Urodynamic and imaging findings in infants with myelomeningocele may predict need for future augmentation cystoplasty.

INTRODUCTION: Urologic issues are persistent and important causes of morbidity and mortality in patients with myelomeningocele. Classically, patients with elevated bladder pressures despite adherence to clean intermittent catheterization (CIC) and pharmacotherapy undergo augmentation cystoplasty (AC). Currently, there is little understanding of which infants are more likely to require AC later. OBJECTIVE: In this context, the authors studied whether unfavorable urodynamic or imaging findings in patients with myelomeningocele during infancy could predict future AC. The authors hypothesized that infants born with elevated bladder pressures, vesicoureteral reflux (VUR), and/or hydronephrosis would be more likely to undergo AC. STUDY DESIGN: The authors retrospectively identified patients with myelomeningocele at their institution who were followed-up since infancy (<1 year of age), with a minimum of eight continuous years of follow-up. Standard care protocol included cystometrogram, voiding cystourethrogram (VCUG), and renal ultrasound during infancy. The primary outcome was AC for elevated bladder pressures despite attempts at more conservative management with medical therapy and CIC. Specifically, the authors evaluated for differences in augmentation rates based on gender, level of lesion, presence of detrusor leak point pressure (DLPP) or end-fill pressure (EFP) greater than 40 cm H2O, presence of hydronephrosis, VUR, initiation of CIC, and initiation of antimuscarinics in infancy. The authors excluded patients who underwent surgical intervention for urinary incontinence. RESULTS: A total of 97 patients met the inclusion criteria. The median follow-up time was 13.8 years. Augmentation cystoplasty was performed for 17 patients (17.5%) at a median age of 114 months (9.5 years). Detrusor leak point pressure/EFP was greater than 40 cm H2O in 34.0% (33/97) of infant cystometrogram studies, while 30.9% (30/97) had VUR on infant VCUG and 20.6% (20/97) had hydronephrosis on infant renal ultrasound. Patients with DLPP/EFP greater than 40 cm H2O or VUR during infancy were more likely to undergo AC (P = 0.02 and P = 0.03, respectively). Binomial logistic regression revealed that DLPP/EFP greater than 40 cm H2O (odds ratio [OR]: 4.28, 95% confidence interval [CI]: 1.34-13.62) and VUR (OR: 3.73, 95% CI: 1.18-11.77) were independent risk factors for future AC. DISCUSSION: Infants with myelomeningocele and elevated bladder pressures and VUR should be closely monitored by urodynamic testing and imaging studies. Parents can be counseled regarding the potentially higher risk for future AC in these patients. Nonetheless, the majority of high-risk infants will safely avoid AC with conservative management.

Localized Inflammatory Myofibroblastic Tumor Involving the Genitourinary System: Adolescent Case Series and Review.

We describe four adolescent cases of inflammatory myofibroblastic tumor involving the genitourinary system. Two patients with masses of the urinary bladder presented with gross hematuria. The third patient presented with left flank pain and a mass encasing the left ureter causing hydronephrosis. The fourth patient presented with a painless, growing palpable mass of the left hemiscrotum. Currently, no standards exist for the management of inflammatory myofibroblastic tumors. Herein, we discuss the work-up and treatment approaches taken in each case.

Impact of Clinical Guidelines on Voiding Cystourethrogram Use and Vesicoureteral Reflux Incidence.

PURPOSE: To prevent over diagnosis and overtreatment of vesicoureteral reflux the 2007 NICE (National Institute for Health and Care Excellence) and 2011 AAP (American Academy of Pediatrics) guidelines recommended against routine voiding cystourethrograms in children presenting with first febrile urinary tract infections. The impact of these guidelines on clinical practice is unknown. MATERIALS AND METHODS: Using an administrative claims database (Clinformatics™ Data Mart) children who underwent voiding cystourethrogram studies or had a diagnosis of vesicoureteral reflux between 2001 and 2015 were identified. The cohort was divided into children age 0 to 2 and 3 to 10 years. Single and multiple group interrupted time series analyses (difference-in-difference) were performed with the guidelines as intervention points. The incidence of vesicoureteral reflux was compared across each period. RESULTS: Of the 51,649 children who underwent voiding cystourethrograms 19,422 (38%) were diagnosed with vesicoureteral reflux. In children 0 to 2 years old voiding cystourethrogram use did not decrease after the 2007 NICE guidelines were announced (-0.37, 95% CI -1.50 to 0.77, p = 0.52) but did decrease significantly after the 2011 AAP guidelines were announced (-2.00, 95% CI -3.35 to -0.65, p = 0.004). Among children 3 to 10 years old voiding cystourethrogram use decreased during the entire study period. There was a decrease in the incidence of vesicoureteral reflux in both groups that mirrored patterns of voiding cystourethrogram use. CONCLUSIONS: The 2011 AAP guidelines led to a concurrent decrease in voiding cystourethrogram use and incidence of vesicoureteral reflux among children 0 to 2 years old. Further studies are needed to assess the risks and benefits of reducing the diagnosis of vesicoureteral reflux in young children.

Pediatric ureteropelvic junction obstruction: can magnetic resonance urography identify crossing vessels?

BACKGROUND: MR Urography (MRU) is an increasingly used imaging modality for the evaluation of pediatric genitourinary obstruction. OBJECTIVE: To determine whether pediatric MR urography (MRU) reliably detects crossing vessels in the setting of suspected ureteropelvic junction (UPJ) obstruction. The clinical significance of these vessels was also evaluated. MATERIALS AND METHODS: We identified pediatric patients diagnosed with UPJ obstruction by MRU between May 2009 and June 2014. MRU studies were evaluated by two pediatric radiologists for the presence or absence of crossing vessels. Ancillary imaging findings such as laterality, parenchymal thinning/scarring, trapped fluid in the proximal ureter, and presence of renal parenchymal edema were also evaluated. Imaging findings were compared to surgical findings. We used the Mann-Whitney U test to compare continuous data and the Fisher exact test to compare proportions. RESULTS: Twenty-four of 25 (96%) UPJ obstructions identified by MRU were surgically confirmed. MRU identified crossing vessels in 10 of these cases, with 9 cases confirmed intraoperatively (κ = 0.92 [95% CI: 0.75, 1.0]). Crossing vessels were determined to be the primary cause of UPJ obstruction in 7/9 children intraoperatively, while in two children the vessels were deemed incidental and noncontributory to the urinary tract obstruction. There was no significant difference in age or the proportions of ancillary findings when comparing children without and with obstructing vessels. CONCLUSION: MRU allows detection of crossing vessels in pediatric UPJ obstruction. Although these vessels are the primary cause of obstruction in some children, they are incidental and non-contributory in others. Our study failed to convincingly identify any significant predictors (e.g., age or presence of renal parenchymal edema) that indicate when a crossing vessel is the primary cause of obstruction.

Cloaca and hydrocolpos: laparoscopic-, cystoscopic- and colposcopic-assisted vaginostomy tube placement.

Hydrocolpos presenting at birth in a neonate with a cloacal malformation may lead to massive distension, with compression of adjacent structures. At times, the hydrocolpos requires urgent drainage. Additionally, these neonates need a divided colostomy to divert their fecal stream and prevent genitourinary contamination. We present a novel approach by which these two procedures can be performed as a single operation guided by a combination of cystoscopy, colposcopy, and laparoscopy. This provides a minimally invasive technique with excellent outcome and potentially superior visualization of the necessary intra-abdominal structures.

Pediatric urethral catheter consultations: understanding driving factors.

PURPOSE: Pediatric urethral catheterization is often straightforward. However, it can be challenging and may require urological consultation. Possible critical factors are patient anatomy and comorbidities, and insertion technique. To better understand pediatric catheter consultations, we reviewed our experience. MATERIALS AND METHODS: All pediatric catheter consultations between July 2009 and June 2012 were identified. A retrospective review was then performed, focusing on demographics, reasons for consultation and difficulty of catheter placement. The 4 categories of difficulty noted were easy, challenging, extremely difficult and could not be placed. Patients were excluded from analysis if catheter placement was not needed, the consultation was for a catheterizable stoma or they were status post urological surgery. Statistical analyses were performed to evaluate associations between patient factors and difficulty of placement. RESULTS: A total of 93 consultations were identified, of which 57% were inpatient, 28% intraoperative and 15% other source. Of the inpatient consultations 75% were from an intensive care unit, the majority (80%) of which were for catheter placement, with the remainder for removal, nondraining catheter, trauma or other. After exclusions 65 patients remained, of whom 80% were male and 32% had a urological comorbidity. By difficulty level 69.2% of cases were easy, 15.4% were challenging, 9.2% were extremely difficult and 6.2% could not be placed. Location of consult, gender, urological comorbidity and history of prematurity were not significantly associated with difficult catheter placement. CONCLUSIONS: Pediatric catheter consultations are largely straightforward. Comorbidities do not significantly impact catheter placement. Correct catheter technique may be more important than patient comorbidities, giving us a basis to shape catheter insertion training within pediatric hospitals.

Urinary NGAL levels correlate with differential renal function in patients with ureteropelvic junction obstruction undergoing pyeloplasty.

PURPOSE: Recent investigations described the use of NGAL, a sensitive biomarker for kidney injury, in the setting of ureteropelvic junction obstruction. We prospectively evaluated urinary NGAL levels in the affected renal pelvis and bladder of children with ureteropelvic junction obstruction undergoing unilateral dismembered pyeloplasty. Our hypothesis was that higher NGAL in the kidney and bladder would correlate with decreased ipsilateral differential function. MATERIALS AND METHODS: We performed a prospective cohort study in patients treated with unilateral dismembered pyeloplasty from 2010 to 2012. Urine was obtained intraoperatively from the bladder and obstructed renal pelvis. A control population of unaffected children was recruited to provide a voided bladder specimen. Bladder NGAL levels were compared between the study and control populations. We tested our study hypothesis by correlating bladder and renal pelvic NGAL levels with the differential renal function of the affected kidney. RESULTS: A total of 61 patients with a median age at surgery of 1.62 years (range 0.12 to 18.7) were enrolled in the study. Median bladder NGAL was 18.6 ng/mg (range 1.4-1,650.8) and median renal pelvic NGAL was 26.2 ng/mg (range 1.2-18,034.5, p = 0.004). Median bladder NGAL was significantly higher than in controls (p = 0.004). The correlation of bladder and renal pelvic NGAL with differential renal function was r = -0.359 (p = 0.004) and r = -0.383 (p = 0.002), respectively. CONCLUSIONS: Bladder NGAL is increased in children with ureteropelvic junction obstruction. Renal pelvic and bladder normalized urinary NGAL levels correlate inversely with the relative function of the affected kidney in cases of unilateral ureteropelvic junction obstruction.

Unusual combination of bilateral testicular microlithiasis and tubular ectasia of the rete testis with left intra- and extratesticular varicocele in a 17-year-old boy.

We describe the rare combination of testicular microlithiasis, unilateral intra/extratesticular varicocele, and tubular ectasia of the rete testis in a 17-year-old boy who presented with testicular pain following a trauma. He had a prior history of undescended testis and orchiopexy in childhood. His workup included a normal abdominal ultrasound and a sperm analysis demonstrating a low sperm count with sperm dysmotility. A follow-up ultrasound was unchanged, and he has been managed conservatively. This combined set of findings has not been previously reported.

Evaluation of methods for urine inhibitory potential for precipitation of calcium oxalate.

Renal lithiasis is a significant medical and social problem. Worldwide recurrence is anywhere from 3% to 5%. Objective of this paper is to evaluate two methods for distinguishing between stone formers and non-stone formers. Urine samples were titrated with calcium and seed crystals were added to facilitate precipitation. Ionic calcium levels were monitored and compared between the two groups. Stone formers showed impaired tolerance to the calcium added and increased precipitation on seed crystals. Both methods discriminated between stone formers and non-stone formers. Further evaluations are needed to establish the better of the two for wider clinical use.

Progression of renal insufficiency in children and adolescents with neuropathic bladder is not accelerated by lower urinary tract reconstruction.

PURPOSE: Children with chronic renal insufficiency and neuropathic bladder resistant to medical management may require lower urinary tract reconstruction before renal transplantation. A low pressure urinary reservoir optimizes the chance of graft survival and may slow native kidney death. We evaluated whether the renal deterioration rate is affected by augmentation cystoplasty. MATERIALS AND METHODS: We performed a retrospective cohort study in children who presented to our institution with chronic renal insufficiency and neuropathic bladders from 2005 to 2009. Chronic renal insufficiency was defined as a glomerular filtration rate of less than 60 ml per minute. As a surrogate for renal function change, we used the inverse creatinine trend with respect to time to determine the progression rate of renal insufficiency before and after augmentation. RESULTS: A total of 11 patients with a mean glomerular filtration rate of 34 ml per minute per 1.73 m(2), mean bladder capacity 168 ml and mean compliance 3.5 ml/cm H(2)O met study inclusion criteria. Bladder augmentation or replacement was done at a mean age of 9.7 years with a resultant mean capacity of 486 ml and compliance of 14.7 ml/cm H(2)O. Mean followup was 4 years before and 1.9 years after augmentation. There was no statistically significant difference between the preoperative and postoperative slopes of inverse creatinine in 8 of 11 patients (73%). Two of the 3 patients (18%) with different preoperative and postoperative slopes had improving renal function after surgery. There was no statistically significant difference in slopes across all patients. CONCLUSIONS: In our series bladder augmentation did not appear to hasten progression to end stage renal disease in patients with severe chronic renal insufficiency and neuropathic bladder.

Influence of pH, temperature and common ion on magnesium hydrogenurate octahydrate solubility.

Physico-chemical investigation of urolithiasis today is mostly focused on applying physico-chemical description of precipitation processes to the stone formation with the aim to distinguish between stone formers and nonstone formers. This is done by calculating supersaturation for different solid phases which can be formed in urine using data obtained by urine analysis and existing solubility product, dissociation and complexation constants. In order for this approach to succeed it is of utmost importance that system is described as detailed as possible, i.e., that all species that can be formed are taken into account. Magnesium hydrogenurate octahydrate, Mg(C5H3N4O3)28H2O (Mg(HU)2.8H2O), is among species which can precipitate in the urine and for which solubility data doesn't exist. In order to fill this void crystals of Mg(C5H3N4O)2.8H2O phase I and phase II have been prepared and characterized. Solubility product constant of Mg(C5H3N4O3)2.8H2O phase I in water at 37 degrees C and phase II at different temperatures, pH and in different solvents have been determined by measuring total concentration of uric acid and magnesium ions in solutions at different time periods. Results show that in water at 37 degrees C thermodynamically less stable phase I is more soluble (Ksp = (5.64 +/- 0.20).10(-9) mol3 dm(-9)) than phase II (Ksp = (1.66 +/- 0.13).10(-9) mol3 dm(-9)). Solubility of Mg(HU)2.8H2O phase II increases with temperature. At equilibrium the solubility of phase II is the lowest in the presence of excess of magnesium ions, while solubility in the presence of uric acid is comparable with the one obtained in water.

Metabolic and physico-chemical urolithiasis parameters in the first morning urine.

The 24-hour urine is golden standard for metabolic assessment of stone formers. However, due to the difficulties in collecting almost 1/3 of the samples can not be used for the analysis. Therefore, we analyzed first morning urine and calculated different risk indexes in order to asses possibility of using it in determining urolithiasis risk. Subjects were divided into 4 groups: male patients (n = 31, age 18-64), female patients (n = 31, age 25-63), male controls (n = 16, age 25-64) and female controls (n = 19, age 21-65). First morning urine pH, concentrations of calcium, magnesium, phosphate, sodium, potassium, chloride, citrate, urate, oxalate, creatinine and glycosaminoglycans were determined. Based on them, ionic concentrations and activity products of calcium oxalate and phosphate were calculated by EQUIL 2. In addition, different risk indices were calculated. The results showed that both patients and control groups had metabolic disorders, but the frequencies of occurrence were statistically independent. Significant difference in concentration of urinary constituents between corresponding patient and control groups was observed only for glycosaminoglycans in female subjects. Ca/Cit ratio and Baggio index could differentiate between both corresponding patients and control groups. The results indicate that interplay between stone formation inhibitors and promotors is responsible for urinary stone formation and that the first morning urine could be used in assessing urolithiasis risk and its prevention.

Rapid antimicrobial susceptibility determination of uropathogens in clinical urine specimens by use of ATP bioluminescence.

We describe the first direct testing of the antimicrobial susceptibilities of bacterial pathogens in human clinical fluid samples by the use of ATP bioluminescence. We developed an ATP bioluminescence assay that eliminates somatic sources of ATP to selectively quantify the bacterial load in clinical urine specimens with a sensitivity of <1,000 CFU per milliliter. There was a log-log relationship between light emission and the numbers of CFU in clinical urine specimens. A clinical study was performed to evaluate the accuracy of the ATP bioluminescence assay for determination of the antimicrobial susceptibilities of uropathogens in clinical urine specimens tested in a blinded manner. ATP bioluminescent bacterial density quantitation was used to determine the inoculation volume in growth medium with and without antibiotics. After incubation at 37 degrees C for 120 min, the ATP bioluminescence assay was repeated to evaluate the uropathogen response to antibiotics. The ability of the ATP bioluminescence assay to discriminate between antimicrobial susceptibility and resistance was determined by comparison of the results obtained by the ATP bioluminescence assay with the results obtained by standard clinical microbiology methods. Receiver operator characteristic curves were used to determine the optimal threshold for discriminating between susceptibility and resistance. Susceptibility and resistance were correctly predicted in 87% and 95% of cases, respectively, for an overall unweighted accuracy of 91%, when the results were stratified by antibiotic. For samples in which the pathogen was susceptible, the accuracy improved to 95% when the results for samples with less than a 25-fold increase in the amount of bacterial ATP in the medium without antibiotics were excluded. These data indicate that a rapid bioluminescent antimicrobial susceptibility assay may be useful for the management of urinary tract infections.

Kinetics of struvite to newberyite transformation in the precipitation system MgCl2-NH4H2PO4NaOH-H2O.

The influence of the initial reactant concentrations on the composition of the solid phases formed in the precipitation system MgCl(2)-NH(4)H(2)PO(4)-NaOH-H(2)O was investigated. The precipitation diagram constructed shows the approximate concentration regions within which struvite, newberyite, and their mixtures exist at 25 degrees C and an aging time of 60 min. It was found that immediately after mixing the reactant solutions, struvite (MgNH(4)PO(4).6H(2)O) precipitated in nearly the whole concentration area, while newberyite (MgHPO(4).3H(2)O) appeared mostly within the region of the excess of magnesium concentration. It was also found that after aging time of 60 min the precipitation domain of struvite alone is much broader than that of newberyite or the domain of their coexistence, and shows that struvite is more abundant in the systems in which the initial concentration of ammonium phosphate is higher than that of magnesium. The kinetics of struvite to newberyite transformation (conversion) was systematically studied under the conditions of different initial reactant concentrations and different initial pH in the systems in which a mixture of both phases precipitated spontaneously. The struvite to newberyite conversion period was found to be strongly related to the ratio of initial supersaturations, S(N)/S(S), rather than to the any particular physical quantity that can describe and predict the behavior of the precipitation system. Experimental data suggest a solution-mediated process as a most possible transformation mechanism. Along with a continuous monitoring of the changes in the liquid phase, the content of struvite in the solid phase was estimated by means of a Fourier transform infrared (FT-IR) method, developed for this particular precipitation system.

Formation and morphology of struvite and newberyite in aqueous solutions at 25 and 37 degrees C.

The influence of the initial reactant concentrations (c(i)(Mg)tot = 5.0 x 10(6) to 5.0 x 10(-1) mol dm(-3), c(i)(P)tot = c(i)(NH4)tot = 1.0 x 10(-3) to 5.0 x 10(-1) mol dm(-3)) and temperature (25 and 37 degrees C) on the composition and morphology of the precipitates formed in the system MgCl2-NH4H2PO4-NaOH-H2O at initial pHi = 7.40 has been investigated. Precipitation diagrams are presented showing the concentration regions within which different morphologies of solid phase have been formed. The solid phases aged for 24 hours were characterized by means of optical microscopy, FT-IR spectrophotometry, X-ray diffractometry and thermogravimetry. It was found that struvite was a predominant phase formed within the concentration region examined and newberyite was obtained only in the region where pH(24h) < 6.5. The influence of the initial pH on the formation and transformation of these two compounds were studied in the region 5.0 < or = pHi < or = 9.0 and the results are discussed.

Metabolic evaluation of urolithiasis patients from eastern Croatia.

Metabolic parameters were determined in fasting blood serum, fasting first morning urine, and 24-hour urine of male patients with recurrent calcium oxalate stones (N = 26, age 39.1 +/- 6.2 years) as well as in male healthy controls (N = 18, age 35.0 +/- 7.1 years), recruited from the eastern part of Croatia. The 24-hour urinary calcium excretion was significantly higher (p < 0.01) for patients (5.6 +/- 2.5 mmol) than for controls (3.7 +/- 1.9 mmol), but potassium excretion was higher (p < 0.01) for controls (74.5 +/- 33.8 mmol) than for patients (49.2 +/- 15.7 mmol). The mean ionic activity product of calcium and oxalate ions, IAP(CaOx), calculated from the fasting first morning urine parameters, was 25% higher for patients than for controls, but the difference was not statistically significant (p > 0.05). Very strong correlation (r = 0.97) was obtained between IAP(CaOx) values and calculated Ogawa indices that were recommended for estimating the potential risk for calcium oxalate stone formation.

Bone mineral density loss in patients with urolithiasis: a follow-up study.

BACKGROUND: Recurrent calcium urolithiasis is often associated with disorders of calcium metabolism. The purpose of this investigation was to assess bone mineral content (BMC) and bone mineral density (BMD) over a period of 1 year in patients with urolithiasis and to determine the factors that could have influenced the changes in bone density during that period. METHODS: The patient group comprised 34 men aged 41.2 plus minus 7.9 years with recurrent urolithiasis. A wide spectrum of biochemical measurements was performed. Bone mineral density (g/cm(2)), bone mineral content (BMC), and bone area (BA) were measured twice during a period of 1 year at the lumbar spine (L2-L4), femoral neck, Ward triangle, and trochanter, using dual energy absorptiometry. Patient results were compared to those obtained from 30 healthy male controls of a comparable age group. RESULTS: Nine patients were hypercalciuric, while the majority of the remaining metabolic parameters were within the reference values. Bone mineral content and bone areas at all regions were lower in patients comparing to controls, but not significantly. The greatest annual reduction of BMD was noticed at Ward triangle (-5.70% in patients and -2.36% in controls), followed by femoral neck (-4.06% patients, -2.03% controls) and trochanter (-3.06% patients, -1.39% controls). There was no significant decrease of the BMD of the spine. Analyzing the influence of age, body mass index (BMI), metabolic parameters, and dietary calcium intake on the annual reduction of bone density, we found that age, hyperuricosuria, and calcium intake were significantly associated with bone loss in that time period. CONCLUSIONS: Bone mass reduction in patients with urolithiasis over a 1-year period did not differ significantly from that in controls and was principally related to age, hyperuricosuria, and calcium dietary restriction but not to increased calcium excretion.