Validation and in silico function prediction of circtial1 as a novel marker of abnormal lung development in nitrofen-induced congenital diaphragmatic hernia (CDH).

Purpose: Circular RNAs (circRNAs) are stable, non-coding RNAs with tissue- and developmental-specific expression making them suitable biomarkers for congenital anomalies. Current circRNA discovery pipelines have focused on human and mouse. We aim to bridge this gap by combining bioinformatics resources and used circtial1 as a model candidate in the nitrofen rat model of congenital diaphragmatic hernia (CDH).

Fetal Tracheal Occlusion Corelates with Normalized YAP Expression and Alveolar Epithelial Differentiation in CDH.

Congenital diaphragmatic hernia (CDH) is characterized by incomplete closure of the diaphragm. While the ensuing compression to the fetal lung causes lung hypoplasia, specific cellular phenotypes and developmental signaling defects in the alveolar epithelium in CDH are not fully understood. Employing lung samples from human CDH, a surgical lamb model and a nitrogen rat model, we investigate whether lung compression impairs alveolar epithelial differentiation and Yes-associated protein (YAP)-mediated mechanosensing.

Artificial intelligence and pediatric surgery: where are we?

Here, we explore the transformative effects of artificial intelligence (AI) and large language models (LLMs), such as ChatGPT and GEMINI, on pediatric surgery, including preoperative, intraoperative, and postoperative care, as well as their influence on medical education and patient communication. We discuss the role of AI in enhancing surgical precision, facilitating personalized care strategies throughout all surgical phases, and improving learning for healthcare professionals and students. AI's application in intraoperative settings, providing real-time decision support and augmenting surgical accuracy, underscores how AI can contribute to safer surgical outcomes.

Yes-associated protein is dysregulated in human congenital diaphragmatic hernia patients during mid and end gestation.

Background: Yes-associated protein (YAP) is implicated in congenital diaphragmatic hernia (CDH). This study aims to investigate the abundance of YAP and its inactive form, phosphorylated YAP (p-YAP), in fetal human lung tissues from CDH cases compared to control cases at mid-gestation and end-gestation.

Methods: Immunofluorescence was performed to assess the abundance of YAP and p-YAP in lung tissues from human CDH and control fetuses who died from causes other than CDH.

Surgical management of the diaphragmatic defect in congenital diaphragmatic hernia: a contemporary review.

Worldwide, 150 children are born each day with congenital diaphragmatic hernia (CDH), a diaphragmatic defect with concomitant abnormal lung development. Patients with CDH with large defects are particularly challenging to treat, have the highest mortality, and are at significant risk of long-term complications. Advances in prenatal and neonatal treatments have improved survival in high-risk patients with CDH, but surgical treatment of large defects lacks standardization.

Cellular origins and translational approaches to congenital diaphragmatic hernia.

Congenital Diaphragmatic Hernia (CDH) is a complex developmental abnormality characterized by abnormal lung development, a diaphragmatic defect and cardiac dysfunction. Despite significant advances in management of CDH, mortality and morbidity continue to be driven by pulmonary hypoplasia, pulmonary hypertension, and cardiac dysfunction. The etiology of CDH remains unknown, but CDH is presumed to be caused by a combination of genetic susceptibility and external/environmental factors.

Experimental congenital diaphragmatic hernia features an alteration of DNA sensing targets cGAS and STING.

Background: The pathogenesis of congenital diaphragmatic hernia (CDH) depends on multiple factors. Activation of the DNA-sensing cyclic-GMP-AMP-synthase (cGAS) and Stimulator-of-Interferon-Genes (STING) pathway by double-stranded DNA (dsDNA) links environmental stimuli and inflammation. We hypothesized that nitrofen exposure alters cGAS and STING in human bronchial epithelial cells and fetal rat lungs.

The RNA-binding protein quaking is upregulated in nitrofen-induced congenital diaphragmatic hernia lungs at the end of gestation.

Background: The RNA-binding protein Quaking (QKI) increases during epithelial-to-mesenchymal transition and its expression is controlled by microRNA-200 family members. Here, we aimed to describe the expression of QKI in the developing lungs of control and nitrofen-induced congenital diaphragmatic hernia lungs (CDH).

Methods: To investigate the expression of QKI, we dissected lungs from control and nitrofen-induced CDH rats on embryonic day 15, 18, 21 (E15, E18, E21).

Microinjection With Nanoparticles to Deliver Drugs in Prenatal Lung Explants - A Pilot Study for Prenatal Therapy in Congenital Diaphragmatic Hernia.

Background: Fetoscopic endoluminal tracheal occlusion (FETO) improves the survival rate in fetuses with severe congenital diaphragmatic hernia (CDH). We hypothesize that prenatal therapies into the trachea during FETO can further improve outcomes. Here, we present an ex vivo microinjection technique with rat lung explants to study prenatal therapy with nanoparticles.

Prevalence of Mental Health Disorders in Individuals With Gastrointestinal Congenital Surgical Anomalies: A Systematic Review and Meta-analysis.

Purpose: Studies examining functional outcomes and health-related quality of life for patients with congenital surgical anomalies (CSA) are increasingly common. However, the prevalence of mental health disorders in this population has not been determined. The purpose of this review is to summarize the reported prevalence of mental health disorders in children born with gastrointestinal CSA.

Mental Health Outcomes of Mothers of Children With Congenital Gastrointestinal Anomalies Are Similar to Control Mothers: A Longitudinal Retrospective Cohort Study.

Purpose: Mothers of infants born with congenital gastrointestinal surgical anomalies experience a unique caregiving role. Whether these challenges result in more mental health diagnoses than the general population is unknown. This study assessed mental health diagnoses in mothers of children born with congenital surgical anomalies (CSA) compared to mothers of children without CSA.

Dysregulation of CITED2 in abnormal lung development in the nitrofen rat model.

Purpose: CITED2 both modulates lung, heart and diaphragm development. The role of CITED2 in the pathogenesis of congenital diaphragmatic hernia (CDH) is unknown. We aimed to study CITED2 during abnormal lung development in the nitrofen model.

Diagnosis and management of congenital diaphragmatic hernia: a 2023 update from the Canadian Congenital Diaphragmatic Hernia Collaborative.

Objective: The Canadian Congenital Diaphragmatic Hernia (CDH) Collaborative sought to make its existing clinical practice guideline, published in 2018, into a 'living document'.

Design And Main Outcome Measures: Critical appraisal of CDH literature adhering to Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. Evidence accumulated between 1 January 2017 and 30 August 2022 was analysed to inform changes to existing or the development of new CDH care recommendations.

Overactivated Epithelial NF-κB Disrupts Lung Development in Congenital Diaphragmatic Hernia.

Abnormal lung development is the main cause of morbidity and mortality in neonates with congenital diaphragmatic hernia (CDH), a common birth defect (1:2,500) of largely unknown pathobiology. Recent studies discovered that inflammatory processes, and specifically NF-κB-associated pathways, are enriched in human and experimental CDH. However, the molecular signaling of NF-κB in abnormal CDH lung development and its potential as a therapeutic target require further investigation.

Post-discharge follow-up of congenital duodenal obstruction patients: a systematic review.

Purpose: Long-term follow-up of congenital duodenal obstruction patients often falls on care providers with little experience of this condition. We performed a systematic review of the long-term outcomes of duodenal obstruction and provide a summary of sequelae care providers should anticipate.

Methods: In 2022, after registering with PROSPERA, Medline (Ovid), EMBASE, PSYCHINFO, CNAHL and SCOPUS databases were searched using the title keyword 'intestinal atresia'.

MicroRNA-200b deficiency is not sufficient to increase susceptibility to allergen-induced airway inflammation and dysfunction in mice.

MicroRNA-200b (miR-200b) has emerged as a therapeutic option for reducing inflammation and airway dysfunction in asthma. miR-200b belongs to a family of miRNAs that regulate epithelial-to-mesenchymal (EMT) transition and IL-33 abundance. In asthma, miR-200b abundance is reduced in the airways and is correlated with disease severity.

Skin Lesions as a Presenting Feature of Dimorphic T-Lymphoblastic Leukemia in an Adolescent Male.

Cutaneous involvement is rare in acute lymphoblastic leukemia/lymphoma, particularly within the T-cell lineage. Review of the literature for cutaneous involvement in T-cell lymphoblastic lymphoma/leukemia identifies mostly case reports, with the majority of cases involving adults. We describe an adolescent male presenting with cervical lymphadenopathy and skin lesions leading to a diagnosis of early T-cell precursor lymphoblastic leukemia.

Educational and mental health outcomes in school-aged children with anorectal malformations: A case-control cohort study.

Purpose: Outcome studies for patients with anorectal malformation (ARM) have focused on fecal incontinence and quality of life, but a comparison of educational outcomes between ARM cases and controls has not been reported. The purpose of this study was to assess real-world educational outcomes, neurodevelopmental disorders and mental health disorders in ARM patients and compare to an age-matched control group.

Methods: We performed a retrospective case-control study of children diagnosed with ARM from 1991 to 2017.

Preventing severe necrotizing enterocolitis: Propensity score analysis of interventions associated with surgical NEC or NEC-associated death.

Purpose: To determine what modifiable interventions used in the neonatal intensive care unit (NICU) are associated with severe necrotizing enterocolitis (NEC) requiring surgical intervention.

Methods: A retrospective review of patients treated for NEC at a tertiary hospital from 1991 to 2016 was performed. Patient characteristics were used to calculate propensity scores for likelihood of exposure to seven interventions: enteral feeds, use of glucocorticoids, nonsteroidal anti-inflammatory drugs (NSAIDs), antacids, antibiotics, or umbilical arterial (UAC) and venous catheters (UVC).

A Tracheal Aspirate-derived Airway Basal Cell Model Reveals a Proinflammatory Epithelial Defect in Congenital Diaphragmatic Hernia.

Congenital diaphragmatic hernia (CDH) is characterized by incomplete closure of the diaphragm and lung hypoplasia. The pathophysiology of lung defects in CDH is poorly understood. To establish a translational model of human airway epithelium in CDH for pathogenic investigation and therapeutic testing.

The impact of intestinal atresia on educational and mental health outcomes in school-aged children: A case-control cohort study.

Purpose: To assess real-world educational outcomes, neurodevelopmental disorders and mental health disorders in patients with intestinal atresia (IA) and compare these to outcomes to age-matched controls.

Methods: We performed a retrospective case-control study of children with IA born between 1991 and 2017. We evaluated educational outcomes using an Early Developmental Instrument, Grades 3, 7 and 8 assessments, Grade 9 completion and performance, high school graduation, and neurodevelopmental and mental health disorders using International Classification of Diseases codes available from a population-based dataset.

Effect of trisomy 21 on long-term gastrointestinal outcomes in duodenal atresia.

Purpose: We aimed to determine if Trisomy 21 (T21) affected gastrointestinal outcomes for children with duodenal atresia (DA).

Methods: We identified children born with DA between 1991 and 2017. Cases were divided into DA with T21 and DA without T21.