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[Long-term blood transfusion in sickle-cell anemia].

Ten homozygous sickle cell patients, aged from 4 to 20 years, underwent a periodic red blood cells transfusion program. Indications were = one stroke or more in 4 cases, repeated severe painful crisis in 2 cases, and for others = chronic anaemia induced by hypersplenism, priapism, chronic leg ulceration, dwarfism with delayed sexual maturation. Transfusion program lasted from 6 months to 6 years. In 7 cases, a phlebotomy preceded the transfusion by the same venipuncture in order to reduce the inevitable iron overload progression. Clinical benefit was clear in the following cases: lack of painful crisis with return to school, leg ulceration healing, growth and weight increase, onset of puberty. Benefit is more difficult to assess in the other observations and, particularly, in stroke. No red cell antibodies were found. An HIV contamination occurred in 3 children. All transfused patients during a period exceeding one year developed iron overload, needing a daily chelation by intramuscular deferoxamine in two of them.

Adolescent↗

[Impotence in diabetes mellitus. Etiological factors and therapeutic possibilities].

The neurovascular causes of diabetic impotence are presented. 55 men presenting impotence and diabetes mellitus were examined in an extended diagnostic program. Nocturnal penile tumescence and Papaverin-test showed psychogenic impotence in 10 of these men, which lead to psychosexual therapy of the couple. Because of regional erectile lesions (Mb. Peyronie, penile trauma, inborn penile deviation, Priapism) 8 further diabetics were successfully operated. The remaining 37 patients with diabetes mellitus showed vascular erectile lesions (increased venous drainage in 7 and decreased arterial inflow in 30 men) and were operated upon with the following methods: Microsurgical arterialisation of the penile vein via a V. saphena-graft to the iliaque artery was done in 4 patients. There was an amelioration in 2 and a longterm failure in the remaining 2 men. Vein ligation of both internal iliaque veins and lateral penile veins in 7 patients resulted in 2 short term improvements and 5 failures. Flexible penile prostheses (AMS- and Jonas-prostheses) in 26 patients showed good results in 24 and infectious complications in 2 of them (Explanation of both prostheses, ones partial penile amputation). Vascular interventions for diabetic impotence seem to be of questionable value and therefore the implantation of penile prostheses should be preferred. In diabetics, infection of the alloplastic implants is particularly dangerous and may lead to septicemia and penile amputation.

Adult↗

[NMR Tomography of the penis].

The magnetic resonance tomogram visualises the corpus spongiosum, the corpora cavernosa, the fascia penis profunda, the septum and the dorsal vein of the penis. These structures can be separated from the skin and from the glans penis. Changes of shape are still the essential criteria of assessment. At present it does not seem possible to be able to differentiate between diseased and healthy areas if the connective tissue structures of the penis (e.g. in induratio penis plastica) do not show any change of shape. Compared with other imaging methods, imaging on different levels offers advantages, and so does the simultaneous imaging of the structures of the penis, of its environment and of the adjacent organs. We can assume that the magnetic resonance tomogram will supply essential additional information for clinical diagnosis and practice in conditions like induratio penis plastica, intersexuality, tumours of the prostate gland, and in postoperative follow-up, for example after surgery for priapism.

Humans↗

Hypersexuality in stroke.

Hyposexuality is a common problem in stroke patients. Some stroke patients, however, may present with hypersexuality. We report three stroke patients who demonstrated hypersexuality and deviant sexual behavior after stroke. Abnormal sexual behavior was noted by members of the stroke rehabilitation team while the patients were in hospital. Details of pre- and poststroke sexual activity were obtained from patients and their spouses. All three patients had temporal lobe lesions on computerized tomography, and all had a history of poststroke seizure activity. The 53-year-old man reported poststroke increase in libido and coital frequency with a tendency towards priapism. The two women (47 and 55 years old) exhibited deviant sexual behavior, mood changes, and hyperphagia. The exact cause of these behavior changes is unknown but they may be related to temporal lobe involvement. Hypersexuality has previously been reported in animals and in patients with temporal lobe seizures.

Cerebrovascular Disorders↗

Heterogeneity of sickle-cell anemia based on a profile of hematological variables.

Factors that influence the heterogeneity of the disease expression of sickle-cell anemia are not well understood. This study examines the ability of a profile of six hematological variables (HB, HCT, RBC, %Hb F, MCV, and %HBA2) to predict the severity of disease measured on 225 patients ranging from 0.2 to 18 years of age. Four subgroups of patients were identified separately in each sex using cluster analysis techniques. In each sex, mean hemoglobin concentration and percent Hb F increased across the four clusters from 7 to 10 gm/dl and from 7% to 16%, respectively. Mean cell volumes were approximately 90, 80, 90, and 75 in groups 1, 2, 3, and 4, respectively; thus MCV did not increase in an orderly progression along with HB and %Hb F. We studied the distribution of four anthropometric, five physical examination, and seven clinical measures of disease severity among clusters. In each sex, subgroups differed significantly (P less than .05) for percent ever hospitalized for sickle-cell anemia, percent ever transfused, and percent with bone-age delays greater than 1 year. In addition, male clusters differed significantly for percent ever having had pneumonia, priapism, or dactylitis, and females differed significantly for height and weight. %Hb F and its inverse relationship with %HBA2 was more highly associated with the measures of severity than the degree of anemia or MCV. This study establishes the utility of a vector of hematological variables as a predictor of heterogeneity of measures of clinical manifestations among young patients with sickle-cell anemia. The role of alpha-thalassemia and genetic factors that affect Hb F levels were considered as possible explanations for the observed heterogeneity.

Adolescent↗

Antidepressant drug therapy and sexual dysfunction in men: a review.

Medical literature concerning sexual dysfunction associated with antidepressant drug therapy in men is reviewed. Available information consists mainly of individual case reports or small series of cases. A complicating factor in understanding this area is the lack of sufficient information concerning sexual dysfunction associated with depression. Both erectile dysfunction and ejaculatory problems have been reported with the use of the clinically available antidepressants. No single agent seems to be implicated more frequently than the other drugs. Changes in libido have also been reported. The authors found no reported cases of priapism, which has been reported as a side effect of antipsychotic therapy.

Antidepressive Agents↗

Transfusion support for haemoglobinopathies.

The indications and management of blood transfusion in the haemoglobinopathies have been reviewed. The sickle cell diseases that require transfusion support are sickle cell anaemia, sickle haemoglobin-C and -D diseases and sickle beta-thalassaemia. Homozygous beta-thalassaemia (Cooley's anaemia) is the major problem among the thalassaemias. The pathophysiology of the sickle cell disorders is largely based on the secondary effects of increased blood viscosity, whereas in the thalassaemias the defect is ineffective haematopoiesis. In the former the major problems occur as manifestations of vaso-occlusive crises with disseminated bone and abdominal pain, priapism, stroke and leg ulcers. Bone infarction and aseptic necrosis occur but the widespread bone changes, underdevelopment and haemochromatosis that complicate the thalassaemia are not prominent. Transfusion therapy in the sickle cell diseases is mainly episodic and is guided by the frequency of crises and the severity of vaso-occlusive complications. Partial exchange transfusion and the maintenance of haemoglobin A concentrations at 40 to 50 per cent is frequently indicated. In the thalassaemias, maintenance of haemoglobin levels is essential for normal growth and development. The problem of haemochromatosis is very serious. With hypertransfusion regimens the haemoglobin and haemotocrit are maintained above 12-13 g/dl and 35 per cent. The resulting benefit appears to be reduced blood volume, less iron turnover, and less intestinal iron absorption. The splenomegaly in these disorders is frequently associated with hypersplenism requiring well-timed splenectomy. Chronic and intensive chelation is necessary to prevent the ravages of iron overload. The availability of automated equipment for in vivo and ex vivo blood cell separation has brought new possibilities for improving the management of these haemoglobinopathies. It is feasible, but not as yet practical, to offer transfusions of neocytes (red cells with a mean age of 30 days) which have a 50 per cent longer survival than routine red cell preparations (mean age of 60 days). Neocytes can be prepared ex vivo from fresh routine blood donations using blood cell separator devices. The result is reduced transfusion requirements. A more recent suggestion for using the new technology is to remove the patient's oldest and most abnormal corpuscles on the basis of buoyant density and replacing them with neocytes . Thus the short-lived abnormal red cells would be removed before they could unload their iron. With automation it is possible to perform these procedures on an outpatient basis.

Adult↗

[Sickle cell anemia in surgical patients. Significance and problems].

Drepanocytosis, a disease notoriously widespread among blacks, is surprisingly frequent in certain Italian islands. Surgical aspects of the disease are discussed: erythrocyte sequestration attacks, pigmentary cholelithiasis, priapism, malleolar ulcers and vaso-occlusive attacks. A knowledge of such aspects is considered fundamental for the purposes of a reasoned differential diagnosis in the field of paediatric pathologies requiring surgery.

Anemia, Sickle Cell↗

The uses of corpus cavernosography. A review.

In a series of 18 corpus cavernosograms (CC) we encountered 12 patients with Peyronie's disease, four normals, one with hypoplastic corpora and one with penile fracture. All examinations were successful and there was one complication, a case of post-procedural priapism. CC is a valuable procedure in the evaluation of impotence.

Adult↗

[5 years' experience with revascularization of the corpus cavernosum by a new microsurgical technic in the treatment of vascular sexual impotence].

Treatment of 138 patients with sexual impotence of vascular origin involved revascularization of the dorsal and/or cavernous arteries of the penis by means of a venous auto or homograft branched on the external iliac or femoral arteries. Arteries requiring revascularization can be clearly detected by peroperative arteriography. Two or three shunts are usually necessary. The branches of the venous graft are firstly anastomosed microsurgically (under a 25 to 30 magnification) to the dorsal and/or cavernous arteries of the penis after their microsurgical dissection. The origin of the graft is then joined to the femoral or terminal iliac arteries. Results were generally excellent and there were very few complications: thrombosis during the first 30 postoperative days in 9 cases, hemorrhage in only one case during the last 3 years, frequent but rapidly regressing edema of the foreskin, and obturation of the shunt after the first month (between the 6th and 32nd months) in 4 patients. Postoperative priapism was never noted. This technique, in contrast to that employing direct implantation of the epigastric arteries into the corpus cavernosum, ensures the return of normal physiological erections. Follow-up after a minimum of 6 months showed that recovery was total in 78.6%, the condition being made worse in only 8.8%.

Angiography↗

Metastatic carcinoma of the penis.

Metastatic carcinoma of the penis is distinctly uncommon and in 80 percent of cases is associated with primary malignancy of the pelvic organs with widespread metastases. Pain and priapism are the most common symptoms at the time of the diagnosis. Both cases presented in this paper responded well to local irradiation, subjectively as well as objectively.

Adenocarcinoma↗

Intracavernous injection therapy for male erectile dysfunction.

Intracavernosal injection therapy has consistently produced erections in 60% to 70% of patients in whom it is tried. About 70% of patients enter long-term therapy and there is a dropout rate of 20% to 50% within the first year. The most effective current regimen is a mixture of papaverine-phentolamine and prostaglandin E1, that also seems to have a relatively low incidence of priapism and fibrous nodule formation. Patient education is vitally important to the success of a program. Patient satisfaction rates are high.

Drug Therapy, Combination↗

[Prostaglandin E1 in the diagnosis and therapy of erectile disorders].

Intracavernous injections (ICI) of prostaglandin E1 (PGE1) of German produce (Schwarz Pharma A. G.) have been tested for diagnosis of erectile disorders in 63 patients (age 32-71, the majority at the age 45-65) and for autotherapy in 22 patients. In 76% of males with erectile impotency PGE1 ICI in a dose 10-20 micrograms result in marked erection which emerged within 5-15 min after the injection and persisted for 90 min on the average in spite of ejaculation. 86% of patients suffering from erectile dysfunction found this method of impotency correction satisfactory as it warrants normal coitus in 91% of cases (dose regimen 10-20 micrograms). ICI of PGE1 in the above dose 1-2 times a week for 2 months is safe, entail no systemic and insignificant local side effects. The risk of priapism and cavernous body fibrosis is minimal. The duration of the effect is related to the dose. The above advantages make PGE1 superior to papaverin and phentolamine. PGE1 ICI are recommended as the treatment of choice for therapy of erectile dysfunctions.

Adult↗

Preliminary results of a comparative study with intracavernous sodium nitroprusside and prostaglandin E1 in patients with erectile dysfunction.

We compared the effect of intracavernous administration of sodium nitroprusside, a nitric oxide donor and, therefore, stimulator of the cyclic guanosine monophosphate pathway, with the activity of prostaglandin E1, which is a stimulator of the cyclic adenosine monophosphate pathway. To date 105 patients with erectile dysfunction have entered the study. As part of the diagnostic evaluation every patient received an intracavernous injection of 20 micrograms prostaglandin E1 and a second injection of sodium nitroprusside at different concentrations (100 micrograms in 10 patients, 300 micrograms in 60 and 400 micrograms in 35). Sodium nitroprusside at a dose of 100 micrograms was not effective for inducing erections. Prostaglandin E1 induced better responses overall than sodium nitroprusside at 300 and 400 micrograms (p < 0.001). The overall duration of erections was also significantly longer with prostaglandin E1 (mean 88.5 minutes) than with 300 micrograms sodium nitroprusside (mean 50.8 minutes, p < 0.001) but did not reach statistical significance compared to 400 micrograms sodium nitroprusside (mean 42.2 minutes). Side effects were minimal with both drugs. Although sodium nitroprusside has several benefits over prostaglandin E1 for intracavernous use (such as lower cost, absence of local pain and shorter action, allowing detumescence after orgasm and decreasing the risk of priapism), prostaglandin E1 still remains the agent of choice for intracavernous use.

Alprostadil↗

A new approach to the pharmacologic treatment of impotence.

A new approach to the use of topical medications to induce penile erections is suggested. Many impotent patients treat erectile dysfunction with injected vasoactive drugs like papaverine, phentolamine and PGE-1. However, many patients fear using needles and the possible side effects from injections are well known (pain, ecchymosis, priapism, fibrosis of corpora cavernosa). In six patients with erectile impotence who sought an alternative to pharmacologic injection therapy, a 1 cm square window was created in Buck's fascia and tunica albuginea and covered with a piece of the deep dorsal vein of the penis. The penile skin overlying the window was marked with India ink and vasoactive medication was applied to this area. The goal of this procedure was to enable the topical erection-inducing medication to pass through the skin of the penis, through the window and into the corpus. All of the patients experienced a satisfactory erectile response after application of nitroglycerin cream as documented by penile Doppler ultrasound evaluation. Two of the six patients currently use only the nitroglycerin cream to induce erections satisfactory for sexual intercourse. Three patients alternate the use of the cream and injections. One patient prefers injection. This procedure may offer a desirable alternative to injection therapy for selected patients with impotence.

Administration, Topical↗

Penile prosthesis insertion with corporeal reconstruction with synthetic vascular graft material.

Difficulty with penile prosthesis insertion may be encountered in patients with severe cavernous scarring or tunica albuginea deficiencies. Eleven patients who underwent penile prosthesis implantation required simultaneous corporeal reconstruction due to prior prosthesis infection and/or erosion in 6, priapism in 2 and Peyronie's disease in 1. One patient underwent prior neophallus construction with a tubularized abdominal wall flap for gender reassignment and 1 had congenitally deficient corporeal bodies. In 4 patients previous additional attempts at prosthesis replacement were unsuccessful. The reconstruction techniques included exposure of the corpora usually through a ventral midline incision and repair with synthetic vascular graft material. Accessory sub-coronal incisions were used when the disease was localized to the distal corpora. Six patients required reconstruction of more than half of the corporeal lengths bilaterally and in 5 smaller portions were repaired. Two patients received a semirigid and 9 an inflatable implant. Mean followup was 46 months (range 5 to 81). One patient required early reexploration for separation of the graft from the tunica and 2 required late surgical revision for distal tip divergence. Healing has been excellent and prosthesis function satisfactory in all patients. Infection, erosion and mechanical failure have not occurred. Penile prosthesis insertion with corporeal reconstruction using synthetic graft material is possible in these cases with acceptable morbidity rates and satisfactory erectile function.

Abdominal Muscles↗

[Erection disorders: efficacy and tolerability after 6 months of intracavernous self-injections of alprostadil. Groupe Français d'Etude de l'Alprostadil].

OBJECTIVES: Self-administered intracavernous injections, notably with alprostadil are currently the most widely used non-psychotherapeutical treatment of erectile dysfunction. We assessed the effectiveness of this treatment after 6 months of self-injections. METHODS: Efficacy, acceptability and tolerance to alprostadil self-administered via intracavernous injections for 6 months was evaluated in 144 men, age 24 to 72 years, and followed monthly after an initial training period and search for the optimal dose. RESULTS: The mean number of self-injections was 5.5 at a mean dose of 18.6 micrograms per injection. From the first to sixth month, the frequency of complete erections after self-injection rose from 62% to 77% and failures declined from 8% to 3%. After six months, 40% of the patients had abandoned the treatment including 9% who abandoned during the first month, usually due to poor acceptance and because of cure in two patients (4.5%). Patients reported sexual intercourse after self-injections as satisfactory in 81% of the cases; 66% of their partners reported satisfactory intercourse. Tolerance assessment revealed pain in 14% of the patients and decreasing effect with time in two patients who discontinued self-injections for this reason. Other undesirable side effects were infrequent, usually related to technical injection errors and decreasing effect with time. One case of priapism and two indurations of the cavernous bodies were also observed. CONCLUSION: Self-administration of intracavernous alprostadil can lead to rigid erection in patients with erectile dysfunction. Patients are generally well satisfied and tolerance is good.

Adult↗

Psychotropic medications and their effects on sexual function: diagnosis, biology, and treatment approaches.

BACKGROUND: The recognition and treatment of sexual side effects caused by psychotropic agents have become topics of increasing clinical concern. Gaps in our understanding of the biology of sex and in our knowledge of the effect of Axis I disorders on sexual functioning have made both recognition of sexual side effects and a coherent treatment approach to these side effects difficult. METHOD: The author reviews case reports, case series, and animal studies derived from a MEDLINE search for English language articles on the topics of the effects of psychiatric disorders on sexual functioning, the biology of sex, rates of sexual dysfunction associated with each medication class, and treatment approaches when these side effects occur. RESULTS: In evaluating sexual function in patients taking psychotropic medications, clinicians should first consider other potential causes of sexual dysfunction. In general, dopamine increases sexual behavior, serotonin inhibits it, while norepinephrine has conflicting effects. Sexual side effects have been described in association with all the major classes of psychotropic medications. Neuroleptics are often associated with sexual side effects. Priapism, seen with neuroleptics and trazodone, should be treated as a urological emergency. Anxiolytics cause mild, nonspecific sexual side effects as do the mood stabilizers. Among the antidepressants, the more powerful serotonergic medications--e.g., the serotonin selective reuptake inhibitors (SSRIs), clomipramine, and MAO inhibitors--may cause more sexual side effects than the tricyclics. Potential strategies to treat antidepressant-induced sexual side effects include lowering the dose, waiting, and switching to another agent. A number of specific antidotes, such as cyproheptadine and yohimbine, have been reported to reverse these side effects in a limited number of cases. CONCLUSION: Clinicians must be aware of and specifically ask about medication-induced sexual side effects. More effective treatments of these side effects must await much needed double-blind studies of various approaches, especially those to treat SSRI-induced sexual dysfunction.

Adult↗