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Browsing by Author "Bennett, William E., Jr."
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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 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 A feasibility study of telemedicine for paediatric sickle cell patients living in a rural medically underserved area(Sage, 2021) Jacob, Seethal A.; Carroll, Aaron E.; Bennett, William E., Jr.; Pediatrics, School of MedicineIntroduction: Sickle cell disease (SCD) is the most common inherited haematological disease, with potentially devastating complications. Improvements in therapies have increased the life span of patients with SCD, but this is contingent on receiving timely evidence-based medical care, including regular evaluations with haematologists, disease-specific education and psychosocial care. Our objective was to evaluate the feasibility of utilizing telemedicine for the provision of subspecialty paediatric SCD care in a rural medically underserved area. Methods: This was a cross-sectional, observational, feasibility study. All patients 0-21 years old with SCD seen at Riley Hospital for Children Comprehensive Pediatric Sickle Cell Clinic who lived within 30 miles of the spoke telemedicine facility were eligible for recruitment. The Telehealth Satisfaction Scale (TeSS) was adapted for the SCD population and administered at each visit. Results: Ten SCD patients, ranging in age from 10 months to 18 years old, initiated telemedicine visits during this timeframe. Some 60% were lost to follow-up or did not attend >50% of scheduled visits prior to beginning telemedicine visits. Following initiation of telemedicine, all Hb SS patients were started and/or maintained on hydroxyurea. Nine out of 10 patients who participated during this timeframe had a 100% follow-up rate. All who participated rated the comfort and ease of using the telehealth system as good or excellent and would do a telemedicine visit again. Discussion: This study provides critical information to determine the feasibility and acceptability of a telemedicine intervention to aid in SCD care. To our knowledge, this is the first study to examine the effectiveness of telemedicine to deliver comprehensive paediatric SCD care. Future research with a larger sample size is needed to confirm findings of our study, including expansion of telemedicine sites to include more urban areas.Item Identifying Patient-Centered Outcomes for Caregivers and Children With Musculoskeletal Infections(Oxford University Press, 2022-12-12) Wood, James B.; Hawryluk, Bridget; Lynch, Dustin; Claxton, Gina; Russell, Kelsey; Bennett, William E., Jr.; Wiehe, Sarah E.; Carroll, Aaron E.; Pediatrics, School of MedicineBackground: Musculoskeletal infections (MSKI), including osteomyelitis and septic arthritis, are among the most common invasive infections in children and have the potential to cause significant morbidity. Guidelines have been developed to optimize care based on clinician-developed endpoints. Patient-centered outcomes have not been defined for children with MSKI. This study identified outcomes most important to caregivers and patients with MSKI. Methods: This was a single-center prospective qualitative study of children 6 months to 18 years of age hospitalized with MSKI from November 2019 to September 2021. Using design-research methods, patients and caregivers participated in interviews and/or completed journals to describe their experiences during acute infection and recovery from MSKI. Results: A total of 51 patient/caregivers were approached to participate in the study, 35 of whom declined to participate, resulting in 8 interviews conducted and 14 journals collected from 16 patient/caregivers. From these, a journey map was created highlighting points of stress during the onset of symptoms, through hospitalization, and returning home with new challenges. In addition, patient-centered outcomes were identified. For caregivers, these included managing mental health, managing responsibilities, and receiving support. Both caregivers and patients shared the importance of understanding of treatment plans and responsibilities. For patients, improving mental and physical health was important. Conclusions: Management of children with MSKI is complex and requires a multidisciplinary team approach. Validation of the outcomes identified and development of a measurement tool are needed. Addressing the patient-centered outcomes we identified in this study can greatly improve the holistic care of children with MSKI.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 Mental Health Screening as the Standard of Care in Pediatric Inflammatory Bowel Disease(AMA, 2019-08) Bennett, William E., Jr.; Pfefferkorn, Marian D.; Pediatrics, School of MedicineItem Parents of healthy children assign lower quality of life measure to scenarios labeled as cancer than to identical scenarios not labeled as cancer(BMC, 2019-02-21) McElderry, Brenna M.; Mueller, Emily L.; Garcia, Abigail; Carroll, Aaron E.; Bennett, William E., Jr.; Medicine, School of MedicineBACKGROUND: While it is commonly understood that a cancer diagnosis evokes feelings of fear, the effect of labeling a child's illness as "cancer" remains unstudied. We hypothesized that lower health utility scores would be assigned to disease states labeled as cancer compared to identical disease states without the mention of cancer. METHODS: In this randomized study, caregivers of healthy children were asked to assign health utility values to different scenarios written as improving, stable, or worsening. Participants from general pediatric clinics at Eskenazi Health were randomly assigned to either the scenarios labeled as "cancer" or "a serious illness". Participants then rated the scenarios using the Standard Gamble, with laddering of health utilities between 0 (a painless death) and 1 (perfect health). We also gathered subject demographics and assessed the subject's numeracy. RESULTS: We approached 319 subjects and 167 completed the study. Overall median health utilities of "cancer" scenarios were lower than "serious illness" scenarios (0.61 vs. 0.72, p = 0.018). Multivariate regression (with an outcome of having a utility above the 75th percentile) showed no significant effects by race, ethnicity, numeracy, or income level. "Cancer" scenarios remained significantly lower after adjustment for confounders using logistic regression, but only for the more serious scenarios (OR 0.92, p = 0.048). CONCLUSIONS: On average, caregivers with healthy children were shown to take more risk with their treatment options and view their child as having a worse quality of life when they knew the disease was cancer. Awareness of this bias is important when discussing treatments with families, particularly when a risk of cancer is present.Item Phelan-McDermid syndrome: a classification system after 30 years of experience(BMC, 2022-01-29) Phelan, Katy; Boccuto, Luigi; Powell, Craig M.; Boeckers, Tobias M.; van Ravenswaaij‑Arts, Conny; Rogers, R. Curtis; Sala, Carlo; Verpelli, Chiara; Thurm, Audrey; Bennett, William E., Jr.; Winrow, Christopher J.; Garrison, Sheldon R.; Toro, Roberto; Bourgeron, Thomas; Pediatrics, School of MedicinePhelan-McDermid syndrome (PMS) was initially called the 22q13 deletion syndrome based on its etiology as a deletion of the distal long arm of chromosome 22. These included terminal and interstitial deletions, as well as other structural rearrangements. Later, pathogenetic variants and deletions of the SHANK3 gene were found to result in a phenotype consistent with PMS. The association between SHANK3 and PMS led investigators to consider disruption/deletion of SHANK3 to be a prerequisite for diagnosing PMS. This narrow definition of PMS based on the involvement of SHANK3 has the adverse effect of causing patients with interstitial deletions of chromosome 22 to "lose" their diagnosis. It also results in underreporting of individuals with interstitial deletions of 22q13 that preserve SHANK3. To reduce the confusion for families, clinicians, researchers, and pharma, a simple classification for PMS has been devised. PMS and will be further classified as PMS-SHANK3 related or PMS-SHANK3 unrelated. PMS can still be used as a general term, but this classification system is inclusive. It allows researchers, regulatory agencies, and other stakeholders to define SHANK3 alterations or interstitial deletions not affecting the SHANK3 coding region.Item Quantitative Risk-Benefit Analysis of Probiotic Use for Irritable Bowel Syndrome and Inflammatory Bowel Disease(Springer, 2016-04) Bennett, William E., Jr.; Department of Pediatrics, IU School of MedicineProbiotics have seen widespread use for a variety of gastrointestinal problems, especially in two common disorders: irritable bowel syndrome and inflammatory bowel disease. Since a wide variety of probiotic preparations has been used, and despite a large number of studies performed, a great deal of heterogeneity exists among them. Straightforward evidence-based recommendations for the use of probiotics in irritable bowel syndrome and inflammatory bowel disease have thus been difficult to formulate. In an effort to improve understanding of the risk–benefit balance of probiotics in these conditions, this study (1) queried the US FDA Adverse Event Reporting System (FAERS) database for all reported adverse drug events related to probiotics in 2013, and (2) constructed risk–benefit planes for both irritable bowel syndrome and inflammatory bowel disease using a geometric approximation of the confidence region between risk and benefit. The results show that adverse events from probiotics vary widely by disease, and when they occur, they are mild and may be difficult to distinguish from the natural history of the underlying disorders they are used to treat. The risk–benefit plane for irritable bowel syndrome straddles the risk–benefit threshold, so patients can expect a balance between a low chance of risk and also a low chance of benefit. The risk–benefit plane for inflammatory bowel disease largely lies above the risk–benefit threshold, so patients may expect more benefit than risk in most cases. More standardized and high-quality research is needed to improve our understanding of risk and benefit for these complex biopharmaceuticals.Item Serologic, but Not Genetic, Markers Are Associated With Impaired Anthropometrics at Diagnosis of Pediatric Crohn's Disease(Wolters Kluwer, 2019-11) Naramore, Sara K.; Bennett, William E., Jr.; Jiang, Guanglong; Kugathasan, Subra; Denson, Lee A.; Hyams, Jeffrey S.; Steiner, Steven J.; Pediatrics, School of MedicineObjectives: Children with Crohn's disease may present with malnutrition and linear growth impairment, which can be secondary to insufficient caloric intake, chronic inflammation, malabsorption, and suppression of growth-promoting hormones. We evaluated clinical, serologic, and genetic data to determine risk factors for impaired anthropometrics in Crohn's disease at diagnosis. Methods: Our study evaluated 772 children newly diagnosed with Crohn's disease, inflammatory phenotype, enrolled in the RISK Stratification Project to determine the factors associated with anthropometric impairment. Data were collected on demographics, growth parameters, disease location, serologic and immunologic markers, and disease severity. We performed a genome-wide association study of genetic polymorphisms associated with inflammatory bowel disease. Regression analysis determined associations between anthropometrics and clinical, serologic, and genetic variables. Results: There were 59 (7%) children with height z score <−2, 126 (14%) with a weight z score <−2, and 156 (17%) with a body mass index z score <−2. Linear growth impairment was associated with hypoalbuminemia (P = 0.0052), elevated granulocyte-macrophage colony stimulating factor autoantibodies (P = 0.0110), and elevated CBir antibodies against flagellin (P = 0.0117). Poor weight gain was associated with female sex (P = 0.0401), hypoalbuminemia (P = 0.0162), and thrombocytosis (P = 0.0081). Malnutrition was associated with hypoalbuminemia (P = 0.0061) and thrombocytosis (P = 0.0011). Children with moderate or severe disease had lower weight (P = 0.02 and P = 1.16×10−6, respectively) and body mass index z scores (P = 2.7 × 10−3 and P = 1.01 × 10−6, respectively) than children with quiescent and mild disease. There was no association between age of diagnosis, Tanner stage, or disease location and having impaired anthropometrics. There was no genome-wide association between the genetic polymorphisms and the serologic variables and anthropometric measurements. Conclusions: This is the largest study evaluating growth in treatment-naïve children with Crohn's disease, inflammatory phenotype. It is the first study to use genome-wide sequencing to assess for genetic determinants of growth impairment. Granulocyte-macrophage colony stimulating factor autoantibodies and CBir antibodies are more likely to be elevated in children with growth impairment. Future investigations should evaluate the relationship between genetic polymorphisms, pathologic immune responses, and the biological pathways regulating growth.