased risk of priapism which has been related to chronic hemolysis. This study investigates the prevalence of priapism in all major hereditary and acquired forms of hemolytic disorders. Patients with hemolytic disorders were identified in the nationwide Danish Hemolysis Cohort. Each patient was age-sex-matched with 50 comparisons from the general population without hemolysis. We identified the episodes of hospital-registered priapism events for both patients with hemolysis disorders and comparisons in the Danish National Patient Register between 1977 and 2016. We identified 4181 male patients with hemolytic disorders and 205,994 male comparisons, with 2,294,027 person-years of total observation time. Totally, 101 episodes of priapism occurred during follow-up period. Six episodes of priapism were recorded in three patients with a hemolytic disorder, all affected by sickle cell disease. Two of these patients had verified genotype HbSS. The incidence rate for first priapism in sickle cell disease was 432.8 per 100,000 person-years [95% CI: 139.6; 1341.8] versus 0.84 per 100,000 person-years [95% CI 0.54; 1.32] in comparisons. Using a large nationwide cohort, we found that only sickle cell disease is associated with priapism among patients with hemolytic disorders. The incidence rate of priapism in patients with sickle cell disease was lower than previously reported.

Stuttering Priapism in a Teenage Boy: Lesson to be Learnt.

Untreated priapism can lead to ischemic damage of the penis and impotence. This case report describes a 14-year-old boy who presented with a history of priapism for 2 months, which was undiagnosed, ridiculed, and ignored even by medical practitioners. The underlying etiology was later identified to be chronic myeloid leukemia. Despite the usage of multimodal treatment, it took 7 days for control of priapism. The young boy is now left with an erectile dysfunction. The case highlights that priapism in children is a medical emergency needing aggressive evaluation and treatment.

Occurrence of priapism after transient right MCAO in Swiss albino mice.

Introduction: There are few reports about sexual problems in animal models after stroke. The aim of this paper is to report the occurrence of priapism after right MCAO in Swiss albino mice. In addition, we compared neurological score and apoptosis between the priapism-affected and unaffected mice. Methods: Swiss albino mice were subjected to 45 min' MCAO and 7 days' reperfusion. Mice were observed before MCAO, then daily for 7 days to assess priapism. Neurological status and apoptosis (TUNEL assay) were assessed and compared in priapism and non-priapism mice. Results: The results showed that the incidence of priapism after MCAO in Swiss albino mice were 65%. Priapism was detectable often at day 2 after stroke. Priapism-affected group had more severe behavioural deficits after stroke compared to non-priapism stroke mice. Conclusion: Priapism after right MCAO is not rare in albino mice and could be considered as a marker of stroke severity. Further studies are needed to assess the incidence of priapism after stroke in other animal species used for stroke studies such as rat.

Molecular Profile of Priapism Associated with Low Nitric Oxide Bioavailability.

Priapism is a disorder in which prolonged penile erection persists uncontrollably, potentially leading to tissue damage. Priapism commonly afflicts patient populations with severely low nitric oxide (NO) bioavailability. Because NO is a primary mediator of erection, the molecular mechanisms involved in priapism pathophysiology associated with low NO bioavailability are not well-understood. The objective of this study was to identify dysregulated molecular targets and signaling pathways in penile tissue of a mouse model of low NO bioavailability that have potential relevance to priapism. Neuronal plus endothelial NO synthase double knockout mice (NOS1/3-/-) were used as a model of low NO bioavailability. Priapic-like activity was demonstrated in the NOS1/3-/- mice relative to wild-type (WT) mice by the measurement of prolonged erections following cessation of electrical stimulation of the cavernous nerve. Penile tissue was processed and analyzed by reverse-phase liquid chromatography tandem mass spectrometry. As a result, 1279 total proteins were identified and quantified by spectral counting, 46 of which were down-regulated and 110 of which were up-regulated in NOS1/3-/- versus WT (P < 0.05). Ingenuity Pathway Analysis of differentially expressed proteins revealed increased protein kinase A and G-protein coupled receptor signaling in NOS1/3-/- penises, which represent potential mechanisms contributing to priapism for secondary to low NO bioavailability.

Functional Consequences of Mannose and Asialoglycoprotein Receptor Ablation.

The mannose receptor (ManR, Mrc1) and asialoglycoprotein receptor (ASGR, Asgr1 and Asgr2) are highly abundant endocytic receptors expressed by sinusoidal endothelial cells and parenchymal cells in the liver, respectively. We genetically manipulated either receptor individually or in combination, revealing phenotypic changes in female and male mice associated with changes in circulating levels of many glycoproteins. Both receptors rise and fall in response to progesterone during pregnancy. Thirty percent of Asgr2(-/-) and 65% of Mrc1(-/-)Asgr2(-/-) mice are unable to initiate parturition at the end of pregnancy, whereas Mrc1(-/-) mice initiate normally. Twenty five percent of Mrc1(-/-)Asgr2(-/-) male mice develop priapism when mating due to thrombosis of the penile vein, but neither Mrc1(-/-) nor Asgr2(-/-) mice do so. The half-life for luteinizing hormone (LH) clearance increases in Mrc1(-/-) and Mrc1(-/-)Asgr2(-/-) mice but not in Asgr2(-/-) mice; however, LH and testosterone are elevated in all three knockouts. The ManR clears LH thus regulating testosterone production, whereas the ASGR appears to mediate clearance of an unidentified glycoprotein that increases LH levels. More than 40 circulating glycoproteins are elevated >3.0-fold in pregnant Mrc1(-/-)Asgr2(-/-) mice. Pregnancy-specific glycoprotein 23, undetectable in WT mice (<50 ng/ml plasma), reaches levels of 1-10 mg/ml in the plasma of Mrc1(-/-)Asgr2(-/-) and Asgr2(-/-) mice, indicating it is cleared by the ASGR. Elevation of multiple coagulation factors in Mrc1(-/-)Asgr2(-/-) mice may account for priapism seen in males. These male and female phenotypic changes underscore the key roles of the ManR and ASGR in controlling circulating levels of numerous glycoproteins critical for regulating reproductive hormones and blood coagulation.

The possible protective effects of dipyridamole on ischemic reperfusion injury of priapism.

PURPOSE: To investigate the protective effects against ischemia reperfusion injury of dipyridamole in a model of induced priapism in rats. MATERIALS AND METHODS: Twenty-four male Sprague-Dawley rats were divided into four groups, control, P/R, P/R+DMSO and P/R+D. 3ml blood specimens were collected from vena cava inferior in order to determine serum MDA, IMA, TAS, TOS and OSI values, and penile tissue was taken for histopathological examination in control group. Priapism was induced in P/R group. After 1h, priapism was concluded and 30 min reperfusion was performed. In P/R+DMSO group 1ml/kg DMSO was administered intraperitoneally 30 min before reperfusion, while in P/R+D group 10mg/kg dipyridamole was administered intraperitoneally 30 min before reperfusion. Blood and penis specimens were collected after the end of 30 min reperfusion period. Sinusoidal area (µm2), tears in tunica albuginea and injury parameters in sinusoidal endothelium of penis were investigated. RESULTS: Histopathological examination revealed no significant changes in term of sinusoidal area. A decrease in tears was observed in P/R+D group compared to P/R group (p<0.05). Endothelial injury decreased in P/R+D group compared to P/R group (p>0.05). There were no significant differences in MDA and IMA values between groups. A significant increase in TOS and OSI values was observed in P/R+D group compared to P/R group. A significant decrease in TAS levels was observed in P/R+D group compared to the P/R group. CONCLUSIONS: The administration of dipyridamole before reperfusion in ischemic priapism model has a potential protective effect against histopathological injury of the penis.

Painful ultrasound detected lesion in the proximal part of the corpus cavernosum: A case of so called "partial priapism"?

Partial priapism is a rare disorder generally described in literature as related to an idiopathic etiology leading to the thrombosis of the corpus cavernosum. Despite his rarity, this condition has been described in the last years with an increased frequency. It is characterized by thrombosis of the proximal segment of one corpus cavernosum with perineal pain as the more frequent clinical manifestation. Few cases were associated with perineal trauma. Instrumental appearance suggests for an hematoma in the interstitium of the proximal part of corpus cavernosum. Therapy is still controversial. We report a case of a 52-years old man referred to our Section of Urology suffering from a perineal pain occurred without trauma, sexual arousal or sexual intercourse, during the working office time. Laboratory revealed a slightly elevated white blood cells count. The full blood count, protein C reactive, electrolytes, international normalized ratio, activated partial thromboplastin time and urinalysis were within normal range. Tunica albuginea was normal. The partial thrombosis of the right corpus cavernosum was hypothesized. Treatment was conservative with non steroidal anti-inflammatory drug. Only the clinical presentation of symptoms and perineal ultrasound scan performed with color sonography leaded to the diagnosis. Eleven days later, at the clinical and ultrasonographic follow-up visit, the patient was asymptomatic with a total clinical and instrumental disappearance of signs of the corpus cavernosum involvement previously described. Considering the rarity of the condition, we performed a literature review.

The reduction corporoplasty: the answer to the improbable urologic question "can you make my penis smaller?".

INTRODUCTION: Aneurysmal dilatation of the corpora cavernosa can occur because of recurrent priapism in the setting of sickle cell disease. AIM: We present the first case of a successful implementation of the reduction corporoplasty technique for treatment of a phallus that was "too large for intercourse." METHODS: We describe the presentation of a 17-year-old male with a history of sickle cell disease with a phallus "too large for intercourse." Patient reported normal erectile function and response with masturbation but also reported inability to penetrate his partner due to the enlarged and disfigured morphology. He had three priapismic episodes since the age of 10 that progressively led to an aneurysmal morphologic deformity of his phallus. Evaluation included a magnetic resonance imaging, which revealed true aneurysmal dilatation of bilateral corpora cavernosa in the middle and distal portions, and diffusely hyperplastic tunica. MAIN OUTCOME MEASURE: The main outcome measure is the successful management of phallic disfiguration. RESULTS: Reduction corporoplasty was performed, and the patient reported intact erectile function without aneurysmal recurrence. CONCLUSIONS: Patients with significant corporal aneurysmal defects secondary to recurrent priapism can be successfully managed with reduction corporoplasty.

Priapism as a result of chronic myeloid leukemia: case report, pathology, and review of the literature.

INTRODUCTION: Priapism is rare-presenting feature in male patients with chronic myeloid leukemia (CML). Several hypotheses for pathogenesis have been described. Management has been controversial; some authors described resolution following priapism-specific interventions, and others recommended addition of CML-specific therapy or even CML-specific therapy alone. AIM: In this report, we describe presentation and management of a man with refractory priapism that was the first presenting manifestation of CML. We also report, for the first time, the pathology sections of the sinusoidal tissue in such cases. Literature is reviewed for similar cases and their outcome. METHODS: A 21-year-old male patient presented with painful priapism that started 6 days earlier and failed aspiration-irrigation. CBC revealed marked leucocytosis. Oncology care diagnosed CML, and treatment with Imatinib was commenced with prior semen cryopreservation. Following remission, a penile prosthesis was implanted, assisted by optical corporotomy. Sinusoidal tissue biopsy was stained by hematoxylin/eosin (H&E) and CD34. MAIN OUTCOME MEASURES: Pathology sections of cavernous tissue following CML-induced priapism. RESULTS: The penile implant survived without complications. H&E examination of the sinusoidal tissue biopsy revealed leukemic infiltration associated with vascular endothelial damage. CD34 staining showed the mixed picture of leukemic infiltrates, intact vascular endothelium with lumena showing leukemic cells, alternating with destroyed vessels, and no vascular lumena and ruminants of endothelial cells. CONCLUSION: Priapism can be the first manifestation of previously undetected CML. The pathological picture of sinusoidal tissue in such cases is presented. In the case at hand, a complete blood picture was helpful in early diagnosis of CML and early initiation of targeted chemotherapy along with the corporal irrigation/aspiration or shunt surgery. It is therefore recommended to have a CBC examined at presentation of any case of ischemic priapism of unknown etiology, early initiation of CML therapy along with aspiration/irrigation, preferably cryopreserving a semen sample before CML therapy.

Erythrocyte density in sickle cell syndromes is associated with specific clinical manifestations and hemolysis.

Dense, dehydrated red blood cells (DRBCs) are a characteristic feature of sickle-cell disease (SCD). DRBCs play a role in the pathophysiology of SCD acute and chronic organ damage because of heightened tendency to undergo polymerization and sickling because of their higher hemoglobin S concentration. Relations between red cell density (assessed with phthalate density-distribution profile method) and several hematologic, biochemical, genetic parameters, and clinical manifestations were studied in a large cohort of homozygous patients. The percentage of DRBCs was significantly higher in patients who experienced skin ulcers, priapism, or renal dysfunction. Presence of α-thalassemia deletions was associated with fewer DRBCs. A multivariable analysis model showed DRBCs to be positively associated with hemolytic parameters such as lactate dehydrogenase and bilirubin and negatively with fetal hemoglobin. The percentage of DRBCs decreased by 34% at 6 months of hydroxycarbamide (xydroxyurea) therapy. Thus, DRBCs are associated with specific clinical manifestations and biologic markers and may be a useful addition to the biologic and clinical evaluation of patients with SCD, because they can easily be measured in a hematocrit tube.

Hematoma or "partial priapism" in the proximal part of the corpus cavernosum.

INTRODUCTION: "Partial priapism" is a rare disorder. Therapy is controversial in literature. AIM: To show that conservative treatment leads to a full functional recovery of this condition. METHODS: This report describes a case of a hematoma or "partial priapism" in the proximal part of the right corpus cavernosum. RESULTS: Complete functional recovery after conservative treatment with nonsteroidal anti-inflammatory drugs. CONCLUSION: "Partial priapism" probably represents a hematoma in the interstitium of the proximal part of the corpus cavernosum. Conservative treatment is advised.

The use of color duplex ultrasound and magnetic resonance imaging in the dissolution of idiopathic recurrent priapism in patient with congenital penile curvature--a case report.

Priapism, penile erection characterized as prolonged and devoid of sexual stimulation or excitement is a rare condition. It is critical to distinguish between low- and high-flow priapism, because the treatment algorithm differs markedly for these 2 conditions. The diagnosis is made clinically and confirmed with color Doppler ultrasonographic imaging (CDUS). We present a 21 year old men with high-flow priapism and left lateral congenital penile curvature. A duplex Winter shunt procedure was employed with corporeal irrigation of heparin solution and adrenalin solution instillation, but the priapism returned 12 hours after. Following several days slow instillation of phenilephrine or adrenaline solution accompanied by oral flutamide therapy resulted in complete detumescence. We used both CDUS and magnetic resonance imaging (MRI) before and after treatment of priapism. Although CDUS has been the primary modality for cross-sectional imaging of the penis, the superior soft-tissue contrast and spatial resolution afforded by MRI provide an opportunity to advance imaging evaluation of this organ.

Priapism in teenage boys following depot testosterone.

Priapism is rare in children and may result in erectile dysfunction and sexual aversion behaviours. Testosterone therapy is commonly regarded as safe in children and is widely used in constitutional delay of growth and puberty, hypogonadism, hypospadias and micropenis. We report two cases of priapism in teenage boys with constitutional delay of growth and puberty after a change in the formulation of depot testosterone. One case required surgical intervention and the other was preceded by stuttering priapism. These cases illustrate the importance of patient and/or parent counselling before testosterone administration and consideration of lower doses in at-risk patients.

The role of angiotensin receptor blocker (losartan) on decreasing fibrotic process of corpora cavernosa in priapism model of wistar rats.

Background: Priapism induces regulation of Transforming Growth Factor-ß1 (TGF-ß1) expression and collagen-type-1 deposition. This will replace the normal corpora cavernosa with fibrotic tissue which eventually resulted in erectile dysfunction. It is also known that the fibrosis process of corpora cavernosa is related to Renin-Angiotensin II System (RAS). Angiotensin II receptor blockers (ARB), especially losartan, inhibit the inflammation process and fibrotic tissue formation. This study evaluated the effect of losartan in reducing fibrosis in priapism by evaluating TGF-ß1 and collagen-type-1 in cavernous tissue and determined the effect of losartan in preventing fibrosis in priapism model of Wistar rats assessed by the metavir score. Methods: A total of eighteen male Wistar rats mean were divided into five groups. For the priapism models, we applied negative pressure on the penis to make an artificial erection to mimic the priapism process. The control groups were observed and the treatment groups were orally given losartan 15 mg/kg/day. Corpora cavernosa was harvested for TGF-ß1 and collagen-type-1 measurement using an enzyme-linked immunosorbent assay (ELISA). The fibrotic tissue of each rat was then collected and assessed histopathologically with the metavir scoring system. Results: Penile TGF-ß1 concentration in the losartan-treated group was not significantly different on day 10 and day 28 of observation (p10=0,30; p28=0,17). Meanwhile, collagen-type-1 concentration was significantly lower compared to control group (p10=0,002; p28=0,01). There was a significant difference in metavir scores in rats that received losartan and those who did not (p<0,05). Conclusion: Losartan could suppress the fibrosis process in the priapism model. It could decrease the collagen type 1 deposition during corpora cavernosa tissue regeneration. Based on the metavir score, the group receiving losartan therapy was better than the control group.

The roles of testicular nuclear receptor 4 (TR4) in male fertility-priapism and sexual behavior defects in TR4 knockout mice.

BACKGROUND: Successful reproductive efforts require the establishment of a situation favorable for reproduction that requires integration of both behavior and internal physiological events. TR4 nuclear receptor is known to be involved in male fertility via controlling spermatogenesis, yet its roles in regulating other biological events related to reproduction have not been completely revealed. METHODS: Male TR4 knockout (TR4 -/-) and wild type mice were used for the sexual behavior and penile dysfunction studies. Mice were sacrificed for histological examination and corresponding genes profiles were analyzed by quantitative RT-PCR. Reporter gene assays were performed. RESULTS: We describe an unexpected finding of priapism in TR4 -/- mice. As a transcriptional factor, we demonstrated that TR4 transcriptionally modulates a key enzyme regulating penis erection and neuronal nitric oxide synthese NOS (nNOS). Thereby, elimination of TR4 results in nNOS reduction in both mRNA and protein levels, consequently may lead to erectile dysfunction. In addition, male TR4 -/- mice display defects in sexual and social behavior, with increased fear or anxiety, as well as reduced mounting, intromission, and ejaculation. Reduction of ER alpha, ER beta, and oxytocin in the hypothalamus may contribute to defects in sexual behavior and stress response. CONCLUSIONS: Together, these results provide in vivo evidence of important TR4 roles in penile physiology, as well as in male sexual behavior. In conjunction with previous finding, TR4 represents a key factor that controls male fertility via regulating behavior and internal physiological events.

Increased adenosine contributes to penile fibrosis, a dangerous feature of priapism, via A2B adenosine receptor signaling.

Priapism is a condition of persistent penile erection in the absence of sexual excitation. Of men with sickle cell disease (SCD), 40% display priapism. The disorder is a dangerous and urgent condition, given its association with penile fibrosis and eventual erectile dysfunction. Current strategies to prevent its progression are poor because of a lack of fundamental understanding of the molecular mechanisms for penile fibrosis in priapism. Here we demonstrate that increased adenosine is a novel causative factor contributing to penile fibrosis in two independent animal models of priapism, adenosine deaminase (ADA)-deficient mice and SCD transgenic mice. An important finding is that chronic reduction of adenosine by ADA enzyme therapy successfully attenuated penile fibrosis in both mouse models, indicating an essential role of increased adenosine in penile fibrosis and a novel therapeutic possibility for this serious complication. Subsequently, we identified that both mice models share a similar fibrotic gene expression profile in penile tissue (including procollagen I, TGF-beta(1), and plasminogen activator inhibitor-1 mRNA), suggesting that they share similar signaling pathways for progression to penile fibrosis. Thus, in an effort to decipher specific cell types and underlying mechanism responsible for adenosine-mediated penile fibrosis, we purified corpus cavernosal fibroblast cells (CCFCs), the major cell type involved in this process, from wild-type mice. Quantitative RT-PCR showed that the major receptor expressed in these cells is the adenosine receptor A(2B)R. Based on this fact, we further purified CCFCs from A(2B)R-deficient mice and demonstrated that A(2B)R is essential for excess adenosine-mediated penile fibrosis. Finally, we revealed that TGF-beta functions downstream of the A(2B)R to increase CCFC collagen secretion and proliferation. Overall, our studies identify an essential role of increased adenosine in the pathogenesis of penile fibrosis via A(2B)R signaling and offer a potential target for prevention and treatment of penile fibrosis, a dangerous complication seen in priapism.-Wen, J., Jiang, X., Dai, Y., Zhang, Y., Tang, Y., Sun, H., Mi, T., Phatarpekar, P. V., Kellems, R. E., Blackburn, M. R., Xia, Y. Increased adenosine contributes to penile fibrosis, a dangerous feature of priapism, via A(2B) adenosine receptor signaling.

Penile necrosis due to priapism developed after circumcision in a patient with protein S deficiency.

INTRODUCTION: Hypercoagulable state is a complex condition with an abnormal propensity for thrombosis. The consequences of it due to thrombosis of veins and arteries are the most common cause of sickness. AIM: The present study is a report of a case describing penis necrosis after circumcision and evaluation of hypercoagulable state as a reason of it. METHODS: l Nine-year-old boy referred from another hospital with the sequelae of the penile ischemia with discoloration of the penis after traditional circumcision. RESULTS: The ischemic event developed 3 weeks after circumcision. Priapism that was treated with needed glandulocavernous shunt initially developed following circumcision. All values of routine blood count and blood biochemical analysis were within normal limits. Further, hematologic studies revealed that there might be a hypercoagulable state as a result of lower protein S level. CONCLUSION: Circumcision is a common procedure frequently performed in many communities around the world. Although it is seen as an easy procedure and performed even by unauthorized medical stuff, it is not complication-free. Severe penile necrosis after circumcision should be treated on an individual basis, necessitating different techniques. Hypercoagulable state should be concerned and evaluated in such a complication. In short, circumcision has to be done by specialists who can handle such serious complications.

High-flow priapism due to a malignant glomus tumor (glomangiosarcoma) of the corpus cavernosum.

INTRODUCTION: The major cause of non-ischemic high-flow priapism is post-traumatic vascular injury leading to an arterio-lacunar fistula. However, rare causes such as tumors may induce priapism. This is the first report of a malignant glomus tumor localized in the corpora cavernosa. AIM: The aim of this case is to emphasize the importance of the initial management of priapism and to suggest new tracks on the tests to be performed when the usual exams are not sufficient. METHOD: We report the case of a hypervascular penile tumor responsible for high-flow priapism as the first clinical symptom of a metastatic glomus tumor. The persistent penile tumescence was initially considered to be a stuttering priapism and treated using an oral α-adrenergic as no provoking event nor fistula was found. After a 2-week reluctance, a penile magnetic resonance imaging (MRI) was performed. RESULTS: The MRI showed a hypervascular lesion at the proximal part of the right corpora. The lesion was considered as a fistula, and a selective embolization was performed. Two weeks after embolization, the patient came back to the emergency room because of syncopes and dyspnea. Examination by cardiac ultrasound and chest computed tomography revealed the presence of cardiac, pulmonary, and subcutaneous malignant glomus tumors (glomangiosarcoma). Patient received three lines of chemotherapy, and the penile tumor was surgically removed because of persistent erectile dysfunction and perineal pain. CONCLUSION: This case supports the use of corporal body blood gas analysis in difficult cases to discriminate high- and low-flow priapism and penile MRI when clinical history, physical examination, and aspiration are not contributory.

Variations and characteristics of the various clinical phenotypes in a cohort of Nigerian sickle cell patients.

BACKGROUND: Sickle cell anaemia affects about 4 million people across the globe, making it an inherited disorder of public health importance. Red cell lysis consequent upon haemoglobin crystallization and repeated sickling leads to anaemia and a baseline strain on haemopoiesis. Vaso-occlusion and haemolysis underlies majority of the chronic complications of sickle cell. We evaluated the clinical and laboratory features observed across the various clinical phenotypes in adult sickle cell disease patients. METHODS: Steady state data collected prospectively in a cohort of adult sickle cell disease patients as out-patients between July 2010 and July 2020. The information included epidemiological, clinical and laboratory data. RESULTS: About 270 patients were captured in this study (165 males and 105 females). Their ages ranged from 16 to 55 years, with a median age of 25 years. Sixty-eight had leg ulcers, 43 of the males had priapism (erectile dysfunction in 8), 42 had AVN, 31 had nephropathy, 23 had osteomyelitis, 15 had osteoarthritis, 12 had cholelithiasis, 10 had stroke or other neurological impairment, 5 had pulmonary hypertension, while 23 had other complications. Frequency of crisis ranged from 0 to >10/year median of 2. Of the 219 recorded, 148 of the patients had been transfused in the past, while 71 had not. CONCLUSION: The prevalence of SLU, AVN, priapism, nephropathy and the other complications of SCD show some variations from other studies. This variation in the clinical parameters across different clinical phenotypes indicates an interplay between age, genetic and environmental factors.