Pediatrics International最新文献

Background: In Japan, as several important vaccines are still categorized as voluntary vaccines that require out-of-pocket payment, the vaccination coverage of voluntary vaccines is lower than that of routine vaccines. Thus, higher voluntary vaccination rates are desired. Herein, we used nationwide birth cohort data to create a voluntary vaccine risk score to identify high-risk individuals who were not vaccinated with voluntary vaccines.

Methods: The data from 74,733; 73,571; and 74,360 infants were analyzed for rotavirus, mumps virus, and influenza virus vaccinations, respectively. The risk score for non-vaccination of voluntary vaccines was created from the regression coefficients of the logistic regression models.

Results: The items included for the score resulted from the analysis were the mother's drug allergy history, mother's depression history, mother's educational background, father's educational background, household income, maternal smoking during pregnancy, paternal smoking during pregnancy, fertility treatment, number of siblings, maternal drinking at 1 month of age, maternal age, and maternal nationality. The mother's drug allergy history, mother's depression history, fertility treatment, maternal drinking at 1 month of age, and maternal nationality were factors not previously reported and associated with taking voluntary vaccine. The receiver operating characteristic curve of the risk score for non-vaccination of voluntary vaccines suggested that a score ≥16 predicted non-vaccinated infants for rotavirus, mumps virus, and influenza virus vaccines with 78.6%, 75.0%, and 74.5% sensitivity and 44.2%, 43.2%, and 37.1% specificity, respectively.

Conclusions: We developed a risk score for non-vaccination of voluntary vaccines consisting of 10 domains with high sensitivity but low specificity.

Background: Children have a greater risk of mental health problems in low/middle-income countries. Determining the risk factors and earlier identification of young children at high risk for behavioral problems are central to cost-effective intervention with positive long-term outcomes. The aim of this study was to examine the prevalence and risk factors of behavioral problems of preschoolers in three different cities located in eastern, western, and central Türkiye, a low/middle-income country.

Methods: In total, 300 children (4-6 years old) without any chronic diseases participated the study. The parents completed the Children's Behavior Checklist/4-18 (CBCL/4-18), the Children's Sleep Habits Questionnaire, the Pittsburgh Sleep Quality Index, and the Beck Depression Inventory. Daily activities were evaluated using questionnaires that were specifically developed for the present study.

Results: Based on the CBCL4-18, 12.3% of children were at high risk for behavioral problems. Paternal unemployment, screen time, maternal depressive symptoms, and maternal and child sleep problems were associated with internalizing behavioral problems; maternal depressive symptoms and watching videos on touch-screen devices were associated with externalizing behavioral problems. Total behavioral problems were associated with maternal unemployment, maternal depressive symptoms, being the eldest child, and child sleep problems.

Conclusion: Preventable risk factors such as daily activities and maternal mental health should be evaluated as well as sociodemographic factors among preschoolers who may be at high risk of behavioral problems. The results of this study contribute toward drawing attention to preventable risk factors in a low/middle-income country.

Background: ZNF384 rearrangements (ZNF384-r) are associated with distinct subgroups of B-cell precursor acute lymphoblastic leukemia (BCP-ALL) and the mixed phenotype of acute leukemia. Types of BCP-ALL with ZNF384-r exhibit common immunophenotypic characteristics, whereas their clinical features are not uniform and TCF3::ZNF384-positive patients show a significantly poorer steroid response and higher frequency of relapse, while EP300::ZNF384-positive patients exhibit a favorable response to conventional chemotherapy. Therefore, we aimed to investigate the differences in biological effects between these two ZNF384-r molecules.

Method: We transduced BCP-ALL cell lines with both TCF3::ZNF384 and EP300::ZNF384 by retrovirus-mediated gene transduction, and examined the biological effects.

Results: Flow cytometric analysis and RT-qPCR revealed down-regulation of CD10 in BCP-ALL cells after transduction with both TCF3::ZNF384 and EP300::ZNF384. The annexin-V binding apoptosis assay indicated that TCF3::ZNF384-, but not EP300::ZNF384-, expressing cells exhibited increased resistance to dexamethasone-induced apoptosis. By means of an oligonucleotide microarray and RT-qPCR, we observed that the transduction of TCF3::ZNF384, but not EP300::ZNF384, leads to significant enhancement of cyclin D2 (CCND2) gene expression in BCP-ALL cells, but no growth advantage was observed.

Conclusion: Our data suggest that the acquisition of dexamethasone resistance in BCP-ALL cell lines is an effect of TCF3::ZNF384 protein distinct from EP300::ZNF384. Other than the common functions of ZNF384-r that contribute to the development of leukemia with a lineage-ambiguous phenotype, TCF3::ZNF384 may exhibit a fusion partner-dependent function distinct from EP300::ZNF384 and participate in the formation of characteristic clinical features of TCF3::ZNF384-expressing ALL patients.

Background: Hereditary angioedema (HAE) is a rare genetic disorder that causes recurrent edema and abdominal/laryngeal attacks. However, the number of pediatric HAE cases is unknown. We aimed to assess the number of pediatric HAE and the actual status of pediatric HAE management in Japan.

Methods: A mail questionnaire survey was conducted on 142 clinicians (representatives of institutes) with previous experience in HAE management. The survey items included the number of pediatric patients who were treated for HAE, history of attacks and its impact on quality of life, the presence of untested pediatric relatives of adult patients, and unmet needs.

Results: In total, 69 representatives (49% of overall institutes) responded. Twenty-five (36% of respondents) had experience in pediatric HAE management. The number of cases managed by individual faculties ranged from 1 to 6, and most physicians (n = 16, 64% of faculties with patient(s)) reported the management of a single case only. There were 26 (8 male,16 female) patients with one or more attacks. Nineteen facilities (28% of respondents) reported one or more pediatric relative(s) of patients who were hesitant to screen. Further, physicians reported various unmet needs.

Conclusions: Pediatric HAE is managed in many facilities in a dispersed manner. In some cases, the pediatric relatives of patients diagnosed with HAE did not undergo screening. This study identified unmet needs and challenges that reflect the absence of specialized pediatric management. Hence, the standardization of pediatric HAE management is an urgent concern in Japan.

Background: The rapid increase in depression among adolescents is an urgent concern. This study examined the association between depression and digital device use, among adolescents. Furthermore, we explored the time thresholds of specific digital device use.

Methods: This cross-sectional study administered self-reported questionnaires to all 467 students of a junior high school in Japan. The Patient Health Questionnaire-9 was used to assess depression (cut-off values 0-4: no or minimal, 5-9: mild, 10-27: moderate to severe). Data on participants' dietary habits, sleep habits, physical activity, and time spent on digital devices (total, gaming, social media, and video watching) were obtained. Multiple logistic regression analysis was used to examine the abovementioned association.

Results: The response rate was 92.9% and data from complete case analyses with 357 participants were analyzed. Of the participants, 11.8% reported depressive symptoms. Lifestyle factors and digital device use were consistently associated with depression. Total time spent on any digital device was not significantly associated with depression, adjusted for selected lifestyles. However, ≥3 h of gaming (Adjusted odds ratio [AOR] 3.32 [95%CI, 1.66-6.65], p = 0.001) and social media use (AOR 2.56 [95%CI, 1.28-5.11], p = 0.008) on non-school days were significantly associated with depression, regardless of adjustment.

Conclusion: Moderate use of digital technology to avoid disrupting lifestyles is desirable for adolescents' mental health. Furthermore, gaming or social media use for ≥3 h was consistently significant factors. Health education to control these factors for <3 h is favorable to maintain mental health.

Background: The increasing prevalence of neurodevelopmental disorders (NDD), including autism spectrum disorder (ASD) and attention-deficit/hyperactivity disorder (ADHD), in children has led to concerns about waiting times for medical evaluation. Thus, Fukuchiyama City Hospital jointly established an ambulatory clinic for children with developmental disorders with the city government in 2010. This study aims to investigate behavioral challenges among patients aged 15 years and older attending the developmental outpatient clinic, particularly those diagnosed with ASD, to highlight challenges related to medical and social treatments.

Methods: The study examined patients aged 15 years and older diagnosed with NDD who visited the outpatient clinic for developmental disorders at Fukuchiyama City Hospital from January to December 2023. It collected data on demographics, duration of outpatient visits, diagnoses, current communication skills, and behavioral problems from medical records.

Results: Out of 68 patients, 45 were diagnosed with ASD. Communication difficulties were prevalent, with 31.1% lacking significant verbal communication skills. Behavioral problems included behavioral deviance, inattention, hyperactivity, impulsivity, harmful behavior toward others, and self-injury. Low levels of developmental quotient (DQ) were associated with maladaptive behaviors, but harmful behaviors were independent of IQ/DQ.

Conclusions: The study highlights the behavioral problems in adolescents and young adult patients with ASD attending developmental clinics. Early and appropriate interventions, such as applied behavior analysis, are critical for addressing these challenging behaviors, especially harmful ones. The support of family and caregivers based on behavior analysis is necessary for enhancing the outcomes and quality of life of individuals with NDD.

Background: To retrospectively examine whether differences in arteriovenous carboxyhemoglobin (a-vCOHb) can be used as a marker for evaluating the severity of neonatal lung disease that develops soon after birth.

Methods: The subjects were 129 newborn neonates who were classified into three groups comprising 48 infants with severe respiratory disorder (group S), 46 with mild-to-moderate respiratory disorder (group M), and 35 in a control group without respiratory disorder (group C). Various parameters including COHb were measured using a blood gas analyzer/oximeter at the time of admission, with sampling of arteries and veins performed at short intervals (within 30 min).

Results: The arterial COHb values were significantly higher in group C than in group S. The a-vCOHb values were significantly higher in groups C and M than in group S. This suggested that a loss of carbon monoxide excretion due to the generation of shunt blood with lung collapse at the alveolar level and a decrease in the total amount of carbon monoxide generated within the lungs resulted in the decrease of a-vCOHb values in group S. Receiver operating characteristic curve analysis of a-vCOHb for predicting the need for respiratory support identified an a-vCOHb cut-off value of 0.3% with a sensitivity of 75% and specificity of 74% (p < 0.001).

Conclusion: An a-vCOHb cut-off value of 0.3% may be useful in determining the severity of lung disease in the early neonatal period.

Introduction: Severe combined immunodeficiency (SCID) due to T-cell deficiency is the most severe form of inborn error of immunity (IEI). It frequently leads to severe and recurrent infections and the first infection or live vaccines can sometimes be fatal. Patients with B-cell deficiency (BCD), such as X-linked agammaglobulinaemia (XLA), also suffer from severe or recurrent infections. Thus, early diagnosis via newborn screening (NBS) is suitable for these types of diseases. We developed a lyophylized TaqMan-based quantitative polymerase chain reaction (qPCR) kit with primers and probes for the simultaneous detection of T-cell receptor excision circles (TREC) and κ-deleting recombination excision circles (KREC). We also developed a fully automated DNA extraction and purification process using Magtration technology from dried blood spots (DBS), enabling high-throughput analysis METHODS: We examined 15,258 stored DBS collected from 2014 to 2015 by this method. Newborn screening samples from children with a known SCID, XLA or ataxia-telangiectasia (AT) were also examined as positive controls.

Results: RPPH1 (internal control), TREC, and KREC all had near-normal distributions. One specimen was below the cut-off for TREC (0.00657%) after exclusion of 36 specimens due to the failure of DNA extraction (0.23%). The TREC levels in the patients with AT and SCID, and KREC levels in the patients with AT and XLA were all below cut-off or absent.

Conclusions: This assay would allow the establishment of qPCR-based NBS in unfamiliar laboratories leading to the early diagnosis of SCID and BCD.