Case report: Klippel-Trenaunay syndrome as a cause of erectile dysfunction.
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Biomedical subjects
Publications and source records attributed to J P Pryor.
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PURPOSE: We investigated the immunological features and possible autoimmune basis of Peyronie's disease. MATERIALS AND METHODS: The sera of 100 patients with Peyronie's disease were tested for circulating autoantibodies, including anti-penis antibodies, by indirect immunofluorescence. Antibody deposition and the immunological activity in Peyronie's plaque tissue from patients with early and long-standing disease were also assessed. RESULTS: Circulating anti-penis antibodies were not found in any patient although antinuclear antibodies were present in 24%. Patients with early Peyronie's disease had IgM antibody deposition, marked T lymphocytic and macrocytic infiltration in the sub-tunical space, increased expression of adhesion molecules by endothelial cells and an increased human lymphocyte antigen class 2 expression by the cellular infiltrate, indicating cellular immune activation. CONCLUSIONS: These results show that some of the features of autoimmunity, in particular the cell mediated response, are present in Peyronie's disease.
The animal epididymis was known to play an essential role in the acquisition of motility and fertilising capacity of testicular spermatozoa. Little was known about the function of the human epididymis and this paper describes studies arising from tissues obtained at the time of surgical procedures on the epididymis and vas deferens. Even though the human epididymis differs from that of other animals in fine structure and luminal contents, its function is similar in that spermatozoa gain motility and fertilising capacity during their passage through it. The poor fertilising capacity (24% of 76 cycles) and 2 (2.6%) conceptions with spermatozoa from an obstructed epididymis or vas deferens compared with a fertilisation rate of 88% (pregnancy in 37.5%) in men without obstruction is evidence of this. The defect in fertilising capacity may be overcome by using the technique of intracytoplasmic sperm injection, where fertilisation and embryo transfer occurred in 95% of the 38 couples with obstruction and 34% of the wives conceived.
Congenital abnormalities of the genitourinary tract often coexist, and cryptorchidism is common in patients who have had imperforate anus. Twenty men who had pull-through procedures for imperforate anus in infancy have been evaluated for infertility. Seven had coexisting renal abnormalities, 4 had had recurrent epididymitis, 3 had had bilateral orchidopexies (at age 7 to 12), 2 had spina bifida, and 1 had a pituitary adenoma. Seven had no ejaculate (aspermia), 11 were azoospermic, 1 was severely oligozoospermic, and 1 had a normal sperm concentration in a small volume of ejaculate. Both vasa were blocked in 5 men, and this appeared to be a result of the original operative procedure. One vas was blocked in another 7 patients who had abnormalities on the contralateral side; three had epididymal blocks after epididymitis, and four had congenital malformations associated with an absent or ectopic kidney. After reconstruction (4), insertion of sperm reservoirs (4), microscopic epididymal sperm aspiration (2), or artificial insemination (1), sperm were retrieved from 9 men (ejaculated by 4) 2 pregnancies occurred. Male infertility after treatment of imperforate anus in infancy can be related to a wide variety of cause, some of which are amenable to treatment.
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PURPOSE: We analyze the long-term outcome of the Nesbit operation for the correction of the penile deformity due to Peyronie's disease. MATERIALS AND METHODS: Between 1977 and 1992, the penile deformity due to Peyronie's disease was corrected in 359 patients using the Nesbit operation. RESULTS: The overall success rate was 82%, which increased to 90% during the last 8 years with better patient selection due to improved preoperative investigation. Complications were minimal and only 6 patients had significant penile shortening that precluded sexual intercourse. CONCLUSIONS: The Nesbit operation remains the procedure of choice to correct the penile curvature of Peyronie's disease.
Malignant mesothelioma is a rare, often aggressive tumor of the tunica vaginalis. We report 2 cases and review the literature concerning current management strategies. It appears that, although adjuvant chemotherapy and radiotherapy have little value, local and nodal recurrence may be successfully treated by local excision.
Surgery is capable of correcting many causes of impotence but it is important for the surgeon to have a thorough understanding of the mechanisms of erection, to understand the limitations of current diagnostic techniques and be aware of the psychological needs of his patients. It is then possible to make the correct choice of operation to enable a man to have intercourse by correcting anatomical abnormalities, by restoring defective blood supply or by overcoming defects in the veno-occlusive mechanism. When these techniques are inappropriate, or fail, then the ability to have intercourse may be restored by the implantation of a penile prosthesis.
Ultra high field 1H-NMR spectroscopic methods have been used to analyse the composition of seminal fluid and its component secretions, prostatic and seminal vesicle fluids from normal human subjects and those with vasal aplasia and non-obstructive infertility. The 1H-NMR spectrum of whole seminal fluid is extremely complex and many resonances are extensively overlapped in single pulse spectra even when measured at 600 or 750 MHz 1H resonance frequency. A combination of 2-D 1H-NMR methods (including J-Resolved and various 1H homonuclear correlation and 1H-13C heteronuclear correlation techniques) were applied at 600 or 750 MHz in order to extensively assign the signals from the organic components of seminal fluid. Prostatic fluid (PF) gives a much less complex metabolite profile than whole seminal fluid and can be completely analysed using 500 MHz 1H-NMR spectroscopy. The 1H-NMR spectra of prostatic fluid are dominated by signals from citrate, spermine and myo-inositol, whereas the spectra of seminal vesicle fluid (SVF) show extensively overlapped signals from complex peptide mixtures together with strong signals for glycerophosphocholine (GPC) and lactate. Whole seminal fluid is a combination of the PF and SVF constituents together with further substances that appear after mixing due to the operation of PF enzymes on SVF, e.g. peptidase activity causes rapid cleavage of peptides to amino acids and GPC is hydrolysed to choline, glycerol and inorganic phosphate. It is also shown that vasal aplasia leads to highly characteristic abnormal metabolite profiles in seminal fluid that can be readily observed in single-pulse 500 and 600 MHz 1H-NMR spectra. Measurement of the molar citrate to choline, or spermine to choline ratios in seminal fluid both show differences of 2 orders of magnitude between vasal aplasia (greater for both ratios) and non-obstructed infertile patients. This work gives an indication of the potential of high field 1H-NMR spectroscopy in the investigation and assessment of the secretory functions of the male genital tract and the evaluation of the infertile male subject.
OBJECTIVE: To assess the factors associated with loss of implant following penile prosthetic surgery and to make alterations in the management of patients to prevent this from occurring. PATIENTS AND METHODS: A retrospective analysis was carried out of the factors associated with post-operative infection and erosion in 188 patients who underwent implantation of penile prostheses. A prospective study was then established in which 62 patients were managed by a set protocol designed to counteract the factors identified as being contributory to loss of implants in the retrospective review. RESULTS: In the retrospective study nine (4.8%) patients lost a prosthesis due to erosion. Erosion was noted to be more common with one type of implant which has since been modified. Infection was the cause of prosthesis loss in 12.2% of patients in the retrospective review. The new protocol resulted in a significantly reduced infection rate in penile prostheses to 1.6% (P < 0.05). CONCLUSION: With attention to simple antiseptic prophylaxis, antibiotic penetration and continuing audit, a significant improvement in the loss of penile prostheses can be achieved along with a reduction in the amount of antibiotics prescribed.
In order to study the pattern of male fertility in Noonan syndrome, and its potential implications for genetic counselling, the genital tract function was studied in 11 adult males with Noonan syndrome. Bilateral testicular maldescent occurred in six. The mean testicular volume was 21 (SD 4) ml. The stretched flaccid penile length was 11.4 (SD 1.2) cm. Puberty was delayed in three. Four of the men had fathered children. The LH and testosterone levels were essentially normal in all men, while the FSH levels were grossly raised in the group with testicular maldescent, with the exception of one man. Semen samples were obtained from five men, and azoospermia or oligozoospermia was present in four of them. Sexual function is not affected in men with Noonan syndrome, but the onset of sexual activity was delayed in men with late onset of puberty. Bilateral testicular maldescent appears to be the main factor contributing to impairment of fertility in men with Noonan syndrome.
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It has been suggested that congenital bilateral absence of the vas deferens (CBAVD), an important cause of male infertility, is a variant of cystic fibrosis (CF). This study describes a defect in chloride conductance across the nasal epithelium of subjects with CBAVD which is dissimilar to that found in patients with CF. It also demonstrates normal sodium transport across the nasal epithelium in these men, in contrast to patients with CF who exhibit increased sodium absorption. The increased frequency of CFTR mutations in these men implicates the CFTR gene in the pathogenesis of this disorder. Genetic analysis of men with CBAVD who were heterozygous for a known CFTR mutation failed to identify a second mutation within any of the exons or introns of the CFTR gene. These results demonstrate that most men presenting with CBAVD are not compound heterozygotes for mutations within the CFTR gene and can be distinguished from individuals with atypical or asymptomatic CF on the basis of the bioelectric properties of their nasal epithelium. We postulate that mutations in the promoter region or at other regulatory sites of the CFTR gene may be responsible for the CBAVD phenotype in a proportion of cases.
A laparoscopic varicocele ligation was performed in 49 men with a clinically proven varicocele. Patients were treated predominantly on a day case or overnight basis with minimal post-operative morbidity. Identification of the testicular artery was aided by a laparoscopic Doppler probe in the majority of patients and preserved in 39 cases. The varicocele persisted in 7 patients, 4 of whom have subsequently had an inguinal exploration confirming a missed testicular vein as the source. In all 7 patients the artery was identified without the use of the Doppler probe, this being suggestive of misidentification of the vessels at laparoscopy. The complications included 4 wound infections, 1 scrotal haematoma and 1 vasal injury which necessitated an open vasovasostomy. Laparoscopic varicocele ligation is a simple and safe technique, causing minimal morbidity and enabling a rapid return to normal activity. The success rate can be improved by accurate identification of the testicular vessels with the use of a laparoscopic Doppler probe.
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