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Item The ability of a limited metabolic assessment to identify pediatric stone formers with metabolic abnormalities(Elsevier, 2018) Chan, Katherine H.; Moser, Elizabeth A.; Whittam, Benjamin M.; Misseri, Rosalia; Cain, Mark P.; Krambeck, Amy; Urology, School of MedicineIntroduction American Urological Association guidelines recommend a urinary metabolic evaluation after the first stone event in all pediatric stone patients. Prior studies identified hypercalciuria and urine hypovolemia as the most common abnormalities in children with urolithiasis. Recent data suggest that hypocitraturia is most prevalent. It was hypothesized that a limited evaluation would detect the majority of clinically significant metabolic abnormalities in pediatric stone formers. Material and methods A retrospective analysis of all children (<18 years of age) with renal/ureteral calculi evaluated at the study institution from 2005 to 2015 was performed. Children with ≥ one 24-h urinary metabolic profile after a clinical visit for renal/ureteral calculi were included. Those with bladder stones and those with undercollection or overcollection or missing urinary creatinine were excluded. Demographics and data from the first urinary metabolic profile and stone analyses were collected. The sensitivity, specificity, and positive and negative predictive value (NPV) of a limited urinary metabolic evaluation consisting of four parameters (24-h calcium, citrate, and oxalate and low urinary volume) were compared to a complete urinary metabolic profile. The number and type of metabolic abnormalities that would have been missed with this limited evaluation weredetermined. Results Of 410 patients, 21 were excluded for age ≥18 years, 13 for bladder stones, 248 for overcollections, 38 for undercollections, and 10 for missing creatinine. This left 80 patients for inclusion: median age 11.4 years, 60% female, and 96.3% white. Of the entire cohort, 69.6% had hypocitraturia, 52.5% had low urine volume, and 22.5% had hypercalciuria. Sensitivity was 87.5%. Specificity could not be calculated because no patients had a normal complete metabolic evaluation. The NPV was zero, and the positive predictive value was 100%, but these are artifacts resulting from the absence of patients with a normal complete metabolic evaluation. Of the 80 patients, 10 had at least one abnormality missed by a limited metabolic evaluation (Table 1). The missed abnormalities were high pH (n = 6), abnormal 24-h phosphorus (low in 1 patient and high in 1 patient), low 24-h magnesium (n = 3), low 24-h potassium (n = 3), and high 24-h sodium (n = 4). Discussion A limited urinary metabolic evaluation would have detected the vast majority of clinically significant metabolic abnormalities in the study sample. Approximately two-thirds of the study patients submitted inadequate 24-h urine specimens. Conclusions A simplified approach to metabolic evaluation in first-time stone formers with a stone analysis available was proposed. This streamlined approach could simplify the metabolic evaluation and reduce health care costs.Item Age-based risk of end-stage kidney disease in patients with myelomeningocele(Elsevier, 2023-04) Adams, Cyrus M.; Misseri, Rosalia; Roth, Joshua D.; Whittam, Benjamin M.; Guckien, Zoe E.; King, Shelly J.; Kaefer, Martin; Rink, Richard C.; Szymanski, Konrad M.; Urology, School of MedicineObjective We aimed to quantify end-stage kidney disease (ESKD) risk after infancy in individuals with myelomeningocele (MMC) followed by urology in the modern medical era and to assess if ESKD risk was higher after surgery related to a hostile bladder. Methods We retrospectively reviewed patients with MMC followed by urology at our institution born ≥ 1972 (when clean intermittent catheterization was introduced) past 1 year of age (when mortality is highest, sometimes before establishing urology care). ESKD was defined as requiring permanent peritoneal/hemodialysis or renal transplantation. Early surgery related to hostile bladder included incontinent vesicostomy, bladder augmentation, detrusor Botulinum A toxin injection, ureteral reimplantation, or nephrectomy for recurrent urinary tract infections. Survival analysis and proportional hazards regression were used. Sensitivity analyses included: risk factor analysis with only vesicostomy, timing of surgery, including the entire population without minimal follow-up (n = 1054) and only patients with ≥ 5 years of follow-up (n = 925). Results Overall, 1029 patients with MMC were followed for a median of 17.0 years (49% female, 76% shunted). Seven patients (0.7%) developed ESKD at a median 24.3 years old (5 hemodialysis, 1 peritoneal dialysis, 1 transplantation). On survival analysis, the ESKD risk was 0.3% at 20 years old and 2.1% at 30 years old (Figure). This was ∼100 times higher than the general population (0.003% by 21 years old, p < 0.001). Patients who underwent early surgery for hostile bladder had higher ESKD risk (HR 8.3, p = 0.001, 6% vs. 1.5% at 30 years). On exploratory analyses, gender, birth year, shunt status and wheelchair use were not associated with ESKD risk (p ≥ 0.16). Thirty-year ESKD risk was 10% after early vesicostomy vs. 1.4% among children without one (p = 0.001). Children undergoing bladder surgery between 1.5 and 5 years old had a higher risk of ESKD. No other statistically/clinically significant differences were noted. Comment Patients with MMC remain at risk of progressive renal damage throughout life. We relied on the final binary ESKD outcome to quantify this risk, rather than imprecise glomerular filtration rate formulas. Analysis was limited by few people developing ESKD, inconsistent documentation of early urodynamic findings and indications for bladder-related surgery. Conclusions While ESKD is relatively uncommon in the MMC population receiving routine urological care, affecting 2.1% of individuals in the first 3 decades, it is significantly higher than the general population. Children with poor bladder function are likely at high risk, underlining the need for routine urological care, particularly in adulthood.Item Characteristics of Female Genital Restoration Surgery for Congenital Adrenal Hyperplasia Using a Large-scale Administrative Database(Elsevier, 2018) Roth, Joshua D.; Casey, Jessica T.; Whittam, Benjamin M.; Bennett, William E., Jr.; Szymanski, Konrad M.; Cain, Mark P.; Rink, Richard C.; Urology, School of MedicineObjective To analyze nationwide information on the timing of surgical procedures, cost of surgery, hospital length of stay following surgery, and surgical complications of female genital restoration surgery (FGRS) in females with congenital adrenal hyperplasia (CAH). Materials and Methods We used the Pediatric Health Information System database to identify patients with CAH who underwent their initial FGRS in 2004-2014. These patients were identified by an International Classification of Diseases, Ninth Revision (ICD-9) diagnosis code for adrenogenital disorders (255.2) in addition to a vaginal ICD-9 procedure code (70.x, excluding vaginoscopy only) or perineal ICD-9 procedure code (71.x), which includes clitoral operations (71.4). Results A total of 544 (11.8%) females underwent FGRS between 2004 and 2014. Median age at initial surgery was 9.9 months (interquartile range 6.8-19.1 months). Ninety-two percent underwent a vaginal procedure, 48% underwent a clitoral procedure, and 85% underwent a perineal procedure (non-clitoral). The mean length of stay was 2.5 days (standard deviation 2.5 days). The mean cost of care was $12,258 (median $9,558). Thirty-day readmission rate was 13.8%. Two percent underwent reoperation before discharge, and 1 (0.2%) was readmitted for a reoperation within 30 days. Four percent had a perioperative surgical complication. Conclusion Overall, 12% of girls with CAH underwent FGRS at one of a national collaborative of freestanding children's hospitals. The majority underwent a vaginoplasty as a part of their initial FGRS for CAH. Clitoroplasty was performed on less than half the patients. Overall, FGRS for CAH is performed at a median age of 10 months and has low 30-day complication and immediate reoperation rates.Item Comparison of Intraoperative and Early Postoperative Outcomes of Caudal Versus Dorsal Penile Nerve Blocks for Outpatient Penile Surgeries(Elsevier, 2017) Chan, Katherine H.; Shah, Aali; Moser, Elizabeth A.; Szymanski, Konrad; Whittam, Benjamin M.; Misseri, Rosalia; Kaefer, Martin; Rink, Richard; Cain, Mark P.; Urology, School of MedicineObjective To compare intraoperative and 1-hour postoperative outcomes in caudal versus dorsal penile nerve block (DPNB) patients undergoing penile surgeries. Material and Methods We performed a retrospective cohort study of males <10 years old undergoing penile procedures (2013-2015) using the Pediatric Regional Anesthesia Network, Pediatric Health Information System databases and our medical records. The primary outcome was a maximum Faces Limbs Activity Crying Consolability pain score > 3. Secondary outcomes were intraoperative/post-anesthesia care unit (PACU) narcotics, pre-incision anesthesia time, adjusted operating room charges and complications. We performed bivariate and multivariable analyses controlling for demographic/procedure characteristics and clustering by surgeon. Results Of 738 patients, (mean age 2.1 years) 74.1% had a caudal. DPNB patients were more likely to have a maximum pain score >3 (19.5% vs. 8.1%, p<0.0001), receive intraoperative (33.0% vs.2.9%, p<0.0001) and PACU narcotics (15.7% vs. 7.5% vs. p=0.0009), had shorter pre-incision anesthesia time (19.5 vs. 27.9 minutes, p<0.0001) and lower adjusted operating room charges ($9,402 vs. $12,760, p<0.0001). In a bivariate logistic regression, DPNB patients had 2.7 times the odds of a maximum pain score > 3 (95% CI 1.7- 4.4, p<0.0001) and 5.2 times the odds of intraoperative/PACU narcotic administration (95% CI 3.3-8.1, p<0.0001). In multivariable analyses, caudal patients had longer pre-incision anesthesia time (27.9 ± 7.4 vs. 19.5 ± 6.6 minutes, p<0.0001) and higher adjusted operating room charges ($12,760 ± 4077 vs. $9,402 ± 3741, p=0.01). Conclusion Caudal blocks may offer a small advantage in the immediate postoperative period although cost-effectiveness is unproven.Item Cost-effectiveness analysis of the management of distal ureteral stones in children(Elsevier, 2019) Chan, Katherine H.; Whittam, Benjamin M.; Krambeck, Amy; Downs, Stephen M.; Misseri, Rosalia; Cain, Mark P.; Bennett, William E., Jr.; Urology, School of MedicineObjective To determine the most cost-effective approach to the management of distal ureteral stones in children given the potential for recurrent renal colic during a trial of passage versus potential stent discomfort and complications of ureteroscopy. Methods We developed a decision tree to project costs and clinical outcomes associated with observation, medical explusive therapy (MET), and ureteroscopy for the management of an index patient with a 4mm distal ureteral stone. We determined which strategy would be least costly and offer the most pain-free days within 30 days of diagnosis. We performed a one-way sensitivity analysis on the probability of successful stone passage with MET. We obtained probabilities from the literature and costs from the 2016 Pediatric Health Information System Database. Results Ureteroscopywas the costliest strategy but maximized the number of pain-free days within 30 days of diagnosis ($5,282/29 pain-free days). MET was less costly than ureteroscopybut also less effective ($615/21.8 pain-free days). Observation cost more than MET and was also less effective ($2,139/15.5 pain-free days). The one-way sensitivity analysis on the probability of successful stone passage with MET demonstrated that ureteroscopyalways has the highest net monetary benefits value and is therefore the recommended strategy given a fixed WTP. Discussion Using a rigorous decision-science approach, we found that ureteroscopy is the recommended strategy in children with small distal ureteral stones. Although it cost more than MET, it resulted in more pain-free days in the first 30 days following diagnosis given the faster resolution of the stone episode.Item Incidence of pathologic postobstructive diuresis after resolution of ureteropelvic junction obstruction with a normal contralateral kidney(Elsevier, 2018) Roth, Joshua D.; Lesier, Jeffrey D.; Casey, Jessica T.; Szymanski, Konrad M.; Whittam, Benjamin M.; Misseri, Rosalia; Rink, Richard C.; Cain, Mark P.; Pediatrics, School of MedicineIntroduction Postobstructive diuresis (POD) after unilateral pyeloplasty or percutaneous nephrostomy (PCN) tube insertion for ureteropelvic junction obstruction (UPJO) in patients with a normal contralateral kidney is not well described. Objective The objective of this study was to determine the incidence and characteristics of POD after relief of unilateral UPJO in patients with a normal contralateral kidney. Study design Children who underwent a unilateral pyeloplasty or PCN for UPJO from 2010 to 2017 with a normal contralateral kidney were retrospectively reviewed. Postobstructive diuresis was defined as urine output (UO) of >300% of expected UO. Patients with a solitary kidney or those who underwent bilateral pyeloplasty or bilateral PCN tube placement were excluded. Results Out of 396 children meeting inclusion criteria, seven (1.8%) developed POD (4 after pyeloplasty and 3 after PCN tube placement). Median age at intervention was 1.7 years (range 11 days–18 years); median weight was 11.4 kg (range 3.7–54.2 kg). Postobstructive diuresis was more likely to occur in patients with grade 4 hydronephrosis (3.0%) and larger kidneys and if a PCN tube was placed before pyeloplasty. There was no significant difference in age, gender, kidney laterality, or function between those who developed POD and those who did not. Postobstructive diuresis was managed with additional intravenous fluids and electrolyte monitoring. Median initial postprocedure UO was 5.9 mg/kg/hr (range 3.2–10.0 mg/kg/hr). In five children who underwent PCN in whom UO could be differentiated between kidneys, median initial postprocedure UO was 6.1 mg/kg/hr (range 2.5–9.1 mg/kg/hr) from the affected side and 0.8 mg/kg/hr (range 0.4–0.9 mg/kg/hr) from the unaffected side. The median length of time to resolution of POD was 3 days (range 2–4 days). One patient developed significant acidosis and lethargy that improved with intravenous fluid management. Mild hyponatremia developed in two, hypokalemia in one, hypophosphatemia in one, acidosis in one, and hypoglycemia in 1 patient. Discussion A low but clinically significant risk of POD occurring after relief of unilateral UPJO in children with a normal contralateral kidney is described. Limitations include retrospective analysis and small sample size due to the rarity of the condition. Conclusion Postobstructive diuresis after decompression of UPJO in patients with a normal contralateral kidney is a rare event (1.8%). However, POD does occur, and patients should be carefully monitored after these procedures given the potential for significant dehydration and electrolyte disturbances.Item Initial collection of an inadequate 24-hour urine sample in children does not predict subsequent inadequate collections(Elsevier, 2018) Chan, Katherine H.; Moser, Elizabeth A.; Whittam, Benjamin M.; Misseri, Rosalia; Cain, Mark P.; Krambeck, Amy; Schwaderer, Andrew; Urology, School of MedicineIntroduction Approximately half of adult stone formers submit specimens that are either under or over collections as determined by 24-h creatinine/kg. Previously identified predictors of inadequate collection in adults include female sex, older age, higher body mass index (BMI), vitamin D supplementation, and weekday collection. Objective The objective of this study is to determine risk factors for inadequate 24-h urinary specimen collection in the pediatric population. Study design A retrospective analysis of all children (<18 years of age) with renal and/or ureteral calculi evaluated at the study tertiary care pediatric center from 2005 to 2015 was performed. Those who had at least one 24-h urinary metabolic profile after a clinical visit for kidney and/or ureteral stones were included; children with bladder stones were excluded. Adequate collections had a urine creatinine of 10–15 mg/kg/24 h. A bivariate analysis of potential factors associated with inadequate collection of the initial urinary metabolic profile, including child demographics, parental socio-economic factors, history of stone surgery, and weekday vs. weekend urine collection, was performed. A mixed-effects logistic regression, controlling for correlation of specimens from the same patient, was also performed to determine whether an initial inadequate collection predicted a subsequent inadequate collection. Results Of 367 patients, 80 had an adequate collection (21.9%): median age, 13 years (interquartile range, 8–16); 61.1% female; 93.5% white; 19.5% obese; and 13.0% overweight. No parental or child factors were associated with inadequate collection (Summary Table). Of inadequate collections, more than 80% were over collections. In the 175 patients with more than one 24-h urinary specimen collection, the effect of an initial inadequate collection on subsequent inadequate collections was not significant after controlling for the correlation of samples from the same patient (p = 0.8). Discussion Any parental or child factors associated with the collection of inadequate 24-h urine specimens in children were not found. An initial inadequate collection does not predict subsequent inadequate collections. It was surprising that >80% of the inadequate collections were over collections rather than under collections. Possible explanations are that children collected urine samples for longer than the 24-h period or that stone-forming children produce more creatinine per 24-h period than healthy children due to hyperfiltration. Conclusion Inadequate collections are very common, and the risk factors for them are unclear. A repeat collection would be suggested if the first is inadequate. Further studies must be planned to explore barriers to accurate specimen collection using qualitative research methodology.Item Is surgical antibiotic prophylaxis necessary for pediatric orchiopexy?(Elsevier, 2018) Rensing, Adam J.; Whittam, Benjamin M.; Chan, Katherine H.; Cain, Mark P.; Carroll, Aaron E.; Bennett, William E., Jr.; Urology, School of MedicineIntroduction Surgeons frequently use surgical antibiotic prophylaxis (SAP), despite limited evidence to support its efficacy. Potential adverse events associated with antibiotic use include allergic reaction (including anaphylaxis), Clostridium difficile infection, and selecting for resistant bacteria. Surgical site infections (SSI) are very rare in patients undergoing clean pediatric urologic procedures. Current guidelines are unclear about the efficacy of surgical antibiotic prophylaxis for prevention of SSI in the pediatric population. Objective It was hypothesized that children who received SAP prior to orchiopexy would have no reduction in surgical site infection (SSI) risk but an increased risk of antibiotic-associated adverse events. Methods A retrospective cohort study was conducted of all males aged between 30 days and 18 years who underwent an orchiopexy (ICD-9 CM 62.5) in an ambulatory or observation setting from 2004 to 2015 using the Pediatric Health Information System database. Inpatients and those with concomitant procedures were excluded. Chi-squared or Fisher's exact tests were used to determine the association between SAP and allergic reaction (defined as a charge for epinephrine or ICD-9 diagnosis code for allergic reaction on the date of surgery) and any of the following within 30 days: SSI, hospital readmission or any repeat hospital encounter. Mixed effects logistic regression was performed, controlling for age, race, and insurance, and clustering of similar practice patterns by hospital. Results A total of 71,767 patients were included: median age was 4.6 years, 61.4% were white, and 49.3% had public insurance; 33.5% received SAP. Of these participants, 996/71,767 (1.4%) had a perioperative allergic reaction and <0.1% were diagnosed with an SSI. On mixed effects logistic regression, those who received SAP had 1.2 times the odds of a perioperative allergic reaction compared with those who did not receive SAP ( P = 0.005). Surgical antibiotic prophylaxis was not associated with decreased rates of SSI, lower hospital readmission, nor a lower chance of a repeat encounter within 30 days. Conclusions In patients undergoing orchiopexy, it was found that SAP did not reduce the risk of postoperative SSI, readmissions, or hospital visits. Patients who received SAP had significantly increased odds of perioperative allergic reaction. This demonstrated that the risks of SAP outweigh the benefits in children undergoing orchiopexy.Item Pediatric Sacral Nerve Stimulator Explanation due to Complications or Cure: A Survival Analysis(Elsevier, 2018) Rensing, Adam J.; Szymanski, Konrad M.; Dunn, Sally; King, Shelly; Cain, Mark P.; Whittam, Benjamin M.; Urology, School of MedicineIntroduction Historically, there have been few treatment options for children with severe, refractory bladder and bowel dysfunction (BBD). Sacral neuromodulation (SNM) continues to show promising results in this challenging pediatric population with recalcitrant lower urinary tract symptoms. At our institution, we have begun offering explantation to those with persistent improvement after >6 months of having device turned off. We hypothesized that 1.) SNM explantation for cure increases with extended follow-up, and 2.) those explanted for cure would have improved symptoms and quality of life when compared to those explanted for complication. Materials & Methods We retrospectively reviewed all consecutive patients <18 years old who underwent SNM placements at our institution (2012-2017). We excluded those without the second stage procedure. Reasons for device explantation were categorized as: cure (resolution of symptoms with the device turned off for at least 6 months), or a complication (e.g. infection, need for MRI, or pain). Non-parametric tests and survival analysis were used for analysis to account for differential follow-up time. Of those explanted, surveys were electronically sent to assess BBD severity, and overall quality of life. Results Of 67 children who underwent a first stage procedure, 62 (92.5%) underwent a second stage procedure. 61 met inclusion criteria (68.9% female, 29.5 % with previous filum section, median age at implantation 10.3 years old). During follow-up (median 2.3 years), 12 patients (19.7 %) had the SNM exchanged/revised due to lead fracture/breakage and return of urinary symptoms. To date, 50 patients remain with their SNM implanted, and 11 have been explanted. Adjusting for follow-up time, the risk of explantation was 6.5% at 2 years (2.2% for cure, 4.3% for complications) (Figure 1). Explantation increased to 24.5% at 3 years (16.5% for cure, 8.0% for complications) and 40.4% at 4 years (32.4% for cure, 8.0% for complications). Questionnaires were collected on patients post explant (median 2.2 years), with improvement in those explanted for cure compared to complication (Figure 2). Discussion SNM explantation for cure is a novel concept previously not described in the literature. Limitations of this study include the relatively small numbers, and lack of objective data in the cohort that remains with SNM device implanted. Conclusion SNM is a safe, viable option for the pediatric patient with refractory bladder dysfunction. Furthermore, SNM explantation for cure is an option with increasing likelihood after two years.Item Radiographic abnormalities, bladder interventions, and bladder surgery in the first decade of life in children with spina bifida(Springer, 2019-07) Rensing, Adam J.; Szymanski, Konrad M.; Misseri, Rosalia; Roth, Joshua D.; King, Shelly; Chan, Katherine; Whittam, Benjamin M.; Kaefer, Martin; Rink, Richard C.; Cain, Mark P.; Urology, School of MedicineBackground Spina bifida (SB) patients are at increased risk for hydronephrosis, bladder storage and emptying problems, and renal failure that may require multiple bladder surgeries. Methods We retrospectively reviewed patients born with SB 2005–2009, presenting to our institution within 1 year of birth. Outcomes at 8–11 years old included final renal/bladder ultrasound (RBUS) results, clean intermittent catheterization (CIC) use, anticholinergic use, surgical interventions, and final renal function. We excluded those without follow-up past age 8 and/or no RBUS or fluoroscopic urodynamic images (FUI) within the first year of life. Imaging was independently reviewed by four pediatric urologists blinded to radiologists’ interpretation and initial findings compared with final outcomes. Results Of 98 children, 62 met inclusion criteria (48% male, 76% shunted). Median age at last follow-up was 9.6 years. Upon initial imaging, 74% had hydronephrosis (≥ SFU grade 1), decreasing to 5% at 10 years (p < 0.0001). Initially, 9% had ≥ SFU grade 3 hydronephrosis, decreasing to 2% (p = 0.13). CIC and anticholinergic use increased from 61% and 37% to 87% and 86%, respectively (p = 0.001 and p < 0.0001, respectively). With follow-up, 55% had surgical intervention and 23% had an augmentation. Of children with a serum creatinine/cystatin-C at 8–11 years old, one had confirmed chronic kidney disease (stage 2). Conclusions Despite initial high incidence of hydronephrosis, this was low grade and resolved in the first decade of life. Additionally, the 8–11-year incidence of kidney disease and upper tract changes was low due to aggressive medical management.