Abstract in English:

Abstract Objective To review current evidence on early diagnosis and initial management of biliary atresia, highlighting advances achieved in recent decades and the persistent challenges that continue to influence clinical outcomes. Sources Relevant literature was identified through structured searches of PubMed and other major biomedical databases, complemented by manual review of reference lists from key original studies, systematic reviews, and position papers addressing biliary atresia and neonatal cholestasis. Summary of the findings Biliary atresia remains a highly time-critical disease in pediatric hepatology, with outcomes strongly determined by age at surgical intervention. Population-based screening strategies, particularly stool color card programs, have shown meaningful reductions in age at surgery in selected regions, although their effectiveness is highly dependent on healthcare system organization and follow-up capacity. However, acholic stools remain a valuable sign for clinicians. Laboratory biomarkers such as gamma-glutamyl transferase and matrix metalloproteinase-7 provide complementary diagnostic support. Imaging modalities and liver biopsy continue to play essential roles in the diagnostic work-up. However, several commonly used tests predominantly reflect advanced disease and may be less informative in the earliest stages. Evidence supporting postoperative adjuvant therapies remains heterogeneous, with ongoing controversies regarding their impact on long-term native liver survival. Conclusions Despite important advances, early diagnosis of biliary atresia remains challenging in many settings. Integrating clinical vigilance with the judicious use of screening strategies, biomarkers, and diagnostic procedures is essential to optimize outcomes. Continued efforts are needed to refine early diagnostic pathways and to address persistent gaps in evidence, standardization, and access to specialized care.

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Abstract Objective To evaluate the association between the interval doses of epinephrine and one-year survival and one-year neurological prognosis after pediatric in-hospital cardiac arrest (IHCA). Methods This observational retrospective cohort study included pediatric patients (0-18 years) who experienced IHCA and received at least two doses of epinephrine from January 2015 to December 2022. Data were collected following the Utstein style. The mean interval between epinephrine doses was categorized as 〈 3, 3-5, or > 5 min. Primary outcome was one-year survival; secondary outcomes were survival to hospital discharge and one-year neurological prognosis, assessed by the Pediatric Cerebral Performance Category. Results 194 patients were eligible. In the univariate adjusted analysis, patients who received epinephrine at intervals shorter than 3 min had a 2.3-fold increased chance of one-year survival (OR 2.3; 95 % CI 1.0-5.5; p = 0.042), although this association was not sustained in the multivariable regression. Longer intervals between epinephrine doses (OR: 0.71; 95 % CI 0.52-0.71; p = 0.03), continuous vasoactive drugs infusion prearrest (OR 0.1; 95 % CI 0.1-0.4; p < 0.001), longer resuscitation duration (OR 0.8; 95 % CI 0.7-0.9; p = 0.028), epinephrine doses (OR 0.68; 95 % CI 0.56-0.81; p < 0.001) were associated with reduced one-year survival. Neurological deterioration was observed in 6 (16.6 %) of the 36 patients after one year (p = 0.001). No association was found between epinephrine dosing intervals (OR 0.72; 95 % CI 0.16-3.14; p = 0.65) and neurological outcomes. Conclusion Epinephrine dosing interval was not independently associated with one-year survival after adjusted analyses. These findings underscore the complexity of pediatric resuscitation and support further multicenter prospective studies.

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Abstract Objective Bronchopulmonary dysplasia (BPD) is a common chronic lung complication in preterm infants, often complicated by cardiopulmonary dysfunction and poor outcomes. This study aimed to evaluate echocardiographic parameter differences at 36 weeks postmenstrual age (PMA) between preterm infants with and without BPD, and explore echocardiography’s utility for BPD identification. Methods A retrospective cohort study included 94 preterm infants (gestational age < 32 weeks) admitted to the NICU (2019-2023). They were grouped by 2018 NICHD criteria: Non-BPD (n = 50, no/mild respiratory support) and BPD (n = 44, requiring prolonged oxygen/mechanical ventilation). Standard transthoracic echocardiography (TTE) at 36 weeks PMA assessed left ventricular (LV), right ventricular (RV), and tissue Doppler imaging (TDI) parameters. Results Gestational age and birth weight were comparable between the two groups (p < 0.01), but BPD infants had longer mechanical ventilation duration (p < 0.01). BPD infants showed significant cardiac dysfunction: reduced LV ejection fraction (LVEF), mitral valve E velocity (MV-E), and MV-E/A ratio; decreased RV tricuspid annular plane systolic excursion (TAPSE) and fractional area change (FAC); and abnormal TDI (lower TV-S′/E′/A′, higher TV-E/E′ ratio) (all p < 0.01). Conclusions Echocardiographic parameters reflecting biventricular systolic/diastolic dysfunction differ significantly between BPD and non-BPD preterm infants, indicating potential for BPD identification.

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Abstract Objective Meconium aspiration syndrome is a common cause of severe respiratory failure in term and post-term neonates. The timing for administering surfactant remains non-standardized. This study aimed to evaluate the predictive value of the lung ultrasound score for the need for surfactant therapy in infants with Meconium aspiration syndrome. Method This prospective multicenter study enrolled 218 neonates with meconium aspiration syndrome. Lung ultrasound was performed within 30 min of admission using a six-zone scoring system (0-18). Surfactant was given based on oxygenation criteria (FiO₂ > 0.5 or OI > 8), with clinicians blinded to ultrasound findings. Predictive performance of the lung ultrasound score for surfactant need was assessed by ROC analysis and compared with traditional indices. Multivariable regression and decision curve analysis were performed. Results Lung ultrasound score demonstrated near-perfect diagnostic accuracy for predicting surfactant need: at the optimal cutoff of 6 points, AUC was 0.999 (95% CI, 0.997-1), with 100% sensitivity. Performance was superior to pH (AUC 0.906) and chest radiograph grade (AUC 0.539), and consistent across subgroups by respiratory support, gestational age, and presence of persistent pulmonary hypertension. Multivariable analysis identified lung ultrasound as an independent predictor of surfactant use. Decision curve analysis confirmed greater net clinical benefit for lung ultrasound-based strategies over a wide range of threshold probabilities. Conclusion A lung ultrasound score of ≥ 6 exhibited near-perfect predictive capacity for guiding surfactant administration decisions (AUC 0.999). This technique provides rapid, non-invasive pulmonary morphological information, facilitating the identification of neonates meeting the criteria for surfactant therapy.

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Abstract Objective Penicillin remains the only safe and effective drug recommended for treating syphilis in pregnant women and congenital syphilis (CS). Nevertheless, alternative therapies are needed when penicillin is contraindicated, such as in allergic reactions, or unavailable due to supply limitations. This study aimed to compare the serological response to the Venereal Disease Research Laboratory (VDRL) test in infants reported with CS and treated with either ceftriaxone or penicillin. Method A non-competitive cohort study was conducted in three public maternity hospitals in Fortaleza, Ceará, Northeast Brazil. Data were extracted from notification forms in the Notifiable Diseases Information System (SINAN), medical records from maternity hospitals and outpatient clinics, and electronic records from primary care units. The serological response of infants treated with ceftriaxone and penicillin was assessed. An adequate response was defined as two consecutive negative VDRL tests performed at the intervals recommended by the Brazilian Ministry of Health. Survival curves were estimated using the Kaplan-Meier method. Results Among 383 infants reported with CS, 56 (14.6 %) underwent VDRL testing in accordance with Ministry of Health guidelines. Of these, 19 (33.9 %) received ceftriaxone and 37 (66.1 %) penicillin. No statistically significant difference in time to VDRL negativity was observed between the groups (log-rank p = 0.73). The median time to negativity was 3 months in both cohorts. Conclusions Serological response to VDRL was comparable between infants treated with ceftriaxone and those treated with penicillin. No cases of kernicterus or other CS-related complications occurred among ceftriaxone-treated infants.

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Abstract Objective This study aimed to investigate the risk factors for acute neurological complications (ANCs) in neonates with bacterial meningitis (NBM). Methods This retrospective study analyzed 68 neonates (aged 0-28 days) with bacterial meningitis admitted to a tertiary pediatric medical center (Children's Hospital, Zhejiang University School of Medicine, China) between November 1, 2020, and June 30, 2025. ANCs were defined as a spectrum of conditions resulting from NBM, including subdural effusion/empyema, ventriculitis, hydrocephalus, brain abscess, and encephalomalacia. The cohort comprised 48 neonates without ANCs and 20 who developed ANCs. Results Compared with neonates without complications, those who developed ANCs were significantly older at symptom onset and demonstrated higher rates of seizures, elevated C-reactive protein (CRP) levels, increased cerebrospinal fluid (CSF) culture positivity, higher rates of Group B Streptococcus (GBS) infection, and more frequent dexamethasone administration. Multivariate logistic regression analysis identified seizures (OR = 10.951, 95% CI: 1.981-60.532, p = 0.006) and GBS infection (OR = 4.803, 95% CI: 1.072-21.523, p = 0.040) as independent risk factors for ANCs. Dexamethasone use (OR = 0.946, 95% CI: 0.185-4.834, p = 0.947) was not significant after adjustment. A predictive model incorporating both factors demonstrated good discriminatory capacity (AUC = 0.814, 95% CI: 0.686-0.942, p < 0.001). Conclusion Seizures and GBS infection are independent risk factors for ANCs in NBM. Dexamethasone administration did not reduce the incidence of ANCs in this cohort.

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Abstract Objectives The increasing prevalence of metabolic dysfunction associated with steatotic liver disease (MASLD) is consistent with the global obesity epidemic. The aim of the study was to investigate the presence of MASLD in a high-risk group of children and to compare cardiovascular risk factors between the groups with and without MASLD, in addition to investigating potential biomarkers. Methods This cross-sectional study was carried out at a public institution with thirty-eight patients (19 girls, 13.2 ± 2.7 y) with excess weight. Blood pressure (BP), abdominal fat, carotid intima-media thickness (CIMT), and blood biochemistry were evaluated. Steatosis was assessed by the controlled attenuation parameter (CAP) obtained via transient elastography, and the participants were grouped as ¨Non-Steatosis¨ (n = 24) and ¨Steatosis¨ (n = 14). Results The “Steatosis group” showed higher BP (Z SBP 1.56 vs. -0.25; Z DBP 1.38 vs. 0.07; p = 0.0001), BMI (z-BMI 3.48 vs. 2.39; p < 0.0001), right CIMT (0.07 vs. 0.06 cm; p = 0.013), basal insulin (41 vs. 14 mcUI/l; p < 0.0001), and HOMA-IR (8.2 vs. 3.1; p < 0.0001), and lower adiponectin (2.75 vs. 6.46 µg/ml; p = 0.004) compared to the “Non-Steatosis” group even after z-BMI adjustments. ROC curves indicated adiponectin (cutoff ≤ 4.5 μg/mL) and insulin (cutoff ≥ 22 mcIU/mL) as biomarkers of steatosis. Conclusion In pediatric age with excess weight, hepatic steatosis was associated with higher BP, severe obesity, and worse glycemic profile. Low adiponectin and high insulin levels appeared as possible biomarkers of MASLD. The study reinforces the need for children with obesity and a worse metabolic profile to be investigated for early diagnosis of MASLD.

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Abstract Objective Persistent candidemia (PC) in children can lead to unfavorable outcomes. However, its risk factors and clinical impact remain poorly understood. This study aimed to identify risk factors associated with PC in pediatric patients. Method We conducted a retrospective analysis of 141 children (0-17 years) diagnosed with candidemia at a children’s hospital in Brazil between 2016 and 2022. Clinical data were collected from medical records. Microorganisms were identified by MALDI-TOF MS, tested for biofilm production, and sensitivity profile. Molecular typing was performed on the three most prevalent species, and ERG11 mutation screening was carried out on fluconazole-resistant isolates. Results PC was identified in 34.8% (n = 49) patients. Independent risk factors included early non-removal of the central venous catheter, parenteral nutrition, and cancer. The overall 30-day mortality was 23.4%, and the candidemia-related mortality was 16.3%. C. parapsilosis was the prevalent species. All isolates except one produced biofilm. One isolate of C. tropicalis, which had the missense mutation Y257H in ERG11, was resistant to fluconazole. Isolates showed high genetic diversity. Conclusions PC was associated with host factors and clinical management rather than aspects of the etiological agent, highlighting the importance of early patient monitoring.

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Abstract Objective To investigate lipid profile alterations and their associated sociodemographic, anthropometric, and lifestyle factors among adolescents living in São Paulo in 2008 and 2015. Methods The authors analyzed data from the ISA-Capital study, a population-based cross-sectional survey, including 448 adolescents (12-19 years). Socioeconomic, anthropometric, lifestyle, and biochemical data were collected. Cardiometabolic risk factors assessed included high blood pressure, obesity, increased waist circumference, dyslipidemia, and lipid profile alterations. Statistical analyses included Chi-square tests, t-tests/Wilcoxon tests, and logistic regression models (p < 0.05). Results Dyslipidemia was the most prevalent risk factor (67.6%; 95% CI 63.1-71.8), with low HDL-c affecting 52.4% (95% CI 47.7-57.0). High blood pressure and obesity were observed in 16.1% (95% CI 12.9-19.7) and 11.7% (95% CI 9.0-15.1) of adolescents, respectively. 7% had 3 or more risk factors. Compared to 2008, adolescents in 2015 were twice as likely to present high blood pressure (OR = 2.04; p = 0.011). Older adolescents had lower odds of obesity (OR = 0.83; p = 0.024) but were more likely to have increased waist circumference (OR = 1.22; p = 0.027) and high blood pressure (OR = 1.17; p = 0.017). Adolescents self-declared as non-white had lower odds of dyslipidemia (OR = 0.59; p = 0.018) and high triacylglycerol (OR = 0.58; p = 0.012). Higher household education was associated with lower risk of presenting dyslipidemia (OR = 0.54; p = 0.004). Physically active adolescents had lower odds of high LDL-c (OR = 0.48; p = 0.045). Conclusion The elevated prevalence of cardiometabolic risk factors, particularly dyslipidemia, underscores the need for targeted preventive strategies among adolescents in São Paulo.

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Abstract Objective This study aims to evaluate the quality of life (QoL) and glycemic control of children and adolescents living with type 1 diabetes mellitus (T1D) before and after an intervention with a multidisciplinary educational program. Method In this prospective interventional study, 47 participants were divided into an intervention group (IG), which underwent a six-month in-person and online multiprofessional education program, and a control group (CG), which received standard in-person follow-up. After three months, all participants in this study had a continuous interstitial glucose monitor (CGM). QoL was assessed by the Pediatric QoL Questionnaire PedsQL 3.0 - Diabetes Mellitus Module. Scores were separated into tertiles. Results Both groups had homogeneous general characteristics and mean HbA1c above the recommended levels. The QoL level in the IG and its guardians at T180 showed no differences but girls showed lower QoL scores at T0. Participants in the CG with intermediate perceptions showed a reduction in their QoL in the category “barriers to treatment” in relation to IG. The IG showed a significant increase in adherence to the carbohydrate counting method (CHCM). QoL, according to the parents’/guardians’ perception, was lower than that of their children. Conclusions The intervention failed to significantly affect glycemic control either in HbA1c or in time in the therapeutic range. The intervention and CGM failed to improve glycemic control and QoL in individuals living with T1D and suboptimal glycemic control. However, it significantly increased adherence to CHCM.

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Abstract Objective To characterize the molecular spectrum of NR0B1 variants associated with X-linked congenital adrenal hypoplasia (X-hypoAC) in a multicenter Brazilian cohort and to highlight the clinical implications of early molecular diagnosis. Methods The authors investigated 12 unrelated families referred to pediatric endocrinology centers across Brazil with a clinical diagnosis of early-onset adrenal insufficiency. Clinical data, biochemical profiles, and follow-up information were collected. Genetic testing of the NR0B1 gene was performed by Sanger sequencing to detect single-nucleotide and small insertion/deletion variants, complemented by MLPA to identify large deletions or rearrangements. Variants were classified according to the American College of Medical Genetics and Genomics (ACMG) guidelines. Results All probands presented with adrenal insufficiency within the first months or years of life, often requiring hospitalization and lifelong glucocorticoid and mineralocorticoid replacement. Molecular analysis revealed a heterogeneous spectrum of NR0B1 pathogenic variants, including frameshift, nonsense, and missense variants, as well as large and small deletions, which together accounted for nearly half of the cases. Clinical follow-up confirmed that, in addition to adrenal insufficiency, affected individuals frequently developed hypogonadotropic hypogonadism during puberty, with infertility documented in adulthood. The identification of novel variants contributes to expanding the mutational spectrum of NR0B1and reinforces the genotype-phenotype variability in X-hypoAC. Conclusions This study represents one of the largest Brazilian series of X-hypoAC. The present findings broaden the molecular landscape of NR0B1 variants and underscore the relevance of early genetic testing to differentiate X-hypoAC from congenital adrenal hyperplasia, optimize long-term clinical management, and provide accurate genetic counseling for affected families.

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Abstract Objective Maternal prenatal vitamin D is linked to offspring’s disease risk, but its specific association with offspring’s immune development is understudied. This study aimed to explore the relationships between maternal prenatal vitamin D, umbilical cord blood cytokines, and offspring’s allergic diseases within 2 years of life. Methods Term infants born in 2023 at Shaoxing Maternal and Child Health Care Hospital (China) were included. Ten cord blood cytokines were quantified by flow cytometry. Logistic regression evaluated maternal vitamin d-child allergy and cytokine-allergy associations, with linear regression for vitamin d-cytokine relationships. Results After 2 years of follow-up, maternal prenatal vitamin D deficiency was linked to higher allergic disease risk in offspring (odds ratio = 1.71, 95% confidence interval:1.03-2.87), and maternal vitamin D levels correlated positively with cord blood interleukin-10 (r = 0.33, P < 0.001). After confounder adjustment, cord blood interleukin-4 (odds ratio = 2.38) and interleukin-10 (odds ratio = 0.78) emerged as independent risk and protective factors for childhood allergic diseases, respectively. Conclusions Normal maternal prenatal vitamin D status is tied to lower allergic disease risk in offspring, with umbilical cord blood cytokines (especially interleukin-4 and interleukin-10) potentially mediating this relationship in early childhood.

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Abstract Objective To describe the development of the FIESP Observatory for Childhood Food and Nutrition Security (FNS), a digital platform designed to organize and integrate public data on children aged 0 to 10 years in the state of São Paulo, Brazil. Methods This descriptive study involved three main stages: (1) a literature review and mapping of national and international observatories on health, nutrition, and childhood to identify best practices and gaps; (2) establishment of an expert committee of professionals in nutrition, public health, and public policies to guide indicator selection and ensure technical quality; and (3) technical development of the platform, including automated data extraction, processing, and integration into thematic dashboards. Public databases from official sources were incorporated, structured across six thematic axes: demographic, socioeconomic, FNS policies, nutritional, health, and educational profiles. Results The observatory was launched in May 2025 and made freely available online. It features six interactive panels and a “Your City” dashboard that consolidates key indicators for each municipality in São Paulo. Since its launch, it has received >9600 visits, with 4755 active users. The tool enables segmented analyses by geography, time, and demographics, providing evidence to support policy design and monitoring. Conclusions The Observatory represents an innovative and scalable model for consolidating childhood FNS data. By offering free, intuitive, and continuously updated access to official indicators, it supports policymakers, researchers, and civil society in addressing childhood food insecurity and advancing the right to adequate and healthy food.