Background:Individuals with cirrhosis frequently require Emergency Department (ED) care, with some experiencing repeated ED use, yet little is known about the patient and caregiver perspectives driving decisions to visit the ED. We aimed to explore perspectives of including high ED utilizers with cirrhosis and their caregivers to identify drivers of ED use and opportunities to optimize care.Methods:Using human-centered design methods, we conducted an in-person group engagement session with 7 adults with cirrhosis and their caregivers, recruited from recent ED encounters. A custom board-game activity facilitated the discussion. Data were analyzed using snippet extraction, affinity mapping, and affinity concept modeling.Results:Seven major themes emerged: (1) Mindset around symptoms, which includes fear, uncertainty, and caregiver burden. (2) Informational needs, including reliance on variable-quality online resources and lack of trusted education. (3) Day-to-day cirrhosis management, particularly challenges related to medications and symptom monitoring. (4) Symptom-driven ED triggers, with some prompting, urgent visits. (5) Decision-making factors, including limited alternatives to ED care and prior experiences, and mismatched patient-caregiver thresholds for seeking care. (6) Expectations of ED care, focused on pain relief and return to baseline health. (7) Challenges during ED care, including long wait times, misdiagnosis concerns, and stigma related to pain treatment. Concept modeling revealed that ED decision-making is a dynamic journey shaped by symptom severity, emotional states, logistical considerations, and evolving patient-caregiver-provider roles.Conclusions:Pain, uncertainty about symptom severity, and lack of accessible real-time clinical support were major drivers of ED utilization in cirrhosis. Interventions addressing these specific needs may reduce avoidable ED use. These findings provide a patient-informed foundation for care delivery redesign in cirrhosis.

Schinzel-Giedion Syndrome (SGS) is an ultra-rare, multisystem, genetic developmental disorder caused by gain-of-function pathogenic variants in the SETBP1 gene. No standard of care (SoC) recommendations currently exist. To assess expert opinion on SoC for individuals with SGS using a modified Delphi method. A multidisciplinary panel of 21 experts from the USA and Europe was assembled. Experts responded to a two-round questionnaire, with a subgroup participating in a virtual workshop, through which recommendations pertaining to the diagnosis, monitoring, treatment, and management of SGS were iteratively developed. Consensus was defined as ≥ 70% of respondents demonstrating agreement/disagreement with 6-point Likert scale questions, or ≥ 70% of respondents selecting a given multiple-choice question option. Overall, 81/94 statements achieved consensus. Experts agreed that the recommendations should be considered applicable to any individual with confirmed SGS or an indicative phenotype and any SETBP1 gain-of-function mutation. Key considerations included early and sustained involvement of a multidisciplinary team, routine monitoring for common tumors, neurologic, renal, genitourinary, pulmonary, musculoskeletal and gastrointestinal manifestations/complications, and facilitation of shared decision-making processes. These recommendations should help guide clinicians and families/caregivers in care decisions to enhance quality and duration of life for individuals with SGS and facilitate shared decision-making.

Background Hospitals are transitioning away from traditional pagers to secure text messaging (STM) applications. STM is perceived to improve efficiency and accessibility. There is limited research on user's impressions of how STM impacts patient safety, provider wellness, and quality of patient care. Objectives To understand the use and perceptions of a clinical STM by pediatric residents at a free-standing quaternary care children's hospital. Methods A survey was conducted of pediatric residents regarding their experience with Diagnotes®. Demographic data were obtained along with use patterns, ability to perform tasks, and perceptions of intended purpose. Further questions evaluated agreement with communication strategies and satisfaction with features. Three open-ended questions asked about experience where STM impacted (1) patient care coordination and (2) patient safety. A final question asked for any additional STM feedback. Results Of 169 surveys, there were 112 respondents (66.3% response rate). Respondents unanimously endorsed daily STM use on their personal mobile devices with good knowledge of basic features. Respondents were overall satisfied with Diagnotes® (73.9%) including the ability to communicate efficiently (84.8%) and effectively (79.5%). Yet only 32.1% were satisfied with Diagnotes® training. Only 59.5% believed Diagnotes® was appropriate for urgent patient care needs and only 43.2% believed its purpose was to inform the team of patient emergencies. Key qualitative themes included improved coordination of patient care tasks through STM, but there were concerns raised around sending and receiving messages, the additional cognitive burden placed by STM, and differences in culture of use that created conflict. Conclusions Diagnotes® is viewed positively including use for effective coordination of patient care and familiarity of functions of Diagnotes®. Barriers included unclear interprofessional expectations for use. Future research should incorporate a broad range of healthcare professionals' perceptions and co-creation of STM best practice guidelines for use, including around urgent or emergent patient care issues.

Phelan-McDermid syndrome (PMS) is characterized by variable clinical presentations such as intellectual disability, autistic features, developmental delays, sleep disturbances, as well as different genetic causes, which range from point mutations in the SHANK3 gene to 22q13 deletions of over 9 Megabases. This extensive clinical and genetic variability in PMS has hindered the development of viable treatment options. Several clinical trials have employed insulin-like growth factor 1 (IGF-1) and human growth hormone (hGH) to alleviate neurological symptoms in PMS with inconsistent and differing results across the PMS population.

INTRODUCTION:The Set Brave Goals app is the first digital health app (DHA) aimed at helping children with spina bifida (SB) aged 8-17 years old to select and track their urinary and fecal continence goals. Developed by children, parents, providers and researchers, its usability, or appropriateness to a purpose ("user-friendliness"), remains unknown. In an alpha testing stage, our aim was to determine the usability of the app prior to clinical use. METHODS:We recruited children with SB and their parents in clinic and via social media. A recruitment goal of 10 children exceeded industry standard of 5 participants (10 participants are expected to identify approximately 96% of usability problems). Participants downloaded and used the app for a week. They completed a questionnaire, including the System Usability Scale (SUS) and closed and open-ended questions. The SUS is a 10-item validated usability questionnaire most widely used in DHA usability testing (scores range from 0 to 100, 100 representing greatest usability). Median/mean SUS scores for DHAs are 68. Mann-Whitney-U and t-tests were used. RESULTS:Ten children with SB participated (median age: 14 years old, 6 female, 8 shunted, 8 using bladder catheterizations). Twenty parents participated (17 mothers, median age: 42 years old). Median SUS score was 77.5 for children and 73.8 for parents, corresponding to "good" to "excellent" usability (Summary Figure). There were no significant differences between child/parent scores (p = 0.69) or those for other DHAs (p = 0.11). It took a median 5-10 min to go through the app (2 parents felt it took too much time). All participants felt the app was easy to understand and use (100%). Most participants would recommend it to children and parents (children: 89%, parents: 80%), and believed it would be valuable to children (90%, 75%) and urologists (80%, 80%). Free text responses related to more varied color schemes, keeping free-text notes within the app and more flexible goal/alarm setting. These changes were incorporated into the app. COMMENT:Usability testing prior to clinical launch identified areas for app improvement. Although this study sample met industry standards, findings are limited by a small group of participants. The app will undergo further refinement during prospective beta testing. CONCLUSIONS:The Set Brave Goals app for children with SB has acceptable usability parameters. This justifies proceeding with wider use to formalize children's continence goal setting/tracking and testing its value to children with SB and their healthcare providers.

Phelan-McDermid syndrome (PMS) is a genetic condition caused by SHANK3 haploinsufficiency and characterized by a wide range of neurodevelopmental and systemic manifestations. The first practice parameters for assessment and monitoring in individuals with PMS were published in 2014; recently, knowledge about PMS has grown significantly based on data from longitudinal phenotyping studies and large-scale genotype-phenotype investigations. The objective of these updated clinical management guidelines was to: (1) reflect the latest in knowledge in PMS and (2) provide guidance for clinicians, researchers, and the general community. A taskforce was established with clinical experts in PMS and representatives from the parent community. Experts joined subgroups based on their areas of specialty, including genetics, neurology, neurodevelopment, gastroenterology, primary care, physiatry, nephrology, endocrinology, cardiology, gynecology, and dentistry. Taskforce members convened regularly between 2021 and 2022 and produced specialty-specific guidelines based on iterative feedback and discussion. Taskforce leaders then established consensus within their respective specialty group and harmonized the guidelines. The knowledge gained over the past decade allows for improved guidelines to assess and monitor individuals with PMS. Since there is limited evidence specific to PMS, intervention mostly follows general guidelines for treating individuals with developmental disorders. Significant evidence has been amassed to guide the management of comorbid neuropsychiatric conditions in PMS, albeit mainly from caregiver report and the experience of clinical experts. These updated consensus guidelines on the management of PMS represent an advance for the field and will improve care in the community. Several areas for future research are also highlighted and will contribute to subsequent updates with more refined and specific recommendations as new knowledge accumulates.

Phelan-McDermid syndrome (PMS), caused by pathogenic variants in the SHANK3 gene or 22q13 deletions, is characterized by intellectual disability, autistic features, developmental delays, and neonatal hypotonia. Insulin-like growth factor 1 (IGF-1) and human growth hormone (hGH) have been shown to reverse neurobehavioral deficits in PMS. We assessed the metabolic profiling of 48 individuals with PMS and 50 controls and determined subpopulations by taking the top and bottom 25% of responders to hGH and IGF-1. A distinct metabolic profile for individuals with PMS showed a reduced ability to metabolize major energy sources and a higher metabolism of alternative energy sources. The analysis of the metabolic response to hGH or IGF-1 highlighted a major overlap between both high and low responders, validating the model and suggesting that the two growth factors share many target pathways. When we investigated the effect of hGH and IGF-1 on the metabolism of glucose, the correlation between the high-responder subgroups showed less similarity, whereas the low-responders were still relatively similar. Classification of individuals with PMS into subgroups based on responses to a compound can allow an investigation into pathogenic mechanisms, the identification of molecular biomarkers, an exploration of in vitro responses to candidate drugs, and eventually the selection of better candidates for clinical trials.

OBJECTIVE:To investigate the microbial patterns and clinical outcomes of pediatric patients undergoing mastoidectomy for acute coalescent mastoiditis and to identify factors associated with poor outcomes and/or prolonged treatment. STUDY DESIGN:Monocentric retrospective cohort study. SETTING:Tertiary referral pediatric hospital in Indiana. METHODS:By cross-referencing database data from the Pediatric Health Information System (PHIS) querying for all inpatient stays (patients younger than eighteen) with a diagnostic code of mastoiditis between January 1st, 2010 and August 31, 2019, and the electronic health record (Cerner) for Riley Hospital for Children, 46 patients with mastoidectomy were included. A two-tailed T-test was used to evaluate continuous parametric data. Statistical significance was determined as P < 0.05. For continuous variables, data was analyzed using continuous logistic regression. A criteria of p > 0.1 was used for inclusion in the multivariate regression. RESULTS:Inclusion criteria was met by 46 patients. From 2010 to 2019, S. pyogenes and S. pneumoniae were the most common bacteria, each isolated in 11 of 42 bacterial isolates (26.2%). There was no growth in 35.4% (17/48) of intra-operative wound cultures. On univariate analysis, patients with negative cultures had longer length of hospital stay (LOS) (7.7 days [6.5] vs. 4.3 [2.8]; p = 0.018) as well as higher rates of PICC (peripherally inserted central catheter) placement (53.3% vs. 19.4%; p = 0.021). There was a statistically significant difference in terms of gender (p = 0.021), with 15 males and 16 females in the positive culture cohort and 13 males and 2 females in the negative culture cohort. On multivariate analysis, which included gender, PICC placement, both intracranial and extracranial complications, duration of antibiotics, and LOS, female gender was the only significant predictor of positive culture status (p = 0.039). CONCLUSION:S. pyogenes and S. pneumoniae were the predominant etiologic agents in acute coalescent mastoiditis between 2010 and 2019, and negative wound cultures were associated with worse clinical outcomes.

BACKGROUND:Premature infants are at increased risk of kidney-related complications, including acute kidney injury (AKI) and chronic kidney disease (CKD). The risk of CKD in prematurely born infants is underrecognized by health care teams and caregivers. Understanding how to communicate the risk of CKD to caregivers is essential for longitudinal clinical follow-up and adherence.OBJECTIVE:This study aimed to determine family caregiver attitudes toward kidney health and risk communication during a neonatal intensive care admission. We also sought to understand caregiver preferences for the communication of information surrounding the risk of CKD in premature infants.METHODS:We augmented standard qualitative group sessions with human-centered design methods to assess parent preferences and clinician perspectives. Caregivers recruited had a prematurely born child who spent time in the neonatal intensive care unit at Riley Hospital for Children in Indianapolis, Indiana, and experienced AKI or another kidney complication, which put them at risk for future CKD. We used a variety of specific design methods in these sessions, including card sorting, projective methods, experience mapping, and constructive methods.RESULTS:A total of 7 clinicians and 8 caregivers participated in 3 group sessions. Caregivers and clinicians readily acknowledged barriers to and drivers of long-term kidney monitoring as well as opportunities for communication of the risk of long-term kidney disease. Caregivers' primary concerns were for both the type and depth of information conveyed as well as the time at which it was communicated. Participants emphasized the importance of collaboration between the hospital care team and the primary care provider. Participant input was synthesized into several prototype concepts and, ultimately, into a rough prototype of a website and an informational flyer.CONCLUSIONS:Caregivers of premature infants are open to communication about kidney health during their neonatal admission. The next phase of this work will translate caregivers' preferences into family-centered communication tools and test their efficacy in the neonatal intensive care unit.

Background 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. Using design-research methods, we identified patient-centered outcomes for caregivers and children with MSKI. Outcomes included managing mental health, managing responsibilities, and receiving support for caregivers, shared understanding of treatment plans and responsibilities, improving mental and physical health of the patient.

Phelan–McDermid syndrome (PMS) is a multi-systemic disorder characterized by both genetic and phenotypic variability. Genetic abnormalities causing PMS span from pathogenic variants of the SHANK3 gene to chromosomal rearrangements affecting the 22q13 region and leading to the loss of up to over nine megabases. The clinical presentation of individuals with PMS includes intellectual disability, neonatal hypotonia, delayed or absent speech, developmental delay, and minor dysmorphic facial features. Several other features may present with differences in age of onset and/or severity: seizures, autism, regression, sleep disorders, gastrointestinal problems, renal disorders, dysplastic toenails, and disrupted thermoregulation. Among the causes of this phenotypic variability, the size of the 22q13 deletion has effects that may be influenced by environmental factors interacting with haploinsufficiency or hemizygous variants of certain genes. Another mechanism linking environmental factors and phenotypic variability in PMS involves the loss of one copy of genes like BRD1 or CYP2D6, located at 22q13 and involved in the regulation of genomic methylation or pharmacokinetics, which are also influenced by external agents, such as diet and drugs. Overall, several non-mutually exclusive genetic and epigenetic mechanisms interact with environmental factors and may contribute to the clinical variability observed in individuals with PMS. Characterization of such factors will help to better manage this disorder.

Phelan-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.

OBJECTIVES: To describe a single-center experience of pediatric patients with hyperammonemia not due to inborn errors of metabolism and determine the association between use of continuous kidney replacement therapy (CKRT) treatment and outcomes. DESIGN: Retrospective cohort study. SETTING: Tertiary-care children's hospital. PATIENTS: All children less than 21 years old admitted to the hospital with hyperammonemia defined as an elevated ammonia levels (>100 µmol/L) not due to inborn error of metabolism. INTERVENTIONS: None. MEASURES AND MAIN RESULTS: Of 135 children with hyperammonemia, the most common reason for admission was infection in 57 of 135 (42%), congenital heart disease in 20 of 135 (14%), and bone marrow transplantation in 10 of 135 (7%). The overall mortality was 61% (82 of 135), which increased with degree of hyperammonemia (17 of 23 [74%] in those with ammonia >250 µmol/L). After multivariable regression, hyperammonemia severity was not associated with mortality (aOR, 1.4; 95% CI, 0.92–2.1; p = 0.11). Of the 43 patients (32%) receiving CKRT, 21 were prescribed standard clearance and 22 high clearance. The most common indications for CKRT were fluid overload in 17 of 43 (42%) and acute kidney injury or uremia in 16 of 43 (37%). Mean CKRT duration was 13 days. There was no difference between standard and high clearance groups in risk of death (76% vs 86%; p = 0.39), cerebral edema on CT scan (19% vs 27%; p = 0.52), nor decrease in ammonia levels after 24 or 48 hours of CKRT (p = 0.20, p = 0.94). Among those receiving CKRT, we failed to find an association between high clearance and decreased risk of death in multivariable analysis (aOR, 1.2; 95% CI, 0.64–2.3; p = 0.55). CONCLUSIONS: In our single-center retrospective study, we failed to find an association between clearance on CKRT and improved survival nor decreased cerebral edema on head imaging. In fact, we failed to find an association between ammonia level and mortality, after controlling for illness severity.

Robotic-assisted pyeloplasty (RAP) is a mainstay in the treatment of ureteropelvic junction obstruction (UPJO) in children. At our institution, to limit planned operating rooms visits we have placed a ureteral stent with an external string (SWES) immediately prior to RAP. In this study, we sought to quantify the operative time, complications, and costs associated with this approach compared to the traditional approach, requiring subsequent stent removal in the operating room. We hypothesized the SWES cohort would have decreased cost, yet with similar operative time and complications. We retrospectively collected all RAPs performed at our institution using the SWES approach (Aug 2012–July 2017). We excluded those with a redo pyeloplasty, and/or a percutaneous nephrostomy tube for post-operative drainage. We collected 30-day costs linked to the patients' MRN using the Pediatric Health Information System (PHIS) database. We compared 30-day healthare costs for all patients following RAP. We compared our SWES group to a national cohort of all pediatric RAP during the same time period. Lastly, we sent an anonymous, electronic survey to urologists of all PHIS institutions to identify the predominant postoperative drainage, nationally. Within our institution, we reviewed all those treated with SWES (n = 85) (Table 1). The median 30-day cost was $10,548 among those with SWES (Table 2). This was significantly less than the overall, national cohort of all pediatric RAP during the same period ($14,119, p < 0.001). There was a 15.5 % rate of unplanned return to the hospital in the SWES group. Of those unplanned returns, 8.2 % (7/85) had unplanned return for a procedure (3 for unplanned stent removal, 2 for nephrostomy tube for persistent obstruction, 1 for omental hernia, and 1 for stent replacement). With a 42.5 % (37/87) response rate, our nationwide survey found 84.6 % primarily leave stents WITHOUT a string, 7.7 % left nephrostomy tubes, and 7.7 % stents with strings. During pediatric RAP, placement of a SWES takes little time, carries a risk of unplanned visit to the operating room, saves the patient a certain, second anesthetic for stent removal, and amounts to a cost savings of approximately 25 %.

Introduction 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.

Background Proactively monitoring infliximab levels is an emerging area of interest in pediatric Crohn’s disease. There are only limited data on therapeutic drug monitoring for children with Crohn’s disease. The goal of our study was to determine the utility of therapeutic drug monitoring in achieving clinical remission in a cohort of pediatric Crohn’s disease patients receiving infliximab. Methods This prospective single-center study enrolled 37 patients with Crohn’s disease at the start of infliximab infusions and monitored trough levels at 6-month intervals for 18 months. Each participant was matched to a historic control for the modified pediatric Crohn’s disease activity index (mPCDAI) at baseline, age and sex. The primary outcome was an mPCDAI score of ≤7.5 at 6, 12 and 18 months. A multivariate logistic regression analysis was performed. Results Data were available for all 37 cases at 6 and 12 months and for 34 cases at 18 months. Demographics and disease characteristics were similar between groups. All 34 cases demonstrated clinical remission at 18 months (100% vs. 88%, P=0.114). Univariate and multivariate analyses did not show statistical significance. Dose intensification was seen more often in the cases at 18 months. Conclusion All of our moderate-to-severe pediatric Crohn’s disease patients who received prospective therapeutic drug monitoring of infliximab were in clinical remission at follow up, but this was not statistically significantly different from the 88% clinical remission rate of the control group.

Background: Hirschsprung disease is a relatively uncommon disorder of the developing gastrointestinal tract that requires surgical intervention to restore long-term function. While readmission for Hirschsprung-related complications is a known concern in these patients, we sought to identify patient-level factors associated with a prolonged hospital stay, increased costs at the time of a pull-through operation, as well as the risk for all-cause inpatient readmission after surgery. We hypothesized that higher level of care requirement during the operative stay, age at operation, and length of stay (LOS) would portend increased readmissions and disease-related complications such as Hirschsprung-associated enterocolitis. Methods: Data was obtained from the Pediatric Health Information System database on all Hirschprung patients who underwent a pull-through operation between 2004 and 2019. Regression analyses were performed on this cohort of 3345 patients. Multivariable regression models were utilized to analyze the key outcome variables of postoperative LOS and adjusted charges. Results: Post-operative LOS was significantly increased by the presence of a surgical complication, congenital/genetic defect, or neurologic/neuromuscular defect. Increased LOS was also seen in Black patients. The cost of pull-through operations was significantly higher in patients admitted to the NICU and ICU during index hospitalization, with a cost increase of approximately $75,000 and $57,000 respectively. Presence of a surgical complication, co morbid congenital/genetic defect, and need for mechanical ventilation were associated with higher odds of inpatient readmission. Conclusion: The management of patients with Hirschsprung disease is longitudinal and complex. Identification of key patient metrics can aid clinicians in developing targeted care and education strategies to minimize readmission and excessive hospital charges. (c) 2021 Elsevier Inc. All rights reserved.