Congenital diaphragmatic hernia: automatic lung and liver MRI segmentation with nnU-Net, reproducibility of pyradiomics features, and a machine learning application for the classification of liver herniation
Conclusion: Our results suggest that automatic MRI segmentation is feasible, with good reproducibility of pyradiomics features, and that a ML system for liver herniation prediction offers good reliability.     Trial registration:https://clinicaltrials.gov/ct2/show/NCT04609163?term=NCT04609163&draw=2&rank=1; Clinical Trial Identification no. NCT04609163.What is Known:• Magnetic resonance imaging (MRI) is crucial for prenatal congenital diaphragmatic hernia (CDH) assessment. It enables the quantification of the total lung volume and the extent of liver herniation, which are essential for stratifying the ...
Source: European Journal of Pediatrics - February 28, 2024 Category: Pediatrics Source Type: research
Neonatal hemodynamics of recipient twins after fetoscopic selective laser coagulation for twin-to-twin transfusion syndrome: An unicist classification
Conclusion:  Four distinct and well-characterized groups of recipients were identified based on their hemodynamics. High blood pressure and heart failure occurred in approximately 20% of the infants and were associated with a time between laser coagulation and birth of less than 2 months.What is Known:• Twin-to-twin transfusion syndrome (TTTS) is characterized by a hemodynamic imbalance that leads to high fetal and neonatal mortality if left untreated. One-third of recipient twins born without prenatal fetoscopic laser coagulation (FSLC) develop a life-threatening cardiac failure.What is New:• Four distinct groups of...
Source: European Journal of Pediatrics - February 28, 2024 Category: Pediatrics Source Type: research
Determinants of health-related quality of life in healthy children and adolescents during the COVID-19 pandemic: Results from a prospective longitudinal cohort study
Conclusion: Recent mental health, more than biological, physical, or social factors, played a key role in determining HRQOL in children and adolescents during pandemic times. Public health strategies to improve mental health may therefore be effective in improving HRQOL in this age group.What is Known:• Assessing health-related quality of life (HRQOL) in children and adolescents aids in understanding how life circumstances impact their well-being.• HRQOL is a complex construct, involving biological, psychological, and social factors. Factors driving HRQOL in children and adolescents are not often studied in longitudina...
Source: European Journal of Pediatrics - February 27, 2024 Category: Pediatrics Source Type: research
FGD1-related Aarskog –Scott syndrome: Identification of four novel variations and a literature review of clinical and molecular aspects
Conclusion: We reported four novel pathogenicFGD1 variations in AAS patients and confirmed the efficacy and safety of growth hormone treatment inFGD1-related AAS patients with growth hormone deficiency. Additionally, our literature review suggested the crucial role of DH domain in FGD1 function.What is Known:• Aarskog–Scott syndrome is a rare genetic disease, and the only known cause is the variant in FGD1 gene. The typical clinical manifestations of AAS include facial, skeletal, and urogenital deformities and short stature.What is New:• We reported four novel FGD1 variants and reported the treatment of growth hormon...
Source: European Journal of Pediatrics - February 27, 2024 Category: Pediatrics Source Type: research
Determinants of health-related quality of life in healthy children and adolescents during the COVID-19 pandemic: Results from a prospective longitudinal cohort study
Conclusion: Recent mental health, more than biological, physical, or social factors, played a key role in determining HRQOL in children and adolescents during pandemic times. Public health strategies to improve mental health may therefore be effective in improving HRQOL in this age group.What is Known:• Assessing health-related quality of life (HRQOL) in children and adolescents aids in understanding how life circumstances impact their well-being.• HRQOL is a complex construct, involving biological, psychological, and social factors. Factors driving HRQOL in children and adolescents are not often studied in longitudina...
Source: European Journal of Pediatrics - February 27, 2024 Category: Pediatrics Source Type: research
FGD1-related Aarskog –Scott syndrome: Identification of four novel variations and a literature review of clinical and molecular aspects
Conclusion: We reported four novel pathogenicFGD1 variations in AAS patients and confirmed the efficacy and safety of growth hormone treatment inFGD1-related AAS patients with growth hormone deficiency. Additionally, our literature review suggested the crucial role of DH domain in FGD1 function.What is Known:• Aarskog–Scott syndrome is a rare genetic disease, and the only known cause is the variant in FGD1 gene. The typical clinical manifestations of AAS include facial, skeletal, and urogenital deformities and short stature.What is New:• We reported four novel FGD1 variants and reported the treatment of growth hormon...
Source: European Journal of Pediatrics - February 27, 2024 Category: Pediatrics Source Type: research
Fluids and body composition during anesthesia in children and adolescents: A pilot study
Conclusion:  Intraoperative administration of isotonic solutions results in a significant fluid accumulation in low-risk schoolchildren during general anesthesia. The results suggest that children without major health problems undergoing short procedures do not need any perioperative intravenous fluid therapy, because they are allowed to take clear fluids up to 1 h prior anesthesia. In future studies, the use of BCM measurements has the potential to be valuable in guiding intraoperative fluid therapy.What is Known:• Most children who undergo common surgical interventions or investigations requiring anesthesia are nowad...
Source: European Journal of Pediatrics - February 26, 2024 Category: Pediatrics Source Type: research
Atopic dermatitis in early life and pain at 10 Â years of age: An exploratory study
Conclusion: Atopic dermatitis symptoms in early life were associated with a higher risk of pain at age 10, suggesting that potential for sensitization during the first decade of life and highlighting the importance of improving the health care of children with atopic dermatitis is worth investigating.What is Known:• Atopic disorders have been associated with many non-atopic comorbidities, including chronic pain.• Pain and atopic dermatitis share common inflammatory pathways. Inflammation, injury to the skin from scratching, fissures, and intolerance to irritants related to atopic dermatitis can cause pain.What is New:â...
Source: European Journal of Pediatrics - February 24, 2024 Category: Pediatrics Source Type: research
The assessment of fatigue and sleep quality among children and adolescents with familial Mediterranean fever: A case-control and correlation study
Conclusion: High disease activity has a significant negative impact on the sleep quality and fatigue levels of patients with FMF. This study emphasizes the importance of assessing fatigue and sleep quality with objective outcome tools periodically in FMF patients throughout the disease course.What is Known:• Fatigue is a common matter that often accompanies rheumatic diseases and causes disability.• Chronic rheumatic diseases often experience poor sleep quality.What is New:• In high correlation with the disease severity of familial Mediterranean fever, sleep quality decreases and fatigue level increases significantly...
Source: European Journal of Pediatrics - February 23, 2024 Category: Pediatrics Source Type: research
Glucocorticoid use and varying doses on the long-term outcomes of offspring born to patients with systemic lupus erythematosus
Conclusion: The use of non-fluorinated glucocorticoids during pregnancy and varying doses did not have a significant impact on the long-term physical, neurological, and social-emotional development outcomes of offspring born to SLE patients. However, the offspring of SLE patients treated with glucocorticoids  >  10 mg/day during pregnancy may be necessary to strengthen the monitoring of height (length) and communication skills in the long term.What is Known:• Fetal exposure to glucocorticoids can have implications for the development of multiple systems and may persist after birth, potentially increasing the risk...
Source: European Journal of Pediatrics - February 23, 2024 Category: Pediatrics Source Type: research
Gingival bleeding is a useful clinical feature in the diagnosis of hereditary bleeding disorders in children
Conclusion: Therefore, gingival bleeding should be considered a useful factor in bleeding history for HBD diagnosis. Adding this symptom to a screening score such as HEMSTOP improves its sensitivity. To confirm our findings, a prospective study is required.  Trial registration: Study registration number: NCT05214300.What is Known:• Screening for hereditary bleeding disorder diseases is a necessity and a challenge in children.• Minor disorders of primary hemostasis are the most common, but often escape standard coagulation tests.What is New:• Gingival bleeding is a frequent symptom that is easy to investigate and m...
Source: European Journal of Pediatrics - February 22, 2024 Category: Pediatrics Source Type: research
Comparison of body mass index and fat mass index to classify body composition in adolescents —The EVA4YOU study
Conclusion:  Classification of normal or pathologic body composition based on BMI and FMI shows good accordance in the clearly normal or pathologic range. In an intermediate range, FMI reclassifies categories based on BMI in more than a quarter of adolescents. Cut-off values to differentiate normal from pathol ogic FMI values on a biological basis are needed.Trial registration: The study is registered atwww.clinicaltrials.gov (Identifier: NCT04598685; Date of registration: October 22, 2020).What is Known:• Chronic non-communicable diseases (NCDs) are the leading cause of morbidity and mortality globally, with major ris...
Source: European Journal of Pediatrics - February 22, 2024 Category: Pediatrics Source Type: research
Post-ligation cardiac syndrome after surgical versus transcatheter closure of patent ductus arteriosus in low body weight premature infants: a multicenter retrospective cohort study
Conclusion: Transcatheter PDA closure may be equally effective but safer than surgical PDA closure in low-body-weight premature infants.What is Known:• Post-ligation cardiac syndrome is a serious and common complication of surgical closure of the ductus arteriosus in preterm infants.• Transcatheter closure of preterm ductus arteriosus is a safe and effective technique that is becoming more and more common worldwide.What is New:• Device closure is safer than surgical ligation for patent ductus arteriosus closure in preterm infants and may be the first-line non-pharmacological therapeutic option in this indication in e...
Source: European Journal of Pediatrics - February 21, 2024 Category: Pediatrics Source Type: research
A significant increase in anthropometric indices during long-term follow-up of pediatric patients with celiac disease, with no endocrine disorders
Conclusion: Over a long-term follow-up, pediatric patients withCeD demonstrate significant increases in weight, height, and BMI-for-age. Younger age at diagnosis is associated with greater improvement in weight and linear growth, emphasizing the importance of early diagnosis ofCeD.What is Known:• Celiac disease (СeD) is likely to be associated with growth impairment and poor weight gain.• Long-term changes in anthropometric indices after diagnosis of CeD are not well characterized.What is New:• Over a long-term follow-up, pediatric patients with CeD demonstrate significant increases in weight, height, and BMI-for-ag...
Source: European Journal of Pediatrics - February 20, 2024 Category: Pediatrics Source Type: research
Hemodynamic evaluation in preterm infants using ultrasonic cardiac output monitor (USCOM)
Conclusion: This is the first study describing reference values for USCOM parameters in hemodynamically stable preterm infants and factors affecting their variability. Further studies to investigate the usefulness of USCOM for the longitudinal assessment of patients at risk for cardiovascular instability or monitoring the response to therapies are  warranted.What is Known:• The ultrasonic cardiac output monitoring (USCOM) has been widely used on adult and pediatric patients and reference ranges for cardiac output (CO) by USCOM have been established in term infants.What is New:• We established reference values for USCO...
Source: European Journal of Pediatrics - February 20, 2024 Category: Pediatrics Source Type: research
