WAGR, Sex Reversal, Bilateral Gonadoblastomas, and Intralobar Nephrogenic Rests: Uncertainties of Pre-Biopsy Chemotherapy in a High Risk Syndrome for Nephroblastoma.

WT1 deletions are associated with nephroblastomas, WT mutations are associated with 46, XY sex reversal. It is unclear why only a few WT1 deletions are associated with sex reversal. .

A Curious Case of Continuous Incontinence.

Obstructed hemivagina, ipsilateral renal anomaly (OHVIRA) is a rare Müllerian duct abnormality with renal manifestations. Classical presentation is an adolescent female with abdominal pain following menarche. Because of its rarity, diagnosis is often delayed.

Trends in Toilet Training and Voiding Habits among Children with Down Syndrome.

Purpose: Children with Down syndrome are at risk for lower urinary tract dysfunction and delayed toilet training. Comparative studies regarding voiding function in the Down syndrome population are lacking. We assessed urinary continence and voiding function in patients with Down syndrome and a control group.

The role of laparoscopic nephrectomy in pediatric xanthogranulomatous pyelonephritis: a case report.

Xanthogranulomatous pyelonephritis (XGP) is a rare chronic renal infection characterized by the destruction of renal parenchyma. Traditional treatment involves open radical nephrectomy, which is challenging due to the inflammatory process associated with XGP. More recently, laparoscopic nephrectomy has been employed successfully in adult XGP.

Projected financial impact of noncoverage of elective circumcision by Louisiana medicaid in boys 0 to 5 years old.

Purpose: Several states, including Louisiana since 2005, no longer cover elective circumcision under Medicaid programs. The recent AAP (American Academy of Pediatrics) policy statement recognizes the medical benefits of circumcision and recommends the removal of financial barriers to this procedure. Cost savings are a factor in the limitation of circumcision coverage, although to our knowledge the actual cost savings to Medicaid programs have not been reported.

Salvage of bilateral asynchronous perinatal testicular torsion.

Purpose: Current management strategies for prenatal torsion include observation alone, delayed contralateral orchiopexy and emergent contralateral orchiopexy. Bilateral torsion is now being reported with more frequency and approximately a third of these patients have asynchronous torsion. We highlight the role of scrotal exploration in neonates diagnosed with bilateral asynchronous testicular torsion.

Bladder regeneration in a canine model using hyaluronic acid-poly(lactic-co-glycolic-acid) nanoparticle modified porcine small intestinal submucosa.

Objective: • To determine if hyaluronic acid (HA) can be incorporated into porcine small intestinal submucosa (SIS) through poly (lactide-co-glycolide-acid) (PLGA) nanoparticles to improve the consistency of the naturally derived biomaterial and promote bladder tissue regeneration.

Methods: • Beagle dogs were subjected to 40% partial cystectomy followed by bladder augmentation with commercial SIS or HA-PLGA-modified SIS. • Urodynamic testing was performed before and after augmentation to assess bladder volume.

Timing of posterior urethral valve diagnosis and its impact on clinical outcome.

Objective: Our goals were to assess long-term outcome for patients with posterior urethral valves (PUV) and determine the impact of the timing of diagnosis.

Patients And Methods: We reviewed the hospital records for patients with PUV treated in 1994-2008. Only those patients diagnosed, treated surgically, and followed clinically at our center were included.

Evaluation of need for salvage continence procedures after failed modern staged repair.

Objectives: To assess our experience with salvage continence procedures in patients with bladder exstrophy.

Methods: We retrospectively reviewed our surgical records of patients with bladder exstrophy who had undergone failed modern staged repair elsewhere and underwent salvage continence operations at our institute from 1996 to 2008.

Results: A total of 16 patients had undergone salvage continence operations.

Enhanced angiogenesis of modified porcine small intestinal submucosa with hyaluronic acid-poly(lactide-co-glycolide) nanoparticles: from fabrication to preclinical validation.

Hyaluronic acid-poly(de-co-glycolide) nanoparticles (HA-PLGA NPs) were synthesized to stabilize the porous structure of porcine small intestinal submucosa (SIS), to improve surface biocompatibility and to enhance performance in tissue regeneration. HA-PLGA NPs were characterized for size, zeta potential, surface morphology, and HA loading. Human microvascular endothelial cells responded to HA-PLGA NPs and HA-PLGA modified SIS (HA-PLGA-SIS) with elevated cell proliferation.

Urologic tissue engineering in pediatrics: from nanostructures to bladders.

The use of tissue engineering to develop new urinary bladder could have direct impact on children suffering from congenital bladder dysfunction. Current bladder tissue engineering protocols rely on scaffolds to support the ingrowth of new cells and the subsequent development of new tissues. The interaction between cells and scaffolds is mediated on the molecular level and nanotechnology is being investigated as a means to improve the biocompatibility of these scaffolds.

Application of a continent fecal reservoir in patients with cloacal exstrophy.

Purpose: Initial enteric diversion in cloacal exstrophy is achieved by ileostomy or end colostomy with formal reconstruction in the form of hindgut pull-through performed in select patients. Those who are not candidates for a pull-through procedure are often left with a permanent incontinent stoma. Additionally due to congenitally deficient intestinal length, some patients with cloacal exstrophy experience short bowel syndrome.

Successful removal of a penile constriction ring in a 14-year-old male.

Penile strangulation is a rarely described medical emergency, especially in the adolescent population. This case demonstrates the successful removal of a constricting metal ring from the penis of a 14-year-old male with a diamond blade equipped orthopedic oscillating saw while under ketamine anesthesia in the emergency department.

Regional variations in small intestinal submucosa evoke differences in inflammation with subsequent impact on tissue regeneration in the rat bladder augmentation model.

Objective: To examine the histological differences in the inflammatory response and regenerative outcomes of distal vs proximal porcine small intestinal submucosa (SIS) grafts in the rat bladder, as SIS from distal small intestine yields reliable and reproducible bladder regeneration, while SIS from proximal portions of small intestine does not provide similar results.

Materials And Methods: In all, 30 Sprague-Dawley rats underwent hemi-cystectomy followed by anastomosis of a bladder patch of SIS prepared from either distal or proximal small intestine. After bladder harvest, immunohistochemistry was used to quantify mast cells, eosinophils, macrophages, and neutrophils (PMNs).

Leukocyte inflammatory response in a rat urinary bladder regeneration model using porcine small intestinal submucosa scaffold.

Small intestinal submucosa (SIS) is a biodegradable scaffold that supports bladder regeneration after partial cystectomy. We sought to define the inflammatory response present in a rat bladder augmentation model using distal ileal SIS. Fifteen Sprague-Dawley rats underwent hemi-cystectomy followed by anastomosis of a bladder patch of SIS.

Our initial experience with the technique of complete primary repair for bladder exstrophy.

Objective: We reviewed our initial results with complete primary repair of exstrophy in regard to continence status and the need for subsequent continence procedures.

Patients And Methods: We performed a retrospective review of our surgical records from 1996 to 2008 to identify all patients with bladder exstrophy managed at our center.

Results: Sixteen children were closed successfully.

Body mass index as a predictive value for complications associated with reconstructive surgery in patients with myelodysplasia.

Purpose: Patients with myelodysplasia often have urological pathology, with 25% to 40% requiring reconstructive procedures to achieve urinary and/or fecal continence. Complication rates from these major reconstructive procedures range between 10% and 50%. Additionally many of these patients have significant comorbidities, including a nonambulatory status that leads to an increased body mass index.

Recent advances in urologic tissue engineering.

Tissue engineering provides exciting therapeutic potential in urology. Urinary bladder regeneration is the most heavily researched aspect of tissue engineering in urology. Recent clinical research demonstrated the feasibility of bladder regeneration in children with neurogenic bladders.

Endoscopic injection of submucosal bulking agents for the management of incontinent catheterizable channels.

Background: Urinary and fecal continence can be achieved by constructing catheterizable continent channels that provide access to the bladder and bowel. Some patients develop persistent stomal leakage. A minimally invasive method of injection with a bulking agent for treatment of stomal incontinence was evaluated.

Use of second look nephroscopy in children undergoing percutaneous nephrolithotomy.

Purpose: We determined the rate of stone clearance in children following percutaneous nephrolithotomy, endoscopic assessment of residual stone and the judicious use of second look nephroscopy.

Materials And Methods: We retrospectively reviewed the charts of all children undergoing percutaneous nephrolithotomy from 1996 to 2007. Cases were reviewed for pertinent details including preoperative and postoperative imaging, specifics of the procedure and followup.

Temporal differentiation and maturation of regenerated rat urothelium.

Objective: To determine if porcine small intestinal submucosa (SIS)-regenerated urothelium expresses markers of urothelial differentiation, uroplakin and zona occludens-1 (ZO-1), and whether their expression correlates with the histological appearance of the urothelium.

Materials And Methods: In all, 15 rats underwent partial cystectomy and bladder replacement with SIS. Regenerated bladders were harvested at either 2, 7, 14, 28, or 56 days after SIS grafting.

Occurrence of urinary tract infection in children with significant upper urinary tract obstruction.

Objectives: Ureteropelvic junction obstruction and obstructive megaureter are common causes of upper urinary tract obstruction. Recent data have demonstrated that the rate of urinary tract infection (UTI) among children with upper tract obstruction not treated with prophylactic antibiotics is >36%. The aim of this study was to evaluate the occurrence of UTI in our patients with ureteropelvic junction obstruction and megaureter to better assess the role of prophylactic antibiotics.

Abdominal sacral colpopexy: surgical pearls and outcomes.

Vaginal vault prolapse is a challenging form of pelvic organ prolapse that occurs in combination with cystocele, rectocele, or enterocele in nearly 75% of affected patients. Clinical presentation will vary depending on the associated defects. Any successful therapy for vaginal vault prolapse will depend on a thorough evaluation of the vaginal compartments and concomitant lower urinary tract function.