A case of a rare variant of Klinefelter syndrome, 47,XY,i(X)(q10).

Klinefelter syndrome is a condition in which a male patient has one Y chromosome and one or more extra X chromosomes. It is the most common sex chromosome disorder. Klinefelter syndrome is distinguished by many clinical features, such as infertility, high gonadotropin and low testosterone levels, increased height, and sparse body and facial hair. We report the case of a 32-year-old man who visited our hospital complaining of male infertility. Semen analysis showed azoospermia, and chromosomal analysis revealed a 47,XY,i(X)(q10) karyotype, which is a rare variant of Klinefelter syndrome. No spermatozoon was found on microdissection testicular sperm extraction, and the testis biopsy histology showed only Sertoli cells and hyalinised seminiferous tubules. 47,XY, i(X)(q10) has an additional isochromosome made of the long arm of the X chromosome, which shares some features of classical Klinefelter syndrome in many aspects, but patients are usually shorter than average height and have normal intelligence. In addition, to the best of our knowledge, no successful sperm extractions from 47,XY, i(X)(q10) patients were reported in the literature. The reports of patients who have undergone microdissection testicular sperm extraction are very rare. Further reports and studies of this chromosomal abnormality are needed.

Successful pregnancy with sperm retrieved from the bladder after the introduction of a low-electrolyte solution for retrograde ejaculation.

OBJECTIVE: To determine the safety and efficacy of the introduction of a low-electrolyte solution into the bladder before masturbation for sperm recovery in patients with retrograde ejaculation. DESIGN: Two case reports. SETTING: Assisted reproduction practice at a university hospital. PATIENT(S): Two men with retrograde ejaculation. INTERVENTION(S): A 0.32-mol/L glucose solution or Ham's modified F-10 medium (GIBCO, Grand Island, NY) with 10% serum was introduced into the urinary bladder before masturbation. The retrieved sperm were used for IUI. MAIN OUTCOME MEASURE(S): The motility of retrieved sperm, achievement of pregnancy, and outcome of pregnancy. RESULT(S): In patient 1, the motility rate of sperm retrieved from the bladder after the introduction of Ham's modified F-10 medium was higher than that after the introduction of a glucose solution. The patient's wife did not become pregnant during 10 IUI cycles after treatment with Ham's modified F-10 medium. However, she became pregnant during the first IUI cycle after treatment with a glucose solution and was delivered of healthy twin infants. The wife of patient 2 also became pregnant during the first IUI cycle after treatment with a glucose solution and was delivered of a healthy female infant. CONCLUSION(S): The introduction of a glucose solution appears to be a safe and simple method for sperm recovery in patients with retrograde ejaculation.

[Mucinous adenocarcinoma of the prostate: a case report].

A 64-year-old man presented to our department with urinary retention. Rectal examination revealed a small and soft prostate. PSA was within the normal limits. Computed tomography showed a low-density area around the prostatic urethra and urethrography revealed an irregular prostatic urethra compressed by the prostate. We performed transurethral resection of prostate (TUR-P). On resectoscopy, jelly-like round substances were seen in the bladder. Prostatic urethra and bladder neck were covered with a jelly-like substance. Pathological diagnosis was mucinous adenocarcinoma of the prostate with bladder neck involvement. One month later after TUR-P, we performed radical cystoprostatectomy. Histological findings showed the cancer, of which 70-80% was composed of extracellular mucin lakes containing floating clumps of tumor cells. Mucin lake was stained with alcian blue and PAS. Immunohistochemical study revealed the tumor cells positive for carcinoembryonic antigen (CEA) and negative for prostatic specific antigen (PSA).

[Brain metastasis of bladder carcinoma after total cystectomy: a case report].

A 74-year-old woman underwent total cystectomy with ureterostomy on March 1993 on a diagnosis of advanced bladder cancer. The pathological diagnosis was transitional carcinoma grade 3, pT3a pN0 pV1, pL2. Two courses of adjuvant chemotherapy with CDDP and MTX were added. Three years and 3 months later, she began to suffer from amnesia and hemiparalysis on her left side. Brain MRI examination revealed a solid tumor, 4 x 4 x 3 cm in size, in the right frontal lobe of the cerebrum. No other metastatic lesions were found. She underwent surgical resection of the tumor and subsequent irradiation to right frontal lobe in the cerebrum. Pathological examination confirmed its origin of bladder cancer. She has been enjoying a disease-free life for 3 years with minimal neurological symptoms.

[Fournier's gangrene in a patient with malignant lymphoma: a case report].

We report a case of Fournier's gangrene. An 83-year-old man underwent biopsy of left inguinal tumor as an outpatient. Pathological diagnosis was malignant lymphoma (diffuse large cell type). Three weeks later, he developed reddened scrotal swelling accompanied with high fever (> 39 degrees C). On admission, antibiotic chemotherapy was initiated. Although his general condition was improved, scrotal necrosis had developed. A computed tomography scan demonstrated thickened subcutaneous tissue over the left lumbar region. Excision of necrotic tissue and sequential irrigation using povidone iodine liquid were effective. The patient then underwent chemotherapy for malignant lymphoma at the internal department. Nine months after the operation, the scrotal skin had completely recovered.

[A case of ureteral polyp in a boy].

We reported a case of ureteral polyp in a 12-year-old boy whose chief complaint was asymptomatic gross hematuria. An excretory urogram revealed severe right hydronephrosis with a filling defect of the upper ureter. Our diagnosis was right hydronephrosis due to the ureteral polyp. A partial ureterectomy with an end-to-end anastomosis was performed. A polyp was found in the excised ureter and the histological diagnosis was a fibroepithelial polyp. Six months after the operation, the right hydronephrosis showed remission.

[A case of retroperitoneal teratoma difficult to distinguish from adrenal myelolipoma].

A 59-year-old man presented to our hospital suspected of having cholelithiasis. Computed tomography (CT) scan revealed a left retroperitoneal solid tumor cephalad to the kidney, 7 x 8 x 9 cm in size with mostly a fatty density area and focal calcification. Magnetic resonance imaging (MRI) on T1 and T2 weighted images showed a high intensity mass. Angiography revealed the hypovascular tumor. Although we had suspected it to be an adrenal myelolipoma, tumorectomy was performed because of its size. Pathological diagnosis was mature teratoma. Although retroperitoneal teratoma contains fas, cyst, soft tissue and calcification, the proportion of fat in the tumor is usually less than that of adrenal myelolipoma. In our case, the tumor contained more fat than the 'typical' retroperitoneal teratoma, which led to the misdiagnosis.

[Evaluation of varicocele by ultrasonography through a water bag in the supine position].

Recently, ultrasonography has been widely used to evaluate varicocele. However, it is difficult to obtain reproducible scrotal images with a hand probe in the standing position. We, therefore, applied the water bag technique during ultrasonographic examination of the scrotal contents in the supine position. The ultrasonographic images thus obtained were compared with physical findings and scrotal scintigraphic findings. Forty-five varicocele patients diagnosed by palpation and ultrasonography were enrolled in this study. An ultrasonographic examination through a water bag in the supine position has the following advantages 1) it is easy to perform and stable images are obtained, 2) varicoceles that are not palpable can be detected and 3) examinations can be repeated both pre- and postoperatively. Of the 45 patients, 39 and 41 had positive findings on the ultrasonographic images and physical examinations, respectively. Four patients had negative findings on physical examination but positive findings on the ultrasonographic images. Thus, ultrasonographic images through a water bag in the supine position proved to be a useful method of confirming the hemodynamics in varicoceles.

[Clinical outcome of bladder preserving therapy for superficial high-grade bladder cancer].

Clinical outcome of bladder preserving therapy for superficial high-grade bladder cancers (pTa-pT1b, G3) was retrospectively studied. Twenty-six patients initially received bladder preserving therapy ('preserved' group), while 19 underwent radical cystectomy as the initial treatment ('cystectomized' group). Tumor recurrence was accompanied by a progression in the tumor grade in 9 patients with low-grade tumors ('grade-up' group). The 5-year survival rate was 87% and 100% in the preserved and cystectomized groups, respectively, with no significant difference. However, the grade-up group had a significantly worse rate of 57%. Failure in bladder preserving therapy was unrelated to the size or number of tumors. Our findings suggest that bladder preserving therapy is a reasonable treatment option for superficial high-grade tumors although a careful followup is mandatory. On the other hand, radical cystectomy should be considered for recurrent, grade-up G3 tumors.

[A case of multiple organ failure with massive intestinal bleeding caused by methicillin-resistant Staphylococcus aureus in a postcystectomy patient--efficacy of mask continuous positive airway pressure training and intraarterial embolization].

A 51-year-old man underwent radical cystectomy with tubeless cutaneous ureterostomy. Methicillin-resistant Staphylococcus aureus (MRSA) enteritis developed postoperatively. MRSA caused critical infections such as pneumonia and sepsis, which subsequently progressed to adult respiratory distress syndrome, massive melena and multiple organ failure. The patient was rescued by intensive management including mask continuous positive airway pressure, systemic vancomycin administration and intraarterial embolization to stop jejunal bleeding.

[The efficacy of re-TUR after 3 weeks of initial TUR treatment for locally advanced bladder cancer].

BACKGROUND: Radical cystectomy was usually performed for the patient with advanced bladder cancer. The choice of surgical procedure whether bladder preserved therapy or radical cystectomy has sometime plagued us in bladder cancer treatment. Because we don't have clear guideline for the treatment of locally advanced bladder cancer (T2-T3a N0M0). We devised a radical treatment for the patients with locally advanced bladder cancer without radical cystectomy. PATIENTS AND METHODS: We performed re-TUR treatment after 3 weeks of initial TUR for 13 patients with grade 2 and locally advanced bladder cancer diagnosed with pelvic CT scan, transurethral ultrasonography or bimanual examination under the anesthesia. RESULTS: We could successfully preserve all cases of bladder with the use of this devised TUR (periods 9-45 months median 22 months). This method has significantly reduced the cystectomy rate in such locally advanced cases compared with the cystectomy rate prior to 1993 (p < 0.001). CONCLUSION: This method has significance on treatment for locally advanced papillary bladder cancer as bladder preserving therapy with careful observation.

In vitro O-methylation of 4-hydroxyestrone monosulfates.

In vitro O-methylation of 4-hydroxyestrone monosulfates has been examined by means of high-performance liquid chromatography with electrochemical detection. When 4-hydroxyestrone or its 3-sulfate was incubated with rat liver homogenate in the presence of S-adenosyl-L-methionine, the 4-methyl ether was formed in twelve times larger amount than the 3-methyl ether. 4-Hydroxyestrone 4-sulfate served for O-methylation to much less extent as a substrate namely, only a small amount of the 4-methyl ether was formed. Enzymic sulfation of 4-hydroxyestrone with rat liver 105000 g supernatant fortified with adenosine 3'-phosphate 5'-phosphosulfate provided solely catechol estrogen 4-sulfates. The participation of catechol O-methyltransferase and aryl sulfatase in the formation of guaiacol estrogens has been discussed.

In vitro metabolic conjugation of catechol estrogens.

In vitro metabolic conjugation of the catechol estrogens, 2-hydroxyestrone and 4-hydroxyestrone, has been investigated by means of HPLC with electrochemical detection. Sulfation of 2-hydroxyestrone and 4-hydroxyestrone with the rat liver 105 000 g supernatant fortified with 3'-phosphoadenosine-5'-phosphosulfate provided the 2- and 4-monosulfates, respectively. Glucuronidation of the two catechols with the rat and human liver 1500 g supernatant in the presence of uridine-5'- phosphoglucuronic acid gave the 2- and 4-glucuronides, respectively. In contrast, incubation with the guinea pig liver 1500 g supernatant yielded both isomeric monoglucuronides . When 2'-hydroxyestrone was incubated with rat liver 1500 g supernatant and S-adenosyl-L-methionine, the 2- and 3-monomethyl ethers were formed in an equal amount, while 4-hydroxyestrone was transformed into the 4-methyl ether in 12 times greater yield than the 3-methyl ether. The participation of sulfation and glucuronidation in the formation of guaiacol estrogens is discussed.

Effects of extracellular ions on the reactivation of human spermatozoa preserved in electrolyte-free solution.

It has previously been reported that human spermatozoa preserved in an electrolyte-free solution can survive for several weeks at 4 degrees C. However, the motility of spermatozoa after preservation cannot be restored when incubated at 37 degrees C, unless reactivated by extracellular alkalisation. Under weak acidic conditions, the reactivation is induced by > or = 10 mmol l-1 Na+ and inhibited by a Na(+)-H+ antiporter inhibitor. The addition of > or = 0.1 mmol l-1 K+ also induces the reactivation. In the present study, the reactivation was induced by > or = 0.1 mmol l-1 Rb+ or > or = 1 mmol l-1 Cs+ at an acidic pH. The maximum motility rate with K+, Rb+ or Cs+ was obtained at 10-20 mmol l-1 and inhibited by 10(-5)-10(-2) mol l-1 ouabain in a dose-dependent manner, while ouabain had no effect on the Na(+)-induced reactivation. The addition of K+ further increased sperm motility reactivated by Na+, which was also inhibited by ouabain. The addition of Ca2+ did not induce the reactivation or increase sperm motility reactivated by Na+ or K+. It was concluded that activation of the ouabain-sensitive Na(+)-K(+)-ATPase and Na(+)-H+ exchange mechanism has an important role in sperm motility.

GnRH analog, leuprorelin acetate, promotes regeneration of rat spermatogenesis after severe chemical damage.

BACKGROUND: Future fertility is a major concern for cancer patients who undergo intensive chemotherapy. There has been controversy about whether hormonal treatments may have protective effects against the severe spermatogenic damage caused by chemotherapy or irradiation. Recently, it has been proposed that gonadotrophin-releasing hormone (GnRH) analogs administered after testicular damage stimulate the recovery of spermatogenesis. In this study, we have investigated the effects of GnRH agonist, leuprorelin, on the damage to spermatogenesis induced by busulfan. METHODS: Fisher rats were treated with busulfan, 25 mg/kg, intraperitoneally. The effects of subcutaneous injections of leuprorelin before or after treatment were evaluated histologically 18 weeks later. RESULTS: The percentage of 'recovered' seminiferous tubules was 27.7 +/- 12.6% in control rats without leuprorelin and 26.9 +/- 10.2% in rats with leuprorelin injected 4 weeks before busulfan. Rats in both groups showed poor recovery of spermatogenesis with an increase of intratesticular fluid. However, rats treated with leuprorelin three times (4 weeks apart) after busulfan showed an improvement of up to 56.5 +/- 12.0% (P < 0.05). A focal but massive necrotic lesion in the testis was observed only in this group of rats. CONCLUSIONS: The results demonstrated that leuprorelin administered after chemical testicular damage enhanced the recovery of spermatogenesis. At the same time, a possible significant side-effect of leuprorelin was noted.