Tertiary Pediatric Center Research Articles

Complications associated with subsequent tunneled central venous access device placement in children: a retrospective cohort study

Central venous access devices (CVADs) are vital instruments in pediatric healthcare, enabling the administration of critical treatments such as chemotherapy and parenteral nutrition. However, despite their advantages, CVADs carry a significant risk of complications, including infections, mechanical failures, and thrombotic events. From the current literature, it is unknown whether previous CVAD placements lead to an increased risk for complications in subsequent CVAD placements. We retrospectively analyzed data regarding tunneled, surgically implanted CVADs in children over a period of 2 years at a single tertiary pediatric center regarding their complications. Between 2021 and 2022, 328 CVAD implantations were performed in 313 children. The average age at implantation was 6.6 ± 5.5 years, while most of the patients were younger than 5 years old. During the study period, a total of 102 complications occurred in 96 patients. Most frequent complications were infections (18.29% of all implantations), followed by dislocation of the catheter tip (6.4 0%) and mechanical dysfunctions (4.27%). No patient died from a complication. The choice of catheter type (Port versus Broviac) showed a significant influence on the occurrence of complications (hazard ratio of 3.964 (95% CI 1.993–7.886; p < 0.001). The risk of infection and dislodgement was also higher when comparing Broviac with Ports implantations (infection: HR = 3.236; 95% CI 1.239–8.454; p = 0.017; dislodgement: HR = 5.781; 95% CI 1.229–27.193; p = 0.026). Cox regression showed a statistically significant higher risk of complications (especially infections) when the catheter was inserted via venous cutdown instead of percutaneous puncture technique (complications: HR = 6.709; 95% CI 1.776–25.337; p = 0.005; infections: HR = 7.28; 95% CI 1.096–48.379; p = 0.04). Cox regression did not show a statistically significant influence on complications for neither of the following factors: age, gender, weight, and oncological/non-oncological diagnosis. The complication rate for patients with previous CVAD was nearly the same as for patients who received a CVAD for the first time (previous CVAD: 29.17%; no previous CVAD: 30.74%). Conclusion: Our study challenges conventional assumptions regarding the impact of previous CVAD placements on complication rates. Nevertheless, ongoing vigilance and adherence to standardized protocols remain crucial in mitigating risks and improving outcomes in pediatric CVAD management.What is Known:• Risk factors predisposing for CVAD-related complications remain insufficiently understood.• It is unknown whether previous CVAD placements lead to an increased risk for complications in subsequent CVAD placements.What is New:• The complication rate for patients with previous CVAD appears to be the same as for patients who receive a CVAD for the first time.

Endoscopic and rectal tube management of pediatric sigmoid volvulus: A case series

AbstractThe process of the sigmoid colon twisting on its mesentery is known as sigmoid volvulus, a diagnosis rarely seen in the pediatric population. Volvulus can lead to blood flow obstruction of the colon and eventually perforation, necrosis, or sepsis. Predisposing factors include chronic constipation, chronic dysmotility, or Hirschsprung disease. This communication demonstrates three patients who presented to a tertiary pediatric care center with a diagnosis of sigmoid volvulus. All three patients underwent immediate endoscopic detorsion and rectal decompression tube placement. Two patients subsequently underwent surgical resection of redundant sigmoid colon. This communication highlights the use of sigmoidoscopy for detorsion of uncomplicated sigmoid volvulus with added support for initial rectal decompression tube placement, contributing to initial patient stabilization and positive patient outcomes.

Acute Pediatric Mastoiditis Numbers and Related Complications Before During and After the COVID-19 Pandemic.

To evaluate acute pediatric mastoiditis numbers and associated complications before, during, and after the COVID-19 pandemic. A retrospective chart review of all children less than 18 years of age presenting to a pediatric tertiary medical center with acute mastoiditis from January 1, 2017 to October 30, 2023 was completed. Exclusion criteria included history of chronic mastoiditis, cholesteatoma and mastoidectomy. Demographic and clinical data were collected and included age, sex, race, ethnicity, duration between onset of symptoms and presentation, white blood cell count and C-reactive protein level, affected ear, intracranial and extracranial complications, procedures, readmission and reoperation, length of hospital stay, and culture results. Patients were classified in relation to the COVID pandemic into three groups: pre-COVID included patients presenting from January 1, 2017, until December 31, 2019, COVID from January 1, 2020, until December 31, 2021, and post-COVID from January 1, 2022, until October 30, 2023. A total of 89 patients, 49 males and 40 females, with a mean age of 53.8±40.7 months were included. Twenty-four (27.0%) patients presented in the pre-COVID, 14 (15.7%) in the COVID, and 51 (57.3%) in the post-COVID period. There was no difference in the mean duration between onset of symptoms and presentation across the groups (5.4±4.4 days pre-COVID, 5.8±6.8 days during COVID, and 8.5±8.1 post-COVID, P=0.16). The post-COVID mean annual number of mastoiditis cases (27.8 cases/year) was higher compared to pre-COVID (8 cases/year) and COVID (7 cases/year). Similarly, the post-COVID mean annual number of cases with complications (24 cases/year) was higher compared to pre-COVID (5 cases/year) and COVID (5.5 cases/year). Of note, the post-COVID mean annual number of cases with intracranial complications (12cases/year) was also higher compared to pre-COVID (1.3cases/year) and COVID (1.5cases/year). There was no difference in the number of cases requiring operation across cohorts (97.8% overall, p=0.07). This study revealed a 3.5-fold increase in the mean annual number of mastoiditis cases and a 4.8-fold increase in the mean annual number of cases with complications post-COVID compared to pre-COVID.

Evaluation of severe hypoglycemia management in children and adolescents with type 1 diabetes in a Belgian tertiary pediatric care center: impact of intranasal glucagon and cost analysis.

To evaluate the management and costs of severe hypoglycemia (SH) in children and adolescents with type 1 diabetes (T1D) in our Belgian tertiary pediatric care center. In the EPI-GLUREDIA study, clinical parameters from children and adolescents with T1D were retrospectively analyzed from July 2017 to June 2024. The characteristics of SH and its treatment were collected during the medical consultation following the SH episode. Between July 2017 and June 2024, 208 cases of SH were recorded in 113 children and adolescents with T1D, with an average age of 13.6years and T1D duration of 6.2years. Oral glucose was the most common treatment (47.4%), while glucagon was used in only 25.4% of cases and more frequently in boys (30.8%) than in girls (18.7%). Notably, only 43% of SH episodes were treated according to international guidelines. A significant increase in glucagon use was observed after reimbursement of its intranasal form in Belgium in January 2022. After 2022, glucagon use significantly increased (28/81 vs. 25/129; p = 0.013), particularly among teachers and educators (18/49 vs. 10/78; p = 0.002). The average direct cost of treating SH was €187.9, with costs ranging from €0 to €1092.5 depending on the treatment method.Conculusion: Our study underscores the difficulty in managing SH in young people with T1D, with only 43% being treated as per guidelines. Since 2022, the increased use of the intranasal form of glucagon in Belgium led to reduced healthcare costs and improved care of patients experiencing SH.

Surgery for Bronchiectasis: Experience and Outcomes atStarship Children's Hospital, Auckland, New Zealand.

Surgical management for bronchiectasis is uncommon. This study reviewed the experience of bronchiectasis surgery and subsequent outcomes at a tertiary paediatric centre over a 20 year period. Retrospective audit of children aged < 18 years who underwent bronchiectasis surgery at Starship Children'sHospital between 2001 and 2021. Cases were identified from clinical coding, with demographics, pre-operative investigations and outcomes obtained from clinical records. Nineteen children (11 females, 42% Pasifika, 26% Māori and 26% New Zealand European) were included. Mean age of bronchiectasis diagnosis was 6.3 years (range 2.1-13.9 years) and mean age of surgery was 8.5 years (range 2.6-15.9 years). Indications for surgery included localised bronchiectasis (n = 7), main burden of multilobar disease in one lobe (n = 5) and persistent lobar collapse (n = 3). Pre-operative investigations included chest computerised tomography scan (68%), bronchoscopy (37%) and overnight oximetry (42%). One child underwent documented pre-operative clinical optimisation. For children with bronchiectasis < 5 years (n = 11), 81% demonstrated improved symptoms, 9% were unchanged and 9% deteriorated. All children with bronchiectasis > 5 years had symptomatic improvement. The mean number of daily symptoms decreased by 2.4 (p < 0.0001). Lobectomy resulted in significant symptomatic improvement in 89% of children. However, pre-operative work-up was variable. The study highlights the importance of establishing a protocol for identification of children with bronchiectasis who would benefit from surgery and developing a consistent preparatory approach to ensure optimal and equitable outcomes.

Prospective Evaluation of Adherence to French Guidelines on Early Onset Sepsis Management.

Early onset sepsis (EOS) is rare but potentially lethal. French guidelines were published in 2017 to improve the care of children. The aim of this study was to qualify and quantify inadequate antibiotic therapy management compared with the recommendations made by the new French guidelines. This prospective observational study was conducted at the Robert Debré Hospital (Paris, France), a tertiary pediatric teaching center. Data were prospectively collected between May and October 2019. We included all neonates born at a gestational age ≥ 34 weeks who needed emergency transportation by the pediatric intensive care unit transport team during the first 72 hours of life. The primary outcome was the rate of antibiotic therapy initiation (or abstention) that did not respect the new French guidelines. One hundred and seven patients were included. The median gestational age was 39 weeks and 6 days. Median weight was 3355 g. Fifty-eight births (54%) had at least 1 risk factor for EOS. Ninety-seven (91%) infants had at least 1 symptom of EOS, and 76 (71%) were treated with antibiotics. Three newborns (3%) had a documented EOS, and all of them survived. Antibiotic therapy initiation or abstention was adequate for 91% of our cohort (n = 107). Physicians prescribed inadequate biologic tests for 67 (63%) infants. There was a high adherence to the French EOS guidelines for antibiotic therapy initiation or abstention. No death related to EOS occurred in our cohort. Nevertheless, a high proportion of children had undergone inadequate biological tests.

Left atrial strain is impaired in pediatric patients with mitral annular disjunction

Abstract Background Mitral annular disjunction (MAD) is characterized by separation of the posterior mitral valve (MV) annulus and left ventricular (LV) myocardium. MAD is reported to have a strong association with ventricular tachyarrhythmia and sudden cardiac arrest (SCA) though the mechanism is not well understood. Purpose We aimed to study left atrial strain (LAS) in pediatric patients with MAD, compared with healthy controls. We hypothesized that patients with MAD will demonstrate decreased LAS compared to controls, suggestive of impaired ventricular diastolic function. Methods We included consecutive patients &amp;lt;21 years old diagnosed with MAD from January to December 2023 at a tertiary pediatric care center, excluding patients with Marfan or Loeys-Dietz syndrome. All available echocardiographic images were reviewed to determine the earliest presence of MAD, defined as a distance between the LV myocardial crest and posterior MV hinge point at end-systole in the parasternal long axis view ≥2 mm. Demographic and clinical data were collected via retrospective chart review. Patients with MAD were matched by age and sex with healthy controls. We compared LV ejection fraction (EF), degree of mitral regurgitation (MR), biplane LA volume and LAS (reservoir, conduit, and contraction) between groups. Mann-Whitney U and Chi-Square tests were used to compare continuous and nominal variables between groups, respectively. Results We included 26 patients with MAD (81% female) diagnosed at a median age of 13.8 years (Q1-Q3: 10.5-17.1). During a median time of 3.8 years (Q1-Q3: 1.1-11.5), 19% (n=5) patients were diagnosed with SCA due to ventricular tachyarrhythmia and an additional 15% (n=4) patients had ventricular tachyarrhythmia without SCA. Patients with MAD more frequently had MR graded mild or greater (p &amp;lt; 0.001) than controls. LAS was significantly decreased in all phases of the cardiac cycle in patients with MAD, including conduit (-28.4% v -37.7% p = 0.003), contraction (-8.1% v -13.8%, p = 0.004) and reservoir (37.0% v 51.9%, p = &amp;lt; 0.001). Mean MAD distance was higher (5.24 v 3.66 mm, p = 0.004) and LVEF was lower (56% v 62%, p=0.032) in patients with MAD and SCA compared to those without SCA. There was a trend toward impaired LAS-contraction (-4.8% v –10.9%, p = 0.091) for patients with MAD and SCA, but this did not reach significance. Conclusions All phases of LAS are impaired in pediatric patients with MAD compared to age- and sex-matched controls, suggesting an underlying diastolic impairment. Patients with MAD and SCA had lower LVEF, greater MAD distance, and a trend of lower LAS-contraction. Future studies are needed to better understand the true prevalence of MAD in the pediatric population, its natural history, and its association with arrhythmia and other cardiovascular outcomes.

Secondary neoplasms in survivors of pediatric acute lymphoblastic leukemia and lymphoblastic lymphoma: a single-center, retrospective study

BackgroundAcute lymphoblastic leukemia (ALL)-based therapeutic regimens have markedly improved the survival of children with ALL and lymphoblastic lymphoma (LBL). However, survivors are at risk of secondary neoplasms. Few studies on such secondary neoplasms have been conducted outside of Europe and the United States. The aim of this study was to evaluate the incidence of, risk factors for, and outcomes of secondary neoplasms in long-term survivors of ALL and LBL at a tertiary pediatric oncology center in Mie prefecture, Japan.ProcedureWe retrospectively reviewed 188 patients with ALL and LBL who were treated with an ALL-based therapeutic regimen at Mie University Hospital from January 1, 1977 to December 31, 2022 and followed up.ResultsTen patients developed secondary neoplasms, with 10-year and 20-year cumulative incidences of 2.9% [standard error (SE) = 1.5%] and 5.5% (SE = 2.3%), respectively. The median interval between the primary-cancer diagnosis and secondary-neoplasm diagnosis was 18.5 years (range: 7.8–41.7 years). All 10 secondary neoplasms were central nervous system (CNS) tumors (6 meningiomas and 4 high-grade gliomas). Radiotherapy (p = 0.007) and CNS involvement in the primary cancer (p &amp;lt; 0.001) increased the risk of secondary neoplasms among long-term survivors. Gliomas occurred significantly earlier than meningiomas (p = 0.047), and three patients died of secondary neoplasms (all gliomas).ConclusionsAs secondary gliomas occurred earlier than meningiomas and are associated with poor outcomes, physicians should take great pains to minimize their risk to improve long-term survival and quality of life.

Experience of home monitoring of children with complex CHD during the COVID-19 pandemic: lessons learnt.

The COVID-19 pandemic presented unique challenges to global healthcare. Face-to-face outpatient care was dramatically reduced. This study implemented a remote consultation service via a mobile app (Pexip) to monitor patients with major CHD. Study design was quasi-experimental and prospective. Remote consultations were carried out at a tertiary paediatric cardiology centre in Northern Ireland. Children with major CHD aged 0-16years in Northern Ireland. The intervention was a Pexip-enabled remote consultation. Primary outcome measures included the number of attendances to hospital both initiated and avoided via remote consultation. Remote consultations were conducted by doctor and/or cardiac specialist nurse or by specialist nurse alone (52% vs. 48%). In the study, 32 patients enrolled; three were non-responders and a further two excluded. There were 201 remote consultations delivered (mean = 7.4). There were 12 admissions to hospital resulting from the remote consultation; the commonest indication was abnormal oxygen saturations (42%). There were 38 hospital attendances avoided, predominantly related to infant feeding and medication advice (both 42%). A significant number of unnecessary hospital attendances were avoided (n = 38). Remote consultation technology proved a user-friendly and valuable adjunct to the provision of ongoing specialist patient care in challenging circumstances. There was a reduction in parental anxiety, and both parents and clinicians found this initiative beneficial to patient care. There was prompt identification of unwell children on remote consultations.

A qualitative study exploring experiences of treatment in paediatric rheumatology - children’s, young people’s, parents’ and carers’ perspectives

BackgroundThere is limited literature in paediatric rheumatology describing holistic lived experiences of medical treatment from perspectives of children and young people (CYP) and their parents or carers (PC). This is important as it could have implications for adherence. This study aimed to explore treatment experiences of CYP and PC in a paediatric rheumatology service.MethodsParticipants were recruited at a day-case unit for intravenous infusions at a tertiary paediatric rheumatology centre. Joint qualitative semi-structured interviews with CYP and PC were used to collect data. Data were transcribed, quality checked and thematically analysed using NVivo 12.4 to identify findings.ResultsThirty-two participants (15 CYP between the ages of 6 and 16 years, 17 PC) took part in interviews lasting 41 min and 43 s, on average. Participants described experiences using infliximab, followed by tocilizumab and abatacept. Participants experienced a wave, oscillating between positive and negative trajectories. Experiences of medical treatments were described as temporary, eventually changing and leading to treatment changes or cessation. Behaviours were influenced through somatic factors (pain, function), social factors (advice from health professionals, encouragement from friends, family and teachers, practicality of using treatment in relation to school, work and finance) and cognitive factors (fear of needles, fear of specific medications, beliefs about necessity).ConclusionsCollectively, findings demonstrate experiences of medical treatment reflect the nature of many paediatric rheumatology conditions, oscillating between periods of positive and negative trajectories. Somatic, social and cognitive experiences can be positive, when treatment is considered ‘successful’. Negative somatic, social or cognitive experiences led to behaviours such as treatment non-adherence. A limitation of the study is interviews were conducted jointly with CYP and PC, which may have influenced what participants were willing to say in front of one another however this does mean findings relate to both CYP and PC and so could be suitable targets for interventions to improve adherence.

P1188 Real-world quality of life experience of subcutaneous infliximab use in children with Inflammatory Bowel Disease (IBD) at a tertiary paediatric centre

Abstract Background Subcutaneous infliximab (SC-IFX) use offers relative pharmacokinetic stability and efficacy and safety profiles comparable to intravenous-infliximab (IV-IFX) in adult patients with IBD (1-4). Regular hospital admissions for infusions have an impact Quality of Life (QoL), impacting work and school commitments as well as increasing financial burden. Aim to assess tolerability and QoL in patients switched from IV to SC infliximab collected 12 weeks after the switch. Methods A prospectively identified cohort of 33 children with IBD commenced 120mg fortnightly SC-IFX dosing between February to July 2024 in an Australian tertiary paediatric IBD service. QoL and medication tolerability questionnaires (SIAQ and Costs For Patient’s questionnaire) included assessment of tolerability, convenience, satisfaction along with data about travel time and costs were completed at week 12. Assessment of disease activity, inflammatory biomarkers and therapeutic drug monitoring occurred 4 weekly for the first 12 weeks. Results 31(94%) patients completed the 12-week study treatment period. 21 patients (68%) responded to the QOL questionnaires. Satisfaction with SC treatment was 20 (95%) across respondents, only 1 patient reporting ‘somewhat dissatisfied’ experience. 18 (85%) described ease of administration, with 3 (14%) finding injections "not easy". Respondents described financial impacts to be greatest, followed by time-commitment for attendance, loss of school and work time for attendance for infusions. Nearly 2/3 caregivers required formal leave from employment to facilitate infusion attendance, and 1/3 of patients required full day off school. 100% described a definite or probable willingness to continue SC injections beyond the 12-week period. Conclusion We describe the largest cohort of children with IBD prescribed SC-IFX with short-term follow-up. There was high treatment persistence with the drug. QOL data demonstrates high patient satisfaction, with positive impacts on patient quality-of-life with treatment described. Our real-world experience of paediatric SC-IFX use suggests this well tolerated and preferable alternative to IV-IFX dosing in children in with IBD. Further large-cohort and long-term paediatric data is required. References 1)Schreiber S, Ben-Horin S, Leszczyszyn J et al. Randomized Controlled Trial: Subcutaneous vs Intravenous Infliximab CT-P13 Maintenance in Inflammatory Bowel Disease. Gastroenterology 2021; 160: 2340-53. 2)Buisson A, Nachury M, Bazoge M et al. Long-term effectiveness and acceptability of switching from intravenous to subcutaneous infliximab in patients with inflammatory bowel disease treated with intensified doses: The REMSWITCH-LT study. Aliment Pharmacol Ther 2024; 59: 526-34. 3)Gianolio L, Armstrong K, Swann E et al. Effectiveness of Switching to Subcutaneous Infliximab in Pediatric Inflammatory Bowel Disease Patients on Intravenous Maintenance Therapy. J Pediatr Gastroenterol Nutr 2023; 77: 235-9. 4)Gianolio L, Armstrong K, Swann E et al. OC5 Effectiveness and safety of switching to subcutaneous infliximab in paediatric inflammatory bowel disease patients on established intravenous biosimilar infliximab maintenance therapy: real world data from a regional cohort. Frontline Gastroenterology 2024; 15: A4-A.

P0985 Real-world experience of subcutaneous infliximab use in children with inflammatory bowel disease (IBD) at a tertiary paediatric centre

Abstract Background Subcutaneous infliximab (SC-IFX) offers comparable efficacy and safety profiles to intravenous infliximab (IV-IFX) use in patients with inflammatory bowel disease (IBD), with relative pharmacokinetic stability demonstrated 1, 2. Minimal experience with SC-IFX use in paediatric IBD has been described3, 4. We aimed to assess the clinical tolerability of SC-IFX use in children with IBD requiring anti-tumour-necrosis factor (anti-TNF) treatment. Methods A prospectively identified cohort of children with IBD commenced 120mg fortnightly SC-IFX dosing between February to July 2024 in an Australian tertiary paediatric centre. Treatment followed switch from stable intravenous infliximab (IV-IFX) biosimilar maintenance dosing, or as part of induction treatment. Baseline and four-weekly biochemical and clinical disease activity assessments were undertaken over a 12 week period of follow-up. Results Thirty-one of thirty-three patients completed the 12-week period of treatment follow-up. Median age was 15 years (IQR 14-16.5 years) and 76% (25/33) were male. Twenty-seven patients (82%) had a Crohn’s disease diagnosis. SC-IFX was prescribed as part of induction dosing in 18% (6/33). Biochemical parameters are outlined in Table 1. Median infliximab level increased by 10mg/L to 17.6mg/L at week 12, with a statistically significant increase between baseline level and all other timepoints over the follow-up period (Figure 1). Sixteen patients (48%) had a serum infliximab level greater than 20mg/L at 12 week follow-up. There was no statistically significant change in clinical disease activity indices (Paediatric Crohn’s Disease Activity Index [PCDAI] or Paediatric Ulcerative Colitis Activity Index [PUCAI]) assessed across study timepoints. No correlation between patient characteristics, including demographics, immunomodulator use and previous (if any) IV-IFX regimen, and biochemical markers at baseline or week 12 was seen. High tolerability and treatment persistence were observed in our patient group without any safety concerns. Conclusion We describe the largest cohort of children with IBD prescribed SC-IFX with short-term follow-up. Serum infliximab level increased by 10mg/L over the study period, without significant change in faecal calprotectin and clinical disease activity indices. We observed high treatment persistence in our patient group. Our real-world experience suggests paediatric SC-IFX use may be a reasonable alternative to IV-IFX dosing in children in with IBD. Further large-cohort studies and long-term paediatric data is required. References 1)Schreiber S, Ben-Horin S, Leszczyszyn J et al. Randomized Controlled Trial: Subcutaneous vs Intravenous Infliximab CT-P13 Maintenance in Inflammatory Bowel Disease. Gastroenterology 2021; 160: 2340-53. 2)Buisson A, Nachury M, Bazoge M et al. Long-term effectiveness and acceptability of switching from intravenous to subcutaneous infliximab in patients with inflammatory bowel disease treated with intensified doses: The REMSWITCH-LT study. Aliment Pharmacol Ther 2024; 59: 526-34. 3)Gianolio L, Armstrong K, Swann E et al. Effectiveness of Switching to Subcutaneous Infliximab in Pediatric Inflammatory Bowel Disease Patients on Intravenous Maintenance Therapy. J Pediatr Gastroenterol Nutr 2023; 77: 235-9. 4)Gianolio L, Armstrong K, Swann E et al. OC5 Effectiveness and safety of switching to subcutaneous infliximab in paediatric inflammatory bowel disease patients on established intravenous biosimilar infliximab maintenance therapy: real world data from a regional cohort. Frontline Gastroenterology 2024; 15: A4-A.

P1311 Mucosal Microbiome Signatures Associated with Postoperative Endoscopic Recurrence in Paediatric Crohn’s Disease

Abstract Background Following ileocecal resection (ICR) for Crohn’s disease (CD), recurrence is frequent even in the biologic era. Gut microbiota is involved in this setting and a few adult studies have suggested that bacterial recolonization after surgery may predict recurrence. Given the inherent risk of postoperative recurrence associated with age and disease duration, studies investigating the role of the gut microbiota in predicting relapse in children undergoing ICR appears critical. We aimed to identify changes in the mucosal microbiome associated with, and predictive of, endoscopic CD recurrence within the first year post-ICR in a paediatric population. Methods Microbiota composition (16S rDNA sequencing) was assessed from ileal and colonic mucosa at the time of ICR and later during endoscopic evaluation from above and below the surgical anastomosis in 33 patients (26 with paired samples) prospectively recruited at a tertiary care paediatric IBD center. Results The study included 33 children (mean age 16±2.7 years) undergoing ICR. At the time of ileo-colonoscopy, 9 (35%) patients had endoscopic recurrence (Rutgeerts’ score≥ i2). ICR strongly modified the gut mucosal microbiota composition with significant changes in β-diversity (R2= 0.03, PERMANOVA test, p=0.01). Bacterial recolonization post-ICR differed according to recurrence status (Figure 1). Post-operatively, recurrence was associated with an increase in Fusobacterium, Dialister genera and in members of the Lachnospiraceae family, and a decrease in Faecalibacterium, Lactobacillus genera and members of the Streptococcaceae family. Microbiota colonizing the neoterminal ileum and subanastomotic colon differed in children with recurrence and showed, compared to remission, elevated and reduced bacterial abundance of the Enterobacter (q=0.1) and Ruminococcaceae (q=0.05) families in colonic samples respectively while the ileal mucosa exhibited profound depletion in Faecalibacterium (q=0.01). Postoperative colonic mucosal relative abundance of Enterobacteriaceae (q=0.06) and Ruminococcaceae (q=0.06) correlated positively and negatively with length of intestinal resection. At the time of surgery, several bacterial taxa able to predict relapse were identified (Figure 2). Conclusion Predicting postoperative CD relapse remains challenging, although several clinical risk factors of recurrence have been identified. Our study demonstrates that bacterial recolonization following ICR might pave the way towards remission or recurrence. Our results highlight, for the first time in a paediatric population, target species that may help clinicians to better define patients at risk of relapse and allow precise modulations of the overall microbial ecosystem towards remission pattern.

Second Primary Neoplasms in Pediatric Cancer Survivors With Single Institution Experience From Turkey.

This study aims to establish the characteristics of second primary neoplasms (SPNs) and the long-term follow-up status of a tertiary pediatric oncology center. Records of 1799 patients followed up in the pediatric oncology division between January 1981 and December 2022 were evaluated retrospectively. Thirty-four (1.9%) cases of secondary neoplasms were identified throughout 42 years. The 5-year and 10-year cumulative incidence was 1% and 4%, respectively. The 3 most common SPNs were thyroid carcinomas (TC), central nervous system (CNS) tumors, and leukemias. The shortest median latent period of SPN detection was 15.5 (2 to 35) months in secondary leukemias, whereas 8 (0 to 17) years in all SPNs. Secondary solid tumors that occurred within the radiation field were TC and meningiomas with a 5.5 (3 to 12) and 16 (6 to 22) years latency period, respectively. Ten patients died; the median death time from the diagnosis of SPN was 10 months in all secondary leukemias and 3.5 months in CNS tumors. The 5-year overall survival was 91%, with a median follow-up time of 13.1 years in all patients with SPN. Considering the SPN-inducing effects of radiotherapy and chemotherapy, patient-protective improvements in treatment protocols are required. Multidisciplinary and long-term follow-up is essential even in adulthood because of the long latency period of some SPN occurring in pediatric cancer survivors.

Study of Children Aged Under 2 Years Admitted With RSV at Four Australian Hospitals [2021-2022

Primary aim was to review severe acute respiratory infections (SARI) hospitalisations caused by respiratory syncytial virus (RSV) in children aged < 2 years in paediatric hospitals in Australia. Secondary aims included RSV subtyping, assessing RSV seasonality and contributing to the World Health Organisation's RSV surveillance programme. We prospectively reviewed the medical records of children (< 2 years of age) with a confirmed SARI who were admitted to one of four major Australian paediatric hospitals and had a respiratory sample analysed by Polymerase Chain Reaction (PCR). A detailed dataset was completed for RSV positive cases. Between 1 January 2021 and 31 December 2022, 2290 RSV (laboratory-confirmed) admissions were identified (53.4% of all SARI admissions). Approximately 50% of all RSV cases were aged 0-6 months. RSV-A predominated in 2021 with peak infections observed in summer while in 2022 RSV-B predominated with peak infections in the more traditional winter months. The median total length of stay (LOS) for RSV positive admissions was 46 h (IQR: 22-82 h). 9% of these children required an ICU admission with a prolonged median LOS 68 h (IQR: 40-112 h). Respiratory support utilisation was consistent over the 2 years. 1.8% required mechanical ventilation; 4.6% continuous positive airway pressure; 23.3% high flow oxygen; and 50.8% low flow oxygen. RSV in children continues to cause a significant disease burden at Australian tertiary paediatric centres. Ongoing hospital surveillance is required to document the impact of RSV preventative therapies that have become available in 2024.

Clinical and Electromyographic Characteristics of Pediatric Laryngeal Dyskinesia.

To report the clinical and laryngeal electromyographic (LEMG) parameters of children with laryngeal dyskinesia (LD) and its prevalence among laryngeal mobility disorder (LMD) requiring full airway examination. Retrospective uncontrolled study. Tertiary pediatric center. Eligible children (<17 years old) were stridulous patients suspected of LMD (16 years period). We included those diagnosed with LD according to previously described criteria by Denoyelle et al, followed up for at least 3 months, who were assessed endoscopically and LEMG. LEMG scores, and the prevalence of LD in all LMD who required airway exam under anesthesia. clinical resolution, McGill scores, and instrumental swallowing test. A total of 44 patients (<17 years) were identified. Of these, 29 were included (25 boys; median age: 9.83 months [0.42-116.58]). LD constituted 18.58% of all patients during the study period. Asymmetry of movement and LEMG between the 2 sides was noted (the right side was more affected). Seventeen had swallowing difficulties (13 abnormal instrumental tests), and 27 snored (mean McGill score 3). The median follow up was 49.96 months (range: 5.25-243.25). Symptoms improved in all patients, but one, by the last follow-up date. LD is prevalent among LMD and is mostly self-limiting. We noted asymmetry of LEMG grades between the 2 sides of the larynx, male predominance, and functional deficits in sleep and swallowing. The diagnosis requires a complete endoscopic exam with LEMG. Conflating this entity with bilateral laryngeal paralysis has likely impacted the documented epidemiology and natural history.

Utility of Monitoring Fasting Phase During Pediatric Colonic Manometry Studies.

Colonic manometry (CM) is a diagnostic procedure used to evaluate pediatric patients with refractory constipation, fecal incontinence, Hirschsprung disease, and pediatric intestinal pseudo-obstruction. Pan-colonic high-amplitude propagated contractions (HAPCs), measured by CM, reflect an intact neuromuscular function of the colon. Current guidelines recommend starting CM with fasting recording for 1-2 h, but no prior evaluation has determined the diagnostic yield of the fasting phase. We aimed to determine the utility of the fasting phase during CM studies. We evaluated CM studies conducted at a tertiary pediatric center (2018-2022). Fasting phases of normal CM studies were evaluated. In 433 included studies 241 (55.7%) females, median age (9.7 years), the average fasting recording lasted 126 min. A total of 193 (44.6%) studies exhibited fasting HAPCs, with 123 (28.4%) being pan-colonic. The presence of pan-colonic HAPCs was based solely on the fasting phase in 11 (2.5%) studies. Patients with fasting pan-colonic HAPCs were younger (median age of 6.9 vs. 9.8 years, p = 0.0001) and had a higher rate of postprandial HAPCs (69.1% vs. 25.2%, p < 0.0001). Most fasting pan-colonic HAPCs presented during the first 60 min (94/123, 76.4%). All studies demonstrated HAPCs after stimulation with bisacodyl. In analyzing just the initial 30 min of fasting on CM, only 2 (0.5%) studies would have been misclassified as abnormal, with no bisacodyl administration in these studies. Shortening the fasting phase minimally affects next-day CM results and could reduce patient inconvenience, hospital-related costs, and potential side effects.

Neurodevelopmental and Mental Health Outcomes in a National Clinical Sample of Youth With Sex Chromosome Trisomies Compared With Matched Controls.

To compare the prevalence of neurodevelopmental and mental health diagnoses in a national sample of youth with sex chromosome trisomies (SCTs) with matched controls. Patients in PEDSnet and a diagnosis code mapping to 47,XXY/Klinefelter syndrome (n = 1171), 47,XYY/Double Y syndrome (n = 243), or 47,XXX/Trisomy X syndrome (n = 262) were matched with controls using propensity scores. Generalized estimating equations computed odds ratios (OR) with 95% confidence intervals (CI) for the prevalence of diagnoses within the neurodevelopmental and mental health composites, psychotropic medication prescriptions, and encounters with behavioral health and therapy providers. Alpha was set at 0.0025 to account for multiple comparisons. Patients with SCTs had higher odds of diagnoses within the neurodevelopmental (OR 6.3, 95% CI, 5.7-7.2) and mental health composites (OR 2.7, 95% CI, 2.3-3.2) compared with matched controls. All neurodevelopmental diagnoses were more prevalent among all SCT groups compared with controls. Within the mental health composite, only the prevalence of anxiety and mood disorder was higher in all SCT groups. A higher proportion of patients with SCTs had psychotropic prescriptions compared with controls (stimulants 13.1% vs 5.2%, selective serotonin reuptake inhibitors 8.7% vs 2.8%, antipsychotics 6.5% vs 2.4%, p < 0.0001 for all). Overall, 48% of patients with SCTs had a clinical encounter with a behavioral health provider vs 16.6% of controls (OR 5.6, 95% CI, 4.1-5.1). Compared with matched controls, youth with SCTs receiving care at US tertiary care pediatric centers have disproportionately high rates of neurodevelopmental and mental health conditions, emphasizing the need for appropriate screening and intervention in these populations.

Echocardiographic predictors of surgical outcomes in pulmonary atresia with intact ventricular septum and development of a discriminatory calculator: an Australian tertiary institution experience.

Pulmonary atresia with intact ventricular septum is a rare congenital cardiac lesion with significant anatomical heterogeneity. Surgical planning of borderline cases remains challenging and is primarily based on echocardiography. The aim was to identify echocardiographic parameters that correlate with surgical outcome and to develop a discriminatory calculator. Retrospective review of all pulmonary atresia with intact ventricular septum cases at a statewide tertiary paediatric cardiac centre was performed between 2004 and 2020. Demographic, clinical, and echocardiographic data were collected. Logistic regression was used to develop a discriminatory tool for prediction of biventricular repair. Forty patients were included. Overall mortality was 27.5% (n = 11) and confined to patients managed as univentricular (11 vs 0, p = 0.027). Patients who underwent univentricular palliation were more likely to have an associated coronary artery abnormality (17 vs 3, p = 0.001). Fifteen surviving patients (51.7%) achieved biventricular circulation while 14 (48.3%) required one-and-a-half or univentricular palliation. Nineteen patients (47.5%) underwent percutaneous pulmonary valve perforation. No patients without tricuspid regurgitation achieved biventricular repair. The combination of tricuspid valve/mitral valve annulus dimension ratio and right ventricle/left ventricle length ratio identified biventricular management with a sensitivity of 93% and specificity of 96%. An online calculator has been made available. Pulmonary atresia with intact ventricular septum is a challenging condition with significant early and interstage morbidity and mortality risk. Patient outcomes were comparable to internationally reported data. Right ventricle/left ventricle length and tricuspid valve/mitral valve annulus dimension ratios identified a biventricular pathway with a high level of sensitivity and specificity. Absent tricuspid regurgitation was associated with a univentricular outcome.

Improving anesthesia resident wellness: a facilitated peer discussion group evaluated with a pre-/post-intervention survey

Background: Many residents report a second victim response following near-miss events during their pediatric anesthesia rotation with consequences for their mental and physical wellbeing. This study investigated the impact of a Better Resident Wellness (BREW) initiative at our tertiary pediatric centre. Methods: We invited anesthesia residents to complete a survey at the start of their pediatric rotation. Questions included the Second Victim Experience and Support Tool (SVEST). During their rotation, residents attended weekly BREW rounds, a one-hour peer discussion facilitated by a psychologist. They provided feedback in a follow-up survey, including repeat SVEST. Results: 33/48 (69%) invited residents completed pre- and post-surveys Oct/2021-Feb/2023: all had attended one or more BREW rounds; 32/33 (97%) considered BREW helpful, safe, and would recommend to future residents; perceived benefits included improved morale (30/33, 91%) and clinical care (23/33, 70%). SVEST indicated a second victim response for 17/32 (53%) at the start and 7/32 (22%) at the end of their rotation (odds ratio 0.25, 95% CI 0.07 to 0.82, p = 0.019), with reduced professional self-efficacy concerns (median difference -0.25, 95%CI -0.50 to 0, p = 0.029). Conclusion: BREW offers anesthesia residents a desirable and beneficial support resource. Other residency programs should consider integrating facilitated peer discussion into their curriculum.