- Browse by Author
Browsing by Author "Rink, Richard C."
Now showing 1 - 10 of 16
Results Per Page
Sort Options
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 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 Interleukin-18 stimulates a positive feedback loop during renal obstruction via interleukin-18 receptor(Elsevier, 2011-10) VanderBrink, Brian A.; Asanuma, Hiroshi; Hile, Karen; Zhang, Honji; Rink, Richard C.; Meldrum, Kirstan K.; Urology, School of MedicinePURPOSE: Interleukin-18 is a proinflammatory cytokine that is an important mediator of obstruction induced renal tubulointerstitial fibrosis independent of tumor necrosis factor-α and β1 activity. We hypothesized that interleukin-18 stimulates a positive feedback loop during obstruction via interleukin-18 receptor to increase interleukin-18 gene expression and protein production. MATERIALS AND METHODS: Male C57BL6 interleukin-18 receptor knockout (The Jackson Laboratory, Bar Harbor, Maine) and control wild-type mice underwent unilateral ureteral obstruction or sham operation and were sacrificed 1 week after surgery. Renal cortical tissue samples were harvested and analyzed for interleukin-18 protein by enzyme-linked immunosorbent assay, and for interleukin-18 and interleukin-18 receptor gene expression by quantitative polymerase chain reaction. The specific cellular localization of interleukin-18 and interleukin-18 receptor expression during obstruction was assessed using dual labeling immunofluorescence staining. RESULTS: Renal interleukin-18 receptor expression increased significantly in wild-type mice in response to obstruction but remained at sham operation levels in interleukin-18 receptor knockout mice. Similarly while interleukin-18 protein and gene expression were significantly increased in wild-type mice in response to obstruction, interleukin-18 levels and gene expression were significantly decreased during obstruction in knockout mice. Obstruction induced interleukin-18 and interleukin-18 receptor production were localized predominantly to tubular epithelial cells and to a lesser extent to the renal interstitium. CONCLUSIONS: Results reveal that interleukin-18 stimulates a positive feedback loop via interleukin-18 receptor during renal obstruction to stimulate interleukin-18 production and gene expression. The predominant cellular source of interleukin-18 production during renal obstruction appears to be tubular epithelial cells rather than infiltrating macrophages.Item Long-term outcomes of catheterizable continent urinary channels: what do you use, where you put it and does it matter?(Elsevier, 2015-08) Szymanski, Konrad M.; Whittam, Benjamin; Misseri, Rosalia; Flack, Chandra K.; Hubert, Katherine C.; Kaefer, Martin; Rink, Richard C.; Cain, Mark P.; Department of Urology, IU School of MedicineIntroduction Appendicovesicostomy (APV) and Monti ileovesicostomy (Monti) are commonly used catheterizable channels with similar outcomes on short-term follow-up. Their relative long-term results have not been previously published. Objective Our goal was to assess long-term durability of APV and Monti channels in a large patient cohort. Study design In this retrospective cohort study, we retrospectively reviewed consecutive patients ≤21 years old undergoing APV and Monti surgery at our institution (1990–2013). We collected data on demographics, channel type, location, continence and stomal and subfascial revisions. Kaplan–Meier survival and Cox proportional hazards analysis were used. Results Of 510 patients meeting inclusion criteria, 214 patients had an APV and 296 had a Monti (50.5% spiral Monti). Median age at surgery was 7.4 years for APV (median follow-up: 5.7 years) and 8.7 years for Monti (follow-up: 7.7 years). Stomal stenosis, overall stomal revisions and channel continence were similar for APV and Monti (p ≥ 0.26). Fourteen APVs (6.5%) had subfascial revisions compared to 49 Montis (16.6%, p = 0.001). On survival analysis, subfascial revision risk at 10 years for APV was 8.6%, Monti channels excluding spiral umbilical Monti: 15.5% and spiral umbilical Monti: 32.3% (p < 0.0001, Figure). On multivariate regression, Monti was 2.09 times more likely than APV to undergo revision (p = 0.03). The spiral Monti to the umbilicus, in particular, was 4.23 times more likely than APV to undergo revision (p < 0.001). Concomitant surgery, gender, age and surgery date were not significant predictors of subfascial revision (p ≥ 0.17). Stomal location was significant only for spiral Montis. Discussion Our study has several limitations. Although controlling for surgery date was a limited way of adjusting for changing surgical techniques, residual confounding by surgical technique is unlikely, as channel implantation technique was typically unrelated to channel type. We did not include complications managed conservatively or endoscopically. In addition, while we did not capture patients who were lost to follow-up, we attempted to control for this through survival analysis. Conclusions We demonstrate, durable long-term results with the APV and Monti techniques. The risk of channel complications continues over the channel's lifetime, with no difference in stomal complications between channels. At 10 years after initial surgery, Monti channels were twice as likely to undergo a subfascial revision (1 in 6) than APV (1 in 12). The risk is even higher in for the spiral umbilical Monti (1 in 3).Item Majority of females with a life-long experience of CAH and parents do not consider females with CAH to be intersex(Elsevier, 2021-04) Szymanski, Konrad M.; Rink, Richard C.; Whittam, Benjamin; Hensel, Devon J.; Life with Congenital Adrenal Hyperplasia Study Group; Urology, School of MedicineIntroduction To assess opinions of females with CAH, and parents of females with CAH, about designating this population “intersex,” particularly in legislation about genital surgery during childhood. Methods We conducted a mixed-methods (quantitative and qualitative) anonymous cross-sectional online survey of females with CAH (46XX, 16+years old) and independently recruited parents of girls with CAH (2019–2020) diagnosed in first year of life from the United States. A multidisciplinary CAH team drafted the survey in collaboration with women with CAH and parents. Fisher's exact test was used to compare female and parent responses. A qualitative thematic approach was used to analyze open-ended answers for emergent categories of reasons why CAH females should or should not be considered as intersex. Results Of 57 females with CAH participating (median age: 39 years, 75.5% of ≥25year olds had post-secondary degree), all had classical CAH and 93.0% underwent genital surgery at median 1–2 years old. While 89.5% did not endorse the intersex designation for CAH, the remaining 5.3% did (5.3% provided no answer, Summary Figure). Most CAH females (63.2%) believed CAH females should be considered separately in “any laws banning or allowing surgery of children's genitals” (19.3% disagreed, 17.5% neutral, 0.0% no answer). Most common themes identified by females with CAH not endorsing an intersex designation were: normal female internal organs, sex chromosomes, personal identity, genital appearance, issues with language, hormones, and those endorsing it: genital appearance, community/group experiences, topic complexity. Overall, 132 parents of females with CAH participated (parent/child median ages: 40/11 years, 81.7% of ≥25year olds had post-secondary degree). All children had classical CAH and 78.8% underwent surgery at median <1 year old. While 95.5% of parents did not endorse the intersex designation for CAH, 2.3% did (2.3% no answer), similar to females (p = 0.29). Most parents (81.1%) believed CAH females should be considered separately in legislation (9.1% disagreed, 6.1% neutral, 3.8% no answer), a slightly higher percentage than females (p = 0.01). Discussion Echoing previously published disagreement with clinically designating CAH females as intersex, majority of CAH females and parents oppose a legal intersex designation. Differing opinions among females and parents strengthen concern about a one-size-fits-all approach to legislation about childhood genital surgery. Differences in opinions between female and parent responses, while statistically significant, were relatively small. Conclusion: Majority of females with CAH and their parents believe CAH should be excluded from the intersex designation and be considered separately in legislation pertaining to childhood genital surgery in childhood.Item An ovulating follicle presenting as a testicular mass in a teenage patient with ovotesticular DSD(Elsevier, 2018-01-31) Roth, Joshua D.; Haddad, Nadine G.; Albright, Eric A.; Cheng, Liang; Rink, Richard C.; Kaefer, Martin; Urology, School of MedicineItem A Proposal for a New Approach to Differences of Sexual Development Research(AUA, 2020-01) Szymanski, Konrad M.; Cain, Mark P.; Rink, Richard C.; Urology, School of MedicineItem Quality of Life Assessment in Spina Bifida for Children (QUALAS-C): Development and Validation of a Novel Health-related Quality of Life Instrument(Elsevier, 2016-01) Szymanski, Konrad M.; Misseri, Rosalia; Whittam, Benjamin; Yang, David Y.; Raposo, Sonia-Maria; King, Shelly J.; Kaefer, Martin; Rink, Richard C.; Cain, Mark P.; Department of Urology, IU School of MedicineObjective To develop and validate a self-reported health-related QUAlity of Life Assessment in Spina bifida for Children (QUALAS-C). Methods We drafted a 27-question pilot instrument using a patient-centered comprehensive item generation and refinement process. It was administered to a sample of children 8-12 years old with spina bifida (SB) recruited online via social media and in person at an outpatient SB clinic (January 2013-September 2014). Healthy controls were recruited at routine pediatrician visits. Validation and final questions were determined based on clinical relevance, high loadings on factor analysis, and domain psychometrics. Children with SB also completed the validated generic Kidscreen-27 instrument. Results Median age of 150 participants was 9.6 years (60.7% male, 72.7% Caucasian), similar to 46 controls (P ≥ .10). There were 97 online and 53 clinic participants (89.0% and 84.2% of eligible, respectively). Face and content validities of the 2-domain, 10-question QUALAS-C were established by patients, parents, and experts. Internal consistency and test-retest reliability was high for the Esteem & Independence and Bladder & Bowel domains (Cronbach's alpha: 0.72-0.76, ICC: 0.74-0.77). Correlations between QUALAS-C domains were low (r = 0.51), indicating that QUALAS-C can differentiate between two distinct health-related quality of life components. Correlations between QUALAS-C and Kidscreen-27 were also low (r ≤ 0.44). QUALAS-C scores were significantly lower in children with SB than without (P < .0001). Conclusion QUALAS-C is a short, valid health-related quality of life tool for children with SB. It will be useful in clinical and research settings.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.