BMC Pediatrics最新文献

Background: Inborn errors of metabolism represent a significant cause of childhood morbidity and mortality. These conditions are frequently missed in low-resource settings due to their anticipated rarity and similarity of symptoms to conditions such as sepsis. We present a case of a neonate with N-acetylglutamate synthase deficiency whose diagnosis and management at our facility were complicated by limited healthcare resources.

Case report: A three-day-old male of South Asian origin born to consanguineous parents presented with lethargy, hypothermia and respiratory distress. He was initially managed for suspected septic shock. However, further investigations revealed severe hyperammonemia for which he was managed with peritoneal dialysis and oral sodium benzoate. His care was coordinated by a multidisciplinary team and included teleconsultation with a metabolic specialist. Once stabilized, he was transferred to our sister institution in Pakistan for further care where genetic analysis revealed a homozygous pathogenic variant (c.1306_1307insT; p.Thr439fs*52) in the N-acetylglutamate synthase gene, confirming the diagnosis of N-acetylglutamate synthase deficiency. However, the baby passed away at 49th day of life.

Conclusion: High index of suspicion is important in diagnosing inborn errors of metabolism. Even in resource-limited setting, a multidisciplinary team with international partnership can optimize the care for patients with rare inborn errors of metabolism. There is also a need to increase awareness, improve diagnostic capacity and establish standardized treatment protocols for rare metabolic disorders in low-resource settings like Tanzania.

Background: Neonatal mortality remains a significant health challenge, particularly in low- and middle-income settings, where respiratory distress is a major contributor to mortality. Characterizations of respiratory care practices in the neonatal intensive care unit (NICU), in lower-middle-income contexts in south-east Asia, are notably sparse in the literature. This study aimed to describe the management practices, morbidity, and mortality of newborns requiring respiratory support at a large level III NICU in Hanoi, Vietnam.

Methods: This prospective, descriptive observational study was conducted at Phu San Hanoi Hospital with about 35 000 births in 2023. Infants born alive, requiring respiratory support, and with a birth weight ≥ 700 g were included. Data were collected from September 1 to November 30, 2023, and analysed using descriptive statistics.

Results: During the study period 17% of infants born at the hospital were admitted to the NICU of which 53% (n = 895) were included in the analysis. The median gestational age was 34.6 weeks, and the median birth weight was 2,150 g. Respiratory distress syndrome (RDS) was the leading cause of respiratory support (41%), followed by transient tachypnoea of the newborn (24%) and early-onset sepsis (13%). Non-invasive respiratory support, especially nasal continuous positive airway pressure (nCPAP) (45%) and nasal intermittent positive airway pressure (NIPPV) (21%), was widely utilized, with NIPPV being more common among larger infants. Invasive mechanical ventilation was required in 23% of infants, with higher rates in lower birth weight groups. The overall mortality rate was 3%, with the highest mortality among extremely low birth weight infants (36%). Surfactant therapy was administered to 37% of infants with RDS which was also the primary indication for surfactant treatment (98%). Of all first-dose surfactant therapies, 92% were given within the first six hours after birth and 49% were administered by the INSURE (intubation-surfactant-extubation) method.

Conclusions: This study provides valuable insights into respiratory care practices and outcomes for infants requiring respiratory support at northern Vietnam's largest obstetrical hospital. It highlights RDS as a significant contributor to neonatal morbidity and identifies opportunities to enhance non-invasive support and surfactant administration techniques, potentially reducing invasive mechanical ventilation and improving neonatal outcomes.

Background: Although neonatal sepsis remains a significant cause of morbidity and mortality in preterm infants, few studies in Vietnam have compared the characteristics of early-onset sepsis (EOS) and late-onset sepsis (LOS).

Objective: To compare the clinical, laboratory, and microbiological characteristics of EOS and LOS in preterm neonates at a tertiary obstetric hospital in Vietnam.

Methods: This cross-sectional study included 106 preterm infants with culture-confirmed sepsis admitted from August 2022 to May 2024. Clinical data, laboratory results and blood cultures were analyzed. Statistical tests and univariate logistic regression were used to explore differences between EOS and LOS.

Results: Of 106 neonates, 24 (22.6%) had EOS, and 82 (77.4%) had LOS. LOS predominated in extremely preterm, very low birth weight infants and was associated with more severe symptoms, including respiratory distress and abnormal heart rate. EOS cases were more likely to have birth weight ≥ 1500 g and maternal conditions such as intrapartum fever, genital infections, contaminated amniotic fluid and prolonged amniotic fluid leakage (> 18 h). Laboratory analysis revealed higher levels of leukopenia, platelet and cerebrospinal fluid protein in EOS, as well as elevated C-reactive protein and blood protein in LOS. Gram-negative bacteria were predominant in both groups. Escherichia coli was most common in EOS (46%), whereas Klebsiella pneumoniae predominated in LOS (38%).

Conclusion: EOS and LOS in preterm neonates exhibit distinct patterns in perinatal factors, clinical manifestations, laboratory findings and microbiological profiles. Differentiating these two entities may aid in timely diagnosis and guide empirical treatment in settings with limited resources.

Background: To demonstrate that the CA19-9/creatinine level could be a potential biomarker in children with obstruction by including ureteropelvic junction obstruction (UPJO) patients and comparing them not only with healthy controls but also with children with non-obstructive hydronephrosis (HN).

Materials and methods: Children with HN and healthy controls with no HN were prospectively enrolled into study from March 2021 to December 2022. The children with HN were divided into two separate groups. Group 1 consisted of children with UPJO, while Group 2 included children with lower urinary tract dysfunction (LUTD). Data collected from study and control groups included age, gender, spot urine CA 19 - 9/Cr ratio at the initial visit and at the 6-month follow-up, the Society of Fetal Urology (SFU) grade, antero-posterior (AP) diameter, renal function and diuretic response on mercaptoacetyltriglycine (MAG-3) dynamic renal scintigraphy and surgical or conservative management selection. Spot urine CA 19 - 9 levels were measured using the ELISA method.

Results: Out of 283 children, Group 1 consisted of 40 children (11 girls, 29 boys; median age: 7 years), Group 2 comprised 18 children (10 girls, 8 boys; median age:8 years) and 225 healthy controls (83 girls, 142 boys; median age: 8 years). The spot urine CA 19 - 9/Cr ratio was significantly higher in the Group 1 and 2 compared to controls (respectively 86.7, 64.5, 47.1 U/mg Cr, p = 0.0001). When the two patient groups with HN were compared with each other, no statistically significant difference was observed in the urinary CA19-9/creatinine levels (p = 0.358). No correlation was found between CA 19 - 9/Cr ratio and HN severity and/or dynamic renal scintigraphy findings. Surgical management of unilateral UPJO revealed a decrease in CA 19 - 9/Cr in the study group.

Conclusion: This study suggests that the spot urine CA 19 - 9/creatinine ratio may serve as a promising, non-invasive biomarker for distinguishing children with unilateral UPJO and non-obstructive HN from healthy controls. Although elevated levels in patients and postoperative decline were observed, its inability to predict HN severity or the necessity for surgical intervention limits its clinical utility.

Numerous studies have found that parents underestimate the overweight and obese weight status of their children, which may lead to future health risks. The purpose of the paper was to examine how parents in Serbia perceive their children's weight status. A nationally representative sample of 6-9-year-old children (n = 2700) was evaluated as part of the World Health Organization's (WHO) European Childhood Obesity Surveillance Initiative (COSI). The children's body mass index (BMI) was categorized as BMI-for-age Z-scores based on the 2007 WHO-recommended growth references. The family questionnaires, which were filled in by parents or caregivers, contained, among others, questions seeking to identify the adults' perceptions of their children's weight status. The weight distribution of the participant children was the following: 2% of the children were in the category of 'thinness', 20.6% were in 'overweight' and 14,7% were in the category 'obese'. A total of 83.2% of the parents accurately perceived their child's healthy weight (normal weight), however, 71% of the parents underestimated the overweight status of their children and 91,2% of the parents failed to acknowledge the obese status of their child according to the WHO definition. Childhood obesity should be continuously monitored; existing health promotion interventions should be more strictly controlled. This study found that parents need more health-related education in the future, with close cooperation of schools and stakeholders in raising healthier generations.

Background: Neonatal hemolytic disease and jaundice represent a persistent global health challenge, accounting for substantial avoidable mortality and long-term neurodevelopmental impairment among infants worldwide, with inequitable impacts across resource regions. This study aimed to evaluate the global, regional, and national burden of hemolytic disease and neonatal jaundice from 1990 to 2021, and project future trends up to 2050.

Methods: Data on prevalence, disability-adjusted life years (DALYs), and risk factors were drawn from the Global Burden of Disease (GBD) 2021 study. Socio-demographic disparities were assessed via the socio-demographic index (SDI). Temporal trends were examined, inequalities were quantified by the slope index of inequality and concentration index, and future burden was projected to 2050.

Results: Globally, the ASPR of neonatal jaundice increased by 41.5% between 1990 and 2021, while ASDR decreased by 56.8%. DALYs declined overall, but absolute case numbers rose due to population growth and improved survival. The burden was consistently higher in males than females and disproportionately concentrated in low and middle SDI regions. Key risk factors included low birthweight, preterm birth, and air pollution. Projections suggest that prevalence will continue to rise through 2050, while mortality is expected to decline further.

Conclusions: A global trend of increasing ASPR but decreasing ASDR from neonatal jaundice-related conditions was found. However, low SDI regions still have a disproportionate burden, underscoring the urgent need for targeted perinatal interventions and mitigation of environmental risks.

Objectives: To create a nomogram for early intra-abdominal hypertension (IAH) detection in pediatric patients following liver transplantation (LT).

Methods: This prospective, observational study was conducted in a tertiary hospital's Liver Transplantation Intensive Care Unit (LICU), prospectively recruiting a cohort of 425 children undergoing liver transplantation between January 2022 and December 2024. Perioperative parameters were electronically extracted via the hospital information system. Intra-abdominal pressure (IAP) quantification employed the standardized transvesical technique. To identify clinically relevant predictors, a LASSO-based feature selection algorithm was applied, leveraging regularization to shrink coefficients of non-informative variables toward zero. The retained covariates were then integrated into a multivariable logistic regression model for nomogram construction. Internal validation included three domains: (1) discrimination performance evaluated by area under the ROC curve (AUC), (2) calibration accuracy by Hosmer-Lemeshow test with calibration curves, and (3) clinical utility assessed through decision curve analysis (DCA) across probability thresholds.

Results: Three independent predictors were identified: Graft-to-Recipient Weight Ratio (GRWR), duration of mechanical ventilation (MV), and central venous pressure (CVP), integrated into a predictive nomogram. DCA showed a significant net benefit, and temporal validation confirmed the nomogram's reliability. The nomogram displayed superior predictive accuracy (AUC = 0.831) with adequate calibration (Hosmer-Lemeshow test p > 0.05). DCA revealed favorable clinical applicability across threshold probabilities, showing positive net benefit values ranging from 10%-80% and 5%-80%, while temporal validation maintained discriminative capacity (AUC = 0.822).

Conclusions: The study devised an effective nomogram for identifying pediatric patients at a heightened risk of IAH following LT, incorporating GRWR, duration of MV and CVP.

Background: This study aimed to assess the utilization and associated factors of second-dose measles vaccine among mothers with children less than two years old in Enderta district, South Eastern Tigray.

Methods: A mixed community-based cross-sectional study was conducted in Enderta district, South Eastern Tigray from January- to March 2020. The sample size of this study was 410 mothers, 10 in-depth interviews, and 2 focus group discussions. Multistage sampling technique for the quantitative data and purposive sampling for the qualitative data was used. Quantitative data were collected by the interviewer-administered questionnaires and entered and analyzed using Epi-data-3.1 and SPSS-20, respectively. Binary logistic regression analysis was done and adjusted odds ratios measured the strength of statistical association at 95% confidence interval. Variables with a P-value < 0.2 in the bivariate analysis were entered into multivariable analysis statistical significance was declared at P-value < 0.05. Thematic analysis was employed for the qualitative data. After the qualitative data was coded, themes were developed. The result of the qualitative data was presented and discussed by triangulating.

Result: Utilization of second dose measles-containing vaccine was 32.4%. Top reasons for not vaccinating second dose measles-containing vaccines to their children were: lack of awareness about the necessity of the vaccine, missing the appointment date, mother being too busy and absence of vaccine supply. In the multivariable analysis; children aged 18-23 months (AOR 0.5; CI (0.3-0.8)), number of children greater than five per family (AOR 2.3; CI (1.3-4.3)), type of health facility (AOR 1.7; CI (1.05-2.9)), children with a history of completed basic vaccines at 12 months (AOR 2.6; CI (1.5-4.3)) and knowledgeable mothers (AOR 1.67; CI (1.04-2.7)) were significantly associated with the taking of second dose measles vaccine of their last child.

Conclusion: Despite the slight improvement from the regional and national Ethiopian Demographic Health Survey reports, utilization of second dose measles vaccine in this study was low.