Correction to: Research on the clinical factors of cardiac iron deposition in children with beta-thalassemia major
(Source: European Journal of Pediatrics)
Source: European Journal of Pediatrics - November 30, 2023 Category: Pediatrics Source Type: research
Extracranial malignant rhabdoid tumors in children: high mortality even with the help of an aggressive clinical approach
Conclusion: EMRTs are highly malignant tumors associated with high mortality rates. The loss of SMARCB1/INI1 gene and the protein expression is observed in the vast majority of eMRTs patients. Patients that suffered MRTK are younger than patients with extrarenal EERT and are more prone to lung metastasis, but there is no significant difference in overall survival, possibly due to the higher rate of R0 resection of primary tumors in MRTK. Trial registration: The study was registered on PROSPERO with registration number CRD42023400985.What is Known:• Malignant rhabdoid tumor (MRT) is a rare and highly malignant tumo...
Source: European Journal of Pediatrics - November 29, 2023 Category: Pediatrics Source Type: research
Foreign body aspiration score (FOBAS) —a prospectively validated algorithm for the management and prediction of foreign body aspiration in children
Conclusion: FOBAS reliably predicts FBA in cases of suspected FBA and improves management and in-hospital decision-making.What is Known:• Foreign body aspiration is a major cause of pediatric morbidity and mortality.• Currently, there is no unified protocol for children referred to the emergency department for suspected FBA, therefore, a well-defined algorithm is needed to improve the decision-making process.What is New:• The pediatric Foreign body aspiration score (FOBAS) is a new, prospectively validated clinical score that shows high sensitivity and specificity for the presence of FBA in children.• FOBAS redu...
Source: European Journal of Pediatrics - November 29, 2023 Category: Pediatrics Source Type: research
The dual role of body mass index on Juvenile Idiopathic Arthritis course: a pediatric experience
Conclusions: Both underweight and OB might negatively affect JIA course. Weight control is fundamental in children with JIA to avoid a more unfavourable course of the disease.What is Known:• Obesity represents a well-known risk factor for JIA severity.• The role of underweight in children with JIA is still poorly explored.What is New:• As observed in children with obesity, underweight young patients with JIA seem to experience a more severe JIA course.• Healthy lifestyle promotion in children with JIA is a crucial step in the management of the disease. (Source: European Journal of Pediatrics)
Source: European Journal of Pediatrics - November 28, 2023 Category: Pediatrics Source Type: research
Working towards risk stratification for ascending aortic dilatation in pediatric Turner syndrome patients: results of a longitudinal echocardiographical observation
Conclusions: Marked progression of aortic diameters leading to the development of dilatation can be observed in TS patients during childhood and stresses the importance of close surveillance during childhood. Main risk factors are BAV and complete monosomy 45X0. A beneficial influence of estrogen substitution can be suspected but needs further investigation.What is known:• Patients with Turner syndrome are at an increased risk for aortic dilatation and dissection.• The presence of BAV and complete monosomy 45X are additional risk factors.What is new:• Aortic dilatation can be detected in pediatric patients with Turne...
Source: European Journal of Pediatrics - November 27, 2023 Category: Pediatrics Source Type: research
Sleep duration is associated with liver steatosis in children depending on body adiposity
Conclusion: An association of sleep time in the relationship between body fat distribution and hepatic steatosis index was observed in children and adolescents with overweight/obesity, which can be relevant in the prevention and treatment of excessive adiposity between 2 and 18 years old. Clinical trial: NCT04805762. Import: As part of a healthy lifestyle, sleep duration might be a modifiable factor in the management of fatty liver disease in children.What is Known:• Sleep is an influential factor of overweight and obesity in children.• Excessive adiposity is associated with liver status in children and adolescents...
Source: European Journal of Pediatrics - November 25, 2023 Category: Pediatrics Source Type: research
Characteristics and outcomes in children with congenital central hypoventilation syndrome on long-term mechanical ventilation in the Netherlands
Conclusion: Our study results suggest that in our cohort, the genotype is not easily associated to the phenotype in CCHS. Consistent with these findings and international literature, we recommend a thorough annual evaluation for all patients with CCHS to ensure optimal management and follow-up.What is Known:• The majority of CCHS patients are dependent on ventilatory support.• Variants in the PHOX2B gene are responsible for the characteristics of CCHS.What is New:• This study provides insight into the clinical course and long-term outcomes of CCHS patients in the Netherlands.• In CCHS, the genotype is not easily as...
Source: European Journal of Pediatrics - November 25, 2023 Category: Pediatrics Source Type: research
Reassessing the role of milrinone in the treatment of heart failure and pulmonary hypertension in neonates and children: a systematic review and meta-analysis
Conclusion: Our meta-analysis suggests potential clinical benefits of milrinone by improving cardiac function, likely driven by its systemic vasodilatory effects. However, questions arise about its inotropic influence and the presence of a lusitropic effect. Moreover, milrinone ’s pulmonary vasodilatory effect appears relatively weaker compared to its systemic actions. Further research is needed to elucidate milrinone’s precise mechanisms and refine its clinical applications in pediatric practice.What is Known:• Milrinone is a phosphodiesterase III inhibitor that has been used to treat a variety of pediatric and neon...
Source: European Journal of Pediatrics - November 24, 2023 Category: Pediatrics Source Type: research
The effect of Pilates-based exercise applied with hybrid telerehabilitation method in children with adolescent idiopathic scoliosis: A randomized clinical trial
Conclusion: Pilates-based exercises applied with the hybrid telerehabilitation method can improve Cobb angle and respiratory muscle strength in patients with AIS. The hybrid telerehabilitation method can be used as an alternative to home-based programs, especially in locations and times where there may be lim ited access to supervised training. Also, the nature of the disease that requires long-term follow-up is another factor where hybrid telerehabilitation may be an advantage.Trial registration: ClinicalTrials.gov ID: NCT05761236.What is Known:• Exercise training is one of the main approaches to treating scoliosis....
Source: European Journal of Pediatrics - November 23, 2023 Category: Pediatrics Source Type: research
Evaluation of non-thyroidal illness syndrome in shock patients admitted to pediatric intensive care unit in a developing country
Conclusion: This study underscores the high prevalence of NTI in PICU shock patients and suggests monitoring thyroid hormone levels for outcome prediction and treatment guidance. Further research is needed to optimize NTI management in critically ill children.What is Known:• Non-thyroidal illness syndrome (NTIS) is a condition observed in critically ill patients.• There has been limited research on NTI in children, and existing studies have generated conflicting results regarding the relationship between thyroid hormones and clinical outcomes in cases of sepsis and septic shock.What is New:• The study has revealed dy...
Source: European Journal of Pediatrics - November 23, 2023 Category: Pediatrics Source Type: research
Hearing impairment after asphyxia and neonatal encephalopathy: a Norwegian population-based study
Conclusions: An Apgar 5-min score < 7 in combination with NICU admission is an independent risk factor for hearing impairment. Children with moderate-severe HIE had the highest risk for hearing impairment.What is Known:• Perinatal asphyxia and neonatal encephalopathy are associated with an increased risk of hearing impairment.• The strength of the association, and how other co-morbidities affect the risk of hearing impairment, is poorly defined.What is New:• Among neonates admitted to a neonatal intensive care unit (NICU), decreased Apgar 5-min scores, and increased severity of neonatal encephalopathy, were ...
Source: European Journal of Pediatrics - November 22, 2023 Category: Pediatrics Source Type: research
Early psychomotor development and growth hormone therapy in children with Prader-Willi syndrome: a review
Conclusion: Early psychomotor development is strongly correlated with the prognosis of patients with PWS; moreover, current studies support that the initiation of interventions at an early age can exert significant beneficial effects on enhancing the cognitive and linguistic development of patients with PWS and allow them to"catch up" with motor development. What is Known:•Prader-Willi syndrome is a rare genetic disorder characterized by multisystem damage, and children with Prader-Willi syndrome are typically characterized by early developmental delays, specifically in the areas of cognitive and motor deve...
Source: European Journal of Pediatrics - November 21, 2023 Category: Pediatrics Source Type: research
Gut microbiota transfer evidence from mother to newborn
Conclusion: In our sample, the maternal gut microbiome was found to be associated with the infant gut microbiome, indicating evidence of ASV-specific transfer from the maternal microbiome to newborns.What is Known:• There is a relationship of early-life microbiota composition with future health outcomes.What is New:• This was the first study to evaluate maternal gut microbiota transfer to newborns in Brazil. (Source: European Journal of Pediatrics)
Source: European Journal of Pediatrics - November 21, 2023 Category: Pediatrics Source Type: research
Magnetic resonance spectroscopy and auditory brain-stem response audiometry as predictors of bilirubin-induced neurologic dysfunction in full-term jaundiced neonates
Conclusion: When the symptoms of ABE are mild and MRI does not show any evident abnormalities, MRS and ABR are helpful in differentiating individuals with ABE from patients with neonatal hyperbilirubinemia. Trial registration: ClinicalTrials.gov, Identifier: NCT06018012.What is Known:• MRS can be used as a diagnostic and prognostic tool for the differential diagnosis of patients with acute bilirubin encephalopathy, from patients with neonatal hyperbilirubinemiaWhat is New:• ABR is a useful diagnostic and prognostic tool in the care and management of neonates with significantly raised bilirubin. It can be used as ...
Source: European Journal of Pediatrics - November 18, 2023 Category: Pediatrics Source Type: research
Steps per day and health-related quality of life in schoolchildren: the mediator role of cardiorespiratory fitness
Conclusion: Steps per day are a good metric to estimate daily physical activity because of its positive relationship with CRF. Moreover, those children taking more than 9000 steps per day are associated with higher levels of physical and psychological well-being. Finally, a substantial part of the improvement in HRQoL achieved through the increase in physical activity (steps per day) is mediated by CRF.What is Known:•Physical activity is known to have a positive impact on health-related quality of life in children. Steps per day are commonly used as a measure of physical activity.• Cardiorespiratory fitness is a reco...
Source: European Journal of Pediatrics - November 18, 2023 Category: Pediatrics Source Type: research
