A Wide Spectrum of Axial Mesodermal Dysplasia Complex With Rhombencephalic Anomaly: A Case Report

Axial mesodermal dysplasia complex (AMDC) arises in variable combinations of craniocaudal anomalies such as musculoskeletal deformities, neuroschisis, or rhombencephalic developmental disorders. To the best of our knowledge, the co-existence of AMDC with associated musculoskeletal anomalies, medullary neuroschisis with mirror movements, and cranial nerve anomalies has not yet been reported. Here, we report the case of a 4-year-old boy whose clinical features were suggestive of Goldenhar syndrome and Poland syndrome with Sprengel deformity. Moreover, he showed mirror movements in his hands suspected of rhombencephalic malformation, and infranuclear-type facial nerve palsy of the left side of his face, the opposite side to the facial anomalies of Goldenhar syndrome. After conducting radiological studies, he was diagnosed with medullary neuroschisis without pontine malformations and Klippel-Feil syndrome with rib anomalies. Based on these findings, we propose that clinical AMDC can be accompanied by a wide variety of musculoskeletal defects and variable degrees of central nervous system malformations. Therefore, in addition to detailed physical and neurological examinations, imaging studies should be considered in AMDC.

What are Doctors' Clinical Opinions Regarding Circumcision? / 대한비뇨기과학회지

PURPOSE: Circumcision is widely practiced in Korea, but the general principles of circumcision are not established. To date, several studies on the public's attitude regarding circumcision have been reported, but there have been few studies on doctor's opinions. This study was designed to evaluate the difference of clinical opinion regarding circumcision between the doctors in South Korea. MATERIALS AND METHODS: A questionnaire was sent to the doctors working in university hospitals and primary clinics. The items of the questionnaire were for and against circumcision, the actual clinical practice of circumcision, the recommendation of circumcision, the reasons for it and the difficulties in practicing circumcision. RESULTS: Of the 1,000 questionnaires we sent out, 725 were returned. Of 233 urologists, 211 (91%) agreed with performing circumcision and of the 420 doctor of other department, 319 (76%) agreed with performing circumcision. A total of 530 doctors (81%) agreed with performing circumcision. Both the urologists and the doctors practicing in other departments selected hygiene as the reason for practicing circumcision, 155 (73%) and 254 (86%), respectively. The responses to the questionnaire revealed corresponding opinions regarding circumcision between the urologists and doctors of other departments. CONCLUSIONS: Most Korean men were found to be circumcised and they were very much satisfied with the results. In other countries, there was a difference in clinical opinions between doctors concerning circumcision; however, Korean doctors revealed a corresponding opinion regarding circumcision.

Study on Penile Length of Korean Young Adults with or without Circumcision / 대한비뇨기과학회지

PURPOSE: There have been a few reports investigating the measurement of penile lengths, but none that compare the results of circumcision with penile length. The aim of this study was to determine the correlation between penile length and circumcision, and the mean penile length of physically and sexually active young men in Korea. MATERIALS AND METHODS: The penile lengths in the flaccid and stretched states were measured, between November 2002 and April 2003, in a random group of 2,335 young men without endocrine disorders or an anomaly of the penis. Patients were classified as circumcised (group 1, n=1,767) or uncircumcised (group 2, n=568). The age, body height, body weight, and flaccid and stretched penile lengths were analyzed. RESULTS: The mean values of the flaccid and stretched penile lengths were 7.92+/-3.00 and 12.03+/-2.66cm, and 8.07+/-1.19 and 11.71+/-1.81cm in groups 1 and 2, respectively, with statistical significance according to the stretched length between the two groups (p<0.05). The flaccid and stretched lengths were correlated with height and weight in group 1 (p<0.05). CONCLUSIONS: In this study, we confirmed the normal range for penile length of young adults in Korea. The results of this study showed that a circumcision did not influence the penile length or increase the penile length postoperatively.

Effect of Delayed Estrogen Replacement on Clitoral Corpus Cavernosal Blood Flow and Histologic Composition in Rabbit / 대한비뇨기과학회지

PURPOSE: A decline in the circulating levels of estrogen impairs clitoral engorgement and leads to histopathological changes in the clitoral corpus cavernosum. The aims of this study were to evaluate the effects of delayed estrogen replacement on the clitoral corpus cavernosal blood flow and histological composition in castrated rabbit. MATERIALS AND METHODS: New Zealand White female rabbits(3.0-3.5kg) were randomly divided into three groups of five; two groups(castration group, estrogen replacement group) were castrated and the control group underwent sham operations. Nine weeks after surgery, the hormone replacement group received subcutaneous injections of estrogen(50microgram/kg/day), whereas the castrated group received vehicles for 4 weeks. The clitoral blood flow(ml/min/100g tissue) was measured by a laser Doppler flow meter before and after pelvic nerve stimulation(PNS). Clitoral tissue was processed for histology with Masson's trichrome stain. The expression of TGF-beta was examined by immunohistochemistry. RESULTS: The clitoral corpus cavernosal blood flow after PNS was significantly decreased in the castrated gruop(5.1+/-1.7) compared with the control group(9.3+/-3.1), and increased in the estrogen replacement group(7.4+/-0.4), but without statistical significance compared with the castrated group. On histomorphometry, the percentage of clitoral cavernosal smooth muscle in the castrated group(49.0+/-4.4) was significantly decreased compared with the control(58.7+/-2.3) and the estrogen replacement groups (62.7+/-4.2). The TGF-beta immunoreactivity was increased in both the castrated and estrogen replacement groups. CONCLUSIONS: Delayed estrogen replacement improved the clitoral corpus cavernosal blood flow and increased the percentage of clitoral cavernosal smooth muscle, even though there was evidence of cavernosal fibrosis in the castrated rabbits. These results suggest that delayed estrogen replacement therapy seems to improve sexual function in menopausal women.

Study on Consciousness of Korean Adults for Circumcision / 대한비뇨기과학회지

PURPOSE: There is controversy surrounding the benefits and risks to health of a circumcision of the male prepuce. The purpose of this study was to provide educational material to the general population and medical doctors to help them understand appropriate opinions relating to a circumcision. MATERIALS AND METHODS: A prospective study, including 1,124 adult men, 772 women, with sons, and 971 doctors, was performed. The subjects were interviewed, in detail, about their cognition of circumcision, their circumcision status, their age at circumcision, how many men received circumcisions, their reasons and thoughts relating to circumcision, the pros and cons and their current knowledge of the health benefits and risks associated with male circumcision. RESULTS: 81% of the general subjects, and 77% of the doctors, taking part in the study, were circumcised. The most common age at circumcision, accounting for 34% of the subjects, was over 18 years old. 77% of the general subjects, and 80% doctors, agreed with circumcision. The greatest motive for a circumcision, accounting for 38% of the subjects, was their parents wish. The satisfaction rate of a circumcision was 93%. Most Koreans (69%) considered that a circumcision is an ordinary operations in males. CONCLUSIONS: In this study, most Korean men were found to be circumcised, and were very much satisfied with the results, and on most occasions the procedure had been performed after adolescence. These results suggest that, in Korea, circumcision has a position as a customary conduct, and in itself is inevitable. Therefore, it is essential to explain the advantages and disadvantages of a circumcision to patients and their parents.

Clinical Significance of Prostatitis in Patients with Benign Prostatic Hyperplasia / 대한비뇨기과학회지

PURPOSE: The clinical significance of prostatitis associated with benign prostatic hyperplasia (BPH) remains to be determined. We determined the differences in the prevalence, pattern, clinical symptoms and outcome in BPH patients both with, and without prostatitis. MATERIALS AND METHODS: This prospective study included 134 consecutive patients with lower urinary tract symptoms related to BPH. The patients were divided into 2 groups relating to the expressed prostatic secretion caused by prostate massage (group 1: more than 10 leukocytes per high power field, group 2: less than 10 leukocytes per high power field). Tamsulosin, a selective alpha1-blocker, and finasteride were administered at doses of 0.2 and 5mg, respectively, once a day for one year. The primary efficacy criteria included, symptomatic improvement (International Prostate Symptom Score: I-PSS), maximum flow rate (Qmax) and residual urine volume. RESULTS: Prostatitis was identified in 67 of 119 patients (56.3%); the other 15 patients failed the prostatic massage. Of the patients with associated prostatitis, 8 (11.9%) showed bacterial growth. The serum PSA level was higher in group 1 than in group 2, but there were no significant differences in the other clinical parameters. There was no significantly difference in the improvement of the total I-PSS after treatment between the two groups. However, in group 1, the rritative symptom was significantly less improved (p<0.05). CONCLUSIONS: Prostatic inflammation is a common finding in patients with symptoms of BPH. In such cases, the response to medical treatment for irritative symptoms was inferior in the BPH only cases. There is a need for more studies to distinguish chronic prostatitis and BPH, and to ascertain any additional treatment requirements.

Comparison of Lower Urinary Tract Symptoms and Urodynamic Study in BPH Patients with or without Prostatitis / 대한비뇨기과학회지

PURPOSE: Benign prostatic hyperplasia (BPH) and prostatitis may present similar clinical manifestations, although the severity of their symptoms is different. The differences between BPH patients with prostatitis and those without prostatitis were evaluated to help select appropriate therapeutic modalities and predict the prognosis. MATERIALS AND METHODS: 50 patients with BPH who were not associated with a neurogenic bladder, urethral stricture, diabetes, urinary tract infection or a previous prostate operation were studied. Of the 50 patients, 27 patients were free of prostatitis, and 23 patients had prostatitis. The mean ages of the two groups were 65.9+/-7.3 and 60+/-8.3 years, respectively. The evaluation was performed using the International Prostate Symptom Score (IPSS), uroflowmetry, the residual urine volume and urodynamic studies. Statistical analysis was performed using the Mann-Whitney test and the chi-square scales. RESULTS: According to the IPSS, the straining score was 4.3+/-1.0/3.2+/-1.8 (p<0.05) and the urgency score was 2.4+/- 1.9/3.8+/-1.1 (p<0.05) in BPH patients without or with prostatitis. In the L-PURR, grade 3 and higher, which indicated moderate to severe obstruction, was found in 10 patients with BPH alone (37.1%) and 6 patients with BPH associated with prostatitis (26.1%) (p<0.05). Detrusor instability was identified in 22% (6/27) of patients with BPH alone and 56.5% (13/23) of those with prostatitis (p<0.05). CONCLUSIONS: BPH patients associated with prostatitis had considerably more irritative symptoms, non-obstructive patterns and detrusor instability than those without prostatitis. These findings suggest that the relief of an obstruction by means of surgery alone should be reconsidered when treating BPH with prostatitis.

Malacoplakia of the Prostate / 대한비뇨기과학회지

Malacoplakia is a rare granulomatous inflammatory disease that most frequently involves the urinary tract, particularly the urinary bladder and may involve the testis, epididymis, lung, bone, colon, prostate, female genital organs, and the retroperitoneum, etc. A malacoplakia of the prostate is a very rare disease that is associated with a chronic urinary tract infection. It is often misinterpreted as granulomatous prostatitis, and may clinically mimic prostate cancer. The major cellular component of malacoplakia is the macrophage and the diagnostic hallmark is a characteristic inclusion, the Michaelis-Gutmann body. We recently experienced a case of malakoplakia of the prostate in a 74-year-old man, that was found incidentally in a TRUS-guided biopsy to confirm a suspected prostate cancer.

Differentiation of Infiltrating Urothelial Tumor of Renal Pelvis from Renal Cell Carcinoma Invading into Renal Pelvis or Calyx / 대한비뇨기과학회지

PURPOSE: It is difficult to differentiate urothelial tumours of the renal pelvis, invading the renal parenchyma, from renal cell carcinomas, invading the renal pelvis or calyx. The purpose of this study was to assess the differences between the two conditions. MATERIALS AND METHODS: We retrospectively reviewed the medical records, and imaging studies, of 17 patients who underwent nephroureterectomy with bladder cuff excision for urothelial tumours of the renal pelvis, with parenchymal invasion, and of 30 patients who underwent radical nephrectomy for renal cell carcinomas, invading into the renal pelvis or calyx. We assessed the differences in clinical symptoms, urine cytology, intravenous urography, and CT findings between the two conditions. Pearson chi-square tests, with continuity corrections, were performed for statistical analyses. RESULTS: Renal cell carcinomas showed gross hematuria in only 10 cases (33%), positive findings of urine cytology in 1 case of 9 cases (11%). CT scans demonstrated contour bulging in 25 cases (83%), preservation of reniform shape in 5 cases (17%), peripheral location of tumour in 25 cases (83%), and abnormal CT nephogram in 1 cases (3%). In contrast, urothelial tumour of the renal pelvis showed gross hematuria in 13 cases (76%), positive findings of urine cytology in 9 cases of 15 cases (60%). CT scans demonstrated contour bulging in 1 cases (6%), preservation of reniform shape in 16 cases (94%), central location of tumour in all cases (100%), and abnormal CT nephogram in 10 cases (59%). There was no significant difference between renal cell carcinomas and urothelial tumours of the renal pelvis in blood chemistry or IVP. There were no cases of renal cell carcinoma concurrently with bladder tumour, while 2 cases (12%) of urothelial tumour of the renal pelvis had bladder tumours at the same time. CONCLUSIONS: The presence of gross hematuria, positive findings in urine cytology, the presence of bladder tumours, and tumour location, renal contour changes and CT nephogram in CT scans may be helpful in distinguishing both disease entities.