Children, Volume 8, Issue 8 (August 2021) – 105 articles

Background: Bronchiolitis may result in respiratory failure diagnosed with arterial blood gas (ABG). ABG is not routinely performed in general paediatric wards but is closely reflected by capillary blood gas (CBG). We sought to assess the usefulness of CBG results in prediction of intensive care unit (ICU) transfer, antibiotic treatment, and length of stay in children hospitalized due to bronchiolitis. Methods: The optimal cutoff values were estimated with an ROC analysis, while a multiple regression model calculated the odds of an ICU transfer, prolonged hospitalization, and antibiotic treatment related with hypercapnia (pCO₂ ≥ 45 mmHg) and acidosis (pH ≤ 7.35). The correlation between the CBG (pH, pCO₂, and SatO₂) and the clinical/laboratory parameters (breath rate, heart rate, pulse oximetry, white blood cells, CRP, and procalcitonin) was calculated. Results: The CBG was performed in 485 children aged 8 days–22 months (median 2 months). The pCO₂ was significantly higher in ICU transferred patients (median 44.8 mmHg vs. 36.2 mmHg, p < 0.01), and showed AUC = 0.773, (95% CI: 0.638–0.907, p < 0.01) for ICU transfer (67% sensitivity, 82% specificity, 10.8% positive and 98.7% negative predictive value at cutoff 41.8 mmHg). Hypercapnia (OR = 6.63, 95% CI: 2.15–20.46, p < 0.01) and acidosis (OR = 5.01, 95% CI: 1.26–19.9, p = 0.022) predicted the ICU transfer independently. The CBG parameters were not related to prolonged hospitalization or antibiotic treatment, and showed only a weak and clinically irrelevant correlation with other laboratory and clinical parameters. Conclusions: Acidosis and hypercapnia indicate patients at risk of an ICU transfer, and the pCO₂ levels (including values lower than hypercapnia) seem to be a promising marker in ICU risk assessment. Full article

Background: Small for gestational age is a pregnancy complication associated with a variety of adverse perinatal outcomes. The aim of the study was to investigate if sFlt-1/PlGF ratio is related to adverse short-term neonatal outcome in neonates small for gestational age in normotensive pregnancy. Methods: A prospective observational study was conducted. Serum sFlt-1/PlGF ratio was measured in women in singleton gestation diagnosed with fetus small for gestational age. Short-term neonatal outcome analyzed in the period between birth and discharge home. Results: Eighty-two women were included. Women with sFlt-1/PlGF ratio ≥33 gave birth to neonates with lower birthweight at lower gestational age. Neonates from high ratio group suffered from respiratory disorders and NEC significantly more often. They were hospitalized at NICU more often and were discharged home significantly later. sFlt-1/PlGF ratio predicted combined neonatal outcome with sensitivity of 73% and specificity of 82.2%. Conclusions: sFlt-1/PlGF ratio is a useful toll in prediction of short-term adverse neonatal outcome in SGA pregnancies. Full article

Since the spread of COVID-19, pediatric patients were initially considered less affected by SARS-COV-2, but current literature reported subsets of children with multisystem inflammatory syndrome (MIS-C). This study aims to describe the cardiac manifestation of SARS-COV-2 infection in a large cohort of children admitted to two Italian pediatric referral centers. Between March 2020 and March 2021, we performed a cardiac evaluation in 294 children (mean age 9 ± 5.9 years, male 60%) with active or previous SARS-COV-2 infection. Twenty-six showed ECG abnormalities: 63 repolarization anomalies, 13 Long QTc, five premature ventricular beats, two non-sustained ventricular tachycardia, and one atrial fibrillation. In total, 146 patients underwent cardiac biomarkers: NT-proBNP was elevated in 57, troponin in 34. An echocardiogram was performed in 98, showing 54 cardiac anomalies: 27 left-ventricular dysfunction, 42 pericarditis, 16 coronaritis. MIS-C was documented in 46 patients (mean age 9 ± 4.8 years, male 61%) with cardiac manifestations in 97.8%: 27 ventricular dysfunctions, 32 pericarditis, 15 coronaritis, 3 arrhythmias. All patients recovered, and during follow-up, no cardiac anomalies were recorded. Our experience showed that cardiac involvement is not rare in children with SARS-COV-2, and occurred in almost all patients with MIS-C. However, patients’ recovery is satisfactory and no additional events were reported during FU. Full article

Early Intervention (EI) is a set of interventions focused on responding to the needs of children with or at risk of developmental problems. This study aimed to investigate the relationships between the perceived quality of service, satisfaction and family quality of life. Methods: to conduct a multi-centre, transversal study with a non-probabilistic sample. The participants (N = 1551) were families from 24 Early Intervention Centres (EICs) located in Spain. Results: The results indicated an adequate fit of the measurement and structural models, with the latter showing a capacity of 73% to predict the family quality of life. The structural model established that the perceived service quality was a positive and significant predictor of satisfaction (β = 0.85; p < 0.001). Both the perceived quality of service (β = 0.28; p < 0.001) and satisfaction (β = 0.33; p < 0.001) obtained a similar positive and significant relationship with family quality of life, which was slightly stronger than satisfaction. Conclusions: This study provided a better understanding of the importance of the services offered in EICs and their influence on the satisfaction and family quality of life of their users. Thus, delving into these relationships was highly relevant for decision-making in the context of EI. Full article

In their attempt to achieve the optimum weight or body shape for their activity, athletes frequently use harmful weight-control practices that may lead to the development of disordered eating or eating disorders. These practices are linked to several medical and mental consequences that may be more serious in adolescent athletes, as their bodies must meet both intensive growth demands and training requirements at the same time. Among other consequences, adolescent athletes may be at nutritional risk, due to their high nutrient needs and unhealthy eating behaviors. A literature review was conducted to examine the main nutritional risks and malnutrition issues faced by adolescent athletes that present disordered eating attitudes or eating disorders. Most studies refer to adult elite athletes, however research on adolescent athletes also indicates that the most common nutritional risks that may arise due to disordered eating include energy, macronutrient and micronutrient deficiencies, dehydration and electrolyte imbalances and changes in body composition that may lead to menstrual abnormalities, and decreased bone mass density. Educational programs and early detection of disordered eating and eating disorders are crucial to avoid the emergence and ensure timely management of nutrition-related problems in the vulnerable group of adolescent athletes. Full article

Background: There is a lack of psychometric evidence about pediatric health-related quality of life (HRQoL) instruments. Evidence on cost effectiveness, involving the measurement of HRQoL, is used in many countries to make decisions about pharmaceuticals, technologies, and health services for children. Additionally, valid instruments are required to facilitate accurate outcome measurement and clinical decision making. A pediatric multi instrument comparison (P-MIC) study is planned to compare the psychometric performance and measurement characteristics of pediatric HRQoL instruments. Methods: The planned P-MIC study will collect data on approximately 6100 Australian children and adolescents aged 2–18 years via The Royal Children’s Hospital Melbourne and online survey panels. Participants will complete an initial survey, involving the concurrent collection of a range of pediatric HRQoL instruments, followed by a shorter survey 2–8 weeks later, involving the collection of a subset of instruments from the initial survey. Children aged ≥7 years will be asked to self-report HRQoL. Psychometric performance will be assessed at the instrument, domain, and item level. Conclusions: This paper describes the methodology of the planned P-MIC study, including benefits, limitations, and likely challenges. Evidence from this study will guide the choice of HRQoL measures used in clinical trials, economic evaluation, and other applications. Full article

In a small preliminary study, phenylketonuria and poor metabolic control were suggested as risk factors for Helicobacter pylori infection in children as detected with an antigen stool test. We aimed to determine Helicobacter pylori prevalence in an adequately sized group of individuals with phenylketonuria and healthy subjects using the standard gold test (urea breath test). Further, we correlated Helicobacter pylori infection with metabolic control. The study comprised 103 individuals with phenylketonuria and 103 healthy subjects on whom a ¹³C urea breath test was performed. Blood phenylalanine levels in the preceding year were analysed. The infection rate did not differ between individuals with phenylketonuria and healthy subjects (10.7% vs 15.5%; p = 0.41). The frequency of testing and phenylalanine concentrations of Helicobacter pylori-positive and Helicobacter pylori-negative patients with phenylketonuria did not differ (p = 0.92 and p = 0.54, respectively). No associations were detected for body mass index or metabolic control. Forward stepwise regression models revealed that age (p = 0.0009–0.0016) was the only independent correlate of Helicobacter pylori infection with a relatively low fraction of the variability of the condition being explained (adjR² = 0.0721–0.0754; model p = 0.020–0.023). In conclusion, Helicobacter pylori infection in phenylketonuria is not more frequent than in the general population. Moreover, it does not depend on metabolic control. Full article

Background: multisystem inflammatory syndrome in children (MIS-C) is a new disease associated with a recent infection with severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2). Affected children can present predominantly with abdominal symptoms, fever and high inflammatory parameters that might lead to a consult by the pediatric surgeon and an indication for surgery. Methods: clinical data of three patients with MIS-C that underwent surgery were collected. Histopathological analysis of the appendix was performed. Results: we present the clinical course of three children with fever, abdominal pain and vomiting for several days. Clinical examination and highly elevated inflammation markers led to indication for laparoscopy; appendectomy was performed in two patients. Because of intraoperative findings or due to lack of postoperative improvement, all patients were reevaluated and tested positive for MIS-C associated laboratory parameters and were subsequently treated with corticosteroids, intravenous immunoglobulins, acetyl salicylic acid and/or light molecular weight heparin. Conclusions: we discuss the implications of MIS-C as a new differential diagnosis and stress the importance of assessing the previous medical history, identifying patterns of symptoms and critically surveilling the clinical course. We implemented an algorithm for pediatric surgeons to consider MIS-C as a differential diagnosis for acute abdomen that can be integrated into the surgical workflow. Full article

Hypothalamic hamartoma (HH) is a rare, congenital, and benign lesion of the tuber cinereum, typically presenting with central precocious puberty (CPP), gelastic seizure, and developmental delay. This study aimed to investigate CPP in HH patients and compare clinical features between before and after gonadotropin-releasing hormone (GnRH) agonist treatment. A total of 30 HH patients under 18 years of age who visited Severance Children’s Hospital between January 2005 and May 2020 were retrospectively reviewed. Fourteen patients were male (46.7%) and sixteen (53.3%) were female, with a mean age at diagnosis was4.2 ± 2.9 years. During follow-up, 24 patients (80.0%) were diagnosed with CPP, 15 patients (50.0%) had gelastic seizure, and 13 patients (43.3%) had developmental delay. The gelastic seizure was significantly associated with sessile type HH rather than pedunculated type HH (85.7% vs. 18.8%, p = 0.001). After GnRH agonist treatment, discrepancies between bone age and chronological age decreased (3.3 ± 1.3 years to 2.0 ± 1.7 years, p = 0.002). Additionally, height standard deviation score for bone age was increased, and predicted adult height increased significantly in females, while males showed an increasing trend. Clinical symptoms of HH were closely associated with the location of HH, and GnRH agonist treatment was safe and effective in the management of CPP caused by HH. Full article

Acanthosis nigricans (AN) is associated with obesity and type 2 diabetes, where insulin resistance (IR) is considered a predisposing factor. IR can also affect patients with type 1 diabetes (T1D). We evaluated the prevalence of AN in patients with T1D compared to subjects with obesity in order to define the interplay between IR and excess weight. We considered 138 pediatric patients who presented with T1D and 162 with obesity. As controls, 100 healthy normal-weight subjects were included. A physical examination with the detection of AN and biochemical assessments was performed. IR was calculated by using the homeostasis model assessment for IR in patients with obesity and the estimated glucose disposal rate in T1D. The AN prevalence was higher in T1D and obese subjects compared with controls in whom AN was not detected (p = 0.02 and p < 0.001, respectively). A greater number of AN cases were observed in subjects with obesity compared with T1D (p < 0.001). Patients with AN were older than subjects without AN (p = 0.005), and they had higher body mass index (BMI) values, waist circumference (WC), fasting triglycerides and blood pressure (all p < 0.001). Thirty-five patients with AN exhibited IR with an association between AN presence and IR in patients with obesity (p < 0.001). In T1D, there was an association between AN and being overweight/obese (p = 0.02), independently of IR. AN is a dermatological condition associated with obesity. In T1D, the presence of AN was significantly associated with overweight status or obesity but not IR. The presence of AN in the absence of IR supports the interplay role between impaired insulin signaling, IR and excess weight in the pathogenic mechanism. Full article

Pediatric chronic pain is common and responsible for significant healthcare burden. However, the mechanisms underlying the development and/or maintenance of pediatric chronic pain remain poorly understood. Allostatic load (AL), or wear and tear on the nervous system following significant or prolonged stress, has been proposed to play a role in the maintenance of chronic pain, but minimal research has examined this possibility. This gap in research is particularly notable given the high exposure to adverse childhood experiences (ACEs; abuse/neglect, etc.) and psychological stress in this population. Accordingly, the current study aimed to preliminarily examine the measurement of AL in a treatment-seeking pediatric pain population. Biomarkers were collected during an already scheduled new patient pain evaluation and included salivary cortisol, dehydroepiandrosterone (DHEA), and C-reactive protein, in addition to waist–hip ratio, body-mass index, and blood pressure. A total of 61 children and adolescents with chronic pain (Mage = 14.47 years; 88.5% female and white/Caucasian) completed study procedures and were included in analyses. Preliminary results indicated that a multifactorial AL composite is feasible to assess for in a tertiary pain treatment setting and that over 50% of youth with chronic pain were classified as high risk for AL (two or more risk factors). Further, it was found that individual AL risk factors were significantly associated with functional disability and that AL may moderate the association between psychosocial and functional outcomes. Given the pilot nature of this study, results should be used to inform future investigations with larger and more diverse pediatric pain samples. Full article

Environmental factors play an important role in the development and exacerbation of allergic rhinitis (AR) in childhood. Indoor air pollution, such as house dust mites and secondhand smoke, can significantly increase the onset of AR, while pet dander may affect the exacerbation of AR symptoms in children. Furthermore, traffic related air pollution and pollen are outdoor air pollutants that can affect immune competency and airway responsiveness, increasing the risk of AR in children. Climate change has increased AR in children, as growth patterns of allergenic species have changed, resulting in longer pollen seasons. More extreme and frequent weather events also contribute to the deterioration of indoor air quality due to climate change. Additionally, viruses provoke respiratory tract infections, worsening the symptoms of AR, while viral infections alter the immune system. Although viruses and pollution influence development and exacerbation of AR, a variety of treatment and prevention options are available for AR patients. The protective influence of vegetation (greenness) is heavily associated with air pollution mitigation, relieving AR exacerbations, while the use of air filters can reduce allergic triggers. Oral antihistamines and intranasal corticosteroids are common pharmacotherapy for AR symptoms. In this review, we discuss the environmental risk factors for AR and summarize treatment strategies for preventing and managing AR in children. Full article

We present a case report of a 12-year old female with a midshaft forearm fracture. Initial conservative treatment with a cast failed, resulting in a malunion. The malunion resulted in functional impairment for which surgery was indicated. A corrective osteotomy was planned using 3D analyses of the preoperative CT-scan. Subsequently, patient-specific guides were printed and used during the procedure to precisely correct the malunion. Three months after surgery, the radiographs showed full consolidation and the patient was pain-free with full range of motion and comparable strength in both forearms. The current case report shows that a corrective osteotomy with patient-specific guides based on preoperative 3D analyses can help surgeons to plan and precisely correct complex malunions resulting in improved functional outcomes. Full article

Play is a key factor for children’s healthy psychological, emotional, social, and cognitive development. During the COVID-19 pandemic, it has been postulated that children’s play was affected, not only regarding the time children spent playing but also in terms of the qualitative characteristics of play. The aim of this review was to investigate how children’s play has changed during the COVID-19 pandemic. A review was conducted in the PubMed, Google Scholar, EMBASE, SCOPUS, ERIC, PsycInfo, and JSTOR databases up to 6 December 2020. Furthermore, references of eligible studies as well as of relevant articles were searched using a snowballing technique. The search retrieved 17 eligible studies, conducted in Europe and North America. In general, outdoor play was reduced during the pandemic; on the other hand, there was an increase in indoor play and in videogames-screen time. COVID-19 was present in children’s pretend play. Children’s play was a key contributor to children’s mood and wellbeing. Furthermore, teachers were especially concerned about how children’s play was affected during the lockdown measures. There is evidence that children’s play habits were affected during the COVID-19 pandemic; further research is required, especially cross-culturally oriented. Full article

During growth, children are influenced by an extensive network, in which more favorable contexts provide better affordance landscapes, and consequently have a better potential to foster child development. We aimed to examine the affordances provided to children using the Affordances for Motor Behavior of Schoolchildren (AMBS) tool, estimating its association with children’s motor competence, as assessed by the Motor Competence Assessment (MCA) battery. Seventy-two Brazilian children were evaluated using the MCA instrument. Their parents/guardians completed the AMBS. The correlations between the two instruments (sub-scales and total scores) were investigated. ANOVAs were used to compare the motor competence performance of children with Low, Average, and High AMBS scores. Positive associations were found between AMBS and MCA, although weak to moderate in nature. In addition, children whose environments were richer in motor affordances (higher AMBS scores) showed significantly higher levels on the MCA. This study provides evidence that AMBS is a valid tool for assessing motor affordances for schoolchildren, and that those affordances are related to children’s motor competence. Full article

Background: While meaningful sound exposure has been shown to be important for newborn development, an excess of noise can delay the proper development of the auditory cortex. Aim: The aim of this study was to assess the acoustic environment of a preterm baby in an incubator on a newborn intensive care unit (NICU). Methods: An empty but running incubator (Giraffe Omnibed, GE Healthcare) was used to evaluate the incubator frequency response with 60 measurements. In addition, a full day and night period outside and inside the incubator at the NICU of the University Hospital Zurich was acoustically analyzed. Results: The fan construction inside the incubator generates noise in the frequency range of 1.3–1.5 kHz with a weighted sound pressure level (SPL) of 40.5 dB(A). The construction of the incubator narrows the transmitted frequency spectrum of sound entering the incubator to lower frequencies, but it does not attenuate transient noises such as alarms or opening and closing of cabinet doors substantially. Alarms, as generated by the monitors, the incubator, and additional devices, still pass to the newborn. Conclusions: The incubator does protect only insufficiently from noise coming from the NICUThe transmitted frequency spectrum is changed, limiting the impact of NICU noise on the neonate, but also limiting the neonate’s perception of voices. The incubator, in particular its fan, as well as alarms from patient monitors are major sources of noise. Further optimizations with regard to the sound exposure in the NICU, as well as studies on the role of the incubator as a source and modulator, are needed to meet the preterm infants’ multi-sensory needs. Full article

This is a retrospective radiographic review to assess post-operative sagittal plane deformities in patients with Spinal Muscular Atrophy type 2 that had been treated with posterior spinal instrumentation. Thirty-two patients with a history of either spinal fusion (N = 20) or growing rods (N = 12) were identified with an average of 7.6 (2.1–16.6) years post-operative follow-up. Forty percent (13/32) of the patients were identified as having obvious “tucked chin” (N = 4), “tipped trunk” (N = 9), or both (N = 3). Sacral incidence was the only parameter that was statistically significant change between pre-operative or immediate post-operative measurements (66.9° vs. 55.2° p = 0.03). However, at final follow-up, the post-operative thoracic kyphosis had decreased over time in those that developed a subsequent sagittal deformity (24.2°) whereas it increased in those that did not (44.7°, p = 0.008). This decrease in thoracic kyphosis throughout the instrumented levels, resulted in a greater lordotic imbalance (30.4° vs. 5.6°, p = 0.001) throughout the instrumented levels in the group that developed the subsequent cervical or pelvic sagittal deformities. In conclusion, sagittal plane deformities commonly develop outside the instrumented levels in children with SMA type 2 following posterior spinal instrumentation and may be the result of lordotic imbalance that occurs through continued anterior growth following posterior instrumentation. Full article

Our objective is to emphasize the important role of continuous glucose monitoring (CGM) in suggesting adrenal insufficiency in patients affected by type 1 diabetes. We describe an adolescent girl with type 1 diabetes and subsequent latent Addison’s disease diagnosed based on a recurrent hypoglycemic trend detected by CGM. In patients with type 1 diabetes, persistent unexplained hypoglycemic episodes at dawn together with reduced insulin requirement arouse souspicionof adrenal insufficiency. Adrenal insufficiency secondary to autoimmune Addison’s disease, even if rarely encountered among young patients, may be initially symptomless and characterized by slow progression up to acute adrenal crisis, which represents a potentially life-threatening condition. Besides glycometabolic assessment and adequate insulin dosage adjustment, type 1 diabetes needs prompt recognition of potentially associated autoimmune conditions. Among these, Addison’s disease can be suspected, although latent or paucisymptomatic, through periodic and careful evaluation of CGM data. Full article

Background: The standard of treatment for infants with hypertrophic pyloric stenosis is still pyloromyotomy. Recently, in most of the pediatric surgery centers laparoscopic pyloromyotomy has become popular. The aim of the present study is to compare the outcomes of treatment in infants with hypertrophic pyloric stenosis between traditional open approach and laparoscopic pyloromyotomy using 3-mm electrocautery hook. Methods: A total of 125 infants, 104 (83.2%) males, with median age 33 (interquartile range, IQR 24, 40) days, who underwent pyloromyotomy because of hypertrophic pyloric stenosis, between 2005 and 2021, were included in the retrospective study. Of that number 61 (48.8%) infants were allocated to the open group and 64 (51.2%) to the laparoscopic group. The groups were compared in regards to time to oral intake, duration of surgery, the type and rate of complications, rate of reoperations, frequency of vomiting after surgery, and the length of hospital stay. Results: No differences were found with regards to baseline characteristics between two investigated groups. Laparoscopic approach was associated with significantly better outcomes compared to open approach: shorter duration of surgery (35 min (IQR 30, 45) vs. 45 min (40, 57.5); p = 0.00008), shorter time to oral intake (6 h (IQR 4, 8) vs. 22 h (13.5, 24); p < 0.00001), lower frequency of postoperative vomiting (n = 10 (15.6%) vs. n = 19 (31.1%)), and shorter length of postoperative hospital stay (3 days (IQR 2, 3) vs. 6 days (4.5, 8); p < 0.00001). In regards to complications and reoperation rates, both were lower in the laparoscopic pyloromyotomy group but the differences were not statistically significant (p = 0.157 and p = 0.113, respectively). The most common complication in both groups was mucosal perforation (open group, n = 3 (4.9%); laparoscopic group, n = 2 (3.1%)) followed by wound infection in open group, n = 3 (4.9%). No cases of wound infection were recorded in the laparoscopic group. Conclusion: Open and laparoscopic pyloromyotomy are equally safe and effective in treatment of hypertrophic pyloric stenosis. Laparoscopic technique is associated with faster recovery, shorter duration of surgery and shorter duration of hospital stay. Full article

Most studies with institutionalised children and adolescents focus on evaluating the impact of negative life events on emotional development. However, few have investigated the relationship between resilience assets and the teenagers’ psychopathological problems. The purpose of the present study was to investigate differences in psychological distress symptoms and in resilience assets in institutionalised and non-institutionalised adolescents. A total of 266 adolescents aged between 12 and 19 years old took part in the study (60.5% female): 125 lived in residential care and 144 resided with their families. Results found a significant and inverse relation between psychopathology and the perception of individual resilience assets, specifically with self-efficacy and self-awareness in the community sample, and with empathy in the institutionalised sample. Overall, and regardless of the age group, adolescents in residential care tend to perceive themselves as significantly less resilient in perceived self-efficacy and empathy, and they report fewer goals and aspirations for the future. The importance of promoting mental health and resilience assets in adolescents, particularly in those in residential care, is discussed. This can be achieved through early interventions that may prevent emotional suffering and deviant life paths, with transgenerational repercussions. Full article

The coronavirus disease 2019 (COVID-19) pandemic is an unprecedented public health crisis worldwide. This pandemic has led to the spread of online misinformation, closure of schools, postponement of re-opening of schools, and restrictions on healthy outdoor activities. These, in turn, have caused a disruption in the daily life of middle school students. This study aimed to identify the relationships between eHealth literacy, awareness of pandemic infectious diseases, and healthy lifestyle in middle school students. For this purpose, we conducted a descriptive, correlational study in two cities in the capital of South Korea. A total of 138 middle school students completed self-reported questionnaires about eHealth literacy, awareness of pandemic infectious diseases, and healthy lifestyle. Middle school students displayed moderate levels of eHealth literacy (3.72 ± 0.97), awareness of pandemic infectious diseases (3.33 ± 0.92), and healthy lifestyle (2.98 ± 0.72). Positive correlations were found between eHealth literacy and awareness of pandemic infectious diseases (r = 0.44, p < 0.001), between eHealth literacy and healthy lifestyle (r = 0.52, p < 0.001), and between awareness of pandemic infectious diseases and healthy lifestyle (r = 0.38, p < 0.001). These findings suggest that eHealth literacy may be an important aspect of increasing the awareness about pandemic infectious diseases and forming healthy lifestyle habits in Korean middle school students during the COVID-19 pandemic. Full article

Epidermal nevus syndrome (ENS) is a congenital disorder characterized by widespread linear epidermal lesions consisting of epidermal nevus and extracutaneous involvements, especially of the central nervous system and skeletal system. Garcia–Hafner–Happle syndrome, also known as fibroblast growth factor receptor 3 (FGFR3)-ENS, is characterized by a systematized keratinocytic EN of soft and velvety type with neurological abnormalities such as seizures, intellectual impairment, and cortical atrophy. We present a case of a 9-year-old Japanese boy afflicted with Garcia–Hafner–Happle syndrome associated with dwarfism and atopic dermatitis. We show the results of physical examination, DNA analysis, and imaging studies and discuss the mutation underlying the child’s disorder. Full article

Child healthcare (CHC) nurses have a key role in promoting and supporting healthy lifestyle behaviors from a young age. Thus, this study aims to investigate the perspectives of CHC nurses regarding discussing food introduction, physical activity/active play, and screen time with parents; explore facilitators and barriers influencing the discussion of healthy lifestyle behaviors with parents; and explore the perspectives of CHC nurses regarding a complementary program to promote healthy lifestyle behaviors from the start of life. A total of fifteen nurses participated in semi-structured interviews, which were recorded, transcribed verbatim, and analyzed using thematic analysis. There were four themes that were generated: parental needs; facilitators and barriers; parental groups; and future working methods. This study found that CHC nurses have seen an increase in the need for support among today’s parents. Time, the need to tailor information, and confidence to address sensitive topics were perceived as the largest barriers during daily work for the nurses. Furthermore, large variations in parental groups were found. Finally, the CHC nurses displayed a willingness and openness to change and develop current working methods using digital solutions. These solutions could possibly ease the workload and at the same time, support parents to create healthy lifestyle behaviors from the start of their child’s life. Full article

Background: Electronic medical records (EMRs) offer a promising approach to mapping and documenting the complex information gathered in paediatric palliative care (PPC). However, if they are not well developed, poorly implemented EMRs have unintended consequences that may cause harm to patients. One approach to preventing such harm is the involvement of users in the participatory design to ensure user acceptance and patient safety. Therefore, the aim of this study is to evaluate the acceptance of a novel patient chart module (PCM) as part of an EMR from the perspective of potential users in PPC and to involve these professionals in the design process. Methods: A qualitative observational study with N = 16 PPC professionals (n = 10 nurses, n = 6 physicians) was conducted, including concurrent think aloud (CTA) and semi-structured interviews. A structured content analysis based on the Unified Theory of Acceptance and Use of Technology was applied. Results: The results can be summarized in terms of general observations, performance expectancy, effort expectancy and facilitating conditions, all of which are likely to have a positive influence on acceptance of the PCM from the user perspective in the context of PPC. Conclusions: The involvement of users in the development of EMRs is important for meeting the requirements in PPC. Further software adaptations are necessary to implement these requirements. Full article

Spinal muscular atrophy (SMA) is a genetic neuromuscular disorder that results in progressive muscle atrophy and weakness. As new therapies for SMA have been developed, newborn screening for SMA can lead to early diagnosis and treatment. The objective of this study was to gather the general population’s view on screening of SMA in newborns in Japan. A questionnaire survey was conducted on two general population groups in Japan. A total of 269 valid responses were obtained. In the general population, about half of the participants had no knowledge about SMA, and more than 90% did not know about new therapies for SMA. Conversely, more than 95% of the general population agreed with screening newborns for SMA because they believed that early diagnosis was important, and treatments were available. This study revealed that the general population in Japan mostly agreed with screening for SMA in newborns even though they did not know much about SMA. Newborn screening for SMA is promising, but it is in very early stages. Therefore, SMA newborn screening should be performed with sufficient preparation and consideration in order to have a positive impact on SMA patients and their families. Full article

(1) Background: Obstructive sleep apnea (OSA) and lower airway anomalies are both highly prevalent in children with Down syndrome (DS). However, little is known on the interaction between both. We aim to investigate the co-occurrence of OSA (defined as obstructive apnea/hypopnea index (oAHI) ≥ 2/h) and lower airway anomalies in children with DS and explore their impact on OSA severity and treatment outcome. (2) Methods: Retrospective analysis of data from airway endoscopy and polysomnography (PSG) in a cohort of children with DS. (3) Results: Data on both lower airway evaluation and PSG were available for 70 patients with DS. Our study population was relatively young (mean age 3.5 years), not obese and presented with severe OSA (mean oAHI 13.1/h). Airway anomalies were found in 49/70 children (70%), most frequently laryngomalacia, tracheomalacia or a combined airway malformation. In the remaining 21 cases (30%), endoscopy was normal. A comparison between both groups showed a similar distribution of gender, age and BMI z-scores. The prevalence of OSA was not significantly higher in DS patients with airway anomalies (89.6% vs 71.4%, p = 0.078). Additionally, OSA severity or treatment choice (conservative, upper airway surgery or CPAP) were not significantly different. Follow-up data (available for 49/70 patients) showed a significant improvement of OSA in both groups. There is a not significant tendency to more patients with persistent OSA among those with lower airway anomalies (34.3% vs 7.1%, p = 0.075). (4) Conclusions: We found no significant differences in OSA severity, treatment choice or outcome between children with DS with and without lower airway anomalies. Further studies should investigate the role of DISE-directed treatment and compare the outcome of different treatment modalities in larger patient groups. Full article

The antibacterial efficacy of antimicrobial filling pastes (AFP) used in the root canal treatment of primary teeth has been widely reported. However, antibiotic resistance as an emerging global problem could impact their current efficacy. This study aimed to evaluate the efficacy of two common AFP on susceptible or resistant bacteria isolated from primary necrotic molars. Microbiological samples were obtained and cultured from the root canals of 34 children. In total, 96 colony-forming units were obtained to determine their resistance to tetracycline, rifampicin, and chloramphenicol. They were identified as S. mutans or E. faecalis using polymerase chain reaction. The antimicrobial activity of CTZ paste (chloramphenicol, tetracycline, zinc oxide, and eugenol) and Guedes-Pinto modified (GPM) paste (rifampicin, prednisolone, iodoform, and camphorated paramonochlorophenol) were tested against the identified and selected microorganisms. Larger size inhibition zones were observed in both species when the tested strains were susceptible to the antibiotics in the AFP preparation. The efficacy of AFP containing antibiotics depends on the antibiotic resistance profile of the strain. Antibiotic resistance and its effect on the AFP were shown, which calls into question the use of simplified endodontic techniques that depend on antibiotics, since in these cases these techniques could not clinically eliminate resistant bacteria from the root canal. Full article

Taking blood via venipuncture is part of the necessary surveillance before and after liver transplantation. The spectrum of response from children and their parents is variable, ranging from a short and limited aversion to paralyzing phobia. The aim of this retrospective, cross-sectional study was to determine the level of anxiety amongst children during venipuncture, to compare the anxiety reported by children and parents, and to identify the factors affecting the children’s and parents’ anxiety in order to develop therapeutic strategies. In total, 147 children (aged 0–17 years, 78 female) and their parents completed questionnaires. Statistical analysis was performed using qualitative and quantitative methods. Results showed that the majority of children reported anxiety and pain during venipuncture. Younger children had more anxiety (self-reported or assessed by parents). Children and parental reports of anxiety were highly correlated. However, the child’s anxiety was often reported as higher by parents than by the children themselves. The child’s general anxiety as well as the parents’ perceived stress from surgical interventions (but not the number of surgical interventions) prompted parental report of child anxiety. For children, the main stressors that correlated with anxiety and pain were factors during the blood collection itself (e.g., feeling the puncture, seeing the syringe). Parental anxiety was mainly related to circumstances before the blood collection (e.g., approaching the clinic, sitting in the waiting room). The main stressors mentioned by parents were the child’s discomfort and their inability to calm the child. Results indicate that the children’s fear of factors during the blood collection, along with the parents’ perceived stress and helplessness as well as their anticipatory anxiety are important starting points for facilitating the drawing of blood from children before and after liver transplantation, thereby supporting a better disease course in the future. Full article

Preventative measures taken worldwide to decrease the transmission of COVID-19 have had a tremendous impact on youth. Following social restrictions, youth with and without physical disabilities are engaging in less physical activity, more increased sedentary behavior, and poor sleep habits. Specifically, youth wheelchair users (YWU) are likely disproportionately affected by COVID- 19 and have a higher risk of contraction due to underlying comorbidities. While we cannot control all of the negative long-term implications of COVID-19 for YWU, participation in positive 24-h activity behaviors can decrease chronic disease risk and the likelihood of long-term complications resulting from infection. This commentary is to extend the discourse on the importance of 24-h activity behaviors by focusing on YWU. Specifically, we discuss the importance of chronic disease prevention, provide a brief overview of 24-h activity behaviors, and outline some of the lessons that can be learned from the COVID-19 pandemic. Full article