Primary sclerosing cholangitis in children with inflammatory bowel disease: An ESPGHAN position paper from the Hepatology Committee and the IBD Porto group.

Objective: We aimed to provide an evidence-supported approach to diagnose, monitor, and treat children with inflammatory bowel disease (IBD) and primary sclerosing cholangitis (PSC).

Methods: The core group formulated seven PICO-structured clinical questions. A systematic literature search from inception to December 2022 was conducted by a medical librarian using MEDLINE and EMBASE.

Human enteric glia diversity in health and disease: new avenues for the treatment of Hirschsprung disease.

Background And Aims: The enteric nervous system (ENS), comprised of neurons and glia, regulates intestinal motility. Hirschsprung disease (HSCR) results from defects in ENS formation, yet while neuronal aspects have been extensively studied, enteric glia remain disregarded. This study aimed to explore enteric glia diversity in health and disease.

Prevention of Stump Overgrowth in Pediatric Patients with Traumatic Crush Injury of the Leg: A Case Report.

Case: Stump overgrowth is the most common complication in skeletally immature amputees. Various techniques including capping the amputated stump have been used to prevent it but have been associated with variable rates of recurrence of bony overgrowth. We report a technique of intercalary tibial shortening prophylactically to avoid stump overgrowth in some specific situations in children with traumatic crush injuries of the leg/foot.

The Newborn Infant Parasympathetic Evaluation Index for Assessment of Procedural Pain and Discomfort in Mechanically Ventilated Pediatric Intensive Care Patients: A Prospective, Exploratory, Observational Study.

Background: The heart rate variability-based Newborn Infant Parasympathetic Evaluation (NIPE) Index is a continuous noninvasive tool for the assessment of pain and discomfort in infants. Little is known about its performance in the pediatric intensive care unit (PICU) setting, where assessment of pain/discomfort is usually based on discontinuous observational scoring systems or personal experience of medical staff.

Aim: Evaluation of the performance of NIPE as an indicator of procedural pain and/or discomfort in conscious mechanically ventilated PICU patients.

Childhood exposure to non-persistent endocrine disruptors, glucocorticosteroids, and attentional function: A cross-sectional study based on the parametric g-formula.

Background: Evidence suggests that endocrine disrupting chemicals (EDCs) may perturb the hypothalamic-pituitary-adrenocortical (HPA) axis, which has a major role in brain development. We aimed to evaluate the effects of childhood exposure to organophosphate pesticides, phenols, and phthalate metabolites, on urinary glucocorticosteroids and inattention in childhood.

Methods: We used data from the Human Early-Life Exposome (HELIX) cohort (2013-2016) and the parametric g-formula to estimate associations between EDCs, glucocorticosteroids, and hit reaction time standard error (HRT-SE), a measure of inattention, and tested for possible effect modification by sex.

The Impact of Slight to Mild Hearing Loss on Academic Performance and Behavior of 9-15-Year-Olds.

Objective: To examine the impact of slight to mild hearing loss in children by studying its association with academic performance and behavioral problems.

Methods: This study was embedded within a prospective birth cohort in Rotterdam, the Netherlands. Participants underwent audiometric and behavioral evaluations between ages 9-11 (April 2012-October 2015) and 13-15 (May 2017-September 2019).

Developing a Core Outcome Set for Netherton Syndrome: An International Multi-Stakeholder e-Delphi Consensus Study.

Introduction: Netherton syndrome (NS; OMIM#256500) is a rare and severe disorder of epidermal maturation and keratinization caused by pathogenic variants in the serine protease inhibitor Kazal type 5 (SPINK5), leading to severe skin barrier impairment. Although effective treatment is crucial for NS patients, there is a lack of knowledge on what the best treatment options are for these patients. Large heterogeneity in reported outcomes and measurement instruments hinders accurate comparison of treatment results across studies and the development of a treatment guideline.

Polysaccharide, Conjugate, and mRNA-based Vaccines are Immunogenic in Patients with Netherton Syndrome.

Background: Netherton syndrome (NS) is a rare, severe genetic skin disorder, currently classified as an inborn error of immunity (IEI) due to previously reported immune dysregulation. We recently reported the results of an immunological evaluation showing no evidence for a relevant B- and/or T-cell mediated immunodeficiency, but immune responses after vaccination were not evaluated in that study. Therefore, we evaluated immune responses to three vaccine platforms in adult NS patients to further investigate the presence of a clinically relevant B- and/or T-cell immunodeficiency.

Combined plasma protein and memory T cell profiling discern IBD-patient-immunotypes related to intestinal disease and treatment outcomes.

Inflammatory bowel disease (IBD) chronicity results from memory T helper cell (Tmem) reactivation. Identifying patient-specific immunotypes is crucial for tailored treatment. We conducted a comprehensive study integrating circulating immune proteins and circulating Tmem, with intestinal tissue histology and mRNA analysis, in therapy-naïve pediatric IBD (Crohn's disease, CD: n = 62; ulcerative colitis, UC: n = 20; age-matched controls n = 43), and after 10-12 weeks' induction therapy.

Preoperative Three-Dimensional Planning of Screw Length is not Reliable in Osteotomies of the Humerus and Forearm.

Objectives: Pediatric upper extremity fractures are seen frequently and sometimes lead to malunion. Three-dimensional (3D) surgery planning is an innovative addition to surgical treatment for the correction of post-traumatic arm deformities. The detailed planning in three dimensions allows for optimization of correction and provides planning of the exact osteotomies which include the advised material for correction and fixation.

Scar Perception in School-aged Children After Major Surgery in Infancy.

Background: The long-term effects of childhood surgery scars on health status, quality of life (QoL), self-esteem, and body image remain uncertain. This study explores these effects in school-aged children.

Methods: We conducted a retrospective cohort study involving 454 children (58% boys; 8-17 years) who had undergone surgical correction of anatomical anomalies or neonatal ECMO.

The Value of Preoperative Rigid Tracheobronchoscopy for the Diagnosis of Tracheomalacia in Oesophageal Atresia Patients.

Background: Oesophageal atresia (OA) is often accompanied by tracheomalacia (TM). The aim of this study was to evaluate its presence in OA patients during routine rigid tracheobronchoscopy (TBS) before primary correction and compare this to postoperative TBS and clinical signs of TM.

Methods: This retrospective cohort study included patients born with OA between June 2013 and December 2022 who had received a TBS before OA correction and had been followed for at least twelve months.

Thyroid hormone levels in children with Prader-Willi syndrome: a randomized controlled growth hormone trial and 10-year growth hormone study.

Context: Several endocrine abnormalities were reported in children with Prader-Willi syndrome (PWS), including hypothyroidism. Growth hormone (GH) treatment may impact the thyroid hormone axis by direct inhibition of T4 or TSH secretion or by increased peripheral conversion of free T4 (FT4) to T3.

Objective: The objective of this study is to evaluate thyroid function during GH treatment in a large group of children with PWS.

Dupilumab treatment decreases MBC2s, correlating with reduced IgE levels in pediatric atopic dermatitis.

Background: A preference for type 2 immunity plays a central role in the pathogenesis of atopic dermatitis (AD). Dupilumab, an mAb targeting the IL-4 receptor α (IL-4Rα) subunit, inhibits IL-4 and IL-13 signaling. These cytokines contribute significantly to IgE class switch recombination in B cells, critical in atopic diseases.

Health-related Quality of Life and Problem Behavior After GH Cessation in Adults Born Small for Gestational Age: A 12-Year Follow-up Study.

Context: Long-term data regarding health-related quality of life (HRQoL) and problem behavior in adults born small for gestational age (SGA) who were treated with GH during childhood are lacking.

Objective: To investigate longitudinal changes in HRQoL and problem behavior in adults born SGA during 12 years after cessation of childhood GH treatment (SGA-GH) and compare these with 3 control groups at age around 30 years.

Participants: One hundred seventy-six SGA-GH adults and 3 untreated age-matched control groups: 50 born SGA with short stature (SGA-S), 77 born SGA with spontaneous catch-up growth to normal height (SGA-CU), and 99 born appropriate-for-gestational-age with normal height (AGA).

Cerebral white matter hyperintensities in adults born small for gestational age at 12 years after cessation of childhood growth hormone treatment: a prospective cohort study including untreated controls.

Background: Increased cerebrovascular morbidity was reported in adults born small for gestational age (SGA) who were treated with growth hormone (GH) during childhood compared to the general population. Yet, previous studies lacked an appropriate control group which is a major limitation. We prospectively studied cerebral white matter hyperintensities (WMHs) in adults born SGA at 12 years after cessation of childhood GH-treatment (SGA-GH), compared to appropriate controls.

Management of Autoimmune Encephalitis in a 7-Year-Old Child With CTLA-4 Haploinsufficiency and AMPA Receptor Antibodies: A Case Report.

Objectives: We report on the therapeutic management of early-onset severe neurologic symptoms in cytotoxic T lymphocyte antigen-4 haploinsufficiency (CTLA-4h) and the presence of antibodies to the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) as an important finding.

Methods: This is a case report from a Dutch academic hospital. Repeated clinical examinations, repeated brain MRI and extended diagnostics on serum and CSF were performed.

Procedural sedation by advanced practice providers in the emergency medical service in the Netherlands: a retrospective study.

Background: Procedural sedation and analgesia (PSA) is a technique of administering sedatives to induce a state that allows the patient to tolerate painful procedures while maintaining cardiorespiratory function, a condition that is frequently desired prehospital. Non-physician prehospital clinicians often have a limited scope of practice when it comes to providing analgesia and sedation; sometimes resulting in a crew request for back-up from physician-staffed prehospital services.".

Micro-CT Imaging of Tracheal Development in Down Syndrome and Non-Down Syndrome Fetuses.

Objectives: Down syndrome (DS) is associated with airway abnormalities including a narrowed trachea. It is uncertain whether this narrowed trachea in DS is a consequence of deviant fetal development or an acquired disorder following endotracheal intubation after birth. This study aimed to compare the tracheal morphology in DS and non-DS fetuses using microfocus computed tomography (micro-CT).