Browsing by Author "Casey, Jessica T."

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    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 Medicine
    Objective 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.
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    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 Medicine
    Introduction 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.
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    Validation of QUALAS-T, a health-related quality of life instrument for teenagers with spina bifida
    (Polish Urological Association, 2017) Szymański, Konrad M.; Misseri, Rosalia; Whittam, Benjamin; Casey, Jessica T.; Yang, David Y.; Raposo, Sonia-Maria; King, Shelly J.; Kaefer, Martin; Rink, Richard C.; Cain, Mark P.; Urology, School of Medicine
    Introduction We aimed to develop and validate a self-reported QUAlity of Life Assessment in Spina bifida for Teenagers (QUALAS-T). Material and methods We drafted a 46-question pilot instrument using a patient-centered comprehensive item generation/refinement process. A group of 13–17 years olds with spina bifida (SB) was recruited online via social media and in person at SB clinics (2013–2015). Healthy controls were recruited during routine pediatrician visits. Final questions were identified based on clinical relevance, factor analysis and domain psychometrics. Teenagers with SB completed the validated generic Kidscreen-27 instrument. Results Median age of 159 participants was 15.2 years (42.0% male, 77.4% Caucasian), similar to 58 controls (p ≥ 0.06). There were 102 online and 57 clinic participants (82.8% of eligible). Patients, parents and an expert panel established face and content validity of the 2-domain, 10-question QUALAS-T. Internal consistency and test-retest reliability were high for the Family and Independence and Bladder and Bowel domains (Cronbach's alpha: 0.76–0.78, ICC: 0.72–0.75). The Bladder and Bowel domain is the same for QUALAS-T , QUALAS-A for adults and QUALAS-C for children. Correlations between QUALAS-T domains were low (r = 0.34), indicating QUALAS-T can differentiate between distinct HRQOL components. Correlations between QUALAS-T and Kidscreen-27 were also low (r ≤0.41). QUALAS-T scores were lower in teenagers with SB than without (p <0.0001). Conclusions QUALAS-T is a short, valid HRQOL tool for adolescents with SB, applicable in clinical and research settings. Since the Bladder & Bowel domains for all QUALAS versions are the same, Bladder and Bowel HRQOL can be measured on the same scale from age 8 through adulthood.