Male pseudohermaphroditism (MPH) is a complex variety of sexual differentiation disorders characterized by deficiency of masculinization of the internal and/or external genital organs in the presence of testicular development as the male gonad. This condition is caused by embryonic failure in the processes of male sexual development, which is a sequence of mechanisms originating from the genetic sex determination triggered by the SRY gene on the Y chromosome, followed by genital sex differentiation influenced by the fetal testis. Resulting phenotypical features of MPH vary from complete female to mostly normal but with some abmiguity in the maleness. Pubertal changes are also important factors related to etiology. Recent elucidation of detailed mechanisms of male differentiation and its derrangements has been achieved in the era of molecular genetics. Classical classification of MPH, mainly based on anatomical and endocrinological findings obviously needs to subject to a complete revision. The newest version of MPH classification is reviewed and discussed in relation to etiological backgrounds of each type of the disorder. Main etiological factors are: failure of the SRY and its related genes involved in the testis determination; failure of anti-mülerian hormone (AMH) for normal involution of the female duct system; disordered production or function of androgen receptors essential for the fetal differentiation of the male genital organs; 5α-reductase deficiency syndrome; defective responsiveness of the testis to gonadotropin due to Leydig cell agenesis; various types of enzyme defects involved in testicular androgen biosynthesis; fetal testicular dysgenesis syndromes occurring at various stages of embryogenesis; and other less clearly defined entities of MPH. Implications are that other types of sexual differentiation disorders than MPH, such as true hermaphroditism, gonadal dysgenesis and some other disorders that have been considered to be distinct entities, may have close linkage to MPH through dysgenetic process of gonadal development with subsequent degeneration and/or tumorigenesis. Molecular basis of these probably related disorders should be elucidated in the near future and some clues to preventive measures for these genetically determined malformations are awaited.
In order to evaluate the significance of repeat transurethral resection (TUR) in differentiating stage A1 prostatic adenocarcinoma from those with stage A2, we performed repeat TUR in 34 patients with an initial diagnosis of stage A1 prostatic adenocarcinoma. It was found that residual adenocarcinoma was present in five cases (14.7%), but the diagnosis was changed from stage A1 to stage A2 in only one case (2.9%). In one patient with final diagnosis of stage A1 carcinoma, bone metastases were detected seven months after the repeat TUR. It was concluded that repeat TUR for stage A1 prostatic adencarcinoma did not yield clinically significant information.
Prader-Willi syndrome is rather a rare disease. However, as it includes 4 features (hypogonadism, hypomentia, hypotonia, and obesity), urologist may see the patients with this syndrome for their gonadal problem. We studied all the 27 cases in our hospital of which data were precisely collected. Among males, 67% of patients had presented themselves first to the department of pediatric internal medicine. One third of the patients were not diagnosed as the syndrome and referred to our clinic because of urological abnormalities. Chromosomal abnormality was seen in 40.9%. We found cryptorchism in all the cases and micropenis in 56%. In females, delayed menarche over 15-year-old was seen in 75%. From endocrinological studies, 75% male showed low reaction against HCG stimulation. Low gonadotropin responses to LH-RH were seen in 80% of all patients. We performed orohiopexy for cryptorchism, but testicular development was poor. And we do not actively ulilize hormonal therapies for these patients.
Recently, nerve sparing radical prostatectomy has became widely considered as the primary goal for maintaining a high standard of quality of life (QOL). However, anatomical localization of the cavernous nerve has not yet been precisely clarified in terms of the terminal end in the corpus cavernous penis distal to the urogenital membrane. Here in atempt to demonstrate the precise localization of the cavernous nerve, in six adult male cadaver. The cavernous nerves ran between the prostatic capsule and the prostatic fascia, through the capsule of the seminal vesicle. The nerves penetrated the membranous urethra at 8mm from the margin of the urethra at the position of 5 and 7 o'clock. Therefore, the following procedures are critical to achieve successful nerve sparing: 1) meticulous division of the seminal-vesicle, 2) precise separation of the neurovascular bundle between the prostatic capsule and fascia, and 3) the careful transaction of the membranous urethra.
A purpose of mucosal random biopsy of urinary bladder is to get imformatons about the extent of cancerous lesion and the existence of rest of canser. For the purpose of determining the care range of the operation and postoperative treatments by getting these informations during the operation, we drew up stamp cytological preparations for mucosal random biopsy of urinary bladder performed during TUR-Bt, and examined potency of application of this method to quick diagnostics. First of all, 15 times of transurethral resections of bladder cancers were performed on 14 cases, and 19 of imprint preparations were made from the excised tumor materials during operation. Then the accuracy rate of the rapid cytology was evaluated based on permanent preparations that were made simultaneously. The accuracy rate of the intraoperative rapid cytology was 84.2%. Next, a similar procedure was performed on 68 materials of random biopsy during the above-mentioned operation. The accuracy rate of imprint cytology was 73.5%. The relatively low accuracy rate of rapid cytology must be attributed to diagnostic difficulty noted when dealing with Papanicoloau class 3. For all above, it was suggested that rapid implint cytology during operation must be applicable to quick diagnostics.
To examine clinical features and the prognostic factors for renal function in patients with autosomal dominant polycystic kidney disease (ADPKD), a total of 118 patients (60 men and 58 women) were followed for 3 to 192 months (mean 77 months). The mean age of men at the diagnosis of ADPKD was younger than that of women. Main Symptoms were hematuria, hypertension and proteinuria. Forty-one % of the patients showed deterioration of renal function at the diagnosis. The rate of residual volume of renal parenchyma on CT findings was correlated well with renal function. Twenty-eight % of the patients preserved good and stable renal functions for over 5 years, while most of others had deterioration in their renal function. Thirty-four % of the patients started dialysis within 79±62 months from the diagnosis. The frequency of end stage renal failure was 7% at 40 years, 21% at 50 years, 36% at 60 years and 63% at 70 years old, respectively. Men needed hemodialysis at younger ages than women. Renal function of the patients with hypertension was worse than that of the patients without hypertension. The ratio of the value of P. S. P. 120 to that of serum creatinine (PSP120/sCr), and the rate of residual volume of renal parenchyma revealed distinct prognostic factors for renal function.
We examined the distribution of interstitial temperatures during balloon lasertherapy, using 4 mongrel canines. The laser equipment (CL50FS, SLT Japan Co. Ltd. Tokyo, Japan) consisted of Nd: YAG laser that produced 360 spindle-shaped radiation from a laser balloon (2cm long) with a water-cooled circulation system. By measuring the temperature 3-5 minutes after the beginning of the treatment, we obtained following results. The temperature of the bladder neck and urethral sphincter remained below 42°C except under extreme conditions. The temperature at a 6mm depth from the prostatic urethra rose as laser energy increased, but was not affected by increased circulation of cooling water. The temperature at a 10mm depth from the prostatic urethra was not affected by either increased laser energy or increased circulation of cooling water. We concluded that the limit of tissue cooling is within 6mm and direct transmission of laser energy is within 10mm. Distribution of the tissue temperature peaked at a 6mm depth in the prostatic urethra. When cooling water was circulated at a 200ml/min and laser-energy was generated at 30W, the temperature distribution demonstrated that at a 4-10mm depth from the prostatic urethra reached over 45°C and at a 6mm depth from the prostatic urethra, a peak of 52°C was obtained. The possibility of thermotherapy of the prostate was suggested.
We investigated the effect of subjective urinary symptoms on quality of life (QOL) in 536 males who participated in a field survey for prostatic diseases in Hokkaido from 1991 to 1993. To quantitatively estimate the subjective urinary symptoms, we used a questionnaire that included questions about irritative symptoms such as “diurnal frequency”, “nocturia”, “sensation of residual urine”, “urgency” and “frequent urination within 2 hrs.”, and obstructive symptoms such as “difficulty to start urination”, “intermittency during urination”, “strain or pushing”, “weak stream” and “stopping or starting urination”. The incidences of participants with poor QOL increased as irritative and obstructive symptoms became apparent (p<0.001). However, some participants, who had only mild urinary symptoms, could not tolerate the situation and felt impairment of QOL. In contrast, others did not feel a decrease of QOL in daily life in spite of severe symptoms. Thus, subjective urinary symptoms did not always correlate with QOL in some individuals. These results indicated that we should keep in mind not only subjective urinary symptoms but their effect on QOL whenever we determine an indication for medical or surgical intervention for patients having urinary symptoms.
At the Second Tokyo National Hospital, treatment of patients with prostatic cancer was carried out under the same protocol over 12 years. The mean age of the patients was 73.6 years, and 50% of them fell into the Stage D category. As the initial treatment, hormonal therapy consisting primarily of diethylstilbestrol diphosphate (DES-P) was given to 93.8% of the patients, and the effectiveness of the initial treatment was observed in 87.4% of them. The pre-treatment tumor marker value was high in 65.6% of the patients, and reverted to within nornal limits after treatment in 51.3% of them. Relapse was observed in 15.0% of the patients. Side effects of hormonal therapy were observed in 20.7% of the patients, and the side effects resulted in death in 1.3% of them. Throughout the duration of the study, 34.4% of the patients resulted in death; 18.8% of these deaths related to cancer and 13.1% of them to other causes. The 5-year survival rate of all patients was 68.1%, and the 10-year survival rate was 52.7%. DES-P which is the primary drug in the protocol employed at our institution was well tolerated in the patients with minimal side effects when given appropriately and was believed to be an extremely effective drug in the treatment of prostatic cancer in Japan.
We performed augmentation gastrocystoplasty in five adult female patients with radiation cystitis after excising the injured bladder wall. The gastric segment was isolated from the body of the stomach with the right gastroepiploic vessels. The ureters were reimplanted into the gastric segment in 3 patients. Postoperatively, all patients were able to urinate from the urethra, although 1 patient needed reoperation for postoperative fistula. Since strongly acid urine developed after meal in all patients, they were placed on oral femotidine 40mg/day. With this medication the urine pH was kept above 5. Follow-up ranged from 6 months to 3 years 2 months. In 3 patients the dosage of famotidine was gradually reduced without any symptoms. In the other 2 patients strongly acid urine caused symptoms despite the medication of famotidine. Of the 2 patients, one developed a small ulcer at the native bladder with occasional urethral pains and hematuria 7 months postoperatively. The ulcer and symptoms were improved by administration of omeprazole for 6 weeks. The other patient who consistently had mild stress incontinence complained of mild urethral and suprapubic pain. These results indicate that gastrocystoplasty is useful for radiation cystitis and postoperative complication secondary to strongly acid urine are controllable by administration of an acid secretion inhibitor.
Hemorrhagic cystitis resulting from radiation to pelvic visceral malignant lesions often might be incurable and there have been established no definitive treatment. We experienced 2 cases with radiation-induced severe hemorrhagic cystitis refractory to conventional therapy. The treatment with hyperbaric oxygen to control hematuria was performed and obtained successful results. Gross hematuria was disappeared and cystoscopic figure was remarkably improved. No remarkable side-effect was observed in both patient. This experience suggested that hyperbaric oxygen could be considered the primary treatment for patient with radiation-induced hemorrhagic cystitis instead of usual treatment.
We report a case of torsion of an intrascrotal testicular tumor. The patient was a 54-year-old man with a chief complaint of swelling and pain in the left scrotum. A testicular scan using 99mtechnetium RBC demonstrated a large cold area of isotope uptake showing loss of blood flow in his left scrotal content. Pre operative diagnosis was acute testicular torsion. A torsed testicle was delivered through an inguinal incision. The testicle appeared to be twisted 720 degrees clock wise. It was swollen and bluish in color. The blood flow did not recover after the relief of twisting. Radical orchiectomy was performed. The histology demonstrated a seminoma. He was free of disease 4 years after surgery. A torsion of testicular tumor in an undescended testis has been reported sporadically. However, the torsion of the intrascrotal testicular tumor is very rare. There have been 5 reported cases in the world literature and this case seems to be the first one in Japan.
An 80-year-old man, who had been treated for colon cancer 25 years ago, presented with gross hematuria. Rectal examination revealed a soft nodule in the right lobe. The serum prostatic specific antigen (PSA) was elevated to 5.2ng/ml, while prostatic acid phosphate (PAP) was normal. Transrectal ultrasound revealed a hypoechoic mass in peripheral zone of the prostate and dilated seminal vesicle. A needle biopsy of the prostate showed mucinous adenocarcinoma. Under the diagnosis of prostatic tumor with semial vesicle involvement, radical prostatectomy was performed. Histological findings showed organ confinded cancer, of which most was composed of extracellular mucin lakes. Immunohistochemical study revealed the tumor cells positive for PSA and PAP. Mucinous adenocarcinoma of the prostate has been known to be clinically different from non-mucinous adenocarcinoma, in that the former is insensitive to hormonal therapy, is rarely associated with elevated PAP and rarely metastasize to the bone. But our analysis of the literatures is Japan showed no significant difference clinically between mucinous and non mucinous prostatic adenocarcinoma. However mucinous adenocarcinoma with signet ring cell rarely responds to hormonal therapy, which should not be classified to true mucinous adenocarcinoma in the current criteria. True mutinous adenocarcinoma could be a variant of prostatic adenocarcinoma, which is peripheral origin and should be treated like non-mucinous adenocarcinoma.