[Fetal megacystis with idiopathic intestinal pseudo-obstruction syndrome: a case report].

A 2,510 g female newborn was delivered by Cesarean section at 33 weeks gestation due to increasing volume of her huge bladder and bilateral hydronephrosis. Just after birth 190 ml of urine was drainaged by catheterization without difficulty. Voiding cystourethrography showed no VUR. She had no ureteral dilation, either. Urodynamic study revealed large bladder capacity and detrusor hypocontractility. Neither neurological nor gastrointestinal abnormality was detected neonatally. Clean intermitted catheterization was performed every 4 hours. At 12 months of her age she had no history of urinary tract infection. However, as she grew up with normal diet, severe constipation became apparent. This is a case of idiopathic intestinal pseudo-obstruction syndrome whose gastrointestinal symptom was masked in the newborn period. Attention should be paid for gastrointestinal tract as well as urinary tract when the body has congenital megacystis with unknown etiology.

Surgical results of complete hypospadias repair in two stages.

Between 1980 and 1992, 403 patients with hypospadias and with no previous operations were treated in Hyogo College of Medicine. Surgery was not performed in children younger than 3 years. The major points in the surgical repair were complete resection of the chordee, normal location of the meatus and reducing complications such as fistula formation or stricture. In order to achieve these aims we performed a two-stage repair, with the second operation at leat 12 months after the first one. In 57 of 245 patients with "chordee without hypospadias" and slight to moderate degree of hypospadias we performed surgery by the Crawford-Ikoma technique, which is characterized by 3 layers of watertight continuous sutures at the time of urethroplasty. The incidence of postoperative fistulae was 1.8%, the rate of strictures was 3.5%. After the Mays-Ikoma technique, where we perform a glandular urethroplasty in addition to chordectomy in the first stage, we diagnosed an urethral fistula in 7.9% and a stricture in 2.3%. A severe degree of hypospadias was seen in 158 patients. After performing the Crawford-Ikoma technique we acknowledged a rate of postoperative fistulae in 3.6% and strictures in 10.7%. The incidence of fistulae after the Mays-Ikoma technique was 8.9% and of strictures 1.1%. Altogether we found persistent chordee in 2 cases.

[Clinical study of primary carcinoma in situ of the bladder].

In our department 14 patients with primary carcinoma in situ of the bladder were treated. Thirteen patients were male and 1 patient was female. Most of the patients complained of irritative vesical symptoms such as painful urination and/or pollakisuria. Cystoscopic examination revealed no overt tumor but some abnormal findings like localized or diffuse hyperemia or fine granular changes were noted. In 4 patients, total cystectomy was performed primarily and 10 other patients were treated at first with intravesical chemotherapy or intravesical BCG. Five of those 10 patients (50%) developed invasive cancer and total cystectomy was performed secondarily in them. Invasive cancer occurred in the bladder wall in 2 patients, in the prostate in 2 patients and in both bladder and prostate in 1 patient. Five-year and 10-year survival rates of 14 patients in this study were 66.7% and 44.4%, respectively.

[Anesthesia-free extracorporeal piezoelectric shock wave lithotripsy for urolithiasis].

In November 1991, a third generation extracorporeal shock wave lithotriptor (Piezolith 2500) was installed in our clinic. We examined the effective and safe aspect of this machine for urolithiasis. From November 1991 to October 1993, we experienced 530 sessions of ESWL treatment on 235 patients (270 cases). All patients except two underwent ESWL treatment without anesthesia, and in situ ESWL treatment was possible on 247 cases. The mean number of shock waves and sessions per patient of renal stone were 3369 and 2.1, respectively. Those of ureteral stone were 3970 and 1.9, respectively. The complete removal rate of renal and ureteral stone 3 months after the last session were 86% and 92%, respectively. This outcome was very satisfactory. On the other hand, endoscopic procedure was needed on 13 patients. In almost all cases hematuria was noted after ESWL, but major complications such as subcapsular hematoma or gastrointestinal hemorrhage were not experienced at all. It was concluded that Piezolith 2500 was very effective and safe in the treatment of urolithiasis.

[Expression of integrin molecule in urological tumor cell lines by using RT-PCR method].

Integrins are heterodimer molecule that are composed of one alpha subunit and one beta subunit. Integrins appear to be the major receptors by which cells attach to extracellular matrices, and some integrins also mediate important cell-cell adhesion event. In recent years signaling pathway via beta subunit of integrin molecule has been clarified, and 8 kinds of integrin beta subunit are known to exist. And so we investigated the expression of integrin beta subunit in various urological tumor cell lines by using RT-PCR method. Materials are composed of 8 renal cell carcinoma cell lines, 2 urinary bladder carcinoma cell lines, a testicular tumor cell line and a prostate tumor cell line. All 12 cell lines express integrin beta 1 subunit. The expression rate of beta 4 subunit in renal cell carcinoma lines are lower than that in other urological tumor cell lines. The expression of beta 6 subunit was observed in renal cell carcinoma cell lines and testicular tumor line. In testicular tumor cell line we also found the expression of beta 2 subunit which expression had been believed to be specific in leukocyte.

[Liver impairment in kidney transplant recipients. 1. Prevalence and clinical significance of hepatitis C].

The prevalence and the clinical significance of hepatitis C (HCV) infection in recipients of kidney transplantation was assessed using second generation anti-HCV antibody (anti-HCV-2). Out of 88 patients whose preoperative sera were available for anti-HCV-2 determination, 27 patients (30.7%) were positive. Transfusion units were significantly larger and the duration of hemodialysis was significantly longer in the patients with anti-HCV-2 than those without. In 10 patients whose preoperative sera were negative for anti-HCV-2, seroconversion was documented after the operation, so that the postoperative positive rate of anti-HCV-2 increased to 42.9% (39/91). Seroconversion from positive to negative was observed in only one patient. Out of 91 patients who were followed-up at least 3 months after operation, 66 patients (72.5%) developed liver dysfunction. According to the criteria of non-A, non-B post-transfusion hepatitis established by the Japanese Society of Digestive Disease, 31 of 66 patients (34.1%) were diagnosed as "definite", 21 patients (23.1%) as "suspicious". The anti-HCV-2 positive rate was 90.3% in the "definite" group, which was significantly higher than the other groups. Liver dysfunction in the patients with anti-HCV-2 had a tendency for a chronic or prolonged course. Out of 20 patients in whom liver dysfunction continued for more than 1 year, 18 patients were positive for anti-HCV-2. It is concluded from this study that the prevalence of hepatitis C is very high in kidney transplant recipients with HCV as the main and most important etiologic factor of liver dysfunction, especially in chronic liver impairment.

Predeposit autologous blood transfusion in elderly patients undergoing transurethral resection of the prostate.

In order to reduce the rate of homologous blood transfusion, predeposit autologous blood transfusion has been introduced in 134 out of 266 patients undergoing transurethral resection of the prostate since February 1988. Nine (6.7%) out of 134 patients who deposited their own blood were also transfused with homologous blood. In contrast, the rate of homologous blood transfusion was 22.7% in 132 patients who did not deposit autologous blood. Overall homologous blood transfusion rate (14.7%) in the past 4.5 years has been much lower than in the previous 4 years (22.2% in 203 patients). In 6 patients in whom blood donation was performed concurrently with administration of recombinant human erythropoietin, decrease of haemoglobin level was significantly less than in those without rHuEPO. Predeposit autologous blood transfusion can reduce homologous blood transfusion rate. It is safe and easily performed, and more effective when combined with rHuEPO administration even in elderly patients undergoing TURP.

[Complete testicular feminization syndrome associated with thermolabile androgen receptor].

Radioreceptor assay and thermostability test for the androgen receptor in two cases with complete testicular feminization syndrome were performed in regard to the fibroblasts cultured from genital skin on the basis of dispersed whole cell binding assay (Eil et al., 1980). No [3H]dihydrotestosterone binding to the androgen receptor was observed in case 1 (receptor negative), while maximum binding capacity and dissociation constant of androgen receptor for [3H]dihydrotestosterone in case 2 were 21000 sites per cell and 1.67 x 10(-10) M (receptor positive). The specific binding of [3H]dihydrotestosterone to the androgen receptor in case 2 decreased remarkably to 6.6% after high temperature (42 degrees C) incubation in comparison with that at 22 degrees C incubation. The specific binding of [3H]dihydrotestosterone to the androgen receptor in normal controls decreased down to 81.6% at high temperature incubation. Thermostability test was useful to demonstrate qualitative abnormality of androgen receptor in receptor positive testicular feminization syndrome.

[BEP (bleomycin, etoposide, cisplatin) therapy for testicular tumors].

We describe our experience with BEP (bleomycin, etoposide, cisplatin) therapy as chemotherapy for testicular tumors in 11 patients. Eight were non-seminomatous testicular cancer patients and 3 were seminoma patients. Three of 8 non-seminomatous testicular cancer patients had no evident metastasis and BEP therapy was performed for prophylaxis of recurrence. Other 5 non-seminomatous testicular cancer patients and 3 seminoma patients had metastatic lesions and BEP therapy was performed to cure these metastatic lesions. Ten of our 11 patients are living and disease-free. One non-seminomatous testicular cancer patient who had brain, lung, eye and bladder metastases and had an extremely elevated human chorionic gonadotropin (hCG) level responded only partially and died later due to disease progression. Side effects in most patients were nausea, vomiting, alopecia and leucopenia and all these side effects were reversible. Neuromuscular toxicity such as paresthesia or abdominal cramp that is sometimes encountered in PVB (cisplatin, vinblastine, bleomycin) therapy was not seen in our patients. Our results support the concept that BEP therapy is better than PVB therapy as an initial chemotherapy for testicular tumors.

[Chromosome abnormalities in hypospadias? An analysis of 131 patients]. / Chromosomenstörungen bei Hypospadien? Eine Analyse bei 131 Patienten.

We performed chromosome studies in 131 patients presenting with hypospadias, with the aim of detecting any causal connections between chromosomal abnormalities and the induction of hypospadias. Autosomal abnormalities were revealed in 6 and sex chromosomal abnormalities in 10 patients. Although a significant causal relationship between the occurrence of hypospadias and chromosomal abnormalities was seen in this study in only two cases of mixed gonadal dysgenesis (45,X/46,XY and streak gonad), the high incidence of chromosomal abnormalities observed (12.2%) seems noteworthy compared with the incidence of only 0.61% in the normal male population.

[Prognostic parameters of scar progression, new scar formation and maturation in primary vesico-ureteral-renal reflux]. / Prognostische Parameter zu Narbenprogression, neuer Narbenbildung und Maturation beim primären vesiko-uretero-renalen Reflux.

We analysed the data recorded for 901 children (1357 kidneys) treated for primary vesicoureteral reflux between 1973 and 1988 at our institution. The retrospective study showed a rate of 7.3% for new renal scarring and progression; analysis by reflux grade revealed progression and new scarring in reflux, 10.9% of cases with grade IV and 16.5% of cases with grade V whereas low grades (I and II) were followed by no scarring at all. A close correlation of new scarring with urinary tract infection and bladder function was demonstrated. Spontaneous resolution of reflux was observed in 33.6% of all patients and in only 2.6% of patients with grade V reflux, as against a 85.5% resolution rate in patients with grade I or II reflux. A different sex distribution was revealed in the age group under 1 year, in which 80% of the patients were boys and the resolution rate was higher than in any other age group.

[Mainz pouch with umbilical stoma].

Between March 1986 and May 1991 the Mainz pouch urinary diversion was performed in 23 patients with bladder cancer. In 12 of these 23 patients, stoma was constructed in the umbilicus. As the efferent stomal limb, the ileum was used in 10 cases and the appendix was used in 2 cases. The skin at the bottom of the umbilicus and the abdominal fascia under the umbilicus were excised round. The stomal limb was pulled through the fascial hole and the stomal margin was sutured to the skin. The cosmetic results of the umbilical stoma were satisfactory in these 12 patients. Pouch capacity ranged from 330 ml to 560 ml and good urinary continence without difficulty of self-catheterization was obtained in 11 patients. In 1 patient difficulty in catheterization occurred due to a pocket-formation in the stomal limb and the operative revision was performed. Stomal stenosis occurred in 1 patient. Acute renal failure followed by intestinal bleeding occurred in 1 patient who was cured with intensive care including hemodialysis. The results of our study show the superiority of the umbilical stoma in the Mainz pouch in regard to good cosmetic appearance, no need to use a Marlex collar, little bending of catheterization route and low incidence of complications such as parastomal hernia or nipple valve prolapse.

Testicular function in patients with hypospadias associated with enlarged prostatic utricle.

Of 485 patients with hypospadias who underwent urethrography, enlarged prostatic utricles were found in 173 (35.7%). Utricles with a higher grade of enlargement were seen mainly in patients with a severe degree of hypospadias. Serum testosterone levels before and after 3 days' treatment with human chorionic gonadotrophin were determined by radioimmunoassay in 97 prepubertal boys with hypospadias and enlarged prostatic utricles. Lower levels of serum testosterone were found after hormonal stimulation in 22 patients with high grade utricular enlargement when compared with 5 controls and 75 patients with low grade enlargement; the difference was statistically significant. This finding supports the hypothesis that utricular enlargement may be due to androgenic insufficiency during the critical period of organogenesis.

[Treatment of 300 patients with bladder cancer].

From September 1973 to September 1989, 300 patients with bladder cancer were treated at the Department of Urology, Hyogo College of Medicine. They were 231 males and 69 females with an average age of 65.3 years old. The overall 5-year survival rate (Kaplan-Meier's method) was 64.7%. The 5-year survival rates were not different between male patients and female patients, or between patients with single tumor and patients with multiple tumors. Patients with vesical irritation symptoms had more unfavorable prognosis than patients with painless hematuria. Size and configuration of the tumors also affected the prognosis. Histological diagnosis was transitional cell carcinoma in 291 patients, squamous cell carcinoma in 7 patients, adenocarcinoma in one patient and undifferentiated carcinoma in one patient. In patients with transitional cell carcinoma, the 5-year survival rates according to histological grades were 93.5% for G1, 77.8% for G2 and 31.6% for G3. The 5-year survival rate according to clinical stage was 94.4% for Ta, 79.7% for T1, 66.7% for Tis, 46.1% for T2, 38.5% for T3 and 26.6% for T4. Transurethral resection of bladder tumor (TUR-b.t.) was performed in 208 patients as an initial operation and the 5-year survival rate in these patients was 78.6%. The 5-year survival rates for total cystectomy (52 patients), partial cystectomy (6 patients) and simple tumor resection (4 patients) were 51.9%, 25.0% and 37.5%, respectively. These findings suggest that superficial tumors (Ta, T1) can be controlled with TUR-b.t. but infiltrating tumors (T2, T3, T4) should be treated more vigorously with multidisciplinary approaches.(ABSTRACT TRUNCATED AT 250 WORDS)

Androgen receptor levels in patients with isolated hypospadias.

The binding of [3H]dihydrotestosterone (17 beta-hydroxy-5 alpha-androstan-3-one) to the androgen receptor was studied in 26 prepubertal boys with isolated hypospadias (no associated anomaly) using fibroblasts cultured from surgical specimens of hypospadiac penile skin and/or chordal tissue. Maximal binding capacities of androgen receptor for [3H] dihydrotestosterone in 10 patients with mild degree (2 glandular + 8 penile), 6 with severe degree (4 penoscrotal + 2 scrotal), chordal tissues of hypospadias (N = 10), and controls (10 endocrinologically normal boys) were, respectively, 11750 +/- 2410, 10720 +/- 2680, 16100 +/- 7570, and 11570 +/- 3830 (mean +/- sd) sites per cell. Dissociation constants in these subjects were 1.09 +/- 0.29, 0.93 +/- 0.22, 1.20 +/- 0.31, and 1.22 +/- 0.41 x 10(-10) mol/l respectively. There were no significant differences as to both maximal binding capacity and dissociation constant of androgen receptor among any groups. Abnormal receptor thermolability was not found in 5 patients with severe degree of hypospadias either. It is, therefore, concluded that prepubertal boys with isolated hypospadias have primarily normal androgen receptor activity.

[Clinical statistics on outpatients, inpatients and operations in 1988].

Statistical studies were made on 1,766 outpatients, 657 inpatients and 687 operative procedures at our department in 1988. The most frequent diseases among the outpatients were urogenital infections followed by anomalies, tumors and stones. The major diseases among the inpatients were congenital urethral stenosis, hypospadias, vesicoureteral reflux, benign prostatic hypertrophy and bladder tumor. A total of 687 operations were performed. The five major operations were optic internal urethrotomy, hypospadias repair, transurethral resection of prostate, ureterocystoneostomy and transurethral resection of bladder tumor.

[Clinical statistics on outpatients, inpatients and operations in 1987].

Statistical studies were made on 1,859 outpatients, 587 inpatients and 601 operative procedures at our department in 1987. The most frequent diseases among the outpatients were urogenital infections followed by anomalies, tumors and stones. The major diseases among the inpatients were hypospadias, vesicoureteral reflux, congenital urethral stenosis, benign prostatic hypertrophy and bladder tumor. A total of 601 operations were performed. The five major operations were hypospadias repair, optic internal urethrotomy, transurethral resection of prostate, ureterocystoneostomy and transurethral resection of bladder tumor.