European Journal of Pediatrics最新文献

Neonatal hyperbilirubinemia is a prevalent condition, with a risk of serious complications. Phototherapy is the standard treatment for significant cases, but its limitations highlight the need for additional options. Zinc sulfate has emerged as a potential adjunctive treatment. Our objective is to evaluate the efficacy of zinc sulfate as an adjunct to phototherapy in neonates with hyperbilirubinemia. PubMed, Embase, and CENTRAL were searched for studies published up to September 2024. Eligible studies were randomized clinical trials (RCTs) enrolling neonates with hyperbilirubinemia that evaluated the combined use of phototherapy and zinc sulfate. This study followed PRISMA guidelines, with independent extraction of data by two reviewers. Risk of bias was assessed using the RoB2 tool, and the quality of evidence was evaluated using the GRADE approach. Eleven RCTs comprising 1,349 neonates were included. A total of 690 (51.1%) neonates received zinc sulfate. Zinc sulfate significantly reduced bilirubin levels at 24 h (MD = -0.76 mg/dL; 95% CI = -1.30 to -0.22; P < .01; I² = 82%), 48 h (MD = -0.88 mg/dL; 95% CI = -1.60 to -0.17; P = 0.02; I² = 76%) and at 72 h (MD = -1.19 mg/dL; 95% CI = -2.29 to -0.09; P = .003; I² = 94%). Subgroup analysis indicated that term neonates with normal birth weight benefited most from the intervention, while preterm and low-birth-weight infants showed no significant difference.Conclusion: Zinc sulfate effectively reduces serum bilirubin levels in neonates with hyperbilirubinemia when used alongside phototherapy, especially in term neonates.Trial registration number and date of registration: PROSPERO, CRD42024586259, 09/13/2024. The Impact of Zinc Sulfate on the Treatment of Neonatal Hyperbilirubinemia: An Updated Systematic Review and Meta-Analysis"; CRD42024586259; Link: https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=586259.

This study aims to develop and validate the Post-COVID Symptom Scale for Children/Youth (PCSS-C/Y), which is a comprehensive tool for measuring the symptom burden of post-COVID-19 conditions-persistent symptoms after SARS-CoV-2 infection, commonly known as Long COVID-and its impact on health-related quality of life among children and adolescents. Parents of children and adolescents, adolescents, and young adults with and without a history of COVID-19 were invited to fill in a questionnaire from October 2022 to June 2023. There were 386 valid parent proxy-reported responses, 433 valid adolescent self-reported responses, and 324 valid young adult self-reported responses included in the final analysis. The PCSS-C/Y demonstrated stable factor structure and good internal consistency in different sampling groups. The scale score was negatively associated with Paediatric Quality of Life Inventory (PedsQL) scores (young adult self-report, adjusted R² = 0.394; adolescent self-report, adjusted R² = 0.219; parent-report, adjusted R² = 0.292), while it was positively associated with Strengths and Difficulties Questionnaire (SDQ) scores (young adult self-report, adjusted R² = 0.195; adolescent self-report, adjusted R² = 0.154; parent-report, adjusted R² = 0.239). The scale can also discriminate the post-infected cases and control cases, Cohen's d = 0.41, 0.50, and 0.38 for adult self-report, adolescent self-report, and parent-report, respectively. Conclusions: The PCSS-C/Y is a valid and reliable tool for quantifying the diverse symptomatology of post-COVID-19 conditions in children and adolescents. It provides quantifiable measurements that enable clinicians to monitor post-COVID-19 symptoms in children and young people and facilitates the development of interventions for post-COVID-19 conditions.

Early debridement of partial-thickness burns and coverage with skin substitutes is currently the standard of care in children, although there is currently no "gold standard" skin substitute. Our aim is to compare the effectiveness of three different skin substitutes, analyzing the medium- and long-term outcomes.

Methods: A retrospective study was conducted on burn patients under 18 years admitted to our Burn Unit between 2015 and 2021, who were divided into 3 groups according to the type of skin substitute used (EZ-derm®, Biobrane®, and Suprathel®). Demographic and clinical data and short- and long-term outcomes were analyzed. Effectiveness was analyzed by escharectomy and grafting rate during acute management and long-term follow-up reintervention rate. A total of 378 patients were included (179 EZ-derm® group, 107 Biobrane® group, and 92 Suprathel® group). No differences in demographics or burn characteristics were observed between the groups. Patients treated with Suprathel® had a significantly shorter hospital stay (median 4 days (IQR 2-9)), a lower rate of escharectomy and grafting during acute management (21.1%), and a lower long-term follow-up reintervention rate (18.5%) when compared to the EZ-derm® group (median stay 9 days (IQR 6-13); escharectomy and graft 24.6% and reintervention 26.8%) and to the Biobrane® group (median stay 9 days (IQR 7-14); escharectomy and graft 32.1% and reintervention 26.2%).

Conclusion: Treatment of partial-thickness burns with Suprathel® is associated with a shorter hospital stay, lower need for escharectomy and grafting, and lower need for long-term reintervention. Therefore, it should be considered the treatment of choice for pediatric partial-thickness burns.

What is known: • Different types of skin substitutes are available for the treatment of skin burns in paediatric patients.

What is new: • Suprathel® is linked to a reduction in hospital stays, a lower need for escharectomy and grafting, and a lower likelihood of requiring long-term re-interventions.

Adolescent binge drinking is increasingly common. This study investigates the anomalies in glucose, sodium, calcium, potassium, and acid-base homeostasis induced by binge drinking in adolescents. The records of teenagers who sought medical attention for binge drinking (ethanol level ≥ 0.80 g/L) at the Pediatric Emergency Department, Ca' Granda Ospedale Maggiore Policlinico, Milan (Italy), spanning the years 2013 to 2023 were retrospectively analyzed. For this analysis, cases were selected if documented blood chemistry encompassed sodium, potassium, total calcium, glucose, acid-base balance, and lactic acid (only for those with metabolic acidosis). Included were 173 adolescents (female-to-male ratio 0.94), 13.2 to 18.4, median 16.4 years of age. Hypoglycemia (≤ 3.3 mmol/L; N = 1, 0.6%), hyponatremia (≤ 134 mmol/L; N = 7, 4.0%), hypernatremia (≥ 146 mmol/L; N = 3, 1.7%), hypocalcemia (≤ 2.19 mmol/L; N = 0) hypercalcemia (≥ 2.61 mmol/L; N = 0), and hyperkalemia (≥ 5.1 mmol/L; N = 0) were infrequent. Acute respiratory acidosis (pCO₂ ≥ 46 mm Hg; pH < 7.40; N = 101, 58%) was the most common acid-base imbalance, followed by respiratory alkalosis (pCO₂ ≤ 34 mm Hg; pH > 7.40; N = 10, 5.6%), and metabolic acidosis (HCO₃^- ≤ 19 mmol/L, pH < 7.40; N = 9, 5.2%). The lactic acid level was increased (≥ 2.1 mmol/L) in all cases with metabolic acidosis. Metabolic alkalosis (HCO₃^- ≥ 28 mmol/L, pH > 7.40) never occurred. Hypokalemia (≤ 3.4 mmol/L; N = 56, 32%) was prevalent, particularly in adolescents with normal acid-base equilibrium or metabolic acidosis, rather than respiratory acidosis or alkalosis.Conclusion: Adolescents who engage in binge drinking often experience a disrupted acid-base balance and hypokalemia, while glucose, sodium and calcium levels are rarely affected. What is known? • Binge drinking is becoming increasingly common among adolescents. • Conflicting data regarding the type and prevalence of biochemical disorders induced by binge drinking are available in this age group. What is new? • Acute respiratory acidosis is prevalent in adolescents with binge drinking, whereas respiratory alkalosis, metabolic acidosis, and hypoglycemia are uncommon. • Hypokalemia develops frequently.

This study aimed to evaluate the association between microcephaly and hearing disorders in children with exposed or suspected exposure to Zika virus (ZIKV) during the intrauterine period. In this cross-sectional study, we enrolled children exposed or suspected of being exposed to ZIKV during intrauterine period, admitted to the hospital between April 2016 and July 2018, and followed up until September 2021. All children underwent at least one automated auditory brainstem response (AABR) test. For analysis, the patients were divided into four groups: those with microcephaly, without microcephaly, suspected ZIKV infection, and controls. Other causes of microcephaly were excluded. Hearing impairment was assessed using the AABR to determine associations with microcephaly or central nervous system (CNS) abnormalities. Of the 134 children included, 34 (25.4%) were diagnosed with congenital Zika syndrome (CZS), of whom 28 (82.4%) had microcephaly, and the remaining six (17.6%) without microcephaly. Among the 28 children with microcephaly, 3 (10.7%) had abnormal AABR. Among CZS children without microcephaly (n = 6), 1 (16.7%) had abnormal AABR (Fisher's exact test p = 0.56).Conclusion: In our study population, that hearing impairment assessed using the AABR test was not associated with microcephaly or severe CNS alterations.

The aim of this study was to translate, adapt, and psychometrically evaluate the Quality of Life in a Child's Chronic Disease Questionnaire (QLCCDQ) for the Swedish context. The QLCCDQ was translated into Swedish and adapted to the Swedish context. Data for psychometric testing were obtained through a survey of parents of children and adolescents (n = 627) with chronic diseases: asthma and type 1 diabetes mellitus, with a total of 173 responses (27.6%). Face and content validity of the instrument was assessed, and floor and ceiling effects were measured. Internal consistency was measured with Cronbach's alpha and an exploratory factor analysis (EFA) was conducted. The EFA gave a two-factor solution with an eigenvalue > 1 explaining 73.9% of total variance for the Swedish version. The new subscales are family life and activities (eight questions) and emotions and symptoms (four questions). Three questions concerning anxiety, worry, and guilt loaded < 0.6 and were excluded.

Conclusion: The study concludes that the Swedish version of the QLCCDQ is a reliable and valid questionnaire. The instrument may be useful for clinical screening of families who have the greatest need for supportive interventions. However, this should be further evaluated.

What is known: • A child's chronic disease influences quality of life of its family members. • Few instruments are designed to measure the impact on families.

What is new: • The Swedish version of the Quality of Life in a Child's Chronic Disease Questionnaire has two subscales compared to the original's five. • The instrument may potentially be useful for clinical screening of families who have the greatest need for supportive interventions.

Communication skills are a vital but often neglected part of paediatric training. To make communication training more responsive to patient needs, children and adolescents should be involved in developing the communication curriculum for healthcare professionals, though this is rarely the case. The present study explored children and adolescents' perceptions of healthcare professionals to identify recommendations for healthcare professionals to contribute to formulating goals, learning objectives, and competencies for an interprofessional paediatric communication curriculum. We used narrative and play-based interviews to include the perceptions of preschool children aged 3-6 years (n = 8) and an online questionnaire to explore those of schoolchildren and adolescents aged 5-18 years (n = 54). We did a thematic analysis of the qualitative interview data and open-ended questionnaire responses, which showed that preschool children found familiar approaches, physical contact, and their parents comforting and that healthcare professionals should use playful methods, child-friendly words, and tangible rewards. Schoolchildren and adolescents preferred healthcare professionals who were friendly, patient, attentive, communicated clearly, and engaged them in conversation. They did not like when healthcare professionals appeared stressed, did not keep their promises, or forced them to do something.

Conclusions: We condensed perceptions of children and adolescents into tips and statements to be used in further development of a communication curriculum for paediatric healthcare professionals. Our findings emphasize that paediatric communication training should focus on healthcare professionals' attitude and appearance, strategies for building trust and engaging patients in treatment and care, the use of age-appropriate communication, and understanding the cognitive development of children and adolescents.

What is known: • Communication is a core competence that all paediatric healthcare professionals must practice and maintain. • Children and adolescents can contribute to curriculum development, but only few studies have done so.

What is new: • The perspectives of children and adolescents indicate that education programmes on paediatric communication should focus on the attitude and appearance of healthcare professionals, strategies to build trust and engage patients, age-appropriate communication, and understanding the cognitive development of children and adolescents. • Children and adolescents aged 3-18 years can contribute to the development of goals, learning objectives, and competencies for paediatric communication training, which may help promote more patient-centred approaches in practice.

To evaluate the effects of cytochrome P450 family 2 subfamily C member 9 (CYP2C9) and cytochrome P450 family 2 subfamily C member 19 (CYP2C19) polymorphisms on the plasma concentrations, efficacy, and safety of lacosamide (LCM) among pediatric patients with epilepsy. This prospective study was conducted at two institutions. It included 215 pediatric patients with epilepsy who were under LCM. LCM plasma concentrations were quantified using validated ultra-performance liquid chromatography. CYP2C9 and CYP2C19 polymorphisms were analyzed in all pediatric patients in our hospital's Institute of Clinical Pharmacy research laboratory through polymerase chain reaction, agarose gel electrophoresis detection, gel recovery, and other steps. Seizure frequencies were recorded 3, 6, and 12 months after initiating LCM therapy and compared with the baseline monthly frequency. Clinical information, including efficacy, toxicity, and concomitant drugs, was collected. A total of 158 pediatric patients (73.5%) responded to LCM therapy. Of them, 77 patients reported adverse events while under LCM. The LCM plasma concentration was linearly correlated with its daily dose (r = 0.26, p < 0.001). Patients with adverse events reported higher LCM plasma concentrations (7.9 ± 4.0 µg/mL) than patients without adverse events (6.8 ± 3.0 µg/mL; p < 0.05). The poor metabolizer (PM) group demonstrated the highest concentration-to-dose ratio (1.7 ± 0.7 μg·mL^-1·kg·mg^-1) than the extensive metabolizer, intermediate metabolizer, and ultra-rapid metabolizer groups (0.8 ± 0.4, 1.0 ± 0.5, and 0.8 ± 0.4 μg·mL^-1·kg·mg^-1, respectively). The PM group comprised the highest proportion of patients with effective LCM (9/11, 81.8%) and adverse events (7/11, 63.6%).

Conclusion: LCM plasma concentrations were strongly associated with its clinical efficacy and toxicity. CYP2C19 polymorphisms affect the plasma concentration and treatment efficacy in pediatric patients with epilepsy. CYP2C19 PMs with two no-function alleles are likely to have higher LCM plasma concentrations.

What is known: • LCM is metabolized by CYP2C19, CYP2C9, and CYP3A4 into pharmacologically inactive O-desmethyl-lacosamide; it primarily undergoes renal elimination. • Plasma LCM concentrations in patients treated with the recommended dose vary widely between and within individuals variability.

What is new: • CYP2C19 polymorphisms affect the plasma concentration and treatment efficacy in Chinese pediatric patients with epilepsy. • CYP2C19 PMs with two no-function alleles are likely to have higher plasma LCM concentrations.

Glutaric aciduria type 1 (GA1) is a rare metabolic disorder characterized by a deficiency in the enzyme glutaryl-CoA dehydrogenase. This study aims to present the clinical, biochemical, genetic, and neuroimaging findings of GA1 patients, emphasizing the importance of early detection and the potential benefits of incorporating GA1 into NBS programs. The demographic, clinical, and laboratory findings of GA1 patients were reviewed retrospectively. This study presents the clinical, biochemical, genetic, and neuroimaging findings of 15 patients (six males, nine females) from 13 families diagnosed with GA1. The median age at diagnosis was 20 months, and the median follow-up period was 72 months. Developmental delay was observed in 66.7% of patients, with 46.7% experiencing seizures and 33.3% suffering from encephalopathic crises. Biochemical analyses showed elevated levels of glutaric acid and 3-hydroxyglutaric acid in 93.3% and 80% of patients, respectively. Genetic testing identified the p.Arg402Trp variant in 53.3% of patients. Neurological evaluations revealed delays in motor and speech development, with 66.7% of patients never achieving the ability to walk. Cranial MRI indicated white matter changes in all patients and basal ganglia involvement in 93.3%. Despite significant biochemical improvements with treatment in glutaric acid levels and head circumference over time, neurological deficits remain unchanged. Growth parameters such as body weight showed significant decreases due to poor neurological outcomes.

Conclusion: The study underscores the importance of early diagnosis and intervention to mitigate severe neurological outcomes. Our findings highlight the need for incorporating GA1 into newborn screening programs to ensure timely diagnosis and treatment.

What is known: • Glutaric aciduria type 1 (GA1) is a rare metabolic disorder caused by a deficiency of glutaryl-CoA dehydrogenase. If untreated, it often leads to severe neurological complications. Early diagnosis and treatment are crucial for improving clinical outcomes in GA1 patients.

What is new: • This study presents comprehensive data from a cohort of 15 Glutaric aciduria type 1 (GA1) patients, detailing their biochemical, genetic, clinical, and neuroimaging findings. Drawing attention to the severe neurological findings in late-diagnosed patients underscores the critical importance of including GA1 in newborn screening programs to enhance early diagnosis and prevent severe outcomes.