Children, Volume 10, Issue 4 (April 2023) – 155 articles

Introduction: Endoscopic procedures are performed more frequently in children due to technological advances that can be safely performed in an adequate setting with a support of a multidisciplinary team. Pediatric indications for ERCP (endoscopic retrograde cholangiopancreatography) and EUS (endoscopic ultrasound) occur mainly due to congenital malformations. In a pediatric case series, we report the application of EUS combined with duodenoscopy, eventually associated with ERCP and minimally invasive surgery, highlighting the importance of defining a tailored dedicated management pathway for each patient. Patients and methods: A series of 12 patients, managed at our Center in the last three years, were evaluated, and their management was discussed. Results: EUS was performed in eight patients and permitted the differential diagnosis of duplication cysts and the visualization of the biliary tree and pancreatic anatomy. ERCP was attempted in five patients: in one case, it permitted the preservation of pancreatic tissue, postponing surgery and in three patients, it was technically unfeasible. MIS (minimally invasive surgery) was performed in seven patients, two with laparoscopic common bile duct exploration (LCBDE). Precise anatomical definition and the possibility of surgical simulation and team sharing were evaluated under VR HMD (Virtual Reality Head Mounted Display) in four cases. Conclusions: Exploration of the common bile duct in children differs from that of the adult population and combines echo-endoscopy and ERCP. The integrated use of minimally invasive surgery in the pediatric area is necessary for the whole management perspective in complex malformations and small patients. The introduction in the clinical practice of a preoperative study with Virtual Reality allows a better survey of the malformation and a tailored treatment. Full article

Background: This study aimed to ascertain the prevalence of dental anomalies and their ability to estimate sex status. Material and Methods: This cross-sectional radiographic study was based on the evaluation of dental anomalies of Saudi children aged between 5 and 17 years. A total of 1940 orthopantomograms (OPG) were screened, of which 1442 were included. All the OPGs were digitally evaluated with ImageJ software. The demographic variables and dental anomaly findings were subjected to descriptive and comparative statistical analysis. Discriminant function analysis was conducted for sex estimation. p value < 0.05 was considered as significant. Results: The mean age of the children in this study was 11.35 ± 0.28 years. At least one dental anomaly was detected in 161 children (11.17%), including 71 males and 90 females. Only 13 children (8.07%) presented with more than one anomaly. The most common dental anomaly detected was root dilaceration (47.83%) followed by hypodontia (31.68%). The least common dental anomaly was infraocclusion (1.86%). The sex prediction accuracy using discriminant function analysis was 62.9% (p < 0.01). Conclusion: The prevalence of dental anomalies was 11.17% with root dilaceration and hypodontia being the most common. The role of dental anomalies in sex estimation was found to be ineffective. Full article

The osseous acetabular index (OAI) and cartilaginous acetabular index (CAI) is often used in diagnosing acetabular dysplasia (AD) in children. We examined the reliability of OAI and CAI in AD diagnostics and compared OAI measurements obtained from radiographs versus MRI. Four raters performed retrospective repeated measurements of the OAI and CAI on pelvic radiographs and MRI scans of 16 consecutive patients (mean age 5 years (2–8)) examined for borderline AD during a period of 2½ years. In MRI, the image selected for analysis by the raters was also registered. Spearman’s correlation, scatter plots, and Bland–Altman (BA) plots were analysed for correlation between OAI on pelvic radiographs (OAIR) and MRI scans (OAIMRI), while intra- and interrater reliability was assessed for OAIR, OAIMRI, CAI, and MRI image selection using intraclass correlation coefficients (ICC). ICC values for inter- and intrarater reliability of OAIR, OAIMRI, and CAI were all above 0.65, with no significant differences observed. ICC values (CI) for individual raters’ MRI image selection was 0.99 (0.998–0.999). The mean difference (95% CI) between OAIR and OAIMRI was −0.99 degrees (−1.84; −0.16), while the mean absolute difference (95% CI) between OAIR and OAIMRI was 3.68 degrees (3.17; 4.20). Absolute differences between OAIR and OAIMRI was independent of pelvic positioning or time interval between radiographs and MRI scans. OAI and CAI had high Intrarater reliability but mediocre interrater reliability. There was an absolute difference of 3.7 degrees in OAI between pelvic radiographs and MRI scans. Full article

Background: In recent months, there has been growing interest in the potential of artificial intelligence (AI) to revolutionize various aspects of medicine, including research, education, and clinical practice. ChatGPT represents a leading AI language model, with possible unpredictable effects on the quality of future medical research, including clinical decision-making, medical education, drug development, and better research outcomes. Aim and Methods: In this interview with ChatGPT, we explore the potential impact of AI on future pediatric research. Our discussion covers a range of topics, including the potential positive effects of AI, such as improved clinical decision-making, enhanced medical education, faster drug development, and better research outcomes. We also examine potential negative effects, such as bias and fairness concerns, safety and security issues, overreliance on technology, and ethical considerations. Conclusions: While AI continues to advance, it is crucial to remain vigilant about the possible risks and limitations of these technologies and to consider the implications of these technologies and their use in the medical field. The development of AI language models represents a significant advancement in the field of artificial intelligence and has the potential to revolutionize daily clinical practice in every branch of medicine, both surgical and clinical. Ethical and social implications must also be considered to ensure that these technologies are used in a responsible and beneficial manner. Full article

Pulmonary arterial hypertension (PAH) is associated with increased right ventricular (RV) afterload, affecting RV remodeling and RV performance, a major determinant of outcome in PAH-patients. In children with PAH, treatment strategy is guided by risk stratification where noninvasive prognosticators are highly needed. The prognostic value of RV characteristics derived by cardiac magnetic resonance (CMR) has been scarcely studied in pediatric PAH. We aimed to identify CMR-derived morphometric and functional RV characteristics prognostic for outcome in children with PAH. From the Dutch National cohort, thirty-eight children with either idiopathic/heritable PAH (IPAH/HPAH) or PAH associated with congenital heart disease (PAH-CHD), who underwent CMR, were included (median (interquartile range) [IQR] age 13.0 years (10.8–15.0), 66% females). Patients had severe PAH, characterized by their World Health Organization Functional Class, increased N-terminal pro-B-type natriuretic peptide and high pulmonary arterial pressure and pulmonary vascular resistance index at time of CMR. RV-ejection fraction (RVEF), indexed RV-mass (RVMi), the ratio between RV and LV mass (RVM/LVM-ratio) and left ventricular eccentricity index (LVEI) all correlated with transplant-free survival from time of CMR. These correlations could not be confirmed in the PAH-CHD group. This study shows that CMR-derived measures reflecting RV function and remodeling (LVEI, RVMi, RVM/LVM-ratio, RVEF) predict transplant-free survival in children with IPAH/HPAH and may be included in risk stratification scores in pediatric PAH. Full article

Background: Suicide-related behaviors increasingly contribute to behavioral health crises in the United States (U.S.) and worldwide. The problem was worsened during the COVID-19 pandemic, especially for youth and young adults. Existing research suggests suicide-related behaviors are a consequence of bullying, while hopelessness is a more distal consequence. This study examines the association of in-school and electronic bullying with suicide-related behavior and feelings of despair among adolescents, adjusted for sociodemographic characteristics, abuse experience, risk-taking behaviors, and physical appearance/lifestyles. Method: Using Chi-square, logistic regression, and multinomial logistic regression, we analyzed the US 2019 Youth Risk Behavior Surveillance System (YRBSS) national component. The YRBSS includes federal, state, territorial, and freely associated state, tribal government, and local school-based surveys of representative sample middle and high school students in the US. The 2019 YRBSS participants comprised 13,605 students aged 12 to 18 years and roughly equal proportions of males and females (50.63% and 49.37%, respectively). Results: We observed a significant association (p < 0.05) between being bullied and depressive symptoms, and the association was more vital for youth bullied at school and electronically. Being bullied either at school or electronically was associated with suicidality, with a stronger association for youth who experienced being bullied in both settings. Conclusion: Our findings shed light on assessing early signs of depression to prevent the formation of suicidality among bullied youth. Full article

The purpose of the study was to analyze caries experience in primary and permanent dentition in children up to 15 years of age located in Banja Luka, Bosnia and Herzegovina. Methods: The research was conducted as a retrospective cross-sectional study. Analyzes and comparisons of caries indices were performed using groups formed according to their gender (male—M and female—F) and age, i.e., the first group-children in early childhood, ≤5 years; the second group, middle childhood 6–8 years; the third group, preadolescents 9–11 years old; fourth group, adolescents 12–15 years old. Results: Overall prevalence of caries in primary dentition was 89.1%, while in permanent dentition, it was 60.7%. The overall mean decayed, missing, and filled teeth— dmft in male participants was 5.4, while in female participants, it was 5.1. By contrast, a higher overall mean DMFT was established in the female participants (2.7 vs. 3.0). Conclusions: We can see a high prevalence in all of the examined groups. In primary dentition, males examined during the course of the study had a higher overall mean dmft and the mean number of untreated decayed primary teeth, whereas females up to age 15 examined during the course of the study had more DMF teeth. Full article

The aim of this insights paper is to propose how the theory of ecological dynamics may invite re-consideration of how sport scientists could support performance, learning and development of children and youth in sports programmes. We seek to outline why learning should be individualised and contextualised, based on the specific needs of learners, such as children and youth, women and disabled athletes in sport. Case examples from individual and team sports are presented to illustrate how constraints can be designed to enrich interactions of children and youth with different performance environments, based on integrating principles of specificity and generality in learning and development. These case examples suggest how a collaborative effort by sport scientists and coaches in children and youth sport may be undertaken in a department of methodology to enrich learning and performance. Full article

An art-based case study was used to illustrate the therapy journey of a child working through issues related to early adoption. The objective of this case was to review art products and clinical notes systematically, exploring main clinical themes and illustrating both challenges related to adoption and the potential of art therapy to support healing within this context. The methods of investigation and report focused on exploring the meaning of narratives, the art products, and the relational dynamic which emerged during sessions. The results are discussed within the context of the relevant literature, emphasizing considerations for working through adoption challenges in art therapy. Full article

Aim of the study: To evaluate the clinical outcomes and complication rate of laparoscopic appendectomy in children operated on during the daytime versus nighttime. Methods: A total of 303 children who underwent laparoscopic appendectomy for acute appendicitis between 1 January 2020 and 31 December 2022 were enrolled in this retrospective study. The patients were divided into two study groups. The first group consisted of the patients who underwent laparoscopic appendectomy during the day shift from 07:00–21:00 (n = 171), while the patients in the second group underwent laparoscopic appendectomy during the night shift from 21:00–07:00 (n = 132). The groups were compared for baseline clinical and laboratory data, treatment outcomes, and complications. The Mann–Whitney U test was used to compare continuous variables, while the Chi-square test was used to compare categorical variables. A two-sided Fisher’s exact test was used when the frequency of events in a certain cell was low. All p values less than 0.05 were considered significant. Results: The proportion of complicated appendicitis was almost the same in both patient groups (n = 63, 36.8% vs. n = 49, 37.1%, p = 0.960). Out of the total number of patients presenting during the daytime and nighttime, 11 (6.4%) and 10 (7.6%) developed a postoperative complication, respectively (p = 0.697). Additionally, rates of readmission (n = 5 (2.9%) vs. n = 2 (1.5%); p = 0.703), redo-surgery (n = 3 (1.7%) vs. n = 0; p = 0.260), conversion to open surgery (n = 0 vs. n = 1 (0.8%); p = 0.435) and length of hospital stay (n = 3 (IQR 1, 5) vs. n = 3 (IQR 2, 5); p = 0.368) did not differ significantly between daytime and nighttime appendectomies. The duration of the surgery was significantly shorter in patients presenting during the day than in those presenting at night (26 min (IQR 22, 40) vs. 37 min (31, 46); p < 0.001). Conclusions: Different shift times did not affect the treatment outcomes or complication rates for children receiving laparoscopic appendectomy. Full article

Visual perception in children can be evaluated using the Test of Visual Perceptual Skills—4th edition (TVPS-4) with normative data developed for the U.S. population. It is widely used by healthcare practitioners in Malaysia, despite reports that children in Asia outperform their U.S. peers in visual perception assessment. We compared TVPS-4 scores among 72 Malaysian preschool children (mean age: 5.06 ± 0.11 years) with U.S. norms and investigated the association between socioeconomic factors and TVPS-4 scores. Malaysian preschoolers had significantly higher standard scores (116.60 ± 7.16) than the U.S. norms (100 ± 15; p < 0.001). They also had significantly higher scaled scores (between 12.57 ± 2.10 and 13.89 ± 2.54) than the U.S. norms (10 ± 3, all p < 0.001) for all subtests. Multiple linear regression analyses revealed that socioeconomic variables were not significant predictors for five visual perception subtests and the overall standard score. The visual form constancy score could be predicted by ethnicity (β = −1.874, p = 0.03). The visual sequential memory score could be predicted by the father’s employment status (β = 2.399, p < 0.001), mother’s employment status (β = 1.303, p = 0.007), and low household income (β = −1.430, p < 0.037). In conclusion, Malaysian preschoolers outperformed their U.S. peers in all TVPS-4 subtests. Socioeconomic variables were associated with visual form constancy and visual sequential memory, but not with the other five subtests or TVPS-4’s overall standard scores. Full article

Handwriting is a complex task that includes planning the content and the execution of handwriting movements on paper or on a tool (e.g., a tablet). This execution depends on the involvement of specific muscles in the hand (distal) and arm (proximal). The present study combines the parallel recording of the writing process on tablets and the related muscle activity with electromyography to investigate the differences in handwriting movements in two groups. A total of 37 intermediate writers (third and fourth graders, mean age 9.6 years, SD 0.5) and 18 skilled adults (mean age 28.6 years, SD 5.5) participated in three handwriting tasks. The tablet data results replicate previous findings in handwriting research for the writing process. The muscle activity data reflected a differential relationship between distinct muscle activity and handwriting performance depending on the handwriting skill (intermediate or advanced writers). Furthermore, the combination of both methods revealed that skilled writers tend to involve rather distal muscles to control the pressure of the writing pen on the surface, whereas developing writers mainly use their proximal muscles to control the velocity of their handwriting movements. This research contributes to a deeper understanding of the underlying processes of handwriting and the development of efficient handwriting execution. Full article

Nutrition screening is an essential process to detect children at risk of malnutrition during hospitalization and provide appropriate nutrition management. STRONGkids is a nutrition screening tool which has been implemented in a tertiary-care hospital service in Bangkok, Thailand. This study aimed to evaluate the performance of STRONGkids in the real-situation setting. Electronic Medical Records (EMR) of hospitalized pediatric patients aged 1 month to 18 years from January to December 2019 were reviewed. Those with incomplete medical records and re-admission within 30 days were excluded. Nutrition risk scores and clinical data were collected. Anthropometric data were calculated to Z-score based on the WHO growth standard. The sensitivity (SEN) and specificity (SPE) of STRONGkids were determined against malnutrition status and clinical outcomes. In total, 3914 EMRs (2130 boys, mean age 6.22 ± 4.72 years) were reviewed. The prevalence of acute malnutrition (BMI-for-age Z-score < −2) and stunting (height-for-age Z-score < −2) were 12.9 and 20.5%. SEN and SPE of STRONGkids against acute malnutrition were 63.2 and 55.6%, stunting values were 60.6 and 56.7%, and overall malnutrition values were 59.8 and 58.6%. STRONGkids had low SEN and SPE to detect nutrition risks among hospitalized children in a tertiary-care setting. Further actions are required to improve the quality of nutrition screening in hospital services. Full article

Introduction: The Performance of Upper Limb version 2.0 (PUL 2.0) is increasingly used in Duchenne Muscular Dystrophy (DMD) to study longitudinal functional changes of motor upper limb function in ambulant and non-ambulant patients. The aim of this study was to evaluate changes in upper limb functions in patients carrying mutations amenable to skipping exons 44, 45, 51 and 53. Methods: All DMD patients were assessed using the PUL 2.0 for at least 2 years, focusing on 24-month paired visits in those with mutations eligible for skipping exons 44, 45, 51 and 53. Results: 285 paired assessments were available. The mean total PUL 2.0 12-month change was −0.67 (2.80), −1.15 (3.98), −1.46 (3.37) and −1.95 (4.04) in patients carrying mutations amenable to skipping exon 44, 45, 51 and 53, respectively. The mean total PUL 2.0 24-month change was −1.47 (3.73), −2.78 (5.86), −2.95 (4.56) and −4.53 (6.13) in patients amenable to skipping exon 44, 45, 51 and 53, respectively. The difference in PUL 2.0 mean changes among the type of exon skip class for the total score was not significant at 12 months but was significant at 24 months for the total score (p < 0.001), the shoulder (p = 0.01) and the elbow domain (p < 0.001), with patients amenable to skipping exon 44 having smaller changes compared to those amenable to skipping exon 53. There was no difference within ambulant or non-ambulant cohorts when subdivided by exon skip class for the total and subdomains score (p > 0.05). Conclusions: Our results expand the information on upper limb function changes detected by the PUL 2.0 in a relatively large group of DMD patients with distinct exon-skipping classes. This information can be of help when designing clinical trials or in the interpretation of the real world data including non-ambulant patients. Full article

Venetoclax, the best established BH3-mimetic, is a practice-changing proapoptotic drug in blood cancers in adults. In paediatrics the data are fewer but exciting results were recently presented in relapsed or refractory leukaemias demonstrating significant clinical activity. Importantly, the in-terventions could be potentially molecularly guided as vulnerabilities to BH3-mimetics were re-ported. Currently venetoclax is not incorporated into paediatric treatment schedules in Poland but it has been already used in patients that failed conventional therapy in Polish paediatric haemato-oncology departments. The aim of the study was to gather clinical data and correlates of all paediatric patients treated so far with venetoclax in Poland. We set out to gather this experience to help choose the right clinical context for the drug and stimulate further research. The questionnaire regarding the use of venetoclax was sent to all 18 Polish paediatric haemato-oncology centres. The data as available in November 2022 were gathered and analysed for the diagnoses, triggers for the intervention, treatment schedules, outcomes and molecular associations. We received response from 11 centres, 5 of which administered venetoclax to their patients. Clinical benefit, in most cases consistent with hematologic complete remission (CR), was reported in 5 patients out of ten, whereas 5 patient did not show clinical benefit from the intervention. Importantly, patients with CR included subtypes expected to show venetoclax vulnerability, such as poor-prognosis ALL with TCF::HLF fusion. We believe BH3-mimetics have clinical activity in children and should be available to pae-diatric haemato-oncology practitioners in well-selected applications. Full article

(1) Background: Vascular endothelial growth factor (VEGF) is essential in vasculo- and angiogenesis due to its role in endothelial cell proliferation and migration. As a vascular proliferative factor, VEGF is one of the hallmarks of cancer and, in adult populations, the relationship between genetic polymorphism and neoplasm was widely investigated. For the neonatal population, only a few studies attempted to uncover the link between the genetic polymorphism of VEGF and neonatal pathology, especially related to late-onset complications. Our objective is to evaluate the literature surrounding VEGF genetic polymorphisms and the morbidity of the neonatal period. (2) Methods: A systematic search was initially conducted in December 2022. The PubMed platform was used to explore MEDLINE (1946 to 2022) and PubMed Central (2000 to 2022) by applying the search string ((VEGF polymorphism*) and newborn*). (3) Results: The PubMed search yielded 62 documents. A narrative synthesis of the findings was undertaken considering our predetermined subheadings (infants with low birth weight or preterm birth, heart pathologies, lung diseases, eye conditions, cerebral pathologies, and digestive pathologies). (4) Conclusion: The VEGF polymorphisms seem to be associated with neonatal pathology. The involvement of VEGF and VEGF polymorphism has been demonstrated for retinopathy of prematurity. Full article

This study’s aim was two-fold: (i) to test the intra-session reliability of the one-leg balance activity test; and (ii) to assess the influence of age on reaction time (RT) and the differences between dominant and non-dominant feet. Fifty young soccer players with an average age of 12.4 ± 1.8 years were divided into two groups: younger soccer players (n = 26; 11.6 ± 0.9 years) and older soccer players (n = 24; 14.2 ± 0.8 years). Each group then completed four trials (two with each leg) of the one-leg balance activity (OLBA) to evaluate RT under a single-leg stance. Mean RT and the number of hits were calculated, and the best trial was also selected. T-tests and Pearson correlations were performed for statistical analysis. Values for RT were lower, and the number of hits was higher while standing on the non-dominant foot (p = 0.01). MANOVA revealed that the “Dominant Leg” factor did not affect the multivariate composite (Pillai Trace = 0.05; F(4, 43) = 0.565; p = 0.689; Partial ETA Squared = 0.050; Observed Power = 0.174). The “Age” factor did not present an effect on the multivariate composite (Pillai Trace = 0.104; F(4, 43) = 1.243; p = 0.307; Partial ETA Squared = 0.104; Observed Power = 0.355). The results of the present investigation demonstrate that RT may be lower while standing on the non-dominant foot. Full article

Restricted and repetitive behaviors and interests (RRBI) are a significant component in diagnosing autism spectrum disorder (ASD). They often pose the main challenge in day-to-day functions for children with ASD and their families. Research addressing family accommodation behaviors (FAB) in the ASD population is scarce, and associations with the characteristics of the children’s behaviors are unclear. This sequential mixed-methods study assessed the correlation between RRBI and FAB within the ASD group to deepen the understanding of parents’ subjective experiences regarding their children’s RRBI. It included a quantitative phase with a follow-up qualitative study. A total of 29 parents of children with autism (5–13 yr) completed the study questionnaires; a total of 15 also were interviewed regarding their children’s RRBI and related FAB. We used the Repetitive Behavior Scale-Revised (RBS-R) to assess RRBI, and the Family Accommodation Scale (FAS-RRB) to assess FAS. In-depth interviews from phenomenological methodology were used in the qualitative phase. We found significant positive correlations between the RRBI and FAB overall and their subscores. Qualitative research supports these findings, adding descriptive examples of the accommodations families make to address the RRBI-related challenges. The results indicate relations between RRBI and FAB and the importance of practically addressing children with autism’s RRBI and their parents’ experiences. Both affect and are affected by the children’s behaviors. Full article

The increasing attendance of paediatric emergency departments has become a serious health issue. To reduce an elevated burden of medical errors, inevitably caused by a high level of stress exerted on emergency physicians, we propose potential areas for improvement in regular paediatric emergency departments. In an effort to guarantee the demanded quality of care to all incoming patients, the workflow in paediatric emergency departments should be sufficiently optimised. The key component remains to implement one of the validated paediatric triage systems upon the patient’s arrival at the emergency department and fast-tracking patients with a low level of risk according to the triage system. To ensure the patient’s safety, emergency physicians should follow issued guidelines. Cognitive aids, such as well-designed checklists, posters or flow charts, generally improve physicians’ adherence to guidelines and should be available in every paediatric emergency department. To sharpen diagnostic accuracy, the use of ultrasound in a paediatric emergency department, according to ultrasound protocols, should be targeted to answer specific clinical questions. Combining all mentioned improvements might reduce the number of errors linked to overcrowding. The review serves not only as a blueprint for modernising paediatric emergency departments but also as a bin of useful literature which can be suitable in the paediatric emergency field. Full article

Antibiotics account for over 10% of the overall drug expense of the National Health System in Italy in 2021. Their use in children is of particular interest on one side, because acute infections are very common in children, while they build their immunologic library of competence; on the other side, although many acute infections are expected and turn out to be of viral origin, caregivers will often ask the family doctor or primary care attending to reassure them by prescribing antibiotic treatment, although it may often be unnecessary. The inappropriate prescription of antibiotics in children may likely be a source not only of undue economic burden for the public health system but also of increasing development of antimicrobial resistance (AMR). Based on those issues, the inappropriate use of antibiotics in children should be avoided to reduce the risks of unnecessary toxicity, increase in health costs, lifelong effects, and selection of resistant organisms causing undue deaths. Antimicrobial stewardship (AMS) describes a coherent set of actions that ensure an optimal use of antimicrobials to improve patient outcomes while limiting the risk of adverse events including AMR. The aim of this paper is to spread some concept of good use of antibiotics for pediatricians or every other physician involved in the choice to prescribe, or not, antibiotics in children. Several actions could be of help in this process, including the following: (1) identify patients with high probability of bacterial infection; (2) collect samples for culture study before starting antibiotic treatment if invasive bacterial infection is suspected; (3) select the appropriate antibiotic molecule based on local resistance and narrow spectrum for the suspected pathogen(s); avoid multi-antibiotic association; prescribe correct dosage; (4) choose the best route of administration (oral vs. parenteral) and the best schedule of administration for every prescription (i.e., multiple administration for beta lactam); (5) schedule clinical and laboratory re-evaluation with the aim to consider therapeutic de-escalation; (6) stop antibiotic administration as soon as possible, avoiding the application of “antibiotic course”. Full article

Positional abnormalities per se do not require treatment, but in their place, the accompanying pulmonary pathology in dextroposition patients and pathophysiologic hemodynamic abnormalities resulting from multiple defects in patients with cardiac malposition should be the focus of treatment. At the time of the first presentation, treating the pathophysiologic aberrations caused by the defect complex, whether it is by improving the pulmonary blood flow or restricting it, is the first step. Some patients with simpler or single defects are amenable to surgical or transcatheter therapy and should be treated accordingly. Other associated defects should also be treated appropriately. Biventricular or univentricular repair dependent on the patient’s cardiac structure should be planned. Complications in-between Fontan stages and after conclusion of Fontan surgery may occur and should be promptly diagnosed and addressed accordingly. Several other cardiac abnormalities unrelated to the initially identified heart defects may manifest in adulthood, and they should also be treated. Full article

Objective: The aim of this paper is to describe the protocol of pilot cluster randomized controlled trial (RCT) that will evaluate the effects of a lifestyle-based intervention. The Archena Infancia Saludable project will have several objectives. The primary objective of this project is to determine the 6-month effects of a lifestyle-based intervention on adherence to 24-h movement behaviors and Mediterranean diet (MedDiet) in schoolchildren. The secondary objective of this project is to test the intervention effects of this lifestyle-based intervention on a relevant set of health-related outcomes (i.e., anthropometric measurements, blood pressure, perceived physical fitness, sleep habits, and academic performance). The tertiary objective is to investigate this intervention’s “halo” effect on parents’/guardians’ 24-h movement behaviors and adherence to the MedDiet. Methods: The Archena Infancia Saludable trial will be a cluster RCT submitted to the Clinical Trials Registry. The protocol will be developed according to SPIRIT guidelines for RCTs and CONSORT statement extension for cluster RCTs. A total of 153 eligible parents/guardians with schoolchildren aged 6–13 years will be randomized into an intervention group or a control group. This project focuses on two fundamental pillars: 24-h movement behaviors and MedDiet. It will mainly focus on the relationship between parents/guardians and their children. Behavior change strategies for dietary and 24-h movement behaviors in schoolchildren will be based on healthy lifestyle education for parents/guardians through infographics, video recipes, brief video clips, and videos. Conclusions: Most of the current knowledge on 24-h movement behaviors and adherence to the MedDiet is based on cross-sectional or longitudinal cohort studies, warranting a need to design and conduct RCTs to obtain more robust evidence on the effect of a healthy lifestyle program to increase 24-h movement behaviors and to improve adherence to the MedDiet in schoolchildren. Full article

Cryptorchidism, defined as the failure of at least one or both testicles to descend into the scrotal pouches, is the most frequent (1.6–9% at birth, 1/20 males at birth) congenital anomaly encountered in newborn males, resulting in one of the most frequent causes of non-obstructive azoospermia in men. Similar to other congenital malformations, cryptorchidism is thought to be caused by endocrine and genetic factors, combined with maternal and environmental influences. The etiology of cryptorchidism is unknown, as it involves complex mechanisms aiming to control the testicular development and descent from their initial intra-abdominal location in scrotal pouches. The implication of insulin-like 3 (INSL-3) associated with its receptor (LGR8) is critical. Genetic analysis discloses functionally deleterious mutations in INSL3 and GREAT/LGR8 genes. In this literature review, we discuss and analyze the implication of INSL3 and the INSL3/LGR8 mutation in the occurrence of cryptorchidism in both human and animal models. Full article

For the treatment of osteosarcoma, cisplatin (CDDP) can be substituted by carboplatin (CBDCA) to reduce toxicity. We report a single institution experience of CBDCA-based regimen. Two to three cycles of CBDCA + ifosfamide (IFO) therapy (window therapy) were administered as neoadjuvant therapy for osteosarcoma. Depending on the response of window therapy, the subsequent protocols were determined; for good responders, surgery is performed, and postoperative therapies with CBDCA + IFO, adriamycin (ADM) and high-dose methotrexate (MTX) were administered; for stable disease, the postoperative regimens were advanced before surgery, and the remaining amount of postoperative chemotherapy is deduced; for progressive disease, CBDCA-based regimen is changed to CDDP-based regimen. From 2009 to 2019, seven patients were treated with this protocol. During the window therapy, two patients (28.6%) were assessed as good responders and completed the regimen as planned. Four patients (57.1%) had stable disease, and the chemotherapy schedules were modified. One patient (14.2%) with progressive disease was shifted to the CDDP-based regimen. At final follow-up, four patients showed no evidence of disease and three patients died of the disease. Since the efficacy during window therapy was limited, a CBDCA-based regimen in the neoadjuvant setting was considered insufficient for performing adequate surgery. Full article

Metabolic syndrome (MetS) is defined by a cluster of several cardio-metabolic risk factors, specifically visceral obesity, hypertension, dyslipidemia, and impaired glucose metabolism, which together increase risks of developing future cardiovascular disease (CVD) and type 2 diabetes mellitus (T2D). This article is a narrative review of the literature and a summary of the main observations, conclusions, and perspectives raised in the literature and the study projects of the Working Group of Childhood Obesity (WGChO) of the Italian Society of Paediatric Endocrinology and Diabetology (ISPED) on MetS in childhood obesity. Although there is an agreement on the distinctive features of MetS, no international diagnostic criteria in a pediatric population exist. Moreover, to date, the prevalence of MetS in childhood is not certain and thus the true value of diagnosis of MetS in youth as well as its clinical implications, is unclear. The aim of this narrative review is to summarize the pathogenesis and current role of MetS in children and adolescents with particular reference to applicability in clinical practice in childhood obesity. Full article

Background: Children and adolescents are likely to be exposed to various types of childhood traumatic experiences (CTEs) with gender-specific patterns. Rural-to-urban migrant children have been demonstrated a greater risk of CTE exposure than local children. However, no study has investigated sex differences in the patterns of CTEs and predictive factors among Chinese children. Methods: A large-scale questionnaire survey of rural-to-urban migrant children (N = 16,140) was conducted among primary and junior high schools in Beijing. Childhood trauma history, including interpersonal violence, vicarious trauma, accidents and injuries was measured. Demographic variables and social support were also examined. Latent class analysis (LCA) was utilized to examine patterns of childhood trauma, and logistic regression was used to examine predictors. Results: Four classes of CTEs were found among both boys and girls, labeled low trauma exposure, vicarious trauma exposure, domestic violence exposure, and multiple trauma exposure. The possibility of various CTEs in the four CTE patterns was higher among boys than girls. Sex differences also manifested in predictors of childhood trauma patterns. Conclusions: Our findings shed light on sex differences in CTE patterns and predictive factors in Chinese rural-to-urban migrant children, suggesting that trauma history should be considered along with sex, and sex-specific prevention and treatment programs should be developed. Full article

Management of children with acute liver failure is challenging. In this retrospective study, paediatric patients diagnosed with ALF at our centre, in the last 26 years, were divided into two groups (G1 = diagnosed from 1997 to 2009; G2 = from 2010 to 2022) and compared to see whether they differed with regard to aetiologies, need for liver transplantation (LT), and outcome. A total of 90 children (median age 4.6 years, range 1.2–10.4; M/F = 43/47) were diagnosed with ALF, by autoimmune hepatitis (AIH) in 16 (18%), paracetamol overdose in 10 (11%), Wilson disease in 8 (9%), and other causes in 19 (21%); 37 (41%) had indeterminate ALF (ID-ALF). Comparing the two periods, the clinical features, aetiologies, and median peak values of INR [3.8 (2.9–4.8) in G1 vs. 3.2 (2.4–4.8) in G2] were similar (p > 0.05). The percentage of ID-ALF tended to be higher in G1 compared to G2 (50% vs. 32% in G2, p = 0.09). The overall percentage of patients diagnosed with Wilson disease, inborn errors of metabolism, neonatal hemochromatosis or viral infection was higher in G2 (34% vs. 13% in G1, p = 0.02). A total of 21/90 patients (23%; 5 with indeterminate ALF) were treated with steroids; 12 (14%) required extracorporeal liver support treatment. The need for LT was significantly higher in G1 compared to G2 (56% vs. 34%; p = 0.032). Among 37 children with ID-ALF, 6 (16%) developed aplastic anaemia (all in G2, p < 0.001). The survival rate at last follow up was of 94%. On a KM curve, the transplant-free survival was lower in G1 compared to G2. In conclusion, we report a lower need for LT in children diagnosed with PALF during the most recent period compared to the first era. This suggests improvements over time in the diagnosis and management of children with PALF. Full article

The Child Friendly Cities Initiative introduced by UNICEF aims to help local governments realise child rights by utilising the UN Convention on the Rights of the Child as its foundation. Using Lundy’s model of child participation, which focuses on spaces, voice, audience, and influence, this study assesses young people’s participation as child councillors in two programmes in one city in Malaysia. Ten young people who were former child councillors in one state in Malaysia participated in this study. This study employed thematic analysis in analysing the data obtained using focus groups. Based on the data presented, it was clear that adult (the responsible party) understanding of meaningful child participation is still weak. This study offers substantial contributions to the limited body of literature on child participation in Malaysia by focusing on the difficulties of former child councillors in engaging in meaningful participation. Thus, more efforts (for example, by using participatory methods) are needed to educate the responsible party on the importance of addressing the power dynamic between children and adults so that children can participate effectively in decision-making processes. Full article

Posterior reversible encephalopathy syndrome (PRES) is a clinical and neuroimaging syndrome that can affect both children and adults and has variable etiology. It is clinically defined by headaches, consciousness disorders, seizures and visual disturbances. Early recognition (clinical and imaging) can lead to appropriate general measures to correct the underlying cause of PRES. In this paper, we report a case of PRES in an eight-year-old boy with bilateral renal hypoplasia and end-stage renal disease (ESRD). Full article