Fatores intervenientes na gerência do cuidado de enfermagem à criança hospitalizada com cardiopatia reumática / Intervening factors in the management of nursing care for children hospitalized with rheumatic heart disease / Factores que intervienen en la gestión del cuidado de enfermería al niño hospitalizado con cardiopatía reumática

Objetivo: analisar os fatores intervenientes na gerência do cuidado de enfermagem à criança hospitalizada com cardiopatia reumática. Método: estudo descritivo-exploratório com abordagem qualitativa, que utilizou a Teoria Fundamentada em Dados e o Interacionismo Simbólico, respectivamente, como referencial metodológico e teórico. A coleta de dados foi realizada em uma instituição especializada em atendimento cardiológico, no munícipio do Rio de Janeiro. Foram entrevistados 19 profissionais de enfermagem através de um roteiro semiestruturado. Resultado: emergiram os seguintes fatores intervenientes na prática da gerência do cuidado: condição socioeconômica da família, comportamento da criança, condições de trabalho, comunicação ineficaz, educação permanente, trabalho em equipe e experiência profissional. Conclusão: os resultados apontam para a necessidade de proposição de estratégias de ação e interação que facilitem a prática gerencial de cuidado à criança com cardiopatia reumática e sua família face aos fatores intervenientes identificados.

Objective: to analyze the factors involved in the management of nursing care for children hospitalized with rheumatic heart disease. Method: this is a descriptive-exploratory study with a qualitative approach, which used Data-Based Theory and Symbolic Interactionism, respectively, as methodological, and theoretical references. Data was collected in an institution specializing in cardiac care in the city of Rio de Janeiro. Nineteen nursing professionals were interviewed using a semi-structured script. Result: the following intervening factors in the practice of care management emerged: the family's socioeconomic status, the child's behavior, working conditions, ineffective communication, continuing education, teamwork, and professional experience. Conclusion: the results point to the need to propose strategies for action and interaction that facilitate management practice in caring for children with rheumatic heart disease and their families, given the intervening factors identified.

Objetivo: analizar los factores que intervienen en la gestión del cuidado de enfermería al niño hospitalizado con cardiopatía reumática. Método: estudio descriptivo-exploratorio con enfoque cualitativo, cuyos marcos metodológico y teórico fueron la Teoría Fundamentada y el Interaccionismo Simbólico, respectivamente. La recolección de datos se realizó en una institución especializada en atención cardiológica, en la ciudad de Río de Janeiro. Fueron entrevistados 19 profesionales de enfermería mediante un cuestionario semiestructurado. Resultado: surgieron los siguientes factores intervinientes en la práctica de la gestión del cuidado: condición socioeconómica de la familia, comportamiento del niño, condiciones de trabajo, comunicación ineficaz, educación continua, trabajo en equipo y experiencia profesional. Conclusión: los resultados indican que es necesario proponer estrategias de acción e interacción que faciliten la práctica de la gestión del cuidado al niño con cardiopatía reumática y a sus familiares, con respecto a los factores intervinientes identificados.

Analysis of outcomes of robot-assisted laparoscopic pyeloplasty in children from a tertiary pediatric center in South India.

Aim: This study aims to analyze the outcomes of robot-assisted laparoscopic pyeloplasty (RALP) in children with pelvi-ureteric junction obstruction (PUJO) over a 10-year period at a tertiary care center in South India. Methods: This study provides a detailed analysis of prospectively acquired data from 2013 to 2023 of all children who underwent RALP at our institution. Pre- and post-operative renal ultrasound and isotope renography were used to assess outcomes. Detailed information on patient demographics, procedural duration, post-operative pain relief, operation steps, and post-surgical follow-up protocols has been provided. The analysis included all patients who completed a 1-year follow-up. Result: Between 2013 and 2023, 201 children underwent RALP. Of these, 185 children completed at least 1 year of follow-up and were included in the analysis. The mean age of the cohort was 4.9 years (1 month to 17 years), with males comprising the majority (77.3%). Twenty-five children (13.5%) were younger than 1 year of age. Left-sided PUJO was found to be more common. The mean console time was 76.5 min (40-180 min), and the average hospital stay was 2.8 days (2-5). After surgery, the mean reduction in antero-posterior diameter of the renal pelvis was more than 50% of its pre-operative value and statistically significant (3.3 ± 0.3 to 1.9 ± 0.9 cm). At the end of 1 year, the overall reduction in renal size was also significant (9.7 ± 2.3 cm pre-operative to 8.9 ± 1.8 cm post-operative). The pre-operative Society of Fetal Urology (SFU) grade of hydronephrosis was compared to the post-operative SFU grade, and the improvement (resolution/downgrading) was found to be statistically significant. The median split renal function in this series was 39% pre-operative and 43% post-operative, and the overall functional improvement after RALP was significant. A successful outcome was observed in 181 children (97.8%). Four children experienced persistent severe hydronephrosis and underwent redo stenting and/or redo pyeloplasty (2.1% failure rate). Post-operative complications, according to the Clavien-Dindo classification, were classified as type 1 in three children and type 3b in two children. There were no conversions to open surgery in the series. Conclusion: RALP emerges as the minimally invasive procedure of choice for children with PUJO at our institution. It is safe, delivering consistently excellent results and minimal complications. Our outcomes are comparable to those of previously published series. We trust that our experience will serve as a roadmap for those centers (especially in South Asia) embarking on a pediatric robotic program.

Grisel's Syndrome After COVID-19 in a Pediatric Patient: A Case Report.

An eight-year-old female presenting with posterior neck pain and torticollis who had been diagnosed with coronavirus disease 2019 (COVID-19) three weeks earlier was radiographed and diagnosed with atlantoaxial rotatory fixation (AARF). Following treatment with non-steroidal anti-inflammatory drugs (NSAIDs), the posterior neck pain improved, and the torticollis was cured. Symptoms returned after two weeks, and computed tomography showed a 3.94 mm atlantodental interval and axis rotation. The patient was diagnosed with AARF relapse; symptoms resolved spontaneously prior to subsequent examination, and no further relapses were observed. This case highlights the need for clinicians to be aware that AARF may develop after COVID-19. Treatment options should be carefully considered.

Age-Related Patterns of Traumatic Facial Fractures in the Appalachian Tri-state Area: A Five-Year Retrospective Study.

Introduction Traumatic facial injuries, leading to facial fractures represent a significant subset of traumatic events, with age emerging as a crucial determinant influencing both their etiology and outcomes. Understanding the age-related patterns of traumatic facial fractures is essential for developing targeted prevention and management strategies. In this context, the Appalachian tri-state area stands as an underexplored region concerning this issue, necessitating comprehensive research to elucidate the nuances of age-related traumatic facial fractures within this geographic context. Methods This retrospective study delves into the age-related patterns of traumatic facial fractures within the Appalachian tri-state area, drawing upon patient records from Cabell Huntington Hospital and Saint Mary's Medical Center spanning a five-year period. The study cohort encompasses 623 patients categorized into three age groups: individuals aged <22 years, those aged 22-65 years, and individuals over 65 years. Data analysis involves meticulous examination of mechanisms of injury, injury severity scores (ISSs), hospital length of stay, and the prevalence of surgical interventions across different age cohorts. Results Out of 623 patients, 104 (16.7%) were under 22 years old, 367 (58.9%) were between 22 and 65 years old, and 152 (24.4%) were over 65 years old. The majority were male (70%). Falls were the most common cause of facial fractures in patients over 65 (78%), while assaults were predominant in the 22-65 age group (24%), and motor vehicle collisions (MCVs) in those under 22 (34%). The median ISS and hospital stay durations were similar across age groups. 28% of patients underwent surgery, with significant variation among age groups (p<0.001): 38% for <22 years, 33% for 22-65 years, and 11% for >65 years. Mandibular fractures were more prevalent in younger patients, with rates of 12% for <22 years compared to 5.3% for >65 years. Logistic regression analysis revealed that patients aged 22-65 had 4.10 times higher odds (95% CI=2.38, 7.45, p<0.001) of undergoing surgery, while those under 22 had 5.14 times higher odds (95% CI=2.73, 10.0, p<0.001) compared to those over 65. Significant associations were found for mandibular and bilateral mandibular outcomes in patients aged 22-65 years. Discussion These findings underscore the imperative for tailored prevention strategies and age-specific treatment protocols to optimize patient outcomes. Fall prevention initiatives for the elderly and interventions addressing sports-related injuries for younger individuals are paramount. Moreover, the study highlights the necessity of specialized care protocols for elderly patients to minimize hospital stay durations and manage age-related comorbidities effectively. Moving forward, further research should address limitations, validate findings, and explore the efficacy of specific interventions, thereby paving the way for enhanced preventive measures and management strategies tailored to the diverse age cohorts affected by traumatic facial fractures in the Appalachian region.

Autosomal Recessive Infantile Hyaline Fibromatosis Identified Using Artificial Intelligence-Assisted Rapid Whole Genome Sequencing: A Rare, Multisystemic, Hereditary Disorder.

Infantile hyaline fibromatosis syndrome (HFS) is an ultra-rare genetic condition characterized by the deposition of hyaline material in the skin, muscle, and viscera. Potential complications include debilitating joint contractures, coarse facial features, recurrent infections, failure to thrive, and death. Here, we present the case of a six-month-old infant with a history of painful extremity contractures, global developmental delay, neck hemangioma, and feeding intolerance presenting to our institution with abdominal distension. The multi-systemic, rapidly progressing, severe nature of her symptoms prompted consultation with inpatient pediatric genetics. Per their recommendation, rapid whole-genome sequencing (rWGS) was done with Fabric GEM®-assisted artificial intelligence (Fabric Genomics, Oakland, California, United States) at Rady Children's Hospital Institute for Genomic Medicine (San Diego, California, United States), revealing homozygous pathogenic variant c.652T>C; P.Cys218Arg in the ANTXR2 gene consistent with HFS. This case was significant not only for its rarity, but also its early manifestation of symptoms, wide range of affected body systems, and severity of symptoms, which together present a fascinating diagnostic dilemma for future clinicians that should be taken into consideration. It also highlights the increasing utility of AI-assisted rWGS as a diagnostic tool for medically complex patients with unknown multisystemic hereditary conditions.

Parental Knowledge, Attitudes, and Practices Towards Pediatric Ear Infections in the Makkah Region of Saudi Arabia: A Cross-Sectional Study.

BACKGROUND:  Pediatric ear infections constitute a significant public health concern worldwide, adversely impacting children's health and well-being. Parents play a crucial role in prevention, ensuring timely healthcare access, and therefore minimizing potential complications. This study aims to assess parental knowledge, attitudes, and practices towards pediatric ear infections in Makkah region. METHODOLOGY:  A descriptive cross-sectional study was conducted among Saudi parents who were ≥18 years old and lived in Makkah region. Convenience sampling was used to recruit 319 participants through social media platforms; data were collected from June to September 2023 via an online self-administered questionnaire. The questionnaire assessed sociodemographic characteristics, along with knowledge, attitudes, and practices related to pediatric ear infections. RESULTS:  A total of 319 parents were included in the study. The majority of the participants were female 228 (71.5%), and 208 (65.2%) had a university education level. The most common age groups were 18-30 years and 31-40 years. More than half of the participants (167, 52.4%) demonstrated adequate knowledge regarding pediatric ear infections. Positive attitudes and practices were reported by 183 (57.4%) and 285 (89.3%) of participants, respectively. Adequate knowledge was significantly higher among participants with younger ages (p<0.05). It was found that having a younger age (18-30 years) was an independent predictor of good knowledge (OR: 1.26 (1.96-3.65), p=0.009) and positive practice (OR: 1.53 (1.01-2.33), p=0.045). CONCLUSION:  We found that the majority of parents in Makkah region had a good level of knowledge regarding childhood ear infections, with an overall positive attitude and practice. The study revealed that younger parents had superior knowledge and younger age was an independent predictor for good knowledge and positive attitude.

Lhermitte-Duclos Disease in an Eight-Year-Old Boy: A Case Report.

Lhermitte-Duclos disease (LDD), also known as dysplastic cerebellar gangliocytoma, is a rare, slow-growing, benign lesion that occurs in the cerebellum and is very uncommon in the pediatric population. There is a lack of literature and evidence about LDD management, and only one systematic review is available. Thus, more case reports and studies are warranted. This study reports a pediatric case diagnosed with LDD and describes the patient's clinical presentation, radiological findings, and histopathological criteria. In addition, important aspects of the disease are discussed to help reach the best management options. The main management option is surgical resection, though a "wait and see" approach is also an alternative, especially for asymptomatic patients. More studies are still needed to determine the best management options.

Surgical Correction of Ankyloglossia Using Diode Laser-Assisted Frenectomy in a Pediatric Patient: A Case Report.

Tongue-tie is a continuation of the lingual frenum that is attached to the tip of the tongue. It is a congenital oral anomaly that could restrict tongue movements, caused by a lingual frenum a membrane that originates from the floor of the mouth to the bottom of the tongue that is too thick and short, which limits the natural ability of the tongue to move and function. The tongue is an auxiliary organ that facilitates speaking, mastication, and deglutition. This condition may result in several difficulties including chewing, breastfeeding, speech, and pronunciation of particular words, as well as possessing social and mechanical consequences. Ankyloglossia can be seen in young age groups. The use of lasers has increased in dentistry in recent years. However, in oral and maxillofacial surgery, the use of lasers has been largely restricted to soft tissues, and less focus is placed on the use of hard tissues. Carbon dioxide (CO2) lasers, erbium-doped yttrium aluminum garnet (Er: YAG) lasers, and Er, the erbium, chromium: yttrium: scandium gallium-garnet (Cr: YSGG) lasers are among the several types of lasers that have been utilized in dentistry for correction of soft tissues as well as for hard tissues.

Use of allopurinol to manage skewed 6-mercaptopurine metabolism in pediatric maintenance acute lymphoblastic leukemia treatment.

BACKGROUND: 6-mercaptopurine is a cornerstone of maintenance therapy for pediatric ALL. Response to 6MP is typically determined by the ANC. Therapeutic ANC range while receiving 6MP is between 500 and 1500/µL. In addition to desired myelosuppression, 6MP is associated with multiple adverse drug effects. Increased doses of 6MP can lead to therapeutic ANC values; however, patients may experience adverse effects before obtaining therapeutic myelosuppression, often deemed "skewed metabolism." Allopurinol may potentially correct skewed 6MP metabolism. PROCEDURE: Pediatric patients with ALL with 6MMP and 6TGN metabolites drawn during maintenance therapy were analyzed for allopurinol use. The primary outcome evaluated the percentage of time spent in therapeutic ANC range before and after allopurinol initiation. In addition, the difference in 6MMP:6TGN ratios before and after allopurinol initiation, incidence of hepatotoxicity, and rates of relapse, were analyzed. RESULTS: Ninety-five patients were included for analysis. Thirty-two (34%) patients received allopurinol. There were no significant differences in baseline demographics between the patients who received allopurinol and those who did not. When comparing ANC values pre- and post-allopurinol initiation, a statistically significant increase in the percentage of time spent in therapeutic range was observed (27% vs. 43%; p = .03). In addition, when comparing metabolite ratios pre- and post-allopurinol initiation, a statistically significant decrease in 6MMP:6TGN metabolite ratio values was observed (86.7 vs. 3.6; p < .0001). CONCLUSIONS: Allopurinol significantly increased the percent time in therapeutic ANC range and can be safely utilized to significantly lower the ratio of 6MMP:6TGN metabolites, alleviating the undesirable side effects of 6MMP, and optimizing the anti-leukemic effects associated with 6TGN.

Improving Parental Health Literacy in Primary Caregivers of 0- to 3-Year-Old Children Through a WeChat Official Account: Cluster Randomized Controlled Trial.

Background: Parental health literacy is important to children's health and development, especially in the first 3 years. However, few studies have explored effective intervention strategies to improve parental literacy. Objective: This study aimed to determine the effects of a WeChat official account (WOA)-based intervention on parental health literacy of primary caregivers of children aged 0-3 years. Methods: This cluster randomized controlled trial enrolled 1332 caregiver-child dyads from all 13 community health centers (CHCs) in Minhang District, Shanghai, China, between April 2020 and April 2021. Participants in intervention CHCs received purposefully designed videos via a WOA, which automatically recorded the times of watching for each participant, supplemented with reading materials from other trusted web-based sources. The contents of the videos were constructed in accordance with the comprehensive parental health literacy model of WHO (World Health Organization)/Europe (WHO/Europe). Participants in control CHCs received printed materials similar to the intervention group. All the participants were followed up for 9 months. Both groups could access routine child health services as usual during follow-up. The primary outcome was parental health literacy measured by a validated instrument, the Chinese Parental Health Literacy Questionnaire (CPHLQ) of children aged 0-3 years. Secondary outcomes included parenting behaviors and children's health outcomes. We used the generalized linear mixed model (GLMM) for data analyses and performed different subgroup analyses. The ß coefficient, risk ratio (RR), and their 95% CI were used to assess the intervention's effect. Results: After the 9-month intervention, 69.4% (518/746) of caregivers had watched at least 1 video. Participants in the intervention group had higher CPHLQ total scores (ß=2.51, 95% CI 0.12-4.91) and higher psychological scores (ß=1.63, 95% CI 0.16-3.10) than those in the control group. The intervention group also reported a higher rate of exclusive breastfeeding (EBF) at 6 months (38.9% vs 23.44%; RR 1.90, 95% CI 1.07-3.38) and a higher awareness rate of vitamin D supplementation for infants younger than 6 months (76.7% vs 70.5%; RR 1.39, 95% CI 1.06-1.82). No significant effects were detected for the physical score on the CPHLQ, breastfeeding rate, routine checkup rate, and children's health outcomes. Furthermore, despite slight subgroup differences in the intervention's effects on the total CPHLQ score and EBF rate, no interaction effect was observed between these subgroup factors and intervention factors. Conclusions: Using a WHO literacy model-based health intervention through a WOA has the potential of improving parental health literacy and EBF rates at 6 months. However, innovative strategies and evidence-based content are required to engage more participants and achieve better intervention outcomes.

Survey Data and Experience from a Pediatric Pathology Workshop in Indonesia: Understanding Practice Needs and Utility of Outreach Teachings.

Objective: We aimed to share the post-workshop survey results of a pediatric pathology course held in Jakarta, Indonesia. Methods: Questionnaires were distributed to participants; responses from practicing pathologists and pathologists-in-training were analyzed. Results: The respondents (107 pathologists of 143 attendees) were predominantly female (83.2%) and 31-60 years of age (77.5%). Over half (71.7%) signed out pediatric and perinatal specimens but only a third (34.3%) were comfortable handling such cases. Most (70.0%) felt that their exposure to pediatric and perinatal cases during their training was inadequate. All respondents thought that the workshop was helpful, and would highly recommend it to their colleagues. Post-workshop, the respondents claimed expansion of differential diagnoses (49.5%) and better understanding of what to include in pathology reports (41.1%). Conclusions: Our experience affirms the need for subspecialty courses to address training gaps in developing countries. Post-workshop surveys are helpful in determining actionable deficiencies and effectiveness of outreach teachings.

Cardiometabolic Health in Adolescents with Obesity: The Role of Protein Intake, Diet Quality, and Physical Activity.

Background: Although adolescents with obesity have an increased risk of cardiometabolic disease, a subset maintains a healthy cardiometabolic profile. Unhealthy lifestyle behaviors may determine cardiometabolic risk. We aimed to characterize the lifestyle behaviors of adolescents with obesity, compare differences between metabolically healthy obesity (MHO) and metabolically unhealthy obesity (MUO), and assess associations between lifestyle behaviors and cardiometabolic profiles. Methods: Participants aged 10-18 years with body mass index (BMI) ≥ 95th percentile were included. Dietary intake (DI) was estimated from 3-day food records, and diet quality (DQ) was assessed using the Healthy Eating Index-Canadian Adaptation. Physical activity (PA), body composition, anthropometrics, blood markers, and blood pressure (BP) were objectively measured. MUO was defined as having high triglycerides, BP, glucose, or low high-density lipoprotein. Regression analyses were performed between lifestyle behaviors and cardiometabolic markers. Results: Thirty-nine participants (BMI z-score 2.8 [2.5-3.5], age 12.5 [10.9-13.5] years, 56.4% female) were included. A high proportion of participants failed to meet lifestyle recommendations, particularly for DQ (94.7%, n = 36), fiber (94.7%, n = 36), and PA (90.9%, n = 30). No differences in lifestyle behaviors were found between MUO (59.0%, n = 22) and MHO (41.0%, n = 16). Protein intake was negatively associated with BMI and waist circumference z-scores, fat mass index, insulin resistance, low-density lipoprotein, and C-reactive protein, whereas higher DQ was associated with lower C-reactive protein. Higher light PA levels were associated with lower total cholesterol and triglycerides. Conclusion: Adolescents with either MUO or MHO displayed low adherence to DQ, DI, and PA recommendations; no differences in lifestyle behaviors were found. Protein intake, DQ, and PA were associated with a healthier cardiometabolic profile.

Urology HEIRS: A Feasibility and Acceptability Study for Video-Based Research on Physician-Family Communication in Pediatric Urology Visits.

PURPOSE: Patient- and family-centered communication is essential to healthcare equity. However, less is known about how urologists implement evidence-based communication and dynamics involved in caring for diverse pediatric patients and caregivers. We sought to evaluate the feasibility and acceptability using video-based research to characterize physician-family communication in pediatric urology. MATERIALS AND METHODS: We assembled a multidisciplinary team to conduct a multi-phase learning health systems project to establish the Urology HEIRS (Health Experiences and Interactions in Real-time Studies) corpus for research and interventions. This paper reports the first phase, evaluating feasibility and acceptability based on consent rate, patient diversity, and qualitative identification of verbal and paraverbal features of physician-family communication. We used applied conversation analysis methodology to identify salient practices across eight pediatric urologists. RESULTS: We recruited 111 families at two clinic sites, of these 82 families (N = 85 patients, ages 0-20) participated in the study with a consent rate of 73.9%. The racial/ethnic composition of the sample was 45.9% non-Hispanic White, 30.6% any race of Hispanic origin, 16.5% non-Hispanic Black/African American, 4.7% any ethnicity of Asian/Asian American, 2.3% some other race/ethnicity, and 24.7% of families used interpreters. We identified 11 verbal and paraverbal communication practices that impacted physician-family dynamics, including unique challenges with technology-mediated interpreters. CONCLUSIONS: Video-based research is feasible and acceptable with diverse families in pediatric urology settings. The Urology HEIRS corpus will enable future systematic studies of physician-family communication in pediatric urology and provides an empirical basis for specialty-specific training in patient- and family-centered communication.

Scope of services provided to childhood cancer patients by the Brazilian Pediatric Palliative Care Network.

Introduction: Brazil is a developing and an Upper Middle Income, categorized by the World Bank. Therefore, it is a country that needs a special vision for children with oncological diseases who require Pediatric Palliative Care. This study aimed to understand the specificities of services that provide oncology services in comparison to those that do not provide oncological care. Methods: This is a descriptive, cross-sectional, and online survey study. A questionnaire was created by a multidisciplinary group of leaders from the Brazilian Pediatric Palliative Care Network and then the survey was distributed using a snowball strategy. Results: Of the 90 services that answered the questionnaire, 40 (44.4%) attended oncologic patients. The Southeast represented most of the services (57.57%), followed by the Northeast, with 18.89% (17 services), the South with 12.22% (11 services), and the Center West with 8.89% (8 services). No differences were observed in access to opioid prescriptions between the services. It was observed that those services that attended oncologic patients had a tendency to dedicate more time to Pediatric Palliative Care. Discussion: The distribution of services that cover oncology and those that do not, are similar in the different regions of Brazil. In Brazil, there are difficulties in accessing opioids in pediatrics: access to opioid prescriptions without differences revealed that even pediatric oncologists might have difficulty with this prescription, and this should improve. It is concluded that education in Pediatric Palliative Care is the key to improvements in the area.

Pediatric dilated cardiomyopathy: a review of current clinical approaches and pathogenesis.

Pediatric dilated cardiomyopathy (DCM) is a rare, yet life-threatening cardiovascular condition characterized by systolic dysfunction with biventricular dilatation and reduced myocardial contractility. Therapeutic options are limited with nearly 40% of children undergoing heart transplant or death within 2 years of diagnosis. Pediatric patients are currently diagnosed based on correlating the clinical picture with echocardiographic findings. Patient age, etiology of disease, and parameters of cardiac function significantly impact prognosis. Treatments for pediatric DCM aim to ameliorate symptoms, reduce progression of disease, and prevent life-threatening arrhythmias. Many therapeutic agents with known efficacy in adults lack the same evidence in children. Unlike adult DCM, the pathogenesis of pediatric DCM is not well understood as approximately two thirds of cases are classified as idiopathic disease. Children experience unique gene expression changes and molecular pathway activation in response to DCM. Studies have pointed to a significant genetic component in pediatric DCM, with variants in genes related to sarcomere and cytoskeleton structure implicated. In this regard, pediatric DCM can be considered pediatric manifestations of inherited cardiomyopathy syndromes. Yet exciting recent studies in infantile DCM suggest that this subset has a distinct etiology involving defective postnatal cardiac maturation, such as the failure of programmed centrosome breakdown in cardiomyocytes. Improved knowledge of pathogenesis is central to developing child-specific treatment approaches. This review aims to discuss the established biological pathogenesis of pediatric DCM, current clinical guidelines, and promising therapeutic avenues, highlighting differences from adult disease. The overarching goal is to unravel the complexities surrounding this condition to facilitate the advancement of novel therapeutic interventions and improve prognosis and overall quality of life for pediatric patients affected by DCM.

Validation of the Subaxial Cervical Spine Injury Classification score in children: a single-institution experience at a level 1 pediatric trauma center.

OBJECTIVE: The Subaxial Cervical Spine Injury Classification (SLIC) score has not been previously validated for a pediatric population. The authors compared the SLIC treatment recommendations for pediatric subaxial cervical spine trauma with real-world pediatric spine surgery practice. METHODS: A retrospective cohort study at a pediatric level 1 trauma center was conducted in patients < 18 years of age evaluated for trauma from 2012 to 2021. An SLIC score was calculated for each patient, and the subsequent recommendations were compared with actual treatment delivered. Percentage misclassification, sensitivity, specificity, positive (PPV) and negative predictive value (NPV), and area under the receiver operating characteristic (ROC) curve (AUC) were calculated. RESULTS: Two hundred forty-three pediatric patients with trauma were included. Twenty-five patients (10.3%) underwent surgery and 218 were managed conservatively. The median SLIC score was 2 (interquartile range = 2). Sixteen patients (6.6%) had an SLIC score of 4, for which either conservative or surgical treatment is recommended; 27 children had an SLIC score ≥ 5, indicating a recommendation for surgical treatment; and 200 children had an SLIC score ≤ 3, indicating a recommendation for conservative treatment. Of the 243 patients, 227 received treatment consistent with SLIC score recommendations (p < 0.001). SLIC sensitivity in determining surgically treated patients was 79.2% and the specificity for accurately determining who underwent conservative treatment was 96.1%. The PPV was 70.3% and the NPV was 97.5%. There was a 5.7% misclassification rate (n = 13) using SLIC. Among patients for whom surgical treatment would be recommended by the SLIC, 29.6% (n = 8) did not undergo surgery; similarly, 2.5% (n = 5) of patients for whom conservative management would be recommended by the SLIC had surgery. The ROC curve for determining treatment received demonstrated excellent discriminative ability, with an AUC of 0.96 (OR 3.12, p < 0.001). Sensitivity decreased when the cohort was split by age (< 10 and ≥ 10 years old) to 0.5 and 0.82, respectively; specificity remained high at 0.98 and 0.94. CONCLUSIONS: The SLIC scoring system recommended similar treatment when compared with the actual treatment delivered for traumatic subaxial cervical spine injuries in children, with a low misclassification rate and a specificity of 96%. These findings demonstrate that the SLIC can be useful in guiding treatment for pediatric patients with subaxial cervical spine injuries. Further investigation into the score in young children (< 10 years) using a multicenter cohort is warranted.

Comparing ventriculoatrial and ventriculopleural shunts in pediatric hydrocephalus: a Hydrocephalus Clinical Research Network study.

OBJECTIVE: When the peritoneal cavity cannot serve as the distal shunt terminus, nonperitoneal shunts, typically terminating in the atrium or pleural space, are used. The comparative effectiveness of these two terminus options has not been evaluated. The authors directly compared shunt survival and complication rates for ventriculoatrial (VA) and ventriculopleural (VPl) shunts in a pediatric cohort. METHODS: The Hydrocephalus Clinical Research Network Core Data Project was used to identify children ≤ 18 years of age who underwent either VA or VPl shunt insertion. The primary outcome was time to shunt failure. Secondary outcomes included distal site complications and frequency of shunt failure at 6, 12, and 24 months. RESULTS: The search criteria yielded 416 children from 14 centers with either a VA (n = 318) or VPl (n = 98) shunt, including those converted from ventriculoperitoneal shunts. Children with VA shunts had a lower median age at insertion (6.1 years vs 12.4 years, p < 0.001). Among those children with VA shunts, a hydrocephalus etiology of intraventricular hemorrhage (IVH) secondary to prematurity comprised a higher proportion (47.0% vs 31.2%) and myelomeningocele comprised a lower proportion (17.8% vs 27.3%) (p = 0.024) compared with those with VPl shunts. At 24 months, there was a higher cumulative number of revisions for VA shunts (48.6% vs 38.9%, p = 0.038). When stratified by patient age at shunt insertion, VA shunts in children < 6 years had the lowest shunt survival rate (p < 0.001, log-rank test). After controlling for age and etiology, multivariable analysis did not find that shunt type (VA vs VPl) was predictive of time to shunt failure. No differences were found in the cumulative frequency of complications (VA 6.0% vs VPl 9.2%, p = 0.257), but there was a higher rate of pneumothorax in the VPl cohort (3.1% vs 0%, p = 0.013). CONCLUSIONS: Shunt survival was similar between VA and VPl shunts, although VA shunts are used more often, particularly in younger patients. Children < 6 years with VA shunts appeared to have the shortest shunt survival, which may be a result of the VA group having more cases of IVH secondary to prematurity; however, when age and etiology were included in a multivariable model, shunt location (atrium vs pleural space) was not associated with time to failure. The baseline differences between children treated with a VA versus a VPl shunt likely explain current practice patterns.