Supravesical Fissure Variant of Bladder Exstrophy in a Female Patient: Case Report and Review of Literature.

Variations from classic bladder exstrophy (BE) are extremely uncommon, resulting in distinctive challenges in both diagnosis and management. The supravesical fissure variant of BE is exceptionally rare and has only been reported in male patients to date. Herein, we report the case and surgical management of a supravesical fissure variant of BE presentation in a newborn female patient and provide a literature review of this exstrophy variant.

Persistent Genital Arousal and Major Depressive Disorder in an Adolescent Male: Case Report and Discussion.

Persistent genital arousal disorder [PGAD] is a distressing physiologic arousal condition with no psychologic etiology. We highlight identification and treatment of PGAD and associated mental comorbidities in a 15-year-old male who presented to Pediatric Urology with PGAD with pain, and resultant depression and suicidal ideation. After failure of conservative pharmacologic management, MRI of the lumbosacral spine demonstrated an L5-S1 annular tear and herniation of the nucleus pulposus. The patient underwent transforaminal steroid injection with reduction in symptoms, and eventually lumbar discectomy with near complete resolution of symptoms. Management of this condition should include a focus on the mental health component.

A Case Report of an Obstructing Ureteral Nephrogenic Adenoma in a Child Managed With Open Ileal Ureter.

Nephrogenic adenoma is a rare, benign lesion that can be encountered anywhere along the urinary tract. It is associated with genitourinary trauma, chronic inflammation, genitourinary surgery, renal transplant, urolithiasis, and radiation. In children, these lesions are almost exclusively found in the bladder. However, we report an unusual case of a 15-year-old boy with no prior urologic history who presented with an obstructing right ureteral nephrogenic adenoma that required an ileal ureter interposition and right ureterectomy.

Fit for purpose - developing a software platform to support the modern challenges of data linkage in Western Australia.

The Western Australia Data Linkage System (WADLS) is maintained and operated by the WA Data Linkage Branch (DLB) at the Western Australian Department of Health. DLB has pioneered a number of data linkage innovations, including the facilitation of genealogical research via the Family Connections system and streamlined data delivery via the Custodian Administered Research Extract Server. DLB's latest innovation is a new data linkage system called "DLS3", which improves DLB's capability and capacity to handle the increasing volume and complexity of its routine operations. DLS3 was built entirely in-house and customised to meet the specific challenges that DLB has encountered throughout over twenty years of experience with a wide variety of linkages. This article describes the development and rollout of DLS3, including its design, architecture, benefits and limitations.

Daily Enema Regimen Is Superior to Traditional Therapies for Nonneurogenic Pediatric Overactive Bladder.

Our objective was to evaluate the efficacy of daily enemas for the treatment of overactive bladder (OAB) in children. This study was a prospective, controlled trial of 60 children with nonneurogenic OAB. The control patients (40) were treated with standard therapies, including timed voiding, constipation treatment with osmotic laxatives, anticholinergics, and biofeedback physical therapy, whereas the treatment patients (20) received only daily enemas and osmotic laxatives. On assessment of improvement of OAB symptoms, only 30% of the traditionally treated patients' parents reported resolution of symptoms at 3 months, whereas 85% of enema patients did. At the onset of the study, the average pediatric voiding dysfunction score of all patients was 14, whereas on follow-up, the average scores for traditionally treated patients and enema-treated patients were 12 and 4, respectively. This study demonstrated that daily enema therapy is superior to traditional methods for the treatment of OAB.

Experimental testicular tissue banking to generate spermatogenesis in the future: A multidisciplinary team approach.

Spermatogonial stem cell (SSC) loss due to cancer treatment, developmental disorder or genetic abnormality may cause permanent infertility. Cryopreservation of ejaculated sperm is an effective method of fertility preservation in adult males at risk of infertility. However this is not an option in pre-pubertal boys because spermatogenesis has not yet started, and it is difficult in adolescents who are not sexually mature. Therefore testicular tissue cryopreservation to preserve SSCs for future generation of spermatogenesis, either in vivo or in vitro, could be an option for these groups of patients. Although SSC transplantation has been successful in several species including non-human primates, it is still experimental in humans. There are several remaining concerns which need to be addressed before initiating trials of human SSC autotransplantation. Establishment of a testicular tissue banking system is a fundamental step towards using SSC technology as a fertility preservation method. It is important to understand the consultation, harvesting the testicular tissue, histological evaluation, cryopreservation, and long term storage aspects. We describe here a multidisciplinary approach to establish testicular tissue banking for males at risk of infertility.

The use of a wearable camera improves autobiographical memory in patients with Alzheimer's disease.

Despite the marked impairment of recent episodic memories in Alzheimer's disease, there have been few attempts to rehabilitate these deficits. We used a novel external memory aid to promote recall of episodic memories in patients with mild to moderate Alzheimer's disease. SenseCam, a small wearable camera, recorded significant events in the lives of six Alzheimer's disease patients. Every two days for two weeks each patient's memory for an event was assessed, followed by a structured review of the SenseCam images. Longer-term recall was tested one and three months later. A written diary control condition followed the same procedure. Across 40 events the SenseCam review method resulted in significantly more details of an event being recalled over two weeks than the written diary method in five out of the six patients. At three months post event, four out of five patients (one had dropped out) recalled significantly more details of events in the SenseCam condition while the other patient showed no significant difference. Viewing SenseCam images of personally experienced events may significantly improve autobiographical memory in patients with even moderate Alzheimer's disease.

Altered perineal microbiome is associated with vulvovaginitis and urinary tract infection in preadolescent girls.

BACKGROUND: Vulvovaginitis has a known association with urinary tract infections (UTIs) in girls. We hypothesize that vulvovaginitis is a major contributor to UTIs in prepubertal girls by increasing periurethral colonization with uropathogens. METHODS: Periurethral swabs and urine specimens were obtained from a total of 101 girls (58 with vulvovaginitis and 43 without vulvovaginitis). Specimens were cultured for bacterial growth. The dominant organism in the periurethral swabs and urine cultures was recorded and antibiotic sensitivity profiles were compared. RESULTS: Periurethral swabs from children with vulvovaginitis were associated with a statistically significant increase in uropathogenic bacteria (79% Enterococcus species or Escherichia coli) as the dominant culture compared with swabs from girls without vaginitis (18%) (p < 0.05). In children with vulvovaginitis, 52% of the urine cultures were positive for UTIs, and the dominant organism in the urine cultures matched the species and antibiotic sensitivity profile of the corresponding periurethral swab. Only 11% of the urine cultures from girls without vulvovaginitis were positive for UTIs. CONCLUSIONS: Vulvovaginitis may cause UTIs by altering the perineal biome such that there is increased colonization of uropathogens.

The association of age of toilet training and dysfunctional voiding.

OBJECTIVE: To determine whether age of toilet training is associated with dysfunctional voiding in children. MATERIALS AND METHODS: We compared patients referred to the urologic clinics for voiding dysfunction with age-matched controls without urinary complaints. Characteristics including age and reason for toilet training, method of training, and encopresis or constipation were compared between both groups. RESULTS: Initiation of toilet training prior to 24 months and later than 36 months of age were associated with dysfunctional voiding. However, dysfunctional voiding due to late toilet training was also associated with constipation. CONCLUSION: Dysfunctional voiding may be due to delayed emptying of the bowel and bladder by children. The symptoms of dysfunctional voiding are more common when toilet training early, as immature children may be less likely to empty in a timely manner, or when training late due to (or in association with) constipation.

Autologous cell seeded biodegradable scaffold for augmentation cystoplasty: phase II study in children and adolescents with spina bifida.

PURPOSE: Augmentation cystoplasty using gastrointestinal segments in children/adolescents with medically refractory neurogenic bladder is associated with significant complications. We evaluated an autologous cell seeded biodegradable scaffold (Tengion®) for bladder augmentation as an alternative to traditional enterocystoplasty in this population. MATERIALS AND METHODS: A phase II prospective study was performed in children with neurogenic bladder due to spina bifida requiring enterocystoplasty for detrusor pressure 40 cm H2O or greater despite maximum antimuscarinic medication. Following open bladder biopsy, urothelial and smooth muscle cells were grown ex vivo and seeded onto a biodegradable scaffold to form a regenerative augment as the foundation for bladder tissue regeneration. Bladder neck sling was the only concomitant surgical procedure permitted. Bladders were cycled postoperatively to promote regeneration. Primary and secondary outcomes at 12 months included change in bladder compliance, bladder capacity and safety. Long-term assessment was done with similar outcomes at 36 months. RESULTS: Compliance improved in 4 patients at 12 months and in 5 patients at 36 months, although the difference was not clinically or statistically significant. There was no clinical or statistical improvement in bladder capacity at 12 or 36 months in any patient. Adverse events occurred in all patients, and most were easily treated. Two patients had low cell growth following bladder biopsy, of whom 1 withdrew from the study and 1 underwent a second biopsy. Serious adverse events of bowel obstruction and/or bladder rupture occurred in 4 patients. CONCLUSIONS: Our autologous cell seeded biodegradable scaffold did not improve bladder compliance or capacity, and our serious adverse events surpassed an acceptable safety standard.

The smartphone as a platform for wearable cameras in health research.

BACKGROUND: The Microsoft SenseCam, a small camera that is worn on the chest via a lanyard, increasingly is being deployed in health research. However, the SenseCam and other wearable cameras are not yet in widespread use because of a variety of factors. It is proposed that the ubiquitous smartphones can provide a more accessible alternative to SenseCam and similar devices. PURPOSE: To perform an initial evaluation of the potential of smartphones to become an alternative to a wearable camera such as the SenseCam. METHODS: In 2012, adults were supplied with a smartphone, which they wore on a lanyard, that ran life-logging software. Participants wore the smartphone for up to 1 day and the resulting life-log data were both manually annotated and automatically analyzed for the presence of visual concepts. The results were compared to prior work using the SenseCam. RESULTS: In total, 166,000 smartphone photos were gathered from 47 individuals, along with associated sensor readings. The average time spent wearing the device across all users was 5 hours 39 minutes (SD=4 hours 11 minutes). A subset of 36,698 photos was selected for manual annotation by five researchers. Software analysis of these photos supports the automatic identification of activities to a similar level of accuracy as for SenseCam images in a previous study. CONCLUSIONS: Many aspects of the functionality of a SenseCam largely can be replicated, and in some cases enhanced, by the ubiquitous smartphone platform. This makes smartphones good candidates for a new generation of wearable sensing devices in health research, because of their widespread use across many populations. It is envisioned that smartphones will provide a compelling alternative to the dedicated SenseCam hardware for a number of users and application areas. This will be achieved by integrating new types of sensor data, leveraging the smartphone's real-time connectivity and rich user interface, and providing support for a range of relatively sophisticated applications.

Halofuginone infused keratin hydrogel attenuates adhesions in a rodent cecal abrasion model.

BACKGROUND: Postoperative adhesion formation continues to be a significant surgical complication, and methods for preventing abdominopelvic adhesions remain limited. Halofuginone (HF) is a type-1 collagen synthesis inhibitor and may enhance the effects of a physical barrier in preventing adhesion formation. We evaluated the effectiveness of a HF infused keratin hydrogel on preventing adhesions in a rat cecal abrasion model. MATERIAL AND METHODS: Laparotomy and standardized cecal abrasion was performed on 58 retired-breeder Sprague Dawley female rats to induce intra-abdominal adhesions. Rats were randomized to: no treatment; Interceed absorbable adhesion barrier; keratin hydrogel alone; or keratin hydrogel infused with 22 µg/mL of HF. Necropsies were performed at postop d-14 to assess the extent and tenacity of adhesions and grade histologic inflammation and fibrosis using a standard scoring system. Serum, liver, kidneys, and lungs were harvested to evaluate tissue HF concentrations. Protein and drug elution curves were generated to assess the release of HF from the hydrogel. RESULTS: Treatment with Keratin-HF hydrogel resulted in significantly fewer abdominal adhesions than any other treatment, and significantly less dense adhesions compared with Interceed or keratin hydrogel alone. Subset histologic analysis did not reveal qualitative differences. HF was undetectable in serum and kidneys, and detected at negligible concentrations in liver and lungs. Keratin-HF hydrogel drug release in phosphate-buffered solution (PBS) was sustained over 7 d and correlated with keratin protein degradation. CONCLUSIONS: Keratin-HF hydrogel is a novel therapeutic agent that may provide a better method for preventing the development of postoperative adhesions using a combined physical barrier and pharmacologic approached.

Occult megarectum--a commonly unrecognized cause of enuresis.

OBJECTIVE: To determine whether occult megarectum remains a commonly unrecognized cause of enuresis and whether treating it will cure enuresis in most children. A landmark study proved constipation was a commonly unrecognized cause of enuresis in 1986 in which constipation was defined as abnormal rectal distension. However, modern recommendations have focused on signs of functional constipation, such as hard or rare stools. METHODS: A retrospective review of 30 consecutive patients seen in our clinic with a chief complaint of nocturnal enuresis was performed, with an analysis of the results of their plain abdominal radiographs. The results of the studies were determined using a novel method termed the rectal/pelvic outlet ratio and Leech criteria. These results were compared with the reported constipation history according to the International Children's Continence Society guidelines, which recommends asking parents and children whether the child's bowel movements occur less often than every other day and whether the stool consistency is hard. Patients diagnosed with megarectum were treated with laxatives, with the goal of restoring normal rectal tone. RESULTS: All patients demonstrated rectal distension according to the rectal/pelvic outlet ratio, and 80% were constipated according to the Leech criteria. Only 10% of the patient or families reported clinical symptoms of constipation. All the adolescent patients in our study and 80% of the younger patients were cured of enuresis with laxative therapy. CONCLUSION: Occult megarectum remains a commonly undiagnosed cause of nocturnal enuresis. Abdominal radiographs represent a simple, noninvasive method to diagnose megarectum and might improve the treatment of nocturnal enuresis.

SenseCam: a wearable camera that stimulates and rehabilitates autobiographical memory.

SenseCam is a wearable digital camera that captures an electronic record of the wearer's day. It does this by automatically recording a series of still images through its wide-angle lens, and simultaneously capturing a log of data from a number of built-in electronic sensors. Subsequently reviewing a sequence of images appears to provide a powerful autobiographical memory cue. A preliminary evaluation of SenseCam with a patient diagnosed with severe memory impairment was extremely positive; periodic review of images of events recorded by SenseCam resulted in significant recall of those events. Following this, a great deal of work has been undertaken to explore this phenomenon and there are early indications that SenseCam technology may be beneficial to a variety of patients with physical and mental health problems, and is valuable as a tool for investigating normal memory through behavioural and neuroimaging means. Elsewhere, it is becoming clear that judicious use of SenseCam could significantly impact the study of human behaviour. Meanwhile, research and development of the technology itself continues with the aim of providing robust hardware and software tools to meet the needs of clinicians, patients, carers, and researchers. In this paper we describe the history of SenseCam, and the design and operation of the SenseCam device and the associated viewing software, and we discuss some of the ongoing research questions being addressed with the help of SenseCam.

SenseCam improves memory for recent events and quality of life in a patient with memory retrieval difficulties.

A wearable camera that takes pictures automatically, SenseCam, was used to generate images for rehearsal, promoting consolidation and retrieval of memories for significant events in a patient with memory retrieval deficits. SenseCam images of recent events were systematically reviewed over a 2-week period. Memory for these events was assessed throughout and longer-term recall was tested up to 6 months later. A written diary control condition followed the same procedure. The SenseCam review procedure resulted in significantly more details of an event being recalled, with twice as many details recalled at 6 months follow up compared to the written diary method. Self-report measures suggested autobiographical recollection was triggered by the SenseCam condition but not by reviewing the written diary. Emotional and social wellbeing questionnaires indicated improved confidence and decreased anxiety as a result of memory rehearsal using SenseCam images. We propose that SenseCam images provide a powerful boost to autobiographical recall, with secondary benefits for quality of life.

Can we use digital life-log images to investigate active and sedentary travel behaviour? Results from a pilot study.

BACKGROUND: Active travel such as walking and cycling has potential to increase physical activity levels in sedentary individuals. Motorised car travel is a sedentary behaviour that contributes to carbon emissions. There have been recent calls for technology that will improve our ability to measure these travel behaviours, and in particular evaluate modes and volumes of active versus sedentary travel. The purpose of this pilot study is to investigate the potential efficacy of a new electronic measurement device, a wearable digital camera called SenseCam, in travel research. METHODS: Participants (n = 20) were required to wear the SenseCam device for one full day of travel. The device automatically records approximately 3,600 time-stamped, first-person point-of-view images per day, without any action required by the wearer. Participants also completed a self-report travel diary over the same period for comparison, and were interviewed afterwards to assess user burden and experience. RESULTS: There were a total of 105 confirmed journeys in this pilot. The new SenseCam device recorded more journeys than the travel diary (99 vs. 94). Although the two measures demonstrated an acceptable correlation for journey duration (r = 0.92, p < 0.001) self-reported journey duration was over-reported (mean difference 154 s per journey; 95% CI = 89 to 218 s; 95% limits of agreement = 154 ± 598 s (-444 to 752 s)). The device also provided visual data that was used for directed interviews about sources of error. CONCLUSIONS: Direct observation of travel behaviour from time-stamped images shows considerable potential in the field of travel research. Journey duration derived from direct observation of travel behaviour from time-stamped images appears to suggest over-reporting of self-reported journey duration.

Halofuginone-coated urethral catheters prevent periurethral spongiofibrosis in a rat model of urethral injury.

BACKGROUND AND PURPOSE: Urethral strictures are from periurethral spongiofibrosis that develops as a result of urethral trauma, disease, or iatrogenic injury. The spongy tissue that surrounds the strictured urethra has an altered ratio of collagen, with increased collagen type I relative to type III. We evaluated the ability of a urethral catheter that was coated with halofuginone (HF), a potent type I collagen inhibitor, to prevent spongiofibrosis formation in a rat model. MATERIALS AND METHODS: HF was coated on silicone catheters and release kinetics were measured. Success of impregnation was evaluated with scanning electron microscopy, serial weights, and drug elution data. Urethral strictures were induced in rats using electrocautery. Half the animals had placement of an HF-coated catheter while the others had uncoated silicone controls. Animals were sacrificed at predetermined time points, and urethral tissue was either processed for staining with Masson trichrome and anti-alpha-1 collagen or digested to determine HF concentration. Serum drug levels were also determined in treated animals. Slides were graded by a pathologist who was blinded to treatment to determine collagen deposition. RESULTS: HF was coated successfully on silicone catheters. Local urethral concentration of HF was tenfold higher than serum concentration in treated rats. Animals with HF-coated catheters had no new type I collagen deposition after urethral injury. Control animals had increased periurethral collagen type I deposition, typical of urethral stricture formation. CONCLUSIONS: HF can be coated successfully on silicone catheters. HF successfully inhibits periurethral type I collagen deposition after urethral injury. This may become an important therapy to prevent urethral stricture formation or recurrence after endoscopic therapy.