Novel erectile analyses revealed augmentable penile Lyve-1, the lymphatic marker, expression.

Purpose: The pathophysiology of penis extends to erectile dysfunction (ED) to conditions including sexually transmitted diseases (STDs) and cancer. To date, there has been little research evaluating vascular drainage from the penis. We aimed to evaluate penile blood flow in vivo and analyze its possible relationship with the lymphatic maker. Materials and Methods: We established an in vivo system designed to assess the dynamic blood outflow from the corpus cavernosum (CC) by dye injection. To analyze lymphatic characteristics in the CC, the expression of Lyve-1, the key lymphatic endothelium marker, was examined by the in vitro system and lipopolysaccharide (LPS) injection to mimic the inflammatory conditions. Results: A novel cavernography methods enable high-resolution morphological and functional blood drainage analysis. The expression of Lyve-1 was detected along the sinusoids. Furthermore, its prominent expression was also observed after penile LPS injection and in the erectile condition. Conclusions: The current in vivo system will potentially contribute to the assessment of penile pathology from a novel viewpoint. In addition, current analyses revealed inducible Lyve-1 expression for LPS injection and the erection state, which requires further analyses on penile lymphatic system.

Paeonol alleviates postmenopause-induced neuropsychiatric symptoms through the modulation of GPR30 in ovariectomized mice.

ETHNOPHARMACOLOGICAL RELEVANCE: The Moutan cortex (MC), the root bark of Paeonia suffruticosa Anderws (Paeoniaceae), has been historically employed in traditional herbal medicine for addressing women's ailments by replenishing kidney Yin. AIM OF THE STUDY: We aimed to explore if paeonol, an active constituent of MC, could ameliorate neuropsychiatric symptoms, such as anxiety, depression, and cognitive impairments, associated with post-menopausal syndrome (PMS) in an ovariectomized (OVX) mouse model. MATERIALS AND METHODS: The experimental design comprised 6 groups, including a sham group, OVX group, paeonol administration groups (3, 10 or 30 mg/kg, p.o.), and an estradiol (E2)-treated positive control group. Behavioral tests including the open field, novel object recognition, Y-maze, elevated plus-maze, splash, and forced swimming tests were conducted. In addition, we investigated the effets of paeonol on the phosphorylated levels of phosphatidylinositol 3-kinase (PI3K), Akt, and mammalian target of rapamycin (mTOR), as well as on the expression levels of G protein-coupled receptor (GPR30) and brain-derived neurotrophic factor (BDNF) in the prefrontal cortex and hippocampus. RESULTS: Paeonol treatment (10 and 30 mg/kg, p.o.) effectively reversed the cognitive decline in OVX mice, measured by the novel object recognition and Y-maze tests, similar to that in the positive control group. Additionally, it alleviated anxiety- and depressive-like behaviors, as evaluated by the elevated plus-maze test, splash test, and forced swimming test. Paeonol restored GPR30 expression levels in the prefrontal cortex and hippocampus, mirroring the effects of E2 administration. Furthermore, it reversed the reduced expression levels of the PI3K-Akt-mTOR signaling pathway in the prefrontal cortex and hippocampus and increased BDNF expression in the hippocampus of OVX mice. CONCLUSION: This research suggests that paeonol would be beneficial for alleviating PMS-associated cognitive impairment, anxiety and depression.

Changes in Autonomic Nervous System Activity in Children With Spina Bifida: A Case-Control Study.

PURPOSE: We compared heart rate variability parameters between patients with spina bifida and a control group during urodynamic studies, with the goal of evaluating the autonomic nervous system dysfunction present in spina bifida. METHODS: Continuous heart rate variability parameters were recorded during 3 successive periods (P0, the 2 minutes prior to the start of filling; P1, from the start of filling to the first desire to void; and P2, from P1 to the end of filling or the start of voiding). The control group consisted of children with vesicoureteral reflux who had undergone video-urodynamic studies. Our study included 11 patients with spina bifida and 9 control participants. RESULTS: At baseline, patients with spina bifida exhibited lower values for the root mean square of successive differences in NN intervals, the percentage of successive R-R interval differences exceeding 50 msec relative to the total number of intervals, and high frequency (HF). In contrast, the low frequency (LF)/HF ratio was elevated in these patients (5.04 ± 4.75 vs. 0.67 ± 0.42, P = 0.014). During bladder filling, LF/HF values increased in the control group (P0, 0.67 ± 0.42; P1, 0.89 ± 0.34; P2, 1.21 ± 0.64; P = 0.018), while they declined in patients with spina bifida (P0, 5.04 ± 4.75; P1, 3.96 ± 4.35; P2, 3.26 ± 4.03; P < 0.001). The HF values were significantly elevated in children with spina bifida during bladder filling (P = 0.002). In the time domain, the standard deviations of all NN intervals were elevated only in the control group during bladder filling. Parasympathetic activity domains were reduced in the children with spina bifida at the initial assessment. CONCLUSION: During the bladder filling phase, parasympathetic activity increased along with fixed sympathetic activity in the spina bifida group. In contrast, the control group exhibited a shift towards a sympathetic preponderance at the conclusion of bladder filling. These observations may be associated with the pathophysiology of neurogenic bladder in spina bifida.

D-Pinitol attenuates postmenopausal symptoms in ovariectomized mice.

AIMS: Menopause is a natural process in women that can lead to post-menopausal syndrome with symptoms such as hot flushes, weight gain, anxiety, cognitive decline, and depression. Hormonal replacement therapy is commonly prescribed. However, it has serious adverse effects. Herbal medicinal products and isoflavones are used as alternatives. D-Pinitol found in Pinaceae and Fabaceae families has anti-inflammatory and antioxidant effects. However, it has not received as much attention as isoflavones. In this study, we investigated whether D-pinitol could alleviate post-menopausal symptoms using an ovariectomized (OVX) mouse model. MAIN METHODS: Female ICR mice were divided into six groups: sham (vehicle), OVX (vehicle), OVX + D-pinitol (10, 30, 100 mg/kg, p.o.), and OVX + estradiol (0.5 mg/kg, s.c.). Treatment with vehicle, D-pinitol, and estradiol began at seven weeks post ovariectomy. We employed several behavioral tests, hot-flush test, and Western blot analysis. KEY FINDINGS: We found that D-pinitol treatment (30, 100 mg/kg, p.o.) reversed cognitive dysfunction in OVX mice (novel object recognition and Y-maze test). Additionally, D-pinitol alleviated anxiety-like behaviors (elevated plus-maze) and reversed depressive-like behaviors (splash test, tail suspension test). It also normalized increased basal tail skin temperature in OVX mice. Moreover, D-pinitol administration reversed decreased expression of ERß and synaptophysin and phosphorylation of ERK and PI3K-Akt-GSK-3ß induced by OVX in the hippocampus and prefrontal cortex. SIGNIFICANCE: These findings indicate that D-pinitol might be a promising candidate for treating post-menopausal symptoms by increasing ERß and synaptophysin expression levels and activation of ERK or PI3K-Akt-GSK-3ß signaling pathway, at least in part.

Emerging structural and pathological analyses on the erectile organ, corpus cavernous containing sinusoids.

Background: The corpus cavernosum (CC) containing sinusoids plays fundamental roles for erection. Analysis of pathological changes in the erectile system is studied by recent experimental systems. Various in vitro models utilizing genital mesenchymal-derived cells and explant culture systems are summarized. Methods: 3D reconstruction of section images of murine CC was created. Ectopic chondrogenesis in aged mouse CC was shown by a gene expression study revealing the prominent expression of Sox9. Various experimental strategies utilizing mesenchyme-derived primary cells and tissue explants are introduced. Main Findings: Possible roles of Sox9 in chondrogenesis and its regulation by several signals are suggested. The unique character of genital mesenchyme is shown by various analyses of external genitalia (ExG) derived cells and explant cultures. Such strategies are also applied to the analysis of erectile contraction/relaxation responses to many signals and aging process. Conclusion: Erectile dysfunction (ED) is one of the essential topics for the modern aged society. More comprehensive studies are necessary to reveal the nature of the erectile system by combining multiple cell culture strategies.

Translation and validation of the Korean version of the QUAlity of Life Assessment in Spina bifida for Teenagers (QUALAS-T-K).

PURPOSE: The Quality of Life Assessment in Spina bifida for Teenagers (QUALAS-T) is a tool used to evaluate health-related quality of life (HRQOL) in adolescents with spina bifida (SB). The purpose of this study was to translate the QUALAS-T into Korean and validate its Korean version (QUALAS-T-K). METHODS: Translation and validation processes were carried out in accordance with a specified protocol, including forward and back translation, a content validity study, and a main study. The tool's reliability was evaluated based on its internal consistency and stability. Factor analysis was conducted, and convergent validity was confirmed using the KIDSCREEN-27. RESULTS: Of the 59 participants, 35 had lipomyelomeningoceles. Confirmatory factor analysis confirmed that QUALAS-T-K had the same structure as QUALAS-T. The QUALAS-T-K showed excellent internal consistency (α: 0.872-0.893, ω: 0.875-0.885), test-retest reliability (ICC:0.84-0.92), and weak to strong correlations with the KIDSCREEN-27. CONCLUSIONS: The QUALAS-T-K, developed by reflecting on the characteristics of SB and considering the applicability of Korean cultural characteristics and clinical practice, is a convenient and reliable tool with excellent internal consistency and stability. This could be a useful tool in clinical and research settings for HRQOL evaluation of adolescents with SB.Implications for RehabilitationOptimizing health-related quality of life (HRQOL) is one of the goals of individuals with spina bifida (SB), and HRQOL measures that reflect the condition specificity of SB should be performed.The QUAlity of Life Assessment in Spina bifida for Teenagers (QUALAS-T), developed in the USA, is a self-reported HRQOL questionnaire used in research and clinical practice for adolescents with SB.This study revealed that the QUALAS-T, translated into Korean, is a valid, convenient, and reliable tool.The Korean version of the QUALAS-T is a useful tool that can be used in clinical and research settings to optimize HRQOL in adolescents with SB.

Outcomes of single-port robotic ureteral reconstruction using the da Vinci SP® system.

PURPOSE: The da Vinci SP® robotic system enables three double-jointed wristed instruments and a fully wristed three-dimensional camera to be placed through a single port. This study presents our experience with robot-assisted ureteral reconstruction using the SP system and reports its outcomes. MATERIALS AND METHODS: Between December 2018 and April 2022, a single surgeon performed robotic ureteral reconstruction using the SP system in 39 patients: 18 underwent pyeloplasty and 21 received ureteral reimplantation. Demographic and perioperative patient data were collected and analyzed. Radiographic and symptomatic improvements were assessed 3 months after surgery. RESULTS: In pyeloplasty group, 12 patients (66.7%) were female and two patients (11.1%) had undergone previous surgery for ureteral obstruction. The median operative time was 152 minutes, the median blood loss was 8 mL, and the median length of stay in hospital was 3 days. There was one case of a complication involving postoperative percutaneous nephrostomy (PCN). In ureteral reimplantation group, 19 patients (90.5%) were female and ten patients (47.6%) had undergone gynecological surgery that caused ureteral obstruction. The median operative time was 152 minutes, the median blood loss was 10 mL, and the median length of stay in hospital was 4 days. We observed one case of open conversion and two cases of complications (colonic serosal tearing and postoperative PCN after ileal ureter replacement). The radiographic results and symptoms successfully improved following both surgeries. CONCLUSIONS: Despite adhesion-related complications, the SP system appears to be safe and effective for use in robot-assisted ureteral reconstruction.

Effects of Androgen Treatment on Growth in Patients with 5-α-Reductase Type 2 Deficiency.

BACKGROUND: Patients with 5-α-reductase type 2 deficiency (5αRD2) require androgen treatment for the growth of normal male external genitalia. Since limited research has been conducted on the effects of androgen treatment on height in individuals with 5αRD2, we investigated the effect of androgen treatment on bone age (BA) and the height status in children with 5αRD2. METHODS: Of the 19 participants who were followed up for an average of 10.6 years, 12 received androgen treatment. BA and height standard deviation scores (SDS) were compared between the treatment and non-treatment groups, as well as between the dihydrotestosterone (DHT) and testosterone enanthate (TE) treatment groups. RESULTS: Despite the above-average height of the 19 patients with 5αRD2, the height SDS relative to BA (htSDS-BA) was below average, particularly in the androgen treatment group. DHT treatment did not lead to an increase in BA or htSDS-BA, whereas TE treatment resulted in BA advancement and decreased htSDS-BA, especially in the prepubertal period. CONCLUSIONS: DHT treatment is more favorable for height than TE treatment in patients with 5αRD2, particularly during the prepubertal period. Therefore, age and the type of androgen used should be carefully considered to minimize the risk of height reduction in these patient groups.

Cross-cultural adaptation and validation of the Korean version of the quality of life assessment in spina bifida for children (QUALAS-C-K).

PURPOSE: This study aimed to translate and cross-culturally adapt the QUAlity of Life Assessment in Spina bifida for Children (QUALAS-C) and validate the Korean version of the QUALAS-C (QUALAS-C-K). MATERIALS AND METHODS: Three urologists translated the QUALAS-C into Korean. Facial and content validity were assessed in the pilot study. Back-translation into English was performed. In the main study, the QUALAS-C-K and Korean version of KIDSCREEN-27 were administered simultaneously. Test-retest reliability was confirmed by re-administering the QUALAS-C-K. Internal consistency was verified using Cronbach's alpha. Factor analysis was performed, and convergent and divergent validity were demonstrated using the Korean version of KIDSCREEN-27. RESULTS: A total of 53 children with spina bifida participated in the main study. Cronbach's alpha for the overall instrument determined good internal consistency (0.72-0.85), the intraclass correlation coefficient showed good stability (0.74-0.77), and the factor analysis converged to the same two-factor structure as in the original version. Construct validity revealed weak-to-moderate associations (r ≤ 0.57) between QUALAS-C-K and K-KIDSCREEN-27, indicating that QUALAS-C-K measures different aspects of the HRQOL than K-KIDSCEEN-27. CONCLUSIONS: The QUALAS-C-K is a reliable and valid instrument for assessing the health-related quality of life of children with SB in Korea.IMPLICATIONS FOR REHABILITATIONHealth-related quality of life (HRQOL) is an important patient-reported outcome among children with spina bifida (SB).The QUAlity of Life Assessment of Spina bifida for Children (QUALAS-C) is a self-reported, age-appropriate, and condition-specific HRQOL questionnaire for children with SB, developed in the United States.Our study demonstrated that the Korean version of the QUAlity of Life Assessment of Spina bifida for Children (QUALAS-C-K) is a valid and reliable tool.The QUALAS-C-K is a succinct and valuable questionnaire that can be used to assess HRQOL of children with SB, particularly focusing on bladder and bowel problems in clinical practice and research.

The Genotype-Phenotype Correlation in Human 5α-Reductase Type 2 Deficiency: Classified and Analyzed from a SRD5A2 Structural Perspective.

The phenotype of the 5α-reductase type 2 deficiency (5αRD2) by the SRD5A2 gene mutation varies, and although there have been many attempts, the genotype-phenotype correlation still has not yet been adequately evaluated. Recently, the crystal structure of the 5α-reductase type 2 isozyme (SRD5A2) has been determined. Therefore, the present study retrospectively evaluated the genotype-phenotype correlation from a structural perspective in 19 Korean patients with 5αRD2. Additionally, variants were classified according to structural categories, and phenotypic severity was compared with previously published data. The p.R227Q variant, which belongs to the NADPH-binding residue mutation category, exhibited a more masculine phenotype (higher external masculinization score) than other variants. Furthermore, compound heterozygous mutations with p.R227Q mitigated phenotypic severity. Similarly, other mutations in this category showed mild to moderate phenotypes. Conversely, the variants categorized as structure-destabilizing and small to bulky residue mutations showed moderate to severe phenotypes, and those categorized as catalytic site and helix-breaking mutations exhibited severe phenotypes. Therefore, the SRD5A2 structural approach suggested that a genotype-phenotype correlation does exist in 5αRD2. Furthermore, the categorization of SRD5A2 gene variants according to the SRD5A2 structure facilitates the prediction of the severity of 5αRD2 and the management and genetic counseling of patients affected by it.

Analysis of factors affecting permanent clean intermittent catheterization and bladder function after primary neurosurgical repair of lipomyelomeningocele.

AIMS: This study was conducted to identify potential risk factors for permanent clean intermittent catheterization (CIC) and incontinence in patients with lipomyelomeningocele (LMMC) and evaluate how LMMC affects bladder function prognosis, measured by urodynamic (UD) score. METHODS: This retrospective study analyzed the electronic health records of patients who underwent primary neurosurgical repair for LMMC at a single tertiary referral center between January 2012 and December 2016 and were followed at least 3 years after surgery. Data regarding bladder function were obtained from medical records for multiple time points, including before surgery, after surgery but before hospital discharge, 3 months after surgery, and at outpatient visits during follow-up. RESULTS: This study enrolled 120 patients. At a mean follow-up of 62.6 ± 13.9 months after primary neurosurgical LMMC repair, 22 (18.3%) patients continued to require CIC for bladder emptying, only 7 (31.8%) of whom maintained bladder continence. A multivariate logistic regression model identified age at the time of surgery and the type of LMMC as significant presurgical prognostic risk factors for permanent CIC. In addition, postoperative urinary retention and a UD score greater than or equal to 5 measured 3 months after surgery were identified as significant postsurgical risk factors for permanent CIC and urinary incontinence. A linear mixed model adjusted for age at the time of surgery showed that patients with a transitional or chaotic LMMC type were more likely to experience gradual bladder function decline than patients with other LMMC types. CONCLUSIONS: This study identified both presurgical (age at the time of surgery, LMMC type) and postsurgical (postoperative urinary retention, UD score greater than or equal to 5 at 3 months postsurgery) risk factors for permanent CIC and urinary incontinence. In addition, LMMC type was identified as a prognostic risk factor for bladder function decline. These results will enhance the current understanding of bladder function outcomes in patients who undergo surgical treatment for LMMC.

Cross-cultural adaptation and validation of the Korean modified version of the QUAlity of Life Assessment in Spina bifida for Young Adults (QAULAS-YA-Km).

PURPOSE: The QUAlity of Life Assessment in Spina bifida (QUALAS) for adults (QUALAS-A) evaluates the health-related quality of life (HRQOL), reflecting the condition specificity of adults with spina bifida (SB). The study's purpose was to translate and cross-culturally adapt the QUALAS-A into Korean and validate a Korean-modified version of the QUALAS for Young Adults (QUALAS-YA-Km). METHOD: Face and content validity were evaluated in the pilot study. Internal consistency and test-retest reliability were confirmed in the main study. Factor analysis was performed, and convergent and divergent validity was verified using the World Health Organization Quality of Life assessment instrument abbreviated version (WHOQOL-BREF). RESULTS: Forty-seven adults had myelomeningocele. Five items with low communality were deleted through the factor analysis, and the domains were renamed. The QUALAS-YA-Km showed good internal consistency (Cronbach's alpha 0.73-0.83) and excellent test-retest reliability (intraclass correlation coefficient 0.84-0.89). The QUALAS-YA-Km showed good convergent and divergent validity, with weak to strong correlations with the WHOQOL-BREF. CONCLUSIONS: Developed with consideration of Korea's cultural characteristics, the QUALAS-YA-Km is a convenient and reliable instrument, with good internal consistency, stability, and construct validity. This can be a useful tool in clinical and research settings for HRQOL optimization in young adults with SB.Implications for RehabilitationOptimizing health-related quality of life (HRQOL) is one of the goals of people with spina bifida (SB), which requires HRQOL measurements that reflect the condition specificity of SBThe QUAlity of Life Assessment of Spina bifida for Adults (QUALAS-A) is a self-reported HRQOL questionnaire for adults with SB developed in the United States, which is used in research and clinical practiceThe present study revealed that the Korean modified version of the QUAlity of Life Assessment of Spina bifida for Young Adults (QUALAS-YA-Km), developed in consideration of the cultural characteristics of Korea, is a valid, convenient, and reliable toolThe QUALAS-YA-Km, is a useful tool that can be used in clinical and research settings for HRQOL optimization in adults with SB.

Partial Lipectomy of the Epididymal Fat Alters Expression of the Steroidogenic Enzymes in the Mouse Testis at Different Postnatal Ages.

The epididymal fat is a type of gonadal adipose tissue, which is localized closely to the testis. Even though it has been suggested that the epididymal fat is necessary for maintenance of spermatogenesis in the testis, the influence of epididymal fat on expression of testicular steroidogenic enzymes has not been examined. In the present research, expressional changes of steroidogenic enzymes in the mouse testis after 2 weeks of the surgical partial lipectomy of epididymal fat at different postnatal ages were determined by real-time polymerase chain reaction analysis. The transcript levels of all molecules at 2 months of postnatal age were significantly increased by the lipectomy of epididymal fat. However, the lipectomy at 5 months of postnatal age resulted in decreases of expression levels of all molecules examined in the testis. Except a reduced transcript level of hydroxysteroid 17-beta dehydrogenase 3, there were no significant changes of expression levels of other steroidogenic enzymes by the lipectomy at 8 months of postnatal age. At 12 months of postnatal age, the lipectomy caused a significant increase of transcript level of steroidogenic acute regulatory protein and a significant decrease of transcript level of hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 1, without any expressional change of cytochrome P450 side chain cleavage, hydroxysteroid 17-beta dehydrogenase 3, and hydroxysteroid 17-beta dehydrogenase 3 in the testis. These findings suggest that the substances derived from epididymal fat could differentially influence on expression of steroidogenic enzymes in the testis during postnatal period.

Expressional Evaluation of C/EBP Family, SREBP1, and Steroid Hormone Receptors in the Epididiymal Fat of Postnatally Developing Mouse.

The differentiation and development of preadipocyte into mature adipocyte are regulated by transcription factors, such as CCAAT enhancer binding protein (Cebp) gene family and sterol regulatory element binding transcription factor 1 (Srebp1). Steroid hormones give influences on the development and function of adipocyte. The present research examined expression patterns of CCAAT enhancer binding protein alpha (Cebpa), CCAAT enhancer binding protein beta (Cebpb), CCAAT enhancer binding protein gamma (Cebpg), sterol regulatory element binding transcription factor 1 (Srebp1), androgen receptor (Ar), and estrogen receptors (Esr) among different epididymal fat parts during postnatal period by quantitative real-time polymerase chain reaction. In the distal epididymal fat, expression of Cebpa, Cebpb, Cebpg, Srebp1, Ar, and Esr2 was increased until 12 months of age, while expression of Esr1 was decreased at 5 months of age and was not detectable after 8 months of age. In the proximal epididymal fat, transcript levels of Cebps and Srebp1 were increased at 8 months of age, followed by decreases of Cebpb and Cebpg transcript levels at 12 months of age. An additional increase of Srebp1 expression was observed at 12 months of age. Expression of Ar and Esr2 were increased until 8 months of age, followed by a drop of Ar expression level at 12 months of age. Expression pattern of Esr1 was similar to that in the distal epididymal fat. In the tail epididymal fat, expression of Cebpa, Cebpg, Srebp1, Ar, and Esr2 was increased with age. Esr1 was not detectable at all. The highest level of Cebpb was observed at 8 months of age. These data suggest the possibility of developmental and functional differentiation among the epididymal fat parts.

Long-term usage pattern and satisfaction survey of continent catheterizable channels.

INTRODUCTION: We investigated the long-term usage pattern and satisfaction of continent catheterizable channels (CCCs). METHODS: From 2005 to 2018, CCCs, including Mitrofanoff and antegrade continent enema (ACE) channels, were made in 67 patients (Mitrofanoff in 21 patients, ACE channels in 43 patients, and both in three patients) in our institution. An online survey was conducted for these patients in order to assess usage pattern, continent status, difficulty in usage, and patient satisfaction. RESULTS: Sixteen (66.7%) out of 24 patients with the Mitrofanoff channel and 39 (84.7%) out of 46 patients with the ACE channel completed the online survey. In the Mitrofanoff channel group, 10 (62.5%) patients had spina bifida, two (12.5%) had Hinman syndrome, one (6.3%) had posterior urethral valves, and three (18.8%) had urethral trauma or atresia. Additionally, the mean age of the patients at the time of surgery was 10.0 years, and the median follow-up duration was 10.9 years. All patients were using the Mitrofanoff channel to perform clean intermittent catheterization (CIC). Eleven patients (68.8%) had difficulty with catheterization, mostly at the stomal site. Most patients conducted CIC more than four times a day (13, 81.3%). Regarding urination status, seven patients (43.8%) responded that they were satisfied and nine (56.2%) responded they were neutral. In the ACE channel group, 35 patients (89.7%) had spina bifida, seven (17.9%) had cloacal anomalies, and 26 (66.7%) had anorectal malformations. The mean age of the patients at the time of surgery was 8.4 years, and the median follow-up period was 7.4 years. Two (5.1%) patients were no longer using their ACE channels, but 15 (38.5%) patients were still using their channels almost daily. Twenty-eight (71.8%) patients complained that performing enema was time-consuming, and seven (17.9%) patients reported pain when performing ACE and fecal incontinence. Most patients were satisfied with their defecation status (23, 59%), 15 (38.5%) were neutral, and one (2.6%) was dissatisfied. CONCLUSIONS: While most patients who had either Mitrofanoff or ACE channels were still using their channels effectively, approximately half of the patients with CCCs demonstrated neutral satisfaction with their current status; this shows a poor result compared to previous reports. Considering the results of our patient-based study, thorough explanations should be provided to patients who are candidates for Mitrofanoff and ACE procedures; additionally, the discomfort related to the procedures should be comprehensively assessed during follow-up consults.

An Analysis of Major Causes of Surgical Failure Using Bähren System in Intraoperative Venography During Varicocelectomy.

PURPOSE: In young patients with varicocele, preservation of the internal spermatic artery may be advantageous for catch-up growth, but it may also increase the likelihood of treatment failure. Intraoperative venography reduces the likelihood that unsealed veins will remain after varicocelectomy. We analyzed the characteristics of remnant veins visualized through intraoperative venography to investigate the cause of surgical failure in artery-sparing varicocelectomy (ASV). MATERIALS AND METHODS: We retrospectively analyzed clinical characteristics and outcomes of patients aged 18 years or younger who underwent varicocelectomy with intraoperative venography from January 2005 to December 2017. During varicocelectomy, intraoperative venography was performed to distinguish veins from other structures. Any unsealed veins that were discovered were ligated and classified using the Bahren system. RESULTS: One hundred and sixty-two patients underwent intraoperative venography: 153 cases (94.4%) were for primary varicocelectomy, and 9 cases (5.6%) were for repeat varicocelectomy. Open varicocelectomy was performed in 105 cases (64.8%), and laparoscopic varicocelectomy was performed in 57 cases (35.2%). Venography revealed remnant veins after the first ligation in 51 cases (31.2%), 46 (90.2%) and 5 (9.8%) of which were Bähren types 3 and 4, respectively. Five patients (3.1%) experienced varicocele recurrence, classified as persistence in 1 patient (0.6%) and relapse in 4 patients (2.5%). CONCLUSION: Remnant collateral veins of the internal spermatic vein (ISV) (Bahren type 3) are the most common cause of failure in ASV. In a few patients, an external spermatic vein merges with the ISV at a higher level (Bahren type 4) and is unidentifiable without venography.

Comparison of intraoperative and short-term postoperative outcomes between robot-assisted laparoscopic multi-port pyeloplasty using the da Vinci Si system and single-port pyeloplasty using the da Vinci SP system in children.

PURPOSE: We compared the intraoperative and postoperative outcomes of single-port robot-assisted laparoscopic pyeloplasty (S-RALP) using the da Vinci SP® system and conventional multi-port robot-assisted laparoscopic pyeloplasty (M-RALP) in pediatric patients. MATERIALS AND METHODS: Multi-port and single-port pyeloplasty have been performed in pediatric patients in our institution since October 2015 and February 2019, respectively. We conducted an entire cohort comparison. Considering the learning curve of M-RALP, we defined the last 15 cases of M-RALP as a subgroup of M-RALP and compared this subgroup with the entire cohort of S-RALP patients. RESULTS: Thirty-one patients who underwent multi-port pyeloplasty and 15 patients who underwent single-port pyeloplasty were enrolled in this study. Age, height, body weight, laterality, surgical indication, and ipsilateral differential renal function were statistically similar in the M-RALP and S-RALP groups. The median operative time (3.0 h vs. 2.4 h; p=0.01) and the median console time (2.2 h vs. 1.5 h; p<0.001) were longer in the M-RALP group than in the S-RALP group. There was no significant difference in operative time or console time between the M-RALP subgroup and the S-RALP group. There were no significant differences in the length of hospitalization, pain score, morphine-equivalent use of analgesics, or postoperative differential renal function in all comparisons. CONCLUSIONS: This study confirmed that pyeloplasty using the da Vinci® SP system can be started by robotic surgeons who can overcome the learning curve. Robot-assisted laparoscopic single-port pyeloplasty is feasible in noninfant pediatric patients.

Comparison between prepubertal and postpubertal patients with obstructed hemivagina and ipsilateral renal anomaly syndrome.

BACKGROUND: Obstructive hemivagina with ipsilateral renal anomaly (OHVIRA) syndrome is a rare, complex congenital anomaly with an unknown prevalence. However, case reports and small studies on OHVIRA syndrome have increased rapidly in the last 20 years, which may be related to increased use of imaging, surgical techniques, and prenatal sonography. OBJECTIVE: This study aimed to analyze and compare patients with OHVIRA syndrome diagnosed in the prepubertal and postpubertal periods to understand the disease characteristics and improve clinical management. STUDY DESIGN: A retrospective cohort study was conducted including 65 patients with OHVIRA syndrome who were diagnosed between January 2004 and September 2018 at a tertiary university hospital. RESULTS: Among the 65 patients, 44 patients were diagnosed with OHVIRA syndrome during the prepubertal period and 21 patients were diagnosed postpubertally. Compared with postpubertally diagnosed patients with OHVIRA syndrome, those diagnosed prepubertally were mostly asymptomatic at initial presentation (82% versus [vs.] 0%, P < 0.001), had a higher incidence of ectopic ureter (68% vs. 24%, P = 0.001), and presented with a higher incidence of multicystic dysplastic kidney (61% vs. 19%, P = 0.01). Approximately half of the patients with prepubertal OHVIRA syndrome (53%) showed spontaneous resolution of hemivaginal fluid within 5 years. Among the patients with postpubertally diagnosed OHVIRA syndrome, those in the pain-dominant group had a larger amount of hemivaginal fluid than those in the painless discharge-dominant group (54% vs. 10%, P = 0.036). Superimposed infection of hemivaginal fluid was markedly present in the discharge-dominant group (9% vs. 75%, P = 0.006). CONCLUSIONS: Clinical characteristics of patients with OHVIRA syndrome are altered based on the time of initial diagnosis. Follow-up and timely intervention should be proceeded accordingly.

Ovotesticular Disorder of Sex Development in Korean Children: A Single-Center Analysis over a 30-Year Period.

STUDY OBJECTIVE: To present clinical features that characterize ovotesticular disorder of sex development (OT-DSD) in the Korean population. Among the patient cohort who were initially suspected to have OT-DSD, the actual OT-DSD patients and those of other disorder of sex development were compared. DESIGN: Retrospective medical chart review of patients who were initially suspected to have OT-DSD from 1984 to 2018 on the basis of clinical examination. SETTING: Tertiary care university hospital. PARTICIPANTS: Of 26 patients with initial diagnosis of OT-DSD, 3 were excluded because of incomplete records, and finally, 23 patients were subjected to analysis. Various examinations were performed before the surgical confirmation of gonad histopathology. INTERVENTIONS: Medical records were reviewed for clinical, anatomical, biochemical, and cytogenic characteristics, gender assignment, medical treatment, and histopathologic diagnosis. MAIN OUTCOME MEASURES: Characteristics of OT-DSD in a Korean population. RESULTS: Among 23 patients suspected to have OT-DSD, 13/23 (56.5%) were diagnosed as OT-DSD after histopathologic confirmation. Of the remaining 10 patients, 5/23 (21.7%) were diagnosed with mixed gonadal dysgenesis, 3 with Turner variant, 1 with 46,XX disorder of sex development, and 1 with Mayer-Rokitansky-Küster-Hauser syndrome. Among the 13 OT-DSD cases, 9 patients presented with the 46,XX karyotype, 1 with the 46,XY, and 3 with the 46,XX/XY karyotype. Nine patients were assigned as male and 4 as female at birth. The most common gonad histology was ovotestis 10/26 (38%), followed by ovary and testis. CONCLUSION: OT-DSD is one of the rarest disorders with various clinical presentations. A patient with ambiguous genitalia must be examined with a multidisciplinary approach with clinical suspicion for OT-DSD. Standardized procedure of evaluation and treatment is crucial.

A retrospective analysis of ureteropelvic junction obstructions in patients with horseshoe kidney.

BACKGROUND: Ureteropelvic junction obstruction (UPJO) is often encountered in patients with a horseshoe kidney (HSK) and may require surgical intervention. This study retrospectively investigated obstruction causes in HSK patients with UPJOs to determine the most suitable surgical method. METHODS: Twenty HSK patients with UPJO who underwent pyeloplasty between July 2000 and June 2020 and were followed-up for more than six months in our institution were included in the study. The clinical characteristics, obstruction causes, and surgical outcomes were analyzed. RESULTS: The median age at the time of the operation was 4.1 years [interquartile range (IQR): 1.8-10.6]. Hydronephrosis (HN) was found prenatally in 5 patients (25.0%). Pyeloplasty was performed by open, laparoscopic, and robotic techniques in 6, 10, and 4 patients, respectively. Sixteen patients (80.0%) had high ureteral insertion. Twelve patients (60.0%) had crossing vessels, and eight had a high ureteral insertion and crossing vessels. The median follow-up duration was 4.0 years (IQR: 1.8-8.9); no patient required additional surgery. The median differential renal function was 38.0% (IQR: 16.9-43.0%) preoperatively and 38.0% (IQR: 13.3-48.2%) postoperatively. CONCLUSIONS: UPJOs in HSKs were primarily caused by a high ureteral insertion and crossing vessels. Dismembered pyeloplasty was successfully performed in all surgical modalities such as the open, laparoscopic, and robotic approaches. Attention must be given to patients with HSKs, even in those without HN, to avoid UPJO development.