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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An intermittent-flow blood cell separator was used to perform a sub-total RBC exchange pheresis with prompt relief or priapism secondary to sickle cell disease. The blood cell separator offers an efficient, practical, safe method of performing exchange transfusion in the adult. Surgical procedures in the treatment of priapism have met with limited success and carry a 50% rate of subsequent impotence. We believe that RBC exchange pheresis offers a superior approach in the treatment of complications of sickle cell crisis, including priapism, and should be instituted in the symptomatic patient before more drastic procedures are undertaken.
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PMID:RBC exchange pheresis for priapism in sickle cell disease. 49 Aug 30

Since priapism often leads to impotence immediate therapy should be given with few exceptions (patients with sickle cell disease, neoplasm and infections). A careful history and a search for the etiology are important. The physician should protect himself against legal retribution by obtaining the patient's signature on an informed and witnessed consent to treat.
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PMID:Priapism. 75 8

There is wide variation in the clinical manifestations of sickle cell disease (SCD) from one affected individual to another. Many investigators have sought to discern parameters that would explain this variability. In the present studies we have attempted to correlate the frequency of painful events and the extent of end organ failure in SCD with rheologic properties of packed suspensions of sickle cells, using a magneto-acoustic ball microrheometer developed in our laboratory. Using this device we have measured the steady-state viscosity, and the viscous and elastic moduli of cell suspensions in 16 individuals with hemoglobin SS disease who were untransfused and in their steady state. The rheologic parameters were then correlated with clinical parameters. The clinical parameters measured were emergency department visits, hospitalizations, hemoglobin, reticulocyte count, age, and end organ failure (nephropathy, avascular necrosis of bone, stroke, retinopathy, resting hypoxemia after acute chest syndrome(s), leg ulcer, and priapism with impotence). The P value for the correlation between the steady state viscosity and end organ failure was .001 with a correlation coefficient (R value) of .73. The P value for the correlation between the viscous modulus of viscosity and end organ failure was .00006 with an R value of .83. The P value for the correlation between the elastic modulus of viscosity and end organ failure was .0006 with an R value of .76. However, there was no significant correlation between any component of packed cell rheology and emergency department visits or hospitalizations for pain.
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PMID:Relationship of clinical severity to packed cell rheology in sickle cell anemia. 182 65

To determine the prevalence and characteristics of priapism associated with sickle cell hemoglobinopathic conditions we interviewed and examined 52 men with sickle cell anemia, 10 with sickle C disease, 3 with sickle B(+)-thalassemia and 19 with sickle cell trait. Of the men 20 with sickle cell anemia (38%) and 1 with sickle B(+)-thalassemia (33%) reported past priapism attacks. Average patient age at onset in the sickle cell anemia patients was 19 years (range 8 to 30 years). Four men had had only 1 attack. The average number of attacks per year in the remaining patients ranged from 1 to 52. Of the patients who reported multiple attacks the average duration of a typical episode and the duration of the longest self-limiting episode was 1.6 and 7.0 hours, respectively. Six patients had experienced 1 attack that persisted for longer than 24 hours. There were no significant differences between the clinical and hematological parameters of the sickle cell anemia patients who did and did not experience priapism or of the priapism patients who had and had not experienced episodes lasting greater than 24 hours. Six patients had been hospitalized for priapism and 4 were treated with a shunting procedure. Of the latter patients 2 were impotent but there was no significant alteration in the sexual function of the other study participants.
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PMID:Priapism associated with the sickle cell hemoglobinopathies: prevalence, natural history and sequelae. 198 2

Priapism is a complication of sickle cell disease, and for those with severe prolonged attacks, it is serious, often resulting in permanent sexual impairment. Treatment is frustrating, and psychological consequences may be profound. A 20-year-old sickle cell patient with intractable priapism associated with intense pain and penile gigantism underwent multiple but unsuccessful medical and surgical treatment regimens. Finally, corpora cavernosa corporectomy was performed. The pathologic findings of extensive vascular thrombosis and stromal fibrosis underscore the irreversibility of this process and explain the inevitable impotence. Pain and sexual impairment were associated with serious psychological difficulties and suicide attempts. Counseling and close follow-up have improved his outlook considerably. He has been pain-free for 1 year, and future management includes consideration of placement of a penile prosthesis.
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PMID:Corporectomy for intractable sickle-associated priapism. 224 76

Priapism lasting more than 24 h in adults with homozygous sickle cell disease usually results in impotence. The dense fibrosis of the corpora cavernosa which destroys the normal vascular erectile system of the penis also complicates insertion of penile prostheses. Our experience in 5 patients is reviewed. Sharp dissection and dilatation were necessary to form a tunnel for the prosthesis, which was more easily excavated anteriorly and posteriorly through a dorsal incision than for the length of the corpora through a traditional perineal-scrotal incision. Damage to the tunica was common and subsequent migration of the prosthesis necessitated an additional 11 procedures under general anaesthesia. Despite these technical difficulties, the procedure allowed satisfactory intercourse. The average interval between onset of impotence and implantation was 4 years. Early implantation before the dense fibrosis develops might give more satisfactory results.
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PMID:Penile prostheses in the management of impotence in sickle cell disease. 235 22

Priapism is characterised by a persistent erection that cannot be relieved by sexual intercourse or masturbation. Although priapism subsides spontaneously in a few days, impotence frequently follows. Both vascular and neural mechanisms are implicated in the pathophysiology of priapism, but it is not clear which initiates the process. Idiopathic cases of priapism are the most frequent (near 50%); other medical conditions that can result in priapism are haematological diseases (mainly sickle cell anaemia and leukaemia), traumatism, and neoplastic processes. Drug-induced priapism comprises about 30% of cases. The drugs most frequently implicated are psychotropic drugs (phenothiazines and trazodone), antihypertensives (mainly prazosin) and heparin. Recently, the intracavernosal injection of vasoactive drugs (papaverine and phentolamine) has been described in patients treated for impotence. With the exception of heparin, an alpha-adrenergic blocking mechanism has been suggested in the priapism-inducing action of these drugs. A significant number of anecdotal case reports link priapism and drugs, and it is possible that certain cases of idiopathic priapism could be reclassified if accurate pharmacological anamnesis were to be performed. Priapism must be considered a urological emergency. Surgical procedures are the most preferred treatment for this condition but, in selected cases, drug treatment seems to be an alternative approach.
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PMID:Drug-induced priapism. Its aetiology, incidence and treatment. 265 50

A 26-year experience with all aspects of priapism is reviewed in 105 children and adults. The etiology of the priapism was idiopathic or drug-induced, or owing to sickle cell disease, trauma, neoplasia, leukemia, papaverine-phentolamine injections and total parenteral alimentation. The pathophysiology of prolonged erection is discussed. Treatment is reviewed in respect to initial studies before the type of shunting procedure required is selected. Various shunt techniques are presented with outcome. Complications and their possible causes are discussed, and the importance of medicolegal risk is emphasized. Impotence is a common sequela of priapism.
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PMID:Experience with 105 patients with priapism: update review of all aspects. 317 72

Review of recent implantations of penile prostheses done at Duke University Medical Center reveals 6 patients implanted for impotence secondary to priapism. Of these 6 patients, 5 had sickle cell anemia-induced priapism and 1 had idiopathic priapism. One patient had implantation of an inflatable prosthesis later followed by a semirigid prosthesis, 3 had implantation of a semirigid prosthesis, and 1 had an unsuccessful attempt at implantation of a semirigid prosthesis. The 5 patients with successful implantation have obtained satisfactory postoperative coital function. We believe that penile prostheses are beneficial in patients with impotence secondary to priapism. The degree of fibrosis encountered suggests that a semirigid prosthesis is more favorable, since the inflatable prosthesis may not overcome corporeal rigidity.
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PMID:Implantation of penile prostheses in patients impotent after priapism. 404 9

In Zaire, homozygote sickle cell disease affects 2% of the population and the heterozygote form 1 subject in 10. In that country, this disorder is the most common cause of priapism. The authors report 23 cases of sickle cell priapism seen over a period of 15 years : 14 cases of homozygote sickel disease and 9 cases of heterozygote sickle disease. Since 1972, all cases of priapism in homozygote sickle disease have been treated with transfusions of concentrated erythrocytes and of hyperosmolar sodium lactate (M6). In heterozygotes, the preferred treatment remains spheno-cavernous anastomosis, which does not cause impotence. In 12 homozygote cases treated by transfusion and alkalinisation, there was only one failure and 11 successes despite the long period between the onset of priapism and the start of treatment (69 hours on average). Despite the long duration of these cases of priaspism, almost all the patients retained an intact virility.
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PMID:[Sickle cell priapism. Therapeutic considerations in the light of 23 cases (author's transl)]. 720 20


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