Pediatric Quality Research Articles

Resuscitation arterial waveform quantification and outcomes in pediatric bidirectional Glenn and Fontan patients.

Resuscitation with chest compressions and positive pressure ventilation in Bidirectional Glenn (BDG) or Fontan physiology may compromise passive venous return and accentuate neurologic injury. We hypothesized that arterial pressure and survival would be better in BDG than Fontan patients. Secondary analyses of the Pediatric Intensive Care Quality of CPR and Improving Outcomes from Pediatric Cardiac Arrest databases. P-values were considered significant if < 0.05. In total, 64 patients had either BDG (42/64, 66%) or Fontan (22/64, 34%) anatomy. Return of spontaneous circulation was achieved in 76% of BDG patients versus 59% of Fontan patients and survival with favorable neurologic outcome in 22/42 (52%) BDG versus 6/22 (27%) Fontan patients, p = 0.067. Twelve of 24 (50%) BDG and 2/7 (29%) Fontan patients who survived to discharge suffered new morbidity as defined by worsening Functional Status Score. More BDG patients achieved adequate DBP (≥25 mmHg for neonates and infants; ≥ 30 mmHg for children) than Fontan patients (21/23 (91%) vs. 5/11 (46%), p = 0.007). Only 27% of Fontan patients survived to hospital discharge with favorable neurologic outcome after CPR, likely driven by inadequate diastolic blood pressure during resuscitation. One half of the BDG patients who survived to hospital discharge had new neurologic morbidity. Cardiac arrest in patients with congenital heart disease (CHD) may present challenges to resuscitation based on the unique cardiovascular physiology resulting from surgical palliation. Recent resuscitation guidelines for CHD patients highlight the lack of data surrounding these special patient populations.1 Univentricular heart disease is palliated by a series of cardiac surgeries that stepwise result in passive pulmonary perfusion from the systemic venous system directly to the pulmonary vascular bed. The bidirectional Glenn (BDG) palliation directly anastomoses the superior vena cava (SVC) to the pulmonary arterial system and leaves normal inferior vena cava (IVC) venous return to the heart.2 The Fontan palliation baffles IVC flow directly to the pulmonary vascular bed which relieves cyanosis due to right to left shunting, but requires systemic ventricular preload to be directly dependent upon pulmonary vascular resistance and intrathoracic pressures.3 IMPACT STATEMENT: Hemodynamic waveforms from 2 large prospective observational studies now allow for exploration of physiology during cardiopulmonary resuscitation for unique anatomy associated with single ventricle congenital heart disease. Fewer patients with Fontan physiology (46%) achieved an adequate diastolic blood pressure (defined as ≥ 25 mmHg for neonates and infants and ≥ 30 mmHg for children) than bidirectional Glenn patients during cardiopulmonary resuscitation (91%, p = 0.007). Only 27% of Fontan patients survived to hospital discharge with favorable neurologic outcome after cardiopulmonary resuscitation. Of the bidirectional Glenn patients who survived, 50% developed a new morbidity as quantified by the Functional Status Score.

Translation and Validation of the Arabic Version of the Cerebral Palsy-Quality of Life Child Self-Reported Questionnaire Among Children in the Saudi Arabian Population.

This study aimed to assess the psychometric properties of the Cerebral Palsy-Quality of Life Child Self-Reported (CP-QoL Child Self-Report) questionnaire after it was translated and culturally adapted into Arabic. This is a cross-sectional study that was conducted during the period from February 2021 to June 2023 in Sultan Bin Abdulaziz Humanitarian City (SBAHC) and the Children with Disability Association in Riyadh, Saudi Arabia, on children with cerebral palsy (CP) between the ages of nine to 12 years. After translation and cross-cultural adaptation, the questionnaire was administered to 65 children. The reliability and internal consistency of the tool were assessed using the intraclass correlation coefficient (ICC) and Cronbach's alpha. The validity was analyzed through the association between the CP-QoL Child Self-Report questionnaire and the Arabic version of the Pediatric Quality of Life Inventory (PedsQL) 3.0-CP module (Mapi Research Trust, Lyon, France) using Pearson's correlation coefficient. The overall internal consistency was 0.870, where it ranged from 0.616 for the pain and impact of disability domain to 0.748 for the social well-being and acceptance domain. The interobserver (test-retest) reliability ranged from 0.540 to 0.779. There was a weak correlation between the domains of the CP-QoL Child Self-Report questionnaire and different domains of PedsQL-CP module. The overall quality of life (QoL) score of the children with CP who participated in the current study was 57.86±4.97. The result of the current study suggests that the English version of the CP-QoL Child Self-Report questionnaire was effectively translated into Arabic and had good psychometric properties in evaluating the QoL of Saudi children with CP aged between nine and 12 years.

Supporting caregivers of children living with disability in a humanitarian context: realist-informed evaluation of the ‘Mighty Children’ programme in Afghanistan

IntroductionTo ensure that humanitarian action is disability-inclusive, evidence is needed to understand how different strategies to support children living with disabilities and their families can work in these settings. Evidence...

Clinical Characteristics of Charcot-Marie-Tooth Disease Type 4J.

Charcot-Marie-Tooth disease type 4J (CMT4J) is caused by autosomal recessive variants in the Factor-Induced Gene 4 (FIG4) gene. Recent preclinical work has demonstrated the feasibility of adeno-associated virus serotype 9-FIG4 gene therapy. This study aimed to further characterize the CMT4J phenotype and evaluate feasibility of validated CMT-related outcome measures for future clinical trials. This cross-sectional study enrolled children and adults with genetically confirmed CMT4J, with 2 documented disease-causing variants in the FIG4 gene. Patients were recruited through the Inherited Neuropathy Consortium network. Disease severity was assessed using standardized CMT-specific outcome measures and exploratory biomarkers including muscle MRI fat fraction, electrophysiology, and neurofilament light chain levels. Descriptive statistics and correlation analyses were conducted to explore relationships between variables. We recruited a total of 19 patients, including 14 pediatric patients (mean age 10.9 ± 3.9 years) and 5 adults (mean age 40.0 ± 13.9 years). The most frequent symptoms were gross motor delay and distal more than proximal muscle weakness, which were observed in 14 of 19 patients. The most common non-neuromuscular symptoms were cognitive and respiratory deficits, each seen in 8 of 19 patients. We denoted asymmetric weakness in 2 patients and nonuniform slowing of conduction velocities in 6 patients. Charcot-Marie-Tooth Disease Pediatric Scale (CMTPedS), Pediatric Quality of Life Inventory, and Vineland Adaptive Behavior Scale scores were affected in most patients. We observed a significant positive correlation between neurofilament light chain levels and CMTPedS, but the study was underpowered to observe a correlation between CMTPedS and MRI fat fraction. We obtained baseline clinical and biomarker data in a broad cohort with CMT4J in pediatric and adult patients. Motor delay, muscle weakness, and respiratory and cognitive difficulties were the most common clinical manifestations of CMT4J. Many patients had nerve conduction studies with nonuniform slowing, and 2 had an asymmetric pattern of muscle weakness. We observed that the neurofilament light chain levels correlated with the CMTPedS in the pediatric population. This study showed feasibility of clinical outcomes including CMTPedS in assessment of disease severity in the pediatric patient population and provided baseline characteristics of exploratory biomarkers, neurofilament light chain levels, and muscle MRI fat fraction. The coronavirus disease 2019 pandemic affected some of the visits, resulting in a reduced number of some of the assessments.

Health-related quality of life and caregiver burden of pediatric patients with inborn errors of metabolism in Japan using EQ-5D-Y, PedsQL, and J-ZBI.

Inborn errors of metabolism (IEM) are known with poor long-term health concerns; however, the health-related quality of life (HRQoL) and the burden placed on families remain unclear. This study investigated the self- and proxy-reported HRQoL of pediatric patients with IEM with or without developmental disabilities and the burden placed on their caregivers. Patients with IEM aged 8-15years and their caregivers were asked to respond to the Pediatric Quality of Life Inventory (PedsQL), EuroQoL five-dimension questionnaire for younger populations (EQ-5D-Y), and Japanese version of the Zarit Caregiver Burden Interview (J-ZBI). We compared EQ-5D-Y scores with matched EQ-5D-Y population norms. Intraclass correlation coefficients (ICC) for self and proxy HRQoL scores of those without developmental disabilities were calculated. Correlation coefficients of HRQoL proxy responses with J-ZBI score were estimated. We included 66 patients with IEM (mean age, 11.5years; males, 41.2%) in the study. The mean (± standard deviation) EQ-5D-Y scores without and with developmental disabilities were 0.957 (± 0.071) and 0.821 (± 0.175), respectively. The EQ-5D-Y scores significantly increased compared with the reference values (p < 0.01, effect size = 0.337). The ICC values were 0.331 and 0.477 for the EQ-5D-Y and PedsQL scores, respectively. HRQoL proxy scores had strong negative correlations with J-ZBI scores. The HRQoL of patients with IEM without developmental disabilities in our study was similar to that of the general Japanese population. The HRQoL of patients with IEM with developmental disabilities was low and associated with a tendency towards an increased burden of care.

Effect of Maternal Knowledge of Asthma Management on Quality of Life and Asthma Control among Children with Asthma: A Cross-Sectional Study.

This study aimed to evaluate the effect of mothers' knowledge about asthma management on quality of life and asthma control among children with asthma in Palestine. This cross-sectional study was carried out by mothers of children with asthma in Palestine in four major public hospitals. Mothers of a total of 220 were selected randomly via a computerized system. Data were collected using Pediatric Asthma Quality of Life (PAQLQ), an Asthma Control Test (ACT), and the mothers' Self-Practices and Knowledge (SPK) of asthma management questionnaire. Statistical analysis was performed using SPSS (V25). A total of 220 mothers of children with asthma were randomly selected and invited to participate but 182 agreed to participate, with a response rate of 83%. The mean age of participant children was 9.7 ± 2.72 years, and the mean age of mothers was 34.5 ± 9.6 years. The mean score of the QoL was 3.91(SD ± 1.61) out of 7. Most participant children had uncontrolled asthma with a total mean score of 14.13(SD ± 5.23) on the ACT. The mean score of mothers' SPK level was 2.12(SD ± 0.83) with a total mean score of 42.13 ± 3.68 out of 68, which indicated a moderate level of knowledge and there was a strong correlation between mothers' SPK and children's QoL. The investigation showed that mothers of children with asthma had moderate SPK, and the children had uncontrolled asthma with poor QoL. These findings suggest developing educational initiatives to enhance parents' asthma-related knowledge and skills to improve their children's asthma-related quality of life and asthma control.

Somatic, emotional, and gastrointestinal symptom severity are increased among children and adolescents with COVID-19.

Post-infectious disorders of gut-brain interaction (PI-DGBI) have significant impact on children and adolescents. The effect of COVID-19 on PI-DGBI-associated symptoms in this population, however, is unknown. We performed electronic medical record searches to identify patients 8-17 years old with a SARS-CoV2 PCR test at Lurie Children's Hospital between November 2020 and March 2021 (cohort 1) and April-October 2021 (cohort 2). Questionnaires were administered to assess symptoms prior to and 3 months following the test. This included the Pediatric Eosinophilic Esophagitis Symptom Score (PEESS), questionnaire of pediatric gastrointestinal symptoms-Rome IV, Nausea Profile (NP), dyspepsia symptom survey (DSS), nausea severity profile (NSP), and Pediatric Quality of Life Inventory (PedsQL). We grouped patients based on the presence of symptoms prior to COVID-19 test or the test result. One hundred and ninety-six parent(s) or guardian(s) in cohort 1 and 274 in cohort 2 completed surveys and self-reported their child's COVID-19 result. Cohort 1 had increased PEESS and DSS scores, lower PedsQL scores, and increased frequency of abdominal pain disorders among patients with symptoms prior to COVID-19 testing. Both cohorts had increased NP and NSP scores among patients with symptoms prior to COVID-19 testing that was highest among patients with a positive COVID-19 test. Abdominal pain and diarrhea prior to COVID-19 testing predicted higher NP scores. Among symptomatic COVID-19 tested children, we found increased severity of nausea-associated somatic, emotional, and gastrointestinal symptoms in the 3 months following the test that was most increased among patients with a positive COVID-19 test.

Rubinstein-Taybi Syndrome Clinical Characteristics from the Perspective of Quality of Life and the Impact of the Disease on Family Functioning.

Background/Objectives: Rubinstein-Taybi Syndrome (RSTS-OMIM, #180849) is a rare genetic disorder associated with distinctive clinical features, including a typical craniofacial appearance, global developmental delay, intellectual disability and broad, angular thumbs and fingers. The main aim of the study was to evaluate the health problems of children with RTST, their quality of life and the impact of the disease on family functioning. In addition, we investigate whether comorbidities, autistic behavior and eating problems affect the children's overall QOL. Methods: A cross-sectional study was performed, including a total of 13 caregivers of children diagnosed with RSTS. A self-reported questionnaire [SRQ], medical records and the Pediatric Impact Module PedsQLTM 2.0, the Pediatric Quality of Life PedsQLTM 4.0 were used to obtain data on QOL and the impact of the disease on family functioning. Results: The overall QOL score for children with RSTS was x = 52.40; SD 13.01. The highest QOL was in emotional functioning (EF; x = 59.23; SD 18.69), while the lowest QOL was in physical functioning (PF; x = 48.56; SD 16.32) and social functioning (SF; x = 48.85; SD 18.84). There was a statistically significant negative correlation (p < 0.03; r = -2.01) between the age of the child and their QOL, indicating that older children had lower QOL scores. The mean overall rating for the impact of RSTS on family functioning was x = 50.00; SD 10.91. Caregivers reported the highest scores for cognitive functioning (CF; x = 64.23; SD 23.70) and family relationships (FR; x = 60.00; SD 17.17). The lowest scores were for daily activities (DA; x = 41.03; SD 17.17) and worry (W; x = 37.69; SD 18.55). Conclusions: This study provides the first comprehensive exploration of the QOL of children with RSTS) and its impact on family functioning.

Demonstrating responsiveness of the pediatric cardiac quality of life inventory in children and adolescents undergoing arrhythmia ablation, heart transplantation, and valve surgery.

Pediatric Cardiac Quality of Life Inventory (PCQLI) is a disease-specific pediatric cardiac health-related quality of life (HRQOL) instrument that is reliable, valid, and generalizable. We aim to demonstrate PCQLI responsiveness in children undergoing arrhythmia ablation, heart transplantation, and valve surgery before and after cardiac intervention. Pediatric cardiac patients 8-18 years of age from 11 centers undergoing arrhythmia ablation, heart transplantation, or valve surgery were enrolled. Patient and parent-proxy PCQLI Total, Disease Impact and Psychosocial Impact subscale scores were assessed pre- and 3-12 months follow-up. Patient clinical status was assessed by a clinician post-procedure and dichotomized into markedly improved/improved and no change/worse/much worse. Paired t-tests examined change over time. We included 195 patient/parent-proxies: 12.6 ± 3.0 years of age; median follow-up time 6.7 (IQR = 5.3-8.2) months; procedural groups - 79 (41%) ablation, 28 (14%) heart transplantation, 88 (45%) valve surgery; clinical status - 164 (84%) markedly improved/improved, 31 (16%) no change/worse/much worse. PCQLI patient and parent-proxies Total scores increased (p ≤ 0.013) in each intervention group. All PCQLI scores were higher (p < 0.001) in the markedly improved/improved group and there were no clinically significant differences in the PCQLI scores in the no difference/worse/much worse group. The PCQLI is responsive in the pediatric cardiac population. Patients with improved clinical status and their parent-proxies reported increased HRQOL after the procedure. Patients with no improvement in clinical status and their parent-proxies reported no change in HRQOL. PCQLI may be used as a patient-reported outcome measure for longitudinal follow-up and interventional trials to assess HRQOL impact from patient and parent-proxy perspectives.

Equity in Pediatric Hospital-Based Safety and Quality Improvement

There are well-documented inequities in the quality of care and health outcomes of minoritized youth. Patient safety and quality improvement (QI) work with an equity focus has been identified as an important strategy to remedy these existing inequities. In this article, we will present evidence of inequities in pediatric hospital-based care, describe root causes with a focus on structural racism, highlight existing frameworks for applying equity principles to patient safety and QI, and provide best practices and recommendations on evaluating patient safety and QI data towards advancing equity in pediatric hospital-based care.

Development and psychometric testing of the Pediatric Atraumatic Care Attitude Scale (PACAS) in pediatric nurses

ObjectiveThe study aimed to develop and validate the Pediatric Atraumatic Care Attitude Scale (PACAS) for pediatric nurses, measuring attitudes toward atraumatic care practices. MethodsThis methodological and correlational study included 336 pediatric nurses from various clinics in Turkey, between January and August 2024. Construct validity was evaluated using exploratory factor analysis (EFA) and confirmatory factor analysis (CFA). Internal consistency was assessed through item-total score correlation, test-retest reliability, and Cronbach's α coefficients. ResultsThe content validity index (CVI) for PACAS items ranged from 0.84 to 1.00. The Kaiser-Meyer-Olkin (KMO) coefficient was 0.978, and Bartlett's test of sphericity was significant (χ2 = 14,110.425, P < .001), supporting factor analysis. EFA revealed a two-factor structure with 31 items, explaining 76.7 % of the variance. The factors, “Knowledge and Awareness” and “Practice and Family Involvement,” had loadings between 0.716 and 0.901 and 0.751–0.962, respectively. CFA confirmed the model fit (χ2/df = 2.77, RMSEA = 0.073, NFI = 0.92, NNFI = 0.97, CFI = 0.94, GFI = 0.91). Cronbach's α was 0.985, with subscale coefficients of 0.978 and 0.983. Test-retest reliability showed strong correlations (r = 0.980, r = 0.985, r = 0.957), with no significant differences between test and retest scores (P > .05). ConclusionsPACAS is a reliable and valid tool for assessing pediatric nurses' attitudes toward atraumatic care, with the potential to enhance pediatric nursing practices by improving adherence to atraumatic care principles. Implications to practicePACAS helps integrate atraumatic care into nursing, enhancing pediatric care quality and outcomes.

Development of a signs and symptoms outcome measure for caregivers of patients with methylmalonic acidemia and propionic acidemia (MMAPAQ)

Background and objectiveMethylmalonic acidemia (MMA) and propionic acidemia (PA) are rare inborn errors of metabolism with shared signs and symptoms that are associated with significant morbidity and mortality. No disease-specific clinical outcomes assessment instruments for MMA and/or PA currently exist to capture the patient perspective in clinical trials. Because patients with these conditions are generally young and have cognitive impairments, an observer-reported outcome (ObsRO) instrument is crucial. We report results from qualitative research supporting development of the Methylmalonic Acidemia and Propionic Acidemia Questionnaire (MMAPAQ), a signs and symptoms ObsRO measure for caregivers of patients with MMA and/or PA. MethodsConcept elicitation (CE) interviews were conducted with 35 participants across 2 studies who were aged ≥18 years and caregivers of patients with a confirmed diagnosis of MMA or PA, and an additional 5 patients aged ≥6 years with MMA or PA in Study 1, to identify core signs/symptoms for inclusion in the MMAPAQ. All interviews were conducted in English. Study 2 included cognitive interviews (CI) with caregivers and clinical experts to further assess content validity. CE and a conceptual framework review were also conducted with clinical experts to further support findings. ResultsA consistent set of signs/symptoms of MMA and PA were reported by eligible caregivers interviewed in study 1 (n = 21) and study 2 (n = 14), representing 11 patients with MMA and 20 with PA. Based on concepts reported in study 1, a draft instrument was constructed and compared with the Pediatric Quality of Life Inventory™ (PedsQL™) and Family Impact module, demonstrating face validity for measuring key signs/symptoms important to patients and caregivers. The PedsQL™ and Family Impact modules were preferred to assess patient and caregiver impacts. Two waves of CE and CIs were conducted in study 2, with wave 1 resulting in removal of 7 items and other revisions to improve clarity, and wave 2 resulting in modification of examples used for 2 items. The final instrument consisted of the following 7 items assessed over the past 7 days using a Likert-type response scale ranging from “never” to “very often”: uncontrollable or involuntary movements, dehydration, rapid breathing at rest, appearing lethargic, appearing disinterested in eating, refusing to eat, and vomiting. ConclusionsThis study establishes the content validity of the MMAPAQ as the first ObsRO questionnaire for measuring core signs and symptoms of MMA and PA in clinical trials and community research. Scoring and psychometric measurement properties of the MMAPAQ will be established in future studies. The PedsQL™ was found to have face validity in measuring concepts that affect the MMA and PA patient populations and should also be considered for use in clinical trials in MMA and PA.

Comparative analysis of health-related quality of life between children with bladder and bowel dysfunction versus lower urinary tract dysfunction and healthy controls.

This study aimed to compare health-related quality of life (HRQoL) between children with bladder and bowel dysfunction (BBD) and lower urinary tract dysfunction (LUTD) alone and healthy controls based on self-report forms and parent-proxy report forms. In this retrospective study, clinical records were reviewed. Children with LUTD, with or without bowel dysfunction, and healthy children were included in this study. The dysfunctional voiding scoring system and Rome IV Diagnostic Criteria were used to assess lower urinary tract symptoms. The Rome IV Diagnostic Criteria was also used to evaluate bowel symptoms. The Pediatric Quality of Life Inventory 4.0 (PedsQL) questionnaire was administered to investigate pediatric HRQoL. Of the total 252 children (mean age, 7.3±2.1 years) who participated in this study, 78 were classified into the BBD group and 174 into the LUTD group. Compared with the control group, the BBD group had significantly lower total PedsQL scores (p<0.001) and psychosocial healthy summary scores (p<0.001). The BBD group had significantly lower emotional functioning scores than the LUTD group (p=0.023). Children with BBD who presented with fecal incontinence (FI) had a significantly lower social functioning score than those without FI (p=0.023). Children with BBD who present with FI are at higher risk of decreased psychosocial HRQoL, and they require special emotional support. These findings underscore the need for effective treatment and follow-up to improve the HRQoL of children with BBD who presented with FI.

The Role and Importance of Quality of Life among Preschool Children as a Prerequisite for a Positively Oriented Upbringing

The aim of this study was to evaluate the health-related quality of life (QoL) in a sample of healthy preschool children in the Republic of Croatia and comment on whether and how the research results open the preconditions for positive individual scenarios of the future related to positively oriented upbringing. The Pediatric quality of life inventory generic core scales (PedsQLTM) was completed by 230 healthy preschool children (5-6 years old) and their parents as a proxy-report. The data was descriptively analysed first. Reliability was assessed by Cronbach’s alpha. The statistically significant difference between the PedsQLTM scales (physical, emotional, social, kindergarten and psychosocial functioning) for preschool children and their parent’s proxy-report were analysed. The PedsQLTM scale score means for children’s self-report ranged 71.32-76.49, with the total score was 74.53. Score means for their parent’s proxy-report scale ranged 73.59-85.46, with the total score of 80.93. All the self-report and proxy-report scales showed satisfactory reliability with Cronbach’s alpha varying between 0.64 and 0.89. Statistically significant difference between scales in self-report and proxy-report was for physical (t=-7.12, p&lt;0.01), social (t=-6.42, p&lt;0.01) and school functioning (t=-2.62, p&lt;0.01) as well as psychosocial (t=-4.82, p&lt;0.01) and total health (t=-6.85, p&lt;0.01). The Croatian PedsQLTM is valid and reliable generic pediatric health-related QoL measurement that can be recommended for children’s self-reports and their parent’s proxy-reports. The results of the research open a precondition for positive individual scenarios of the future related to positively oriented upbringing.

Stigma, seizure self-efficacy, and quality of life in children with epilepsy.

In addition to seizure care, it has been well-established that a holistic approach to the treatment of children with epilepsy that addresses the social, behavioral, and psychological dimensions also benefits their quality of life (QoL). The aim of this study was to investigate the patient and parental perceived stigma, seizure self-efficacy of children with epilepsy, and the relationship with quality of life in terms of sociodemographic and epilepsy-related factors. The study group consisted of children, aged between 8 and 18years and with a diagnosis of epilepsy of at least six months duration and their parents. Pediatric Quality of Life Inventory (PedsQL), Child-Parent Stigma Scales, and the Seizure Self-Efficacy Scale for Children (SSES-C) were used for evaluation. One hundred and forty-four children (mean age 12.6 ± 2.9years) took part, of whom 48.6% were female. The mean Child Stigma Scale score was 1.77 ± 0.82, Parent Stigma Scale score 2.41 ± 0.75, SSES-C score was 3.37 ± 0.98, and PedsQL score was 72.8 ± 18.6 for children self-reports (CsR) and 73.2 ± 18.8 for parent proxy-reports. The intraclass correlation coefficient for parent-child PedsQL indicated a good level of agreement. There was a significant negative correlation between the Child Stigma Scale and the PedsQL-CsR scores. A significant positive correlation was identified between the SSES-C scores and the PedsQL-CsR scores. Perceptions of stigma in children with epilepsy and their parents were high in this study population. Of note, the elevated stigma perception reported by the patients had a detrimental impact on seizure self-efficacy. This relationship may affect the children's QoL and further complicates epilepsy management in this patient group.

Subjective and objective sleep quality assessment in adolescent patients with inflammatory bowel disease

ObjectivesThere is a link between inflammatory bowel disease (IBD) and poor sleep quality that is presumed to be multifactorial. The purpose of this study was to identify factors that impact sleep quality in this group including clinical disease activity, depression, anxiety, quality of life, and disordered social media use through questionnaires. MethodsThis prospective study analyzed sleep quality in adolescent patients ages 13 to 18 with a diagnosis of IBD using objective data from wrist actigraphy and subjective report from the Pittsburgh Sleep Quality Index (PSQI). Additional screeners including the Short Pediatric Crohn's Disease Activity Index or Pediatric Ulcerative Colitis Activity Index, Pediatric Quality of Life Inventory Gastrointestinal Symptoms Module, Generalized Anxiety Disorder 7-item screener, Patient Health Questionnaire depression screener, and Social Media Disorder scale were also collected. ResultsTwenty-three subjects enrolled and 16 completed questionnaires. 62.5 % of participants were in clinical remission and the remaining 37.5 % had mild to severe clinical IBD activity. Poor subjective sleep quality was associated with a shorter duration of sleep time and an increased clinical disease activity score. Patients in clinical remission slept for a longer duration than those with mild IBD activity. ConclusionsThis study did not identify a significant relationship between sleep quality and psychosocial factors in the adolescent population with IBD, though their influence cannot be discounted. There was a connection between the presence of disease activity and shorter sleep duration. Similarly, PSQI scores correlated with sleep time but not sleep quality.

Use of Degludec in Youth With Type 1 Diabetes and Recurrent Diabetic Ketoacidosis

This article describes a pediatric diabetes center quality improvement initiative to switch youth with type 1 diabetes and diabetic ketoacidosis (DKA) from insulin glargine to longer-duration insulin degludec to determine whether this change would reduce DKA recurrence. Overall the change in DKA recurrence with degludec was not statistically significant. However, subgroup analysis showed that race/ethnicity and insurance status were significantly associated with change in DKA rates. The association of degludec and decreased rates of repeat DKA in Hispanics and privately insured individuals require further exploration.

Correlation between Fatigue and Quality of Life in Adolescent Oncology Patients in Jordan

Background Adolescence represents a distinct and multifaceted developmental stage characterized by profound physical and cognitive transformations. Adolescents undergoing cancer treatment frequently have unique needs that remain unmet, leading to a notable decline in their quality of life. Cancer treatment often results in problems like fatigue, which affects patients' quality of life. Assessing quality of life and fatigue among adolescent cancer patients is essential for enhancing nursing care and improving outcomes. Objective This study aimed to investigate the correlation between fatigue and quality of life among adolescent cancer patients in Jordan. Methods A “cross-sectional” study was performed, with the participation of 127 Jordanian adolescent cancer patients, and data was collected through a nameless online survey of Jordanian adolescent cancer patients. The study commenced in February 2024 and concluded in June 2024. Demographic data and responses from two self-reported questionnaires—the Arabic versions of “Pediatric Quality of Life Inventory” “Multidimensional Fatigue Scale” and “Generic Core 4.0”—were collected. Descriptive statistics of means, frequencies, standard deviations, and percentages were used with demographics while inferential statistics were conducted using independent t-tests, Spearman's rank correlation coefficients, and Kruskal-Wallis tests in SPSS, version 26. Results Significant and potent correlations were found between various types of fatigue “general, sleep/rest, cognitive, and total” and quality of life “physical, emotional, social, school, psychosocial health”. Notably, the total fatigue scores showed a potent correlation with the total summary scores (r=0.946), with a p-value significant at the &lt;0.001 level for all correlations, indicating that adolescents with cancer experienced high fatigue and low quality of life. Conclusion The study demonstrated a potent link between fatigue and quality of life. The study's outcomes can help enhance guidelines for routine evaluation of fatigue and quality of life by nurses and healthcare providers in adolescent cancer patients to implement and evaluate interventions aimed at reducing fatigue in patients, highlighting fatigue as a significant concern. Future research should explore interventions such as exercise, leisure activities, and sleep to alleviate fatigue. Longitudinal research is essential to assess fatigue at diverse times of the day and week; this could help develop tailored fatigue management strategies to augment the quality of life among adolescent oncology patients.

Sleep Deterioration in Survivors of Community-Acquired Pediatric Septic Shock at 3-Month Follow-up

AbstractChildren who survive septic shock are at high risk of new morbidity. The objective of this study is to evaluate the occurrence and risk factors associated with new or worsening sleep disturbance, comparing baseline to 3 months following admission among children surviving septic shock. A secondary analysis of the Life After Pediatric Sepsis Evaluation prospective cohort study was performed. This study included children &lt;18 years admitted to 12 U.S. Pediatric Intensive Care Units with community-acquired septic shock requiring vasoactive–inotropic support and invasive or noninvasive ventilation who survived discharge. The primary outcome of sleep deterioration was characterized as any increased trouble in sleeping measured by a question from the Pediatric Quality of Life Inventory (PedsQL) at baseline compared with the response at the 3-month follow-up. Child and parent variables were evaluated for association with sleep deterioration using univariable and multivariable analyses. Of the original 389 patients, 229 survived for 3 months and completed the PedsQL. The final cohort included 111 children who had available follow-up data at month 3 and did not report baseline sleep challenges. Overall, 25% (28/111) of children reported declines in sleep at 3 months. There were no patient or illness characteristics associated with sleep decline at 3 months in univariable analysis. In multivariable models controlling for age, sex, insurance type, baseline complex conditions, and immunocompromise, no measures of illness severity were associated with deterioration in sleep at 3 months. Sleep deterioration is common in survivors of community-acquired septic shock but detection may be difficult to identify without routine screening.

Quality of life in SCN1A-related seizure disorders across the lifespan.

This cohort study aims to describe the evolution of disease features and health-related quality of life per life stage in Dravet syndrome and other SCN1A-related non-Dravet seizure disorders which will enable treating physicians to provide tailored care. Health-related quality of life and disease features were assessed cross-sectionally in participants with a SCN1A-related seizure disorder, categorized per age group for Dravet syndrome, and longitudinally over seven years follow-up (2015-2022). Data were collected from questionnaires, medical records, and semi-structured telephonic interviews. Health-related quality of life was measured with the Paediatric Quality of Life Inventory, proxy-reported for participants with Dravet syndrome and for participants with non-Dravet aged younger than 18 years old and self-reported for participants with non-Dravet over 18 years old. Associations between health-related quality of life and disease features were explored with multivariable regression analyses, cross-sectionally in a cohort of 115 patients with Dravet and 48 patients with generalized epilepsy with febrile seizures plus and febrile seizures (non-Dravet) and longitudinally in a cohort of 52 Dravet patients and 13 non-Dravet patients. In the cross-sectional assessment in 2022, health-related quality of life was significantly lower in Dravet syndrome, compared to non-Dravet and normative controls. Health-related quality of life in the School and Psychosocial domain was significantly higher in older Dravet age groups. A higher health-related quality of life was associated with fewer behavioural problems [β = -1.1; 95% confidence interval (CI), (-1.4 to -0.8)], independent walking (β = 8.5; 95%CI (4.2-12.8)), compared to the use of a wheelchair), and fewer symptoms of autonomic dysfunction (β = -2.1, 95%CI (-3.2 to -1.0)). Longitudinally, health-related quality of life was significantly higher seven years later in the course of disease in Dravet participants (Δ8.9 standard deviation (SD) 18.0, P < 0.05), mediated by a lower prevalence of behavioural problems (β = -1.2, 95%CI (-2.0 to -0.4)), lower seizure frequency (β = -0.1, 95%CI (-0.2 to -0.0)) and older age (β = 0.03, 95%CI (0.01-0.04)). In summary, health-related quality of life was significantly higher at older age in Dravet syndrome. This finding may reflect the benefits of an advanced care strategy in recent years and a ceiling of severity of disease symptoms, possibly resulting in an increased wellbeing of parents and patients. The strong association with behavioural problems reinforces the need to incorporate a multidisciplinary approach, tailored to the age-specific needs of this patient group, into standard care.