Psychoeducation for breast cancer: A systematic review and meta-analysis

BackgroundPsychoeducation has emerged as an intervention for women with breast cancer (BC). This meta-analysis evaluated the effectiveness of psychoeducation on adherence to diagnostic procedures and medical treatment, anxiety, depression, quality of life (QoL), and BC knowledge among patients with BC symptoms or diagnosis and BC survivors.MethodsA systematic literature search (in PubMed, Embase, PsycINFO and Cochrane) for randomised controlled trials (RCTs) comparing the effects of psychoeducation to control among patients with BC symptoms or diagnosis and BC survivors. Effects were expressed as relative risks (RRs) and standardized mean differences (SMDs) with their 95% confidence intervals.ResultsTwenty-seven RCTs (7742 participants; 3880 psychoeducation and 3862 controls) were included. Compared with controls, psychoeducation had no significant effect on adherence to diagnostic procedures and medical treatment (RR 1.553; 95% CI 0.733 to 3.290, p = .16), but it significantly decreased anxiety (SMD -0.710, 95% CI -1.395 to −0.027, p = .04) and improved QoL with (SMD 0.509; 95% CI 0.096 to 0.923, p < .01). No effects were found for psychoeducation on depression (SMD -0.243, 95% CI -0.580 to 0.091, p = .14), or BC knowledge (SMD 0.718, 95% CI -0.800 to 2.236, p = .23).ConclusionWe demonstrated that psychoeducation did not improve adherence to diagnostic procedures and treatment, depression and BC knowledge but was valuable for reducing anxiety and improving QoL. Future studies may explore the effectiveness of psychoeducation in promoting adherence across various types of cancer.     

Mother,father and child traumatic stress reactions after paediatric burn: Within-family co-occurrence and parent-child discrepancies in appraisals of child stress

Aim

The current study examined occurrence and within-family associations of traumatic stress reactions after child burn injury, while in the same model addressing the role of parents’ own symptoms in their reports of child symptoms.

Association between Kasai portoenterostomy at low caseload centres and transplant complications in children with biliary atresia

BackgroundKasai portoenterostomy (KPE) is the preferred treatment for biliary atresia (BA) patients. It has been shown that the center caseload of KPE impacts on native liver survival. We aimed to define the impact of KPE caseload on complications at the time of liver transplantation (LT).MethodsRetrospective data collection of LT for BA performed in our tertiary center between 2010 and 2018. The patients were grouped according to the caseload of the center that performed KPE: Group A (≥5 KPE/year) and Group B (<5 KPE/year). We analyzed total transplant time (TTT), hepatectomy time, amount of plasma and red blood cell (RBC) transfusions, occurrence of bowel perforations at LT.ResultsAmong 115 patients, Group A (n 44) and Group B (n 71) were comparable for age, sex, PELD score, TTT. The groups differed for: median hepatectomy time (57 min, IQR = 50–67; vs 65, IQR 55–89, p = 0.045); RBC transfusions (95 ml, IQR 0–250; vs 200 ml, IQR 70–500, p = 0.017); bowel perforations (0/44 vs 15/71, p = 0.001). One-year graft loss in Group A vs Group B was 1/44 vs 7/71 (p = 0.239), whereas deaths were 0/44 vs 5/71 respectively (p = 0.183); 5/15 patients who had a perforation eventually lost the graft.ConclusionsThis study found an association between KPE performed in low caseload center and the incidence of complications at LT. These patients tend to have a worse outcome. The centralization of KPE to referral center represents an advantage at the time of LT.Mini abstractWe studied the impact of Kasai portoenterostomy (KPE) caseload on complications at the time of liver transplantation (LT), in 115 patients. We found an association between KPE performed in low caseload center and increased bowel perforations and blood transfusions. We suggest to centralize to experienced center all children requiring KPE.     

Performance anxiety amongst trauma and orthopaedic surgical trainees

BackgroundThis study aims to assess performance anxiety amongst orthopaedic trainees. Operating is equivalent in its skill level, both dexterous and mental, to that of elite sport. This study uses a slightly altered version of the validated Sports Competition Anxiety Test (SCAT), making it relevant to operating, rather than to sport, to evaluate peri-operative stress and anxiety in orthopaedic surgeons.MethodsThe SCAT questionnaire was sent to trainees across 3 UK. deaneries via email. A score of <17 suggests low-levels of anxiety, 17–24 suggests medium-levels of anxiety and >24 suggest high-levels of anxiety. Data was anonymised except from training grade and sex.Results109 of 273 (40%) responded to the survey, 71% of respondents were male (n = 77). The mean SCAT score amongst Core Surgical Trainees was 16.9 (n = 21, range 12–23), Specialist Trainees years 3–5 was 18.7 (n = 51, range 12–28), Specialist Trainees years 6–8 was 16.8 (n = 26, range 11–24) and consultants was 16 (n = 11, range 11–28). Across all groups, when sub-divided by sex, females had higher mean scores, this was statistically significant for Specialist Trainees years 3–5 (p = 0.029) and Specialist Trainees years 6–8 (p = 0.042) groups.DiscussionSurgical performance anxiety exists amongst orthopaedic surgeons, with females scoring higher than males. Five respondents scored “high-levels of anxiety” including 2 consultants level surgeons, suggesting experience does not entirely eliminate anxiety. Stress and anxiety are known to lead to surgical mistakes and “burnout” amongst surgeons which is highly topical at present. It is important to highlight this significant issue and it could be taken forward as a national survey to evaluate further.     

Sleep quality and depression–anxiety in mothers of children with two chronic respiratory diseases: Asthma and cystic fibrosis

BackgroundSleep quality and psychological well being of parents are expected to be influenced by the child's health and disease status. The aim of this study was to compare sleep quality and depression–anxiety parameters in mothers of children with cystic fibrosis (CF) asthma and healthy controls.MethodsThe study included mothers of 62 children with asthma, 21 children with CF and 35 healthy children. All mothers filled in the Pittsburgh Sleep Quality Index (PSQI) questionnaire and hospital anxiety depression scale (HADS).ResultsComparison of the three groups with Kruskall Wallis analysis demonstrated that subjective sleep, sleep efficiency and total PSQI scores were significantly different between the groups (p = 0.02, p = 0.01 and p = 0.04 respectively). Comparisons of the groups in pairs with Mann Whitney U test with Bonferroni correction revealed that subjective sleep quality scores in mothers of children with asthma were significantly higher than the ones in the control group (1.0 ± 0.9 vs 0.6 ± 0.7, p = 0.015). The other PSQI scores as well as the anxiety and depression scores were higher in CF and asthma groups when compared to the control group but did not reach statistical significance. Anxiety and depression scores were significantly correlated with PSQI total score in CF (rho = 0.54 and 0.49 respectively) and asthma groups (rho = 0.45 and 0.60 respectively) but not in the control group.ConclusionIn conclusion, presence of a chronic respiratory disease in a child may be associated with disturbed sleep quality and increased depression and anxiety in mothers.     

Clinical implications of serum autotoxin in regular follow up after pediatric living donor liver transplantation for biliary atresia

Background: Pediatric patients sometimes develop graft fibrosis after living donor liver transplant (LDLT). Autotaxin is a recently developed serum marker for hepatic fibrosis. We studied the relationship between serum autotaxin levels and histological findings in patients after LDLT for biliary atresia (BA). Methods: Information on patients aged <19 years who received LDLT for BA and were followed for at least 1 year after LDLT was gathered. Autotaxin levels were compared with pathological fibrosis scores. Results: The study included 52 patients, of whom 4 patients had no fibrosis (F0), 36 patients had F1 fibrosis, and 12 patients had F2. The median serum autotaxin level was 0.89 mg/L. In patients with portal vein (PV) complications such as stenosis or thrombosis (n = 7), the mean autotoxin level was 1.25 mg/L compared with 0.95 mg/L in patients without PV complications (p = 0.004). Among patients without PV complications, the mean autotaxin level was 0.90, 0.88, and 1.18 mg/L in F0, F1, and F2 fibrosis, respectively. The mean autotaxin was higher in F2 fibrosis than in F0 or F1 fibrosis (p<0.05). Autotoxin had a high area under the curve (0.86) with the cut-off level of 0.897 mg/L. Conclusion: Serum autotaxin is a novel marker for liver fibrosis in patients after pediatric LDLT for BA.Type of study: Study of Diagnostic Test.Level of evidence: Level II.     

Long-term surgical and patient-reported outcomes of Hirschsprung Disease

BackgroundInformation is needed regarding the complex relationships between long-term functional outcomes and health-related quality of life (HRQoL) in Hirschsprung's Disease (HSCR). We describe long-term outcomes across multiple domains, completing a core outcome set through to adulthood.MethodsHSCR patients operated at a single center over a 35-year period (1978–2013) were studied. Patients completed detailed questionnaires on bowel and urologic function, and HRQOL. Patients with learning disability (LD) were excluded. Outcomes were compared to normative data. Data are reported as median [IQR] or mean (SD).Results186 patients (median age 28 [18–32] years; 135 males) completed surveys. Bowel function was reduced (BFS 17 [14–19] vs. 19 [19–20], p < 0•0001;$\eta$² = 0•22). Prevalence and severity of fecal soiling and fecal awareness improved with age (p < 0•05 for both). Urinary incontinence was more frequent than controls, most of all in 13–26y females (65% vs. 31%,p = 0•003). In adults, this correlated independently with constipation symptoms (OR 3.18 [1.4–7.5],p = 0.008). HRQoL outcomes strongly correlated with functional outcome: 42% of children demonstrated clinically significant reductions in overall PedsQL score, and poor bowel outcome was strongly associated with impaired QOL (B = 22•7 [12•7–32•7],p < 0•001). In adults, GIQLI scores were more often impacted in patients with extended segment disease. SF-36 scores were reduced relative to population level data in most domains, with large effect sizes noted for females in General Health (g = 1.19) and Social Wellbeing (g = 0.8).ConclusionFunctional impairment is common after pull-through, but bowel function improves with age. Clustering of poor functional outcomes across multiple domains identifies a need for early recognition and long-term support for these patients.     

Brain natriuretic peptide as a cardiac marker of transient radiotherapy-related damage in left-sided breast cancer patients: A prospective study

PurposeOur study evaluated brain natriuretic peptide (BNP) changes over time after adjuvant radiotherapy (RT) in women with left-sided breast cancer investigating its correlation with heart dosimetric parameters.MethodsForty-three patients underwent clinical cardiac examination, electrocardiogram (ECG), echocardiography and BNP measurement before RT (T0) and 1 (T1), 6 (T6) and 12 months (T12) after. After T12 cardiac assessment was performed annually in each patient. Mean values and standard deviation (SD) of BNP, left ventricular ejection fraction (LVEF), V20, V25, V30, V45 and mean dose were calculated. Normalized BNP (BNPn) was calculated as follows: BNPnT1 = BNPT1/BNPT0, BNPnT6 = BNPT6/BNPT0, BNPnT12 = BNPT12/BNPT0. Absolute BNP and BNPn values were used for data analysis.ResultsMedian follow-up from the end of RT to the last check-up was 87 months (range 37–120 months). Minimum follow-up was 74 months except for two patients, who died at respectively 37 and 47 months after RT. In all patients LVEF did not change significantly (p = 0.22) after RT. BNP increased significantly (p < 0.001), particularly 1 and 6 months after RT. It slightly decreased after 12 months. BNP did not correlate with V20, V25, V30, V45, mean dose and MHD. All BNPn correlated significantly (p < 0.05) with V20, V25, V30, V45, mean dose and MHD. Four patients had a cardiac event; in the only subject who developed myocardial infarction, V20, V25, V30 and V45 were the highest and BNP increased from T1 and persisted high even at T12.ConclusionOur results confirm that BNP could be a useful minimally invasive marker of early RT related cardiac impairment.     

Lessons learned from lower urinary tract complications of anorectoplasty for imperforate anus with rectourethral/rectovesical fistula: Laparoscopy-assisted versus posterior sagittal approaches

PurposeTo report the sequelae of and preventive strategies for selected lower urinary tract (LUT) complications, i.e., posterior urethral diverticulum (PUD), intraoperative LUT injuries, postoperative dysuria, and fistula recurrence in male imperforate anus (IA) with rectourethral/rectovesical (RU/RV) fistula after laparoscopy-assisted anorectoplasty (LAARP) or posterior sagittal anorectoplasty (PSARP).Methods153 boys with IA and RU/RV fistula treated 1986–2019 by LAARP (n = 56) or PSARP (n = 97) at two unrelated institutes were studied retrospectively.ResultsAfter mean follow-up of 17.0 years (range: 36.5 days-32.0 years), the overall incidences of LUT complications were: LAARP (6/56; 10.7%); PSARP (7/97; 7.2%); p = 0.55, comprising PUD: LAARP (n = 5), PSARP (n = 0); p = 0.006; injuries: LAARP (n = 0), PSARP (n = 5); p = 0.16; dysuria: LAARP (n = 1), PSARP (n = 1); p>0.999; and recurrence: LAARP (n = 0), PSARP (n = 1); p>0.999. Mean onset of PUD was 5.1 years (range: 1.0–15.1 years). Treatment: PUD: surgery (n = 2/5), conservative (n = 3/5); injuries: intraoperative repair (n = 5/5); dysuria: conservative (n = 2/2), and recurrence: redo PSARP (n = 1/1).ConclusionsStrategies devised to improve dissection accuracy resolved the specific technical issues causing LUT complications (remnant RU fistula dissection in LAARP and blind posterior access in PSARP). Currently, the incidence of new cases of PUD and LUT injuries is zero.Level of Evidence: Level III     

Socioeconomic and clinical factors associated with prolonged hospital length of stay after traumatic brain injury

BackgroundHospital length of stay (HLOS) after traumatic brain injury (TBI) is a metric of injury severity, resource utilization, and access to services. This study aimed to evaluate socioeconomic and clinical factors associated with prolonged HLOS after TBI.MethodsRetrospective data from adult hospitalized patients diagnosed with acute TBI at a US Level 1 trauma center between August 1, 2019 - April 1, 2022 were extracted from the electronic health record. HLOS was stratified by Tier (1: 1–74th percentile; 2: 75–84th; 3: 85–94th; 4: 95–99th). Demographic, socioeconomic, injury severity, and level-of-care factors were compared by HLOS. Multivariable logistic regressions evaluated associations between socioeconomic and clinical variables and prolonged HLOS, using multivariable odds ratios (mOR) and [95% confidence intervals]. Estimated daily charges were calculated for a subset of medically-stable inpatients awaiting placement. Statistical significance was assessed at p < 0.05.ResultsIn 1443 patients, median HLOS was 4 days (interquartile range 2–8; range 0–145). HLOS Tiers were 0–7, 8–13, 14–27, and ≥28 days (Tiers 1–4, respectively). Patients with Tier 4 HLOS differed significantly from others, with increased Medicaid insurance (53.4% vs. 30.3–33.1%, p = 0.003), severe TBI (Glasgow Coma Scale 3–8: 38.4% vs. 8.7–18.2%, p < 0.001), younger age (mean 52.3-years vs. 61.1–63.7-years, p = 0.003), low socioeconomic status (53.4% vs. 32.0–33.9%, p = 0.003), and need for post-acute care (60.3% vs. 11.2–39.7%, p < 0.001). Independent factors associated with prolonged (Tier 4) HLOS were Medicaid (mOR = 1.99 [1.08–3.68], vs. Medicare/commercial), moderate and severe TBI (mOR = 3.48 [1.61–7.56]; mOR = 4.43 [2.18–8.99], respectively, vs. mild TBI), and need for post-acute placement (mOR = 10.68 [5.74–19.89], while age was protective (per-year mOR = 0.98 [0.97–0.99]). Estimated daily charges for a medically-stable inpatient was $17126.ConclusionsMedicaid insurance, moderate/severe TBI, and need for post-acute care were independently associated with prolonged HLOS ≥28 days. Medically-stable inpatients awaiting placement accrue immense daily healthcare costs. At-risk patients should be identified early, receive care transitions resources, and be prioritized for discharge coordination pathways.     

Evaluation of atelectasis using electrical impedance tomography during procedural deep sedation for MRI in small children: A prospective observational trial

Study objectiveTo investigate the variation of poorly ventilated lung units (i.e., silent spaces) in children undergoing procedural sedation in a day-hospital setting, until discharge home from the Post-Anesthesia Care Unit (PACU).DesignProspective, single-center, observational cohort trial.SettingThis study was conducted at the radiology department and in PACU at Bern University Hospital (Switzerland), a tertiary care hospital.PatientsWe included 25 children (1–6 years, ASA I-III) scheduled for cerebral magnetic resonance imaging scan, spontaneously breathing under deep sedation. Children planned for tracheal intubation, supraglottic airway insertion, or with contraindication for propofol were excluded.InterventionAfter intravenous or inhaled induction, deep sedation was performed with 10 mg/kg/h Propofol. All children received nasal oxygen 0.3 ml/kg/min.MeasurementsThe proportion of silent spaces and the global inhomogeneity index were determined at each of five procedural points, using electrical impedance tomography: before induction (T1); before (T2) and after (T3) magnetic resonance imaging; at the end of sedation before transport to the PACU (T4); and before hospital discharge (T5).Main resultsThe median [interquartile range (IQR)] proportion of silent spaces at the five analysis points were: T1, 5% [2%–14%]; T2, 10% [7%–14%]; T3, 12% [5%–23%]; T4, 12% [7%–24%]; and T5, 3% [2%–11%]. These defined significant changes in silent spaces over the course of sedation (p = 0.009), but no differences in silent spaces from before induction to before discharge from the PACU (T1 vs. T5; p = 0.29). Median [IQR] global inhomogeneity indices were 0.57 [0.55–0.58], 0.56 [0.53–0.59], 0.56 [0.54–0.59], 0.57 [0.54–0.60] and 0.56 [0.54–0.57], respectively (p = 0.93). None of the children reported anesthesia-related complications.ConclusionDeep sedation results in significantly increased poorly ventilated lung units during sedation. However, this does not significantly affect ventilation homogeneity, which was fully resolved at discharge from the PACU.Trial registration: clinicaltrials.gov, identifier NCT04507581     

Pediatric burn-trauma patients have increased length of stay compared to trauma-only patients: A propensity matched analysis

BackgroundTrauma is the leading cause of mortality in children. Burn injury involves intensive resources, especially in pediatric patients. We hypothesized that among pediatric trauma patients, combined burn-trauma (BT) patients have increased length of stay (LOS) and mortality compared to trauma-only (T) patients.MethodsThe Pediatric Trauma Quality Improvement Program (2014–2016) was queried and BT patients were 1:2 propensity-score-matched to T patients based on age, gender, hypotension on admission, injury type and severity.Results93 BT patients were matched to 186 T patients. There were no differences in matched characteristics. BT patients had a longer median LOS (4 vs 2 days, p < 0.001) with no difference in mortality (1.1% vs 1.1%, p = 1.00), intensive care unit (ICU) LOS (3 vs 3 days, p = 0.55), or complications including decubitus ulcer (0% vs 1.1%, p = 0.32), deep vein thrombosis (0% vs 0.5%, p = 0.48), extremity compartment syndrome (1.1% vs 0%, p = 0.16), and urinary tract infection (1.1% vs 1.1%, p = 1.00).ConclusionPediatric BT patients had twice the LOS compared to a matched group of pediatric T patients. There was no difference between the cohorts in ICU LOS, complications or mortality rate. When evaluating risk-stratified quality metrics such as LOS, concomitant burn injury should be incorporated.     

Outcomes of gastrostomy placement with and without concomitant tracheostomy among ventilator dependent children

IntroductionSimultaneous gastrostomy tube (GT) and tracheostomy placement in young children offers potential benefit in limiting anesthetic exposure, but it is unknown whether combining these procedures introduces additional morbidity. This study compared outcomes after combined GT and tracheostomy placement versus GT placement alone among similar ventilator-dependent patients.MethodsVentilator-dependent children <2-years-old who underwent GT placement alone (MV-GT), simultaneous GT and tracheostomy placement (GT+T), and GT placement alone with a pre-existing tracheostomy (T-GT) were identified using 2012–2018 NSQIP-Pediatric Participant User Files. Multiple logistic regression models were used to compare outcomes while adjusting for other group differences.ResultsAmong 1100 children, 351 underwent MV-GT, 494 GT+T, and 255 T-GT. Major complications occurred in 23.6%, 17.0%, and 14.5% of the respective groups (p = 0.01). Major complications with GT+T were similar to T-GT (adjusted odds ratio [aOR]=1.19, 95%CI:0.78–1.83, p = 0.4) and lower than MV-GT (aOR=0.67, 95%CI:0.47–0.95, p = 0.02). Severe complications including mortality, cardiac arrest, and stroke were similar between the three groups (p = 0.8).ConclusionsChildren <2-years-old undergoing GT+T did not experience higher post-operative complications compared to children undergoing T-GT or MV-GT. Utilizing GT+T to limit anesthetic exposure may be reasonable within this high-risk population.Type of StudyTreatment StudyLevel of EvidenceLevel III     

Influence of the CYP2J2 Gene Polymorphisms on Chronic Obstructive Pulmonary Disease Risk in the Chinese Han Population

IntroductionCytochrome P450 (CYP) 2J2 is a major enzyme that controls epoxyeicosatrienoic acids biosynthesis, which may play a role in chronic obstructive pulmonary disease (COPD) development. In this study, we aimed to assess the influence of CYP2J2 polymorphisms with COPD susceptibility.Material and methodsA case–control study enrolled 313 COPD cases and 508 controls was to investigate the association between CYP2J2 polymorphisms and COPD risk. Agena MassARRAY platform was used to genotype CYP2J2 polymorphisms. Odds ratios (OR) and 95% confidence intervals (CI) were calculated to evaluate the association between CYP2J2 polymorphisms and COPD risk.ResultsWe observed rs11207535 (homozygote: OR = 0.08, 95%CI = 0.01–0.96, p = 0.047; recessive: OR = 0.08, 95%CI = 0.01–0.94, p = 0.044), rs10889159 (homozygote: OR = 0.08, 95%CI = 0.01–0.92, p = 0.043; recessive: OR = 0.08, 95%CI = 0.01–0.90, p = 0.040) and rs1155002 (heterozygote: OR = 1.63, 95%CI = 1.13–2.36, p = 0.009; dominant: OR = 1.64, 95%CI = 1.15–2.35, p = 0.006; additive: OR = 1.45, 95%CI = 1.09–1.92, p = 0.011) were significantly associated with COPD risk. Allelic tests showed T allele of rs2280274 was related to a decreased risk of COPD and T allele of rs1155002 was associated with an increased COPD risk. Stratified analyses indicated the effects of CYP2J2 polymorphisms and COPD risk were dependent on gender and smoking status (p < 0.05). Additionally, two haplotypes (A_rs11207535C_rs10889159T_rs1155002 and A_rs11207535C_rs10889159C_rs1155002) significantly decreased COPD risk.ConclusionIt suggested CYP2J2 polymorphisms were associated with COPD susceptibility in the Chinese Han population.     

A National Registry Analysis of the Association of Perioperative Regional Anesthesia with hospital length of stay following open reduction and internal fixation of the ankle

Study objectivesTo evaluate if using regional anesthesia for post-operative pain control for patients who underwent ankle ORIF is associated with a decrease in length of stay.DesignMulticenter retrospective cohort study.SettingInpatient perioperative.Patients12,468 inpatients (2007 to 2016) who received ankle ORIF with and without regional anesthesia for pain control.InterventionsRegional Anesthesia for postoperative pain control.MeasurementsHospital length of stay along with multiple covariates.Main resultsThe median [quartiles] hospital length of stay of the non-regional anesthesia and regional anesthesia cohorts were 1 day [0, 2 days] and 0 days [0, 1 day], respectively (p < 0.0001). On multivariable Cox regression analysis, the use of regional anesthesia was associated with decreased time to hospital discharge (HR 1.09, 95% CI 1.03–1.14, p = 0.002). Using 1:2 propensity score matching, the median [quartiles] hospital length of stay between the non-regional anesthesia and regional anesthesia matched cohorts were 0 days [0, 1 day] (range = 0–56 days) and 0 day [0, 1 day] (range = 0–33 days), respectively (p = 0.013).ConclusionThe use of regional anesthesia for post-operative pain control was associated with a decreased length of stay for patients undergoing ankle ORIF.     

A beautiful struggle: Parent-perceived impact of short bowel syndrome on child and family wellbeing

BackgroundDespite considerable improvements in outcomes for children with short bowel syndrome (SBS), many clinicians remain pessimistic about long-term quality of life (QoL) for this population.MethodsThe validated FaMM tool was used to measure parent-perceived impact of the child's condition on child and family life. Partnered disease-specific survey questions relevant to child's overall wellbeing and family function were additionally completed and reported. The cross-sectional surveys were distributed to a convenience sample of parents of children with SBS. Child and family wellbeing were described and compared across child age group and involvement of an intestinal rehabilitation program (IRP). Multivariate regression analyses investigated associations between outcomes and IRP management. Open-ended responses were analyzed to investigate perceived impact of the child's SBS on the parent.ResultsSeventeen parents completed both surveys; 71% perceived child QoL as higher today than what they had originally been told to expect. Child daily life and family difficulty scores suggest parents perceived both to be fairly “normal”. While acknowledging effort invested in condition management, parents perceived high competence in managing their child's condition; 56% perceived personal growth resulting from their child's SBS journey. IRP management was associated with better child daily life (4.11, p = 0.015), family difficulty (-4.85, p = 0.048), and family management ability (4.28, p = 0.014) scores.ConclusionsMany parents perceive child and family life with SBS to be fairly “normal”, manage their child's care with great competence, and report personal growth because of their child's SBS journey. Additional research inclusive of diverse patient and parent backgrounds is warranted.Level of evidence: prognosis study; Level IV     

Association between maternal labour epidural analgesia and autistic traits in offspring

Study objectiveStudies investigating associations between maternal epidural analgesia (MEA) and autism spectrum disorder (ASD) in the offspring are conflicting and lack prospective neurobehavioral follow-up assessments for autistic traits. We aim to prospectively investigate associations between MEA and autistic traits in the offspring.DesignProspective neurobehavioral observational cohort study.SettingSingaporean tertiary healthcare institutions.PatientsParticipants recruited were singleton non-IVF children, >36 weeks gestation, delivered via normal vaginal delivery by mothers >18 years of age, delivered in Singapore from June 2009–September 2010 and followed up over 7 years.InterventionsExposure to maternal epidural analgesia during delivery.MeasurementsThe primary outcome is an abnormal Social Responsiveness Scale (SRS) T score at 7 years (≥60 points). Secondary outcomes include the diagnosis of ASD and abnormal scores for autistic traits assessed via a neurobehavioral battery comprising: CBCL (child behavioural checklist), Q-CHAT (Quantitative Checklist for Autism in Toddlers), and Bayley-III. Multivariable analyses adjusting for maternal and offspring characteristics were performed.Main results704 out of 769 mother-child dyads recruited fulfilled the criteria for analysis. 365/704 mothers received MEA.The incidence of an abnormal SRS score at 7 years in offspring exposed to MEA was 19.9%, and 26.1% in non-exposed offspring (p = 0.154). Multivariable analysis did not demonstrate a significant association between MEA and abnormal SRS scores at 7 years (O.R.0.726, 95% C·I. 0.394–1.34, p = 0.305). After adjustment for maternal and fetal demographics, exposure to MEA was not significantly associated with an abnormal screen in all other tests for autistic traits.The clinical incidence of ASD was 1.76% in children without exposure to MEA, and 2.32% in children with MEA exposure (p = 0.506).ConclusionsMEA is not significantly associated with the development of ASD and autistic traits in offspring, assessed over 7 years. Results should be taken into perspective given our wide confidence intervals and small cohort size.     

Oesophageal atresia with very low birth weight: Clinical characteristics and long-term outcome

Aim of the StudyAn assessment of the clinical data and outcome of patients with oesophageal atresia (OA) with very low birth weight (VLBW) was completed.MethodWith ethical consent, we reviewed the records of 327 successive patients with OA from 1980 to 2020. Main outcome measures survival and oesophageal repair were compared between patients with VLBW(≤1500 g) and with BW>1500 g.ResultsThirty-four (10%) patients had VLBW. Gross types of OA in VLBW were similar as in other patients: A (15%/7%), B (3%/3%), C (78%/82%), D (3%/4%), E (0%/7%), F (0%/1%) (p = 0.16–0.99). In VLBW the incidence of congenital heart disease (CHD) (47%) and trisomy 13/18 and Cri du Chat (15%) were higher than in BW>1500 g (23% and 1%), (p = 0.001 both). In VLBW one-month mortality was 35% vs 4% in patients with BW>1500 g (p < 0.001), overall mortality 56% and 8% (p < 0.001), respectively. Cause of one-month mortality in VLBW (12 patients) were CHD w/wo chromosomal abnormality (n = 7), cerebral hemorrhage (n = 2), gastric perforation (n = 1), anastomotic leakage (n = 1) and pulmonary hemorrhage (n = 1). Of VLBW patients 79% and of other patients 99% underwent oesophageal repair (p < 0.001). Repair in VLBW patients included early (n = 18) or delayed (n = 5) end-to-end anastomosis and reconstruction (n = 4). Anastomotic complications occurred in 24% of patients with VLBW and in 17% with BW>1500 g, (p = 0.31). From 1980–2000 to 2001–2020 survival in VLBW changed from 11% to 81% (p = 0.002). During 2001 – 2020 all VLBW patients underwent repair.ConclusionOA with VLBW had high incidence of life-threatening associated anomalies and decreased survival. Recently survival and rate of oesophageal repair have improved significantly.     

Urine sodium to urine creatinine ratio as a marker of total body sodium in infants with intestinal failure

PurposeUrine sodium (UNa) is a measure of total body sodium in infants with intestinal failure (IF) but can be misleading as it does not reflect volume status. Urine sodium to urine creatinine ratio (UNa:UCr) may offer a more accurate measure, but is not routinely used. This study compares UNa:UCr to UNa as a maker of sodium status in infants with IF.MethodsA retrospective review of infants with IF, from a single center, from 2018 to 2020 was conducted (REB H20–00,816). IF etiology, intestinal anatomy, nutritional intake, urine electrolytes and anthropometrics were collected. Linear mixed effects models adjusting for repeated measures were used to associate UNa and UNa:UCr with weight gain and sodium intake.ResultsTwenty-two infants with a median gestational age of 31 weeks were included. IF etiology included gastroschisis (41%), necrotizing enterocolitis (23%), and intestinal perforation (14%). Infants had an average of 3 paired UNa and UNa:UCr measures for a total of 74 paired measurements. UNa:UCr more strongly correlated with sodium intake compared to UNa (R = 0.25, p = 0.032 vs. R = 0.10, p = 0.38). Overall, neither UNa (p = 0.21) nor UNa:UCr (p = 0.16) were significantly correlated with weight gain. However, for infants receiving ≤50% nutrition enterally, weight gain correlated with UNa (p = 0.01) and UNa:UCr (p = 0.01). UNa:UCr >35 predicted adequate growth regardless of enteral intake (92% sensitivity, 59% specificity).ConclusionUNa:UCr is a measure of total body sodium that correlates with sodium intake in infants with IF. Our study indicates UNa:UCr >35 is associated with adequate growth and can be used to guide further validation studies.     

Impact of Molecular Subtyping and Immune Infiltration on Pathological Response and Outcome Following Neoadjuvant Pembrolizumab in Muscle-invasive Bladder Cancer

BackgroundThe PURE-01 study (NCT02736266) evaluated the use of pembrolizumab before radical cystectomy (RC) in muscle-invasive bladder cancer (MIBC).ObjectiveTo evaluate the ability of molecular signatures to predict the pathological complete response (CR: ypT0N0) and progression-free survival (PFS) after pembrolizumab and RC.Design, setting, and participantsWe analyzed the expression data from patients with T2–4aN0M0 MIBC enrolled in the PURE-01 study (N = 84) and from patients of a retrospective multicenter cohort treated with cisplatin-based neoadjuvant chemotherapy (NAC; N = 140).InterventionNeoadjuvant pembrolizumab or NAC and RC.Outcome measurements and statistical analysisImmune signatures and molecular subtyping (The Cancer Genome Atlas, consensus model, and genomic subtyping classifier [GSC]) were evaluated in relation to CR and PFS. Multivariable logistic regression analyses for CR were used, adjusting for gender and clinical T stage.Results and limitationsThe Immune190 signature was significant for CR on multivariable logistic regression analyses (p =  0.02) in PURE-01, but not in the NAC cohort (p =  0.7). Hallmark signatures for interferon gamma (IFNγ; p =  0.004) and IFNα response (p =  0.006) were also associated with CR for PURE-01, but not for NAC (IFNγ: p =  0.9 and IFNα: p =  0.8). In PURE-01, 93% of patients with the highest Immune190 scores (>1st quartile) had 2-yr PFS versus 79% of those with lower scores; no difference was observed in NAC patients, as well as for the other hallmarks in both groups. The neuroendocrine-like subtype had the worst 2-yr PFS in all three subtyping models (33%) and the GSC claudin-low subtype had the best, with no recurrences in 2 yr. Basal subtypes (across classifications) with higher Immune190 scores showed 100% 2-yr PFS after pembrolizumab therapy (p = 0.04, compared with basal-Immune190 low). Statistical analyses are limited by the small number of events and short follow-up.ConclusionsHigher RNA-based immune signature scores were significantly associated with CR and numerically improved PFS outcomes after pembrolizumab, but not after NAC. These data emphasize that RNA profiling is a potential tool for personalizing neoadjuvant therapy selection.Patient summaryWe used gene expression profiling to evaluate the association between immune gene expression and response to neoadjuvant immunotherapy, compared with standard chemotherapy, in patients with muscle-invasive bladder cancer (MIBC). We found a significant association between immune gene expression and response to pembrolizumab, but not chemotherapy. We conclude that gene expression profiling has the potential to guide personalized neoadjuvant therapy in MIBC.